Differential Diagnosis in Neurology

Topic 1. Vascular Disease

1.7. Hemorrhagic Vascular Disease

The major categories of hemorrhagic vascular disease are:

  • Hypertension
  • Aneurysm, arteriovenous malformation, venous malformation, cavernous hemangioma
  • Medical causes of platelet or clotting factor dysfunction
  • Hemorrhagic transformation of ischemic stroke
  • Anticoagulation
  • Drug abuse (methamphetamine, cocaine, PPA)
  • Thrombolytic agents
  • Congophilic angiopathy (CCA)
  • Cold exposure
  • Post carotid endarterectomy syndrome
  • Severe migraine
  • Tumor
  • Surgery
  • Moyamoya disease
  • Trauma
  • Alcohol

Hypertensive Hemorrhage

Hypertensive hemorrhage occurs in very specific places the most common of which are:

  • Basal ganglia – 60% (caudate 10%; putamen 50%; GP1 40%)
  • Thalamus – 20%
  • Cerebellum – 15%
  • Pons – 5%
  • Lobar – 10%
  • Pontine – 5%
  • Medulla – rare <1%
  • Midbrain – rare <1%

If hemorrhages are not found in these locations, they are most likely caused by systemic illness, congenital vascular anomalies, anticoagulation or drugs.

Clinical Manifestations of Intraparenchymal Hemorrhage

The onset of intraparenchymal hemorrhage is most often during wakefulness as a patient pursues the usual activities of daily living. Maneuvers that cause a sudden rise in blood pressure such as heavy lifting, coitus, prolonged vomiting, coughing or straining at stool have been noted to cause hemorrhage with increased frequency. Unusual causes of hemorrhage are related to specific circumstances. Approximately 30% of ICH from aneurysm, AVM, and cavernous angioma occur during sleep.

Most patients when first examined are alert (50%) with the remaining being lethargic (25%), stuporous or comatose (25%). The onset is gradual over minutes to hours and the neurologic deficit is steadily progressive. CT and MRI serial evaluations have demonstrated that expansion occurs in the first 6 hours in approximately 20% of patients while 15% expand during the next 6–12 hours and 6% between 6–24 hours. Expansion may be dramatic. In patients who suffer hemorrhage from a bleeding diathesis expansion may occur insidiously over days.

Focal deficits and consciousness are preserved at the ictus. Headache and vomiting may be absent. In subarachnoid hemorrhage, headache is severe, reaches maximum intensity almost instantly and focal neurologic signs are minimal. Loss of consciousness in SAH is much more frequent at the ictus than that which occurs with intraparenchymal hemorrhage. Embolism produces the greatest neurologic deficit at onset, is accompanied by a higher percentage of seizures than ICH and a lesser degree of headache. Patients who suffer emboli, particularly to the stem of the left MCA appear stunned. Ischemic vascular disease is more frequent during sleep or the early morning and is accompanied frequently with deficits of a cortical nature such as aphasia or denial of contralateral space. Headache is common in ischemic disease with posterior circulation ischemia causing it more frequently than that from the anterior circulation. Rarely is it a predominant feature.

Headache from intraparenchymal lesions occurs frequently as the hematoma develops and stretches the overlying dura, displaces the sinuses and major blood vessels at the base of the brain or spills into the subarachnoid space. Early in the course of the hemorrhage, the stretched dura may project pain to the overlying skull and scalp but most often the headache is projected frontally. The anterior and middle fossa dura and the forehead are innervated by V1 (somatic visceral convergence) and thus lesions in these fossa project to somatic representations of V1. Displacement of the posterior 1/3 of the sagittal sinus may also cause frontal headache. The dura of the posterior fossa is innervated by the posterior roots of C1–C2 and cranial nerves IX and X. Hematomas in the posterior fossa usually project pain to the basiocciput, posterior cervical areas or the trapezius ridge. They may project to the brow, the somatic representation of C2. Displacement of the tentorium and irritation of it's under surface (innervated by C1–C2, IX and X) may cause pain in the inner ear. The upper leaflet of the tentorium is innervated by V1. Many patients who have suffered an intraparenchymal bleed are too lethargic to describe their headache accurately. Headache is most common with lobar and cerebellar hematomas due to their close approximation to the dura and may be accompanied by signs of meningeal irritation. Headache increases with hematoma enlargement and is associated with vomiting and deterioration of the level of consciousness. Head tilt to the side of the hematoma, unilateral forced eye closure (ipsilateral to the side of the hematoma) and C2 headache are most suggestive of a cerebellar hemorrhage.

Severe neck stiffness, photophobia, sore eyes, Brudzinski and Kerning's signs suggest caudate hemorrhage with direct rupture into the ventricular system. Hyperthermia, rarely greater than 103° may be present. Thalamic and cerebellar hemorrhage may also be associated with early ventricular extension. Larger putaminal hemorrhages rarely rupture into the ventricular system at onset.

The focal deficits and topography of each type of hemorrhage will be described in detail. Specific neurological deficits suggest a characteristic hemorrhage:

  • a deeply comatose patient with head and eyes deviated to the side of the lesion, a flaccid hemiparesis and hemisensory defect with periodic breathing is characteristic of a deep pallidal hemorrhage
  • inability to walk, dysarthria, nystagmus with quadriparesis not plegia suggests a cerebellar hemorrhage
  • a patient whose eyes are down and in with a greater sensory than motor deficit is sentinel for a thalamic bleed
  • an elderly patient with arm, face weakness who has suffered a seizure suggests a lobar congophilic hemorrhage

Vomiting and nausea may occur at the ictus of an intraparenchymal hemorrhage due to the sudden increase of ICP that is transmitted to the mechanosensitive emetic center of the floor of the IV ventricle or distortion of the IV ventricle itself. Vomiting stops as pressure is relieved by displacement of CSF into the spinal subarachnoid space, overbreathing that effects a decrease of pCO2 and consequent reduction of cerebral blood flow as well as intracranial shifts of brain parenchyma. As the hematoma expands further, vomiting and nausea return due to exhaustion of compensatory mechanisms.

Persistent vomiting out of proportion to dizziness is suggestive of an ischemic lesion of the brainstem. Vomiting with change of head position is characteristic of a mass in the IVth ventricle. Inability to vomit in the face of a strong urge to do so is characteristic of an intrinsic medullary lesion. A smooth gradual deterioration of neurological function over minutes to hours with concomitant headache and vomiting is most consistent with supratentorial intraparenchymal hemorrhage. Headaches are most often seen with larger hemorrhages whereas deep parenchymal lesions may be asymptomatic. Approximately 30% of patients suffer headaches at the ictus, another 20% develop it during evolution of their neurologic deficits and up to 40% may have no headache. Many of the latter patients are lethargic or obtunded at the time of evaluation. Lobar and cerebellar hemorrhages have the highest incidence of headaches due to their proximity to the meninges. Patients with cerebellar hemorrhage vomit shortly after the ictus. Approximately 50% of patients with posterior circulation headaches vomit. Ischemia in this distribution causes vomiting in 1/3 of patients which tends to be more persistent and is not associated with obtundation and stupor. Patients with migraine have severe persistent vomiting accompanied by dizziness and vertigo but minimal focal deficits and no depression of consciousness. Bickerstaff's migraine (vasospasm of the vertebrobasilar arteries) may be associated with transient loss of consciousness and there have been reports of migraine patients who become comatose.

Seizures occur in approximately 15% of patients who suffer an intraparenchymal bleed. They are most common in lobar hemorrhages which occur at the grey-white junction and excite the overlying cerebral cortex. Seizures are rare in putaminal hemorrhages. They suggest an underlying cavernous angioma, arteriovenous malformation or transformed embolic stroke. A 10 ml hemorrhage is minimal, 45 ml is severe and greater than 60 ml is usually fatal.

Topographical Syndromes

Caudate Hemorrhage (Approximately 7% of ICH)

  • Medial hemorrhages:
    • Blood dissects into the ipsilateral frontal horn
    • Dense hemorrhage is noted at the caudate head
    • Ventricular dilatation occurs ipsilaterally > contralaterally
  • Clinical Features:
    • Small hemorrhage: no prominent motor, sensory or visual loss
    • Symptoms: mimic intraventricular bleed or SAH: neck stiffness, severe headache, nausea and vomiting, decreased level of consciousness

Lateral Caudate Hemorrhage

  • Larger than medial hemorrhage
  • Spreads laterally or posterolaterally into the putamen or anterior or posterior limb of the internal capsule
  • Clinical Features
    • Headache, vomiting, decreased level of consciousness
    • Hemiparesis, hemisensory loss, ipsilateral conjugate deviation of the eyes with inability to look to the contralateral side (may be dolled across the midline in 12 to 24 hours); in general, minimal sensory loss
    • Less severe and transient signs if the hemorrhage is limited to the internal capsule
    • If blood spreads inferiorly; Horner's syndrome
      • Horner's syndrome (ipsilaterally)
    • R caudate hemorrhage: neglect or mania
    • L caudate hemorrhage: aphasia, confusional state, disorientation
    • Memory loss (if hemorrhage involves internal capsule and AV nucleus of the thalamus

Differential Diagnosis

  • HCVD
  • Infarct with hemorrhagic transformation (lenticulostriate, recurrent artery of Heubner)
  • Arteriovenous malformation
  • Carotid aneurysm rupture
  • Moyamoya disease (with hemorrhage)
  • Ependymal vascular malformation

Striatocapsular Hemorrhage

  • Clinical features:
    • Mild dysarthria
    • Contralateral hemiparesis
    • Contralateral hemisensory deficit
    • Lacunar type syndrome
Anterior type
  • 11%; Heubner's artery territory
  • Small caudate hematoma
  • Ruptures into lateral ventricle
  • Clinical presentation:
    • Severe headache (similar to subarachnoid hemorrhage)
    • Contralateral hemiparesis <50%
    • Acute confusion; abulia; (approximately one week)
    • DH: transcortical motor aphasia (transient)
Middle type
  • Medial lenticulostriate artery territory
  • <10%; moderate in size (<30 cc; spreads anterolaterally)
  • Primary involvement globus pallidus and medial putamen
  • Conjugate deviation of the eyes to the side of the lesion
  • Contralateral hemiparesis (mild to moderate)
  • Anomia; global aphasia (transient)
Posteromedial type
  • Anterior choroidal artery territory
  • 4% of striatocapsular hemorrhage
  • Small hematoma in posterior limb of internal capsule (anterior 1/2)
  • Spreads rostrally; pressure on medial putamen or lateral thalamus
Posterolateral type
  • Posterior medial branches of the lateral lenticulostriate arteries territory
  • Approximately 33% of striatocapsular hemorrhage
  • Areas affected
    • Posterior 50% of the putamen; moderate in size (35 cc); extend often to the anterior putamen
    • Posterior limb of the internal capsule (retrolenticular portion); rupture into anterior horn of the lateral ventricle
  • Clinical presentation:
    • Impaired consciousness
    • Cheyne–Stokes or periotic respiration
    • 2–3 mm reactive pupils
    • Severe flaccid contralateral hemiparesis
      • Arm, leg, face, equally involved
    • Dominant hemisphere
      • Anterior > posterior aphasia
    • Non-dominant hemisphere
      • Severe visuospatial neglect
Lateral type
  • Lateral branches of the lateral lenticulostriate arteries
  • Approximately 20% of striatocapsular hemorrhage
  • Areas involved:
    • Elliptical hematoma between the putamen and insular cortex
    • Often ruptures into the lateral ventricle
  • Clinical features
    • Contralateral hemiparesis
    • Neglect for contralateral space (NDH)
    • Language dysfunction (DH)

Massive Hemorrhage

  • Approximately 25% of lenticulo striato hemorrhage
  • Entire striatocapsular territory involved
  • Clinical features
    • Severe flaccid contralateral hemiparesis
      • Arm, face, leg equally involved
    • Lethargy to coma
    • Ipsilateral conjugate eye variation
      • Wrong way eyes observed in a percentage of patients
    • Poor prognosis

Putaminal Hemorrhage

  • General characteristics:
    • Most common location for basal ganglia hemorrhage in hypertensive patients (40%)
    • Largest hemorrhages involve:
      • Medial and most anterior portion of the posterior putamen
      • Anterior 2/3 of the posterior limb of the internal capsule
      • Putative mechanism: rupture of Charcot–Bouchard aneurysms of the large lateral lenticulostriate arteries
  • Clinical Manifestations
    • Abrupt onset of neurologic deficit while awake
    • Approximately 30% have headache at onset and 20% more develop it as the process evolves
    • Vital signs:
      • Level of consciousness:
        • 50% alert at presentation; others obtunded to comatose
        • Blood pressure elevated: 220–240 to 110–140 mmHg
        • Pulse rate tends to be slow and full at 60–70/minute, a rapid thready pulse appears with herniation
        • Respiratory pattern
          • Most often periodic respiration, inspiratory and expiratory build up and decline with irregular apneic periods. The longer the apneic periods the graver the prognosis. Rarely Cheyne–Stokes pattern.
        • Pupils:
          • At presentation are usually diencephalic at 2–3 mm and reactive to light, change with evolution of the hematoma and consequent herniation
    • Posture:
      • Early at presentation:
        • Pronated arm with early cortical thumb (adducted) contralateral to the lesion; externally rotated leg contralaterally
        • Right-sided lesion
          • Patient angled in bed neglecting contralateral space (nondominant hemisphere)
    • Mental status:
      • Left sided lesion (dominant hemisphere)
        • Fluent aphasia with intact repetition unless the arcuate fasciculus is disrupted right sided lesion
      • Non-dominant hemisphere
        • Visual neglect for left space
        • Motor impersistence
        • Constructional dyspraxia
    • Cranial nerve examination:
      • Contralateral VII paralysis
      • Head deviated to the side of the lesion
      • Eyes slightly below the midline if the midbrain oculomotor center for upgaze riMLF is compressed
        • Conjugate deviation of the eyes to the side of the lesion
        • If the patient is alert pupils may be normal; as obtundation supervenes pupils are diencephalic at 2–3 mm
    • Motor examination:
      • Flaccid contralateral hemiparesis
        • Internal capsule involvement with brain shock
        • More anteriorly in the posterior limb faciobrachial paresis; leg less involved (topographically and bilaterally innervated)
        • Rarely patients may have contralateral increased tone if the putamen is primarily involved
        • Rarely; ipsilateral adventitial movement occur (descending pyramidal or extrapyramidal pathways are stimulated)
        • Motor impersistence with right sided lesions
    • Sensory examination:
      • Contralateral hemisensory deficit to all modalities
      • Neglect of left sided space with right sided lesions
    • Reflexes:
      • Depressed on the paretic side
      • Positive Babinski or intermediate toe on the paretic side; bilateral Babinski signs if there is severe pressure

Transtentorial Herniation

  • Anatomy:
    • Compression and torque of the midbrain at the level of the tentorium
    • Obliterated ambient cisterns
    • Trapped contralateral temporal horn
    • Rupture of midline penetrating arteries of the pons, midbrain and thalamus (Duret's hemorrhage)
    • Compression and infarction of the PCA under the ipsilateral tentorium
    • Lateral pineal shift of 10 mm
    • Downward pineal shift of 7 mm

Clinical Manifestations

  • Vital signs:
    • Stupor to coma
    • Blood pressure of 90–60 mm/Hg as herniation occurs
    • Central neurogenic hyperventilation (20–40 breaths/minute)
    • Thready pulse at 100–120 beats/minute
    • Temperature 103–105° Fahrenheit dependent on presence of intraventricular blood or posterior hypothalamic damage
  • Cranial nerve impairment:
    • Paralyzed horizontal gaze; eyes below the midline
    • Ipsilateral pupil smaller then dilates; may become oval as III nerve is compressed under P-COM
    • Posture and reflexes:
      Bilateral decerebrate posture
      Bilateral Babinski response

Anterior Putaminal Hemorrhage

  • Anatomy:
    • Anterior putamen
    • Anterior limb of internal capsule
  • Clinical characteristics
    • Mild hemiparesis
    • No sensory loss

Posterior Putaminal Hemorrhage

  • Anatomy
    • Involves posterior 1/3 of the internal capsule
    • Extreme posterior extension of the putamen
  • Clinical manifestation
    • Hemisensory loss greater than hemiparesis
    • Inferior quadrantanopia or hemianopia with left dominant posterior putaminal hemorrhage
      • Wernicke's type aphasia:
        • If the hemorrhage extends into the isthmus of the temporal lobe
        • Undercuts or involves posterior speech areas. Inferior temporal gyrus thought to be involved in semantic aphasia. Dissection of blood into periventricular white matter

Rare Presentations of Putaminal Hemorrhage

  • Pure motor hemiplegia
  • Hemichorea
  • Hemiballism
  • Sensorimotor stroke

Asymptomatic Putaminal Hemorrhage

  • 1.5% of stroke patients (hemorrhagic transformation)
  • 9.5% of putaminal hemorrhage

Globus Pallidus Hemorrhage

  • Medial lenticulostriate artery territory
  • Hypertensive patient with Cushing reflex
    • BP 220/120 – 240/120–130 mmHg
    • Approximately 50% obtunded within minutes to hours after ictus. Progressive deterioration of level of consciousness
  • Periodic or Cheyne's-Stokes respiration
  • Pupils 2–3 mm poorly reactive to light
  • Pulse rate of 50–60 beats/minute full at onset
  • Eyes conjugately deviated below the horizontal 5–10°
  • Head and eyes deviated to the side of the lesions
  • Dense contralateral hemiparesis: arm, face, and leg relatively equally involved; flaccid tone (destruction of medial components of posterior limb of the internal capsule)
  • Dense hemisensory deficit to all modalities (disruption of medial portion of the posterior limb of the internal capsule); thalamic projections
  • Depressed reflexes contralaterally; may be increased ipsilaterally
  • Contra or bilateral Babinski's signs
  • Progression to decerebrate rigidity and central herniation

Thalamic Hemorrhage

  • General characteristic
    • 4 major categories dependent on involved vascular territories
      • Thalamogeniculate artery (P1)
      • Thalamoperforate or thalamic subthalamic perforate artery (P1)
      • Tuberothalamic (polar arteries)
        • originate from the P-COM
      • Lateral posterior choroidal artery (P3)
    • Most hemorrhages are posterior to descending pyramidal tracts
      • Sensory loss greater than motor
  • Sentinel clinical characteristics:
    • Ocular manifestations
    • Thalamic sensory loss
    • Thalamic aphasia
    • Behavioral or cognitive deficits
    • Thalamic motor abnormalities

Ocular Clinical Findings

  • Paralysis of upgaze (pressure at the level of the superior colliculus, oculomotor center for upgaze the ri MLF (rostral interstitial nucleus of the medial longitudinal fasciculus)
  • Eyes "down" and "in" in primary gaze (as if looking at the tip of the nose)
  • Vertical skew deviation
    • Eyes are down on the side of the lesion
    • Abnormal displacement is constant in all fields of gaze
  • Disconjugate gaze:
    • Limited abduction (pseudo VI nerve palsy)
    • Visual fixation from the adducted eye
    • Increased convergence vectors neutralize abduction (visual fibers descend into the anterior medial thalamus) and cause eyes to look down and in
  • Pupils:
    • 2–3 mm poorly responsive to light
    • Afferent arc disrupted
  • Hemorrhage may extend into:
    • Diencephalic mesencephalic junction
    • Quadrigeminal plate
  • Wrong way eyes

Thalamic sensory loss

  • All primary modalities affected including vibration:
    • Rarely also occurs with lesion of the dorsal column nuclei
    • May split the frontal bone of the forehead
  • Patient aware of a line that bisects the body:
    • Bilateral sensory loss around the umbilicus
    • 20% of patients describe the quality as numb or heavy; 80% describe active paresthesias, formication or burning (VPL)
    • May have bilateral intraoral numbness or sensory loss (VPM)
    • Tips of all fingers may be numb (VPI as fibers destined for VPL are separated here)
    • After 6 weeks to 3 months Déjérine Roussy syndrome may appear:
      • Decreased primary modality sensation in the involved area
      • Static and dynamic mechano allodynia of the involved area
      • Thermal hyperalgesia particularly to cold in some component of the area of sensory loss
      • Hyperpathia in some component of the area of sensory loss:
        • Pain threshold increased
        • Once threshold is exceeded in pain reaches maximum intensity too rapidly
        • Pain is overwhelmingly severe
        • Poorly localized with a deep visceral quality
        • Not stimulus bound (continues after the stimulus is withdrawn)
        • Occurs spontaneously

Thalamic aphasia

  • Nuclear involvement
    • Anterior ventral
    • Dorsomedial
    • Pulvinar
  • Clinical characteristics:
    • Normal conversation early
    • Fluent aphasia
    • Jargon intrusions
    • Relatively good comprehension
    • Repeat or duplicate words or syllables at the ends of words in spoken and written language
    • Paraphasic errors
    • Anomia
    • Perseveration

Behavioral and cognitive dysfunction

  • Anterior ventral nuclear lesion
    • Abulia
    • Waxing and waning levels of consciousness throughout the day
  • Dorsomedial nuclear lesion
    • Amnestic syndrome
    • Anosognosia
    • Visual spatial dysfunction

Thalamic motor dysfunction

  • Thalamic drift: prominent metacarpal pharyngeal flexion with the thumb adducted into the palm
  • Ataxia of stance (area x involved)
  • Mild limb ataxia (disruption of frontal cortical – thalamic distributed network); Brun's ataxia variant
  • Contralateral choreatic or dystonic movements
  • Contralateral severely abnormal posture (patient lying on an arm behind his back)

Topographic Hemorrhages of the Thalamus

Posterolateral (thalamogeniculate artery territory)

  • Largest and most common thalamic hemorrhage
  • Tthalamic sensory and motor involvement
  • Internal capsule may be compressed with consequent sensorimotor deficits
  • Leg sensation and eye movements transiently and minimally involved

Anterior or anterior lateral hemorrhage

  • Tuberothalamic (polar artery) territory
  • Apathy
  • Abulia
  • Hypersomnolence
  • Waxing and waning level of consciousness
  • Thalamic aphasia

Posteromedial (thalamoperforate or thalamic subthalamic-thalamoperforate arteries)

  • Thalamic eye signs:
    • Pupillary involvement
    • Vertical gaze dysfunction
  • Impaired consciousness
  • Ruptures into the III ventricle
  • Compresses the diencephalic mesencephalic junction
  • Early hydrocephalus

Posterior dorsal lesions

  • Posterior choroidal artery territory
  • Pulvinar involved
  • Aphasia
  • Minimal transient sensorimotor involvement (overlap blood supply to posterior limb of the internal capsule)
  • Rare: neglect, apraxia, amnesia

Lobar Hemorrhage

General characteristics

  • 10% of ICH; higher frequency in patients <45 years
  • Develop beneath the grey white junction
  • After absorption of blood products there is a "slit like scar"
  • Expand linearly along white matter pathways
  • Undercuts the overlying cerebral cortex
    • Epileptic genic
    • 15–30% seizures at onset
  • Parietal and occipital lobes more frequently affected than frontal lobes

Clinical signs and symptoms of lobar hemorrhage

  • Sudden onset during activity
  • Headache
  • Vomiting in 30–40% during the first hour
  • Seizures 15–30% at onset, focal and status epilepticus (frontal lobe most common)
  • 5–20% present in coma

Differential diagnosis of lobar hemorrhage

  • HCVD (patients <45 years)
  • Cerebral amyloid angiopathy (>65 years)
  • Arteriovenous malformation (<30 years)
  • Cavernous angioma
  • Coagulopathy
  • Illicit drug use
  • Trauma
  • Aneurysm

Frontal Hematoma

  • General Clinical Manifestation
    • Headache – 80%
    • Vomiting – 80%
    • Seizure – 25–30%
    • Impaired consciousness (lethargy to obtundation) – 50%
    • Gaze preference 30–40%

Topographic Diagnosis of Frontal Hematoma

Dorsal anterior
  • Cognitive behavioral features
    • Abulia
    • Initiate nothing
    • If stimulated:
      • Short terse answers
      • Slow processing of information
  • Frontal eye field involvement(BA 8, BA 10)
    • Eyes conjugately deviated to the side of the lesion; may be dolled across the midline after 12–24 hours from the ictus
  • Dorsolateral prefrontal cortex involvement (DLPC)
    • Left sided executive frontal lobe dysfunction
      • Poor memory retrieval
    • Right side (nondominant)
      • Disinhibition
      • Poor judgment
      • Witzelsucht (inappropriate jocularity)
      • bilateral lesions, bowel and bladder incontinence (second frontal gyrus)
  • Area 4 involvement
    • Brachiocephalic hemiparesis
    • Head and eye deviation to the side of the lesion (if extends to BA 8 and BA 10)
    • Lesions above the frontal horn or the lateral ventricle
      • Frontal headache
      • Contralateral predominant leg weakness
  • Inferior Frontal Hematoma
    • Below frontal horns of lateral ventricle
    • Impaired consciousness
    • Contralateral brachiocephalic hemiparesis and hemisensory loss
    • Rare presentation
      • No motor or sensory loss
      • Abulia
  • Orbital Frontal Hematoma
    • Impaired consciousness
    • Agitated delirium
    • Most often etiology is head trauma
      • Windshield injury in MVA
      • Falls (associated basilar skull fracture)
    • Semantic language dysfunction
    • Autonomic dysregulation
      • Cardiac arrhythmia (BA 11, BA 12)
      • Hypertension

Paracentral Hematoma

  • Primary involvement of Brodmann's area 4,3,1 and 2
  • Contralateral brachiocephalic motor and sensory deficits
  • Anterior (BA 44) aphasia
    • Transcortical motor
    • Decreased ability to write out of proportion to weakness (Exner's area 45)
  • Focal and generalized seizures
  • Lesion above the frontal horn
    • Geadache (frontal and primarily ipsilateral)
    • Contralateral leg weakness

Differential Diagnosis of Frontal Hematoma

  • HCVD – 20%
  • AVM – 25–30%
  • Cavernous angioma – 25–30%
  • Aneurysm – 25–30%
  • Anticoagulation – 10%
  • Congophilic angiopathy – 5–10%
  • Trauma – 10%
  • Illicit drug use – 10%

Parietal Hematoma

  • General manifestations:
    • Unilateral headache
      • Temporoparietal early; then frontal projection
    • Seizures (20–30% at onset)
    • Contralateral cortical sensory loss
    • Contralateral parietal motor dysfunction (loathness to move extremity)
    • Neglect of the contralateral visual field

Topographic Parietal Hemorrhage

  • Anterolateral parietal hemorrhage
    • Parietal movement disorder
      • Updrift of the affected hand with "piano playing" fingers
      • Loathness to move
      • Sensory ataxia during all components of movement
      • Abnormal arm position at rest
      • Imprecise fine movement
    • Noncongruent inferior quad anopsia in 50% of patients (lesion must extend greater than a depth of 1 cm)
    • Posterior aphasia (dominant hemisphere)
    • Neglect of contralateral space (nondominant hemisphere)
  • Anterior medial parietal hemorrhage
    • Similar clinical syndrome to lateral hemorrhage
    • Depressed consciousness
    • May extend into the thalamus

Large Posterior Parietal Hematoma

  • Seizure at onset
    • Aura of contralateral spacial tilt
  • General deficit:
    • Posture copy
    • Two point discrimination
    • Graphesthesia
    • Stereognosis
    • Localization of a body part or limb in space
    • Extinction of double simultaneous stimulus (contralateral to the lesion)
    • Alloesthesia
    • Point localization

Nondominant Parietal Hemisphere Hematoma

  • Constructional apraxia
  • Dressing apraxia
  • Posterior type of alien hand (NDH > DH)
  • Contralateral spatial neglect
  • Anosognosia
  • Asomatognosia
  • Geographical confusion (NDH > DH)
  • Depression of opticokinetic nystagmus (eyes deviate to side of the lesion)
  • Geometric and colored patterns that move in contralateral visual field (NDH = DH)

Dominant Left Parietal Lobe Hemorrhage

  • Transcortical sensory aphasia
  • Von Gerstmann syndrome components
    • Right left confusion
    • Finger agnosia
    • Acalculia
    • Dysgraphia
    • Failure to cross the midline
  • Dysgraphia and dyslexia (BA 37, BA 40, BA 41)
  • Alexia without agraphia (BA 39, BA 40)
  • Bilateral failure to localize body parts
  • Poor visual manual dexterity (BA 5, BA 7 projects to BA 4)
  • Parietal motor dysfunction
  • Bilateral parietal hemorrhage
    • Parietal avoidance behavior

Left Inferior Parietal Hematoma

  • Deficits of reading, writing and calculation
  • Nominal aphasia (supramarginal gyrus)

Right Inferior Parietal Hematoma

  • Constructional dyspraxia
  • Poor visual spatial relationships
  • Inability to draw or copy

Temporal Hematoma

  • General characteristics:
    • Agitation and/or delirium (R > L)
    • Wernicke's type aphasia (DH)
    • Herniation with slight or without hemiparesis
    • Insidious decline of consciousness followed by III nerve paralysis
    • Headache
      • Preauricular
      • Periorbital
  • Left temporal lobe hematoma
    • Fluent aphasia
    • Anomia, paraphasic errors, poor comprehension
  • Right (nondominant) temporal hematoma
    • Confusion without focal signs
  • Posterior left hematoma:
    • Wernicke's aphasia
    • Right homonymous quadrantanopsia
    • Minimal hemiparesis or hemisensory deficit
  • 20% of temporal lobe hematomas have seizure
  • Inferior basal temporal hematoma
    • Blood in the basal cisterns or cortical sulci suggests AVM or aneurysm
  • Medial extension of temporal lob hematoma
    • Basal ganglia internal capsule compression or destruction
    • Contralateral hemiplegia and hemisensory deficit
    • Wernicke's or transcortical sensory aphasia
    • Horizontal gaze palsy contralaterally
    • Right temporal lobe hematoma with extension to the parietal lobe
      • Left sided spatial hemi inattention and neglect
    • Partial III nerve paralysis
      • Poor pupillary light response
      • Partial III nerve paralysis (uncal entrapment of the nerve; compression under the posterior communicating artery)

Occipital Lobe Hematoma

  • General characteristics
    • Headache in or around the ipsilateral eye
    • If visual fields' deficit is present:
      • Patient is aware of visual loss but may ascribe it only to the contralateral temporal field (may miss ipsilateral nasal cut)
    • Visual neglect of contralateral field if the hematoma extends into the parietal lobe
    • Rare weakness

Medial Occipital Hemorrhage

  • Headache (C2 distribution or radiates to the ipsilateral eye)
  • Congruent homonymous hemianopia if above and below the calcarine fissure
    • Dense
    • Respects the midline
  • Visual field variants depend on topography
    • Checkerboard deficit (lesions above and below calcarine sulcus)
    • Superior or inferior quadrantanopsia
    • Anterior lesions (spared temporal crescent)
    • Occipital pole lesions (loss of macular vision)
  • No depression of consciousness
  • No memory loss
  • No weakness

Lateral Occipital Hemorrhage

  • Headache at onset (similar to medial occipital lobe radiation)
  • No visual field deficit
  • No sensorimotor or behavioral deficit
  • if extension to parietal lobe or medially:
    • Depressed level of consciousness
    • Contralateral neglect
    • Agitation
    • Congruent contralateral homonymous hemianopia

Primary Intraventricular Hemorrhage

Differential Diagnosis

  • Subependymal vascular malformation
  • Cavernous angioma
  • Dissection of caudate hematoma
  • Angioma of the choroid plexus (children)
  • Vertebral artery aneurysm (IV ventricle)

Clinical Signs and Symptoms

  • Similar to subarachnoid hemorrhage
    • Stiff neck
    • Early vomiting
    • Lethargy
    • Bilateral hyperreflexia
    • Positive Babinski's sign

Infratentorial Intraparenchymal Hemorrhage

Cerebellar Hemorrhage

  • 10% of intracranial hemorrhage
  • Anatomy
    • Initiated in the area of the dentate nucleus
    • At the level of the anastomosis of distal PICA and SCA
    • Long perforating branches of the SCA may be involved
    • Rarely:
      • Vermis hemorrhage:
        • Medial branches of PICA and the SCA
      • Hemorrhage extension
        • Ipsilateral hemisphere
        • Opposite hemisphere or vermis
        • Rarely to the brainstem
        • Cerebellar peduncles with rupture into IVth ventricle
Early Symptoms and Signs
  • Inability to walk, rarely to sit or stand (pressure on descending pontine and middle cerebellar peduncle fibers)
  • Falling to the side of the lesion
  • Vomiting
  • Headache (occipital, neck, C2 radiation)
  • Dysarthria
  • Rotary nystagmus (greater to the side of the lesion, occasionally gaze paretic to the opposite side); floccular nodular lobe involvement
  • Rare loss of consciousness at the ictus
Evolving Neurologic Signs
  • VI nerve palsy (side of the lesion)
    • Ipsilateral forced eye closure (to prevent diplopia)
    • Head tilt (C2 root irritation) to the ipsilateral side
  • Smaller ipsilateral pupil
  • Failure to check greater than dysmetria or terminal 1/3 ataxic arm movement
  • Severe gait ataxia
  • Quadriparesis without quadriplegia
  • Increased lower extremity reflexes without Babinski's signs

Large Hemorrhage

  • Early brainstem compression:
    • All signs and symptoms noted with smaller lesions but telescoped in time
    • Stupor to coma
    • Lateral gaze palsy (eyes deviated to side of the lesion) pressure on the para pontine reticular formation paralyzes contralateral conjugate gaze)
    • Quadriparesis (basilar strokes there is severe quadriplegia)
    • Bilateral Babinski's signs
    • Bilateral pontine pupils (<0.5 mm; reactive to light)
    • Central neurogenic hyperventilation (CNH)
Terminal Signs and Symptoms
  • Caudal cerebellum involved
    • Medullary cardiovascular and respiratory centers involved
      • Respiratory arrest
      • Vasomotor collapse
      • Decerebration
      • Coma
  • Radiographic evaluation
    • Obliteration of the cerebellopontine angle cisterns
    • Displacement of the IV ventricle
    • Hydrocephalus
    • Rarely upward herniation with vermian compression of the collicular plate
      • Gaze palsy > pupillary defects
    • Hydrocephalus of lateral and third ventricles

Small Hemorrhages with Survival

  • Mild gait ataxia
  • Rotary nystagmus
  • Signs and symptoms of hydrocephalus
    • Dementia
    • Abulia
    • Gait ignition failure in addition to ataxia
    • Hyperreflexia legs > arms

Lateral Cerebellar Hematoma

  • Cranial nerve involvement
    • V, VI, VII and VIII
    • Concomitant ataxia

Cerebellar Vermian Hemorrhage

  • Medial PICA and SCA vessels rupture
  • Rare
  • Severe headache
  • Vomiting
  • Quadriparesis
  • Coma

Differential Diagnosis of Cerebellar Versus Basilar Artery Occlusion

Basilar Artery OcclusionCerebellar Hemorrhage
1. herald hemiplegia1. sudden onset
2. quadriplegia2. quadriparesis
3. "ocular bobbing"3. rotary or horizontal nystagmus
4. horizontal gaze paralysis4. conjugate deviation to the ipsilateral side
5. no cerebellar signs5. failure to check > dysmetria
6. collapse6. severe gait ataxia
7. Early Central Neurogenic Hyperventilation7. late CNH
8. Early pupils changes < 0.5 mm8. 1 mm pupils ipsi > contralateral side
9. Early coma9. late coma

Differential Diagnosis of Cerebellar Hemorrhage

  • HCVD (PICA/SCA vessels; dentate nucleus)
  • AV malformation
  • Hemangioblastoma (lateral lobes)
  • Cavernous angioma (may be multiple)
  • Associated with Von Hippel Lindau (retinal and spinal angiomas)
  • Metastasis
  • Anticoagulation (INR > 5 on Coumadin)
  • Ischemia transformation of infarct
  • Trauma
  • Venous malformation (usually don't bleed)

Primary Pontine Hemorrhage

  • Location:
    • Midventral pons (at the level of Vth nerve or junction of basis pontis and tegmentum)
    • Oval shape: destroys the center of the ventral tegmentum and basis pontis
    • Dissects:
      • Rostrally to the midbrain
      • Rarely to the medullary
      • Commonly into the IVth ventricle
      • Rarely ruptures through the pial surface with spread to the clivus
  • Caused by rupture of paramedian basilar penetrating artery

Clinical Signs and Symptoms

  • Decreased level of consciousness or coma early in the course
  • Hypertension early to vascular collapse during evolution (pressure on vasomotor center of lateral medulla)
  • Central neurogenic ventilation early (20–40 breaths/min to apneustic gasp prior to death)
  • Early hemiparesis
  • Quadriplegia with increased tone of all extremities during evolution
  • Headache (basiocciput) and vomiting (occasional)
  • Gradual asymmetric onset (occasional)
  • Asymmetric weakness of face and extremities
  • Abnormal movements:
    • Shaking tremors (partial decerebration)
    • Shivering and fasciculation (chest wall)
    • Dystonic posture versus decerebration
  • Weakness or paralysis of the face, pharynx, palate and tongue
  • Ocular manifestations
    • Pinpoint pupils < 0.5 mm that are reactive to light (destruction of descending sympathetics vs activation of parasympathetics)
    • Anisocoria
    • Skew deviation (greater part of hemorrhage to the side of down eye)
    • Bilateral paralysis of horizontal gaze
      • Destruction of the PPRF
    • Ocular bobbing (eyes driven down and float up; vestibular tonic drive of upgaze mechanism)
      • May be unilateral
    • Up-bobbing (may be unilateral)
    • Ocular dipping (rapid down and upward movements), may be unilateral
    • 1 and 1 1/2 syndrome (ipsilateral destruction of the PPRF with concomitant destruction of the posterior crossing fibers of the MLF; patient can only abduct the contralateral eye)
    • Pontine exotropia (lateral and down deviation of the contralateral eye), the only voluntary eye movement is contralateral abduction
    • Posterior MLF syndrome (convergence is preserved)
    • Paralysis of upgaze if there is midbrain extension and destruction of the riMLF

Rarer Symptoms and Signs

  • Prior to coma:
    • Bilateral deafness (trapezoid body)
    • Severe dizziness and vertigo
    • Gustatory abnormalities (dysgeusia)
    • Preservation of reflex horizontal gaze
    • Locked in syndrome
    • Hallucinations (between 3–50 days following the ictus)
    • Hyperhidrosis
    • Neurogenic bladder (destruction of the pontine micturition center)
    • Hypo or hyperthermia (>105° Fahrenheit)

Prognosis

  • Most often death in 24–48 hours, rarely a patient survives 7–10 days

Unilateral Basilar or Tegment basilar Hemorrhage

  • Paramedian hemorrhage
    • Exclusively or predominantly on one side of the pons
    • Basotegmental hemorrhage is usually larger than those confined to one side of the pons
    • Small lateral basal hematomas
      • Present as:
        • Pure motor hemiparesis
        • Ataxic hemiparesis
        • Clumsy hand dysarthria syndrome
      • Dissection of lateral basal lesions into the tegmentum
        • Cranial nerve V, VI, VII deficit
        • Contralateral hemiparesis

Differential Diagnostic Points Separating Hemorrhage Versus Lacunar Strokes

  • Occipital headache (hemorrhage)
  • Nausea or vomiting (hemorrhage)

Paramedian Tegmental Hemorrhage

  • Position and higher sensory modality loss in the contralateral arm, face and leg
  • Ipsilateral 1 1/2 syndrome
  • Motor dysfunction of the contralateral extremities
    • Ataxia
    • Decerebration
    • Dystonia

Lateral Pontine Tegmental Hemorrhage

  • Rupture of small penetrating vessels from long circumferential arteries
  • Pupillary defects:
    • Anisocoria (smaller ipsilateral pupil, normal reactivity)
  • Ocular motility defects:
    • Paralysis of ipsilateral conjugate gaze
    • 1 1/2 syndrome (if posterior MLF affected)
    • Intranuclear ophthalmoplegia (posterior type)
    • Ipsilateral VIth nerve deficit
    • Pontine exotropia
    • Ocular bobbing, dipping
  • Cranial nerve deficits:
    • V, VI, VII
  • Moderate hemiparesis
    • Bilateral weakness may occur at ictus; ipsilateral deficit clears
    • Limb and truncal ataxia, ipsilateral > contralateral, may be bilateral

Clinical Differential Points of Lateral vs Paramedian Lesion

  • Quadriparesis or quadriplegia (paramedian)
  • Contralateral hemisensory deficit: (lateral)
    • Lemniscal modalities and spinothalamic deficits contralaterally
  • Rare findings:
    • Decreased hearing (trapezoid body); paramedian lesion
    • Dysarthria (posterior 1/3 of ventral tegmentum)
    • Dysphagia (corticobulbar fibers X, XII)
    • Decreased ipsilateral V (corresponding decrease of corneal reflex)
    • Bilateral ptosis (rare)
    • Convergent and divergent eye movements associated with:
      • Ipsilateral facial numbness
      • Contralateral sensory deficits
      • No motor abnormality

Differential Diagnosis of Primary Pontine Hemorrhage

  • Hypertensive cardiovascular disease
    • Middle aged patients
  • Vascular malformations (younger patients)
    • Von Hippel Lindau disease
    • Zona Bannayan syndrome
    • Cobb's syndrome
    • Cavernous angioma
    • Capillary telangiectasia (rarely bleed)
  • Anticoagulation
  • Coagulopathy
  • Trauma (associated with basilar skull fracture)

Medullary Hemorrhage

  • Approximately 0.5–1% of ICH
  • Bleeding can occur from:
    • Direct penetrating arteries of the distal vertebral artery
    • Branch arterial rupture from PICA, AICA or anomalies of the vertebral artery (basilarization of the vertebral artery)
      • Hemorrhage may be involved with concomitant cerebellar infarct
    • Dorsal branches of PICA
      • Supply the dorsal medulla
      • Concomitant cerebellar infarction if these arteries rupture
  • Clinical Signs and Symptoms
    • Partial or complete lateral medullary syndrome depending on specific arterial rupture

Secondary Pontine Hemorrhage

  • Diencephalic herniation
    • Sretch and rupture of paramedian pontine penetrators from the basilar artery (Duret's hemorrhage)
    • Associated midline thalamic and midbrain hemorrhages
  • Blood dyscrasia
  • Coagulopathy
  • Sudden severe increase of intracranial pressure

Prognostic Factors for Intraparenchymal Hemorrhage

  • Size and level of consciousness
    • <10 cm3 – excellent prognosis
    • <45 cm3 – fair to good prognosis
    • >60 cm3 – poor prognosis
    • >85 cm3 – death
  • Other predictors of prognosis
    • intraventricular hemorrhage
    • hydrocephalus
    • edema
  • 30 day mortality – approximately 45%
    • 50% of deaths occur in the first 48 hours
  • Pineal displacement laterally <10 mm, <5 mm ventrally good prognostic sign
  • Lower mortality in lobar ICH than deep hemispheric hemorrhage

Neurologic Deterioration in Non-comatose Patients with Supratentorial Intra-Parenchymal Hemorrhage

  • Occurs in approximately 1/3 of patients
  • Greatest first hospital day
  • Neurologic deterioration:
    • >45 ml (60%)
    • 10 ml (19%)
  • 30 day mortality of patients with neurologic deterioration approximately 50%; 3% in those that are stable
  • Cerebral edema related to large established hemorrhages is the predominant cause of deterioration
  • Prognosis by size of hematoma volume
    • 50 ml or > – 78% deteriorated
    • 20–50 ml – 33% deteriorated
    • <20 ml – 16% deteriorated
  • Hemorrhage growth 0.8–13%
    • Progressive deterioration
      • Secondary to perifocal edema

Major Diagnostic Features of Intraparenchymal Hemorrhage

  • Gradually progressive neurologic deficit
    • Minutes to hours
    • Headache and vomiting
  • Exceptions: severe early headache
    • Caudate hemorrhage with intravascular extension
    • Primary intraventricular hemorrhage
    • Early severe motor deficits
      • GPe/GP1 hemorrhage
      • Large putaminal hemorrhage (usually mid portion)
Imaging Analysis (CT/MRI)
  • Early < 24 hours
    • regular and smooth
  • First 48 hours
    • blood fluid levels
  • First 72 hours
    • Mild edema and mass effect
  • 3–20 days
    • Resolution of the mass which starts at the periphery
    • Irregular contents that enhance to be differential from metastatic lesion, lymphoma (particularly in an HIV patient), abscess and inflammatory lesion; often hemorrhage is ring enhancing
  • Specific locations
    • Multiple subcortical white matter hemorrhage
      • Leukemia
      • Coagulopathy
      • Disseminated intravascular coagulation
    • Cerebellum
      • Anticoagulation (Coumadin)
    • Lobar in the elderly (65–90 years)
      • Congophilic angiopathy
  • Nine weeks:
    • Enhancement and mass effect have dissipated

Hypertension

  • General features:
    • Most common cause of ICH
    • Blood pressure is 220–240 mmHg systolic and 110–120 mmHg diastolic
      • In several studies the peak systolic pressure is 160–190 mmHg
    • Pressure may be confounded by the Kocher–Cushing reflex; secondary hypertension due to the effects of pressure on the vasomotor center
    • Many patients present without a prior history of hypertension
      • Examination may reveal:
        • Enlarged aortic knob (uncorked)
        • Thickened and tortuous peripheral blood vessel (radial and axillary arteries)
        • Hypertensive changes in the retinal arteries
        • Loud A2 and S4 gallop
        • Sustained cardiac impulse
      • Hypertension induced degenerative changes in vessel walls
        • Fibrinoid degeneration
        • Microaneurysm:
  • Penetrating arteries of the basal ganglia, pons, thalamus and cerebellum
  • Also noted in arteries that supply the grey-white junction
  • Associated concomitantly with lipohyalinosis and fibrinoid degeneration
  • Possible relationship exists between iris microaneurysms and those found on cerebral blood vessels

Non-Hypertensive Causes of Intracranial Hemorrhage (ICH)

General Characteristics

  • Acute changes in blood pressure and blood flow:
    • Precipitate rupture of penetrating arteries without prior hypertension
    • Microaneurysm on penetrating vessels rupture: Predilection for lenticulostriate arteries (Charcot-Bouchard aneurysm).
    • Fibrinoid degeneration of penetrating vessels after sustained hypertension rupture.
    • Microaneurysms:
      • May bleed repeatedly (hemosiderin laden macrophages)
      • Located in penetrating vessels of:
        • Lenticulostriate (MCA)
        • Pons
        • Cerebellum
        • Grey white cortical junction
    • Penetrating arteries may rupture:
      • Not at the site of microaneurysms
      • Generalized lipohyalinosis of entire artery
  • Exposure to cold weather.
    • Stimulation of the sympathetic vasoconstrictor response
  • Dental procedures.
    • Traction on the trigeminal nerve.
      • Changes in heart rate and blood pressure
  • Increased cerebral blood flow:
    • Carotid endarterectomy (hyperperfusion syndrome)
    • Following surgical repair of congenital heart defects
    • Cardiac transplantation
    • Post migraine
  • Older patients:
    • ICH at lower pressure than younger patients:
      • Possible relationship to amyloid angiopathy
  • Biphasic presentation of ICH.
    • Onset of new hypertensive state.
    • Prolonged hypertension.
Underlying medical conditions that are associated with ICH:
  • Coagulopathy.
  • Vasculitis.
  • Subacute bacterial endocarditis.
  • Tumor.
  • Trauma.
  • Thrombolytic treatment.
  • Transformed ischemic stroke.
    • Frequently multiple
    • Not in classic hypertensive ICH locations. (centrum semiovale)

Vascular Malformations

The major vascular malformations that are clinically relevant are:

  • Arterio-venous malformations
  • Dural arteriovenous malformation
  • Cavernous hemangiomas
  • Venous malformations
  • Capillary telangiectasia

Parenchymal arteriovenous malformations are abnormal connections of arteries and veins with no capillary bed. The arteries themselves have poorly developed internal elastic lamina and often become stenotic and occluded. In a sizable percentage, these vessels harbor aneurysms. The natural history of an AVM is to enlarge, recruit blood vessels from neighboring circulations and to bleed. They most often present with seizures due to the abnormal neurons and glial tissue within the malformation. They may present with seizures, hydrocephalus or chronic migrainous headaches. These headaches are stereotyped and do not switch sides during different attacks Some patients note fluctuating bruits concomitant with their pulse. Approximately 2% have a progressive neurologic deficit.

Dural arteriovenous malformations are abnormal arteriovenous shunts within leaflets of the dura mater usually within or near walls of the dural venous sinuses. Headache may be migrainous or localized to the side of the lesion. Patients suffer increased intracranial pressure, hemorrhage, pulsatile, tinnitus and seizures. Headache may be worse in the supine position. They may become symptomatic during pregnancy.

Cavernous angiomas have no entrapped brain tissue, appear anywhere in the brain, but are often periventricular. They are frequently multiple and especially common in Mexican Americans. They usually are not associated with vascular headache. They most often clinically present with recurrent bleeding with focal neurologic deficit (parenchymal type) seizures and subarachnoid hemorrhage. They have a characteristic "popcorn" appearance in MRI from the mixed signal of old and new hemorrhage. They often hemorrhage at night.

Venous hemangiomas are the most common type of vascular malformation. They are composed of anomalous veins and have no direct arterial input. They most often are linear tubular structures in the cerebellum with connection to the IVth ventricle and may demonstrate a "caput medusa". They most often are asymptomatic except when occluded during surgical procedures. In these instances, they may cause cortical infarction.

Telangiectasia are comprised of capillaries separated by normal brain parenchyma. They are most often seen in the cortex and the pons. They are rarely symptomatic.

Perimesencephalic hemorrhage has recently been recognized as a form of hemorrhage that presents frequently as a subarachnoid bleed without focal findings. Blood on CT is most prominent in prepontine and perimesencephalic listerics. The process only seems to occur once and has an excellent prognosis. It may represent venous hemorrhage.

Parenchymal Arteriovenous Malformations

  • General Features:
    • 10% as common as subarachnoid hemorrhage
    • Peak presentation in 2nd and 3rd decade
    • Increased incidence of aneurysms with AVM; usually along feeding blood vessels
    • Some afferent vessels become stenotic and occluded
    • Arteries within the arteriovenous malformation are thin walled with poorly developed internal elastic lamina; there is hypertrophy of the media and endothelial thickening of the afferent vessels
    • The natural history of arteriovenous malformation
      • Enlarge, grow and recruit vessels from neighboring circulations
    • Composition of the arteriovenous malformation:
      • Abnormal gliotic parenchyma is intermixed between component blood vessels
      • No capillary bed
      • Abnormal neurons
    • Parietoccipital > frontal > temporal lobe
  • Clinical Signs and Symptoms:
    • 48% present with seizures
    • 2% have progressive neurologic deficits
    • Chronic headache:
      • Migrainous; often are stereotyped and do not switch sides
      • May have associated increased ICP and papilledema
    • Rare presentation with hydrocephalus:
      • Common with great vein of Galen location in children
      • Prior small bleeds block CSF absorption
    • Fluctuating bruit:
      • Concomitant with heart beat
      • Rarely heard over the malformation
      • Jugular venous hum (heard in the neck)
    • Hemi asymmetry of the side opposite the lesions
      • Most severe if the parietal lobe is involved
    • Hemorrhage is not strictly limited to the subarachnoid space
    • Cortical or ventricular surfaces most likely to rupture
    • Focal deficits specific to location
    • May have asymptomatic bleeds (hemosiderin deposits on gradient ECHO MRI sequence)
    • Rupture 2.2% per year
    • Mean risk of death from rupture is 29%
    • Meningeal signs if rupture is primarily in the subarachnoid space
Imaging Evaluation
  • MRI
    • Honey combed areas
    • Serpiginous flow voids
    • Brain atrophy and ventricular dilatation
    • Adjacent cysts
    • Intralesional calcification
  • Arteriographic features definitive
    • Enlarged feeding vessels from several circulations (most frequent)
    • Tortuous enlarged draining veins
    • Central arterial venous complex of vessels
    • Rapid arterial to venous shunts
  • Negative arteriogram with AVM malformations:
    • Spontaneous thrombosis
    • Obliteration with hemorrhage
    • Cavernous angioma or dural anterior venous malformation
      • No feeding arteries
    • Spinal AVM
      • May mimic intracranial signs and symptoms
        • Headache and stiff neck
  • Features Increasing the Risk of Hemorrhage:
    • Small malformation
    • Exclusively deep venous drainage
    • High intranidal pressure
  • AVM of the lateral ventricle:
    • <40 years of age
    • Small
    • Intraventricular hemorrhage
    • Lateral ventricle usually normal sized
    • Caudate and choroid are other sources of intraventricular hemorrhage

Dural Arteriovenous Malformation

General Features

  • Abnormal arteriovenous shunts within leaflets of the dura mater.
    • Usually within or near the walls of dural venous sinuses.
  • Comprise 10–15% of AVM's.
  • Pattern of drainage:
    • Intradural sinuses.
    • Cortical or deep venous system.
  • DAVM's with prominent cortical venous systems have a higher incidence of bleeding than those without.
  • Symptoms of these malformations are primarily those of venous hypertension that is due or related to:
    • Increased arterial input and therefore increased flow through the draining veins.
    • Venous outflow obstruction.
  • Congenital DAVM's.
    • Vein of Galen type.
      • Due to retention of en embryonic median parencephalic vein; if it persists it becomes the sac for the aneurysmally enlarged vein of Galen.

Etiology of DAVM

  • Trauma; associated with skull fractures.
  • Female hormones: influence placental and uterine vascular growth.
  • Dural sinus thrombosis; female > males 2:1 with thrombosis of the lateral sinus and increased venous drainage enlarging dural veins.
  • May become symptomatic with pregnancy.
  • Thrombosis or stenosis within the draining dural sinus:
    • Enlarges the DAVM.
    • Increases venous hypertension.

Clinical Signs and Symptoms

  • Headache (global and frontal); may be migrainous or localized to the side of the lesion.
  • Increased intracranial pressure.
  • Hydrocephalus.
  • Hemorrhage.
    • Signs and symptoms depend on the drainage pattern:
      • Solely dural
      • Drainage pattern into the cortical or deep venous system
    • Intraparenchymal > subdural > subarachnoid in location
    • 30% mortality
    • Pulsatile tinnitus.
    • Seizures.
    • Cerebral edema with venous hypertension and sinus occlusion.
    • Postural headache (worse in supine position).

Location of DAVM

  • Most common are related to the lateral sinus and involve the transverse or sigmoid portions; approximately 60% of DAVM's.
    • Feeding vessels of lateral sinus fistulae:
      • Occipital, middle meningeal, accessory meningeal from the external carotid.
      • Ascending pharyngeal (arch of the aorta)
      • Meningohypophyseal trunk of the cavernous carotid artery.
      • Dural branches of the vertebral arteries.

Cavernous Sinus DAVM

  • General Features
    • Occur in approximately 16% of DAVM; 2% of patients with head trauma.
    • Abnormal shunting of blood from the internal and external carotid into the cavernous sinus.
      • Type A
        • Direct carotid cavernous fistula
        • Large shunted blood volume
        • Dramatic symptoms
      • Type B; indirect CCF
        • No direct communication between ICA-cavernous sinuses
        • Branches of ICA or ECA and the cavernous sinus
        • Type C
          • Fed by dural branches of ECA
        • Type D
          • Branches of ICA/ECA
      • Orbital venous hypertension causes:
        • Proptosis
        • Scleral arterialized venous blood; the arteries reach the iris whereas in infection they don't.
        • Chemosis.
        • Papilledema.
        • Glaucoma.
      • Cavernous sinus drainage into:
        • Petrosal veins and sinuses
        • Basal vein of Rosenthal
        • Results in brainstem venous hypertension
      • Cranial nerve III, IV, VI and first division of V (V1) may be compromised.
      • Etiology.
        • Trauma.
          • MVA with head injury
          • Gunshot wounds
        • Rupture of ICA infraclinoid aneurysm.
  • Etiology
    • Ehlers Danlos type IV
    • FMD
    • Arteriosclerosis
    • HCVD
  • Indirect Fistulas
    • Post menopausal
    • Spontaneous
  • Direct Fistulas
    • Trauma
  • Differential Diagnosis
  • Tumors
    • Pituitary adenoma
    • Metastasis
    • Craniopharyngioma
    • Nasopharyngeal cancer
    • Hyperthyroidism
    • Retrobulbar masses
      • Hemangioma
      • Bone tumor
      • Mucocele
  • Rare DAVM's
    • Incisura of the tentorium 8%
    • Cerebral convexity – sagittal sinus
    • Orbital-anterior falx
    • Drainage into the vein of Galen, straight sinus or posterior falx
    • Acquired adult vein of Galen – DAVM
      • Related to dural sinus occlusion
      • Supplied by middle meningeal and vertebral artery, PCA and meningohypophyseal branches of the ICA
      • Shunt is into the wall of the vein of Galen
      • Occasional association of a vein of Galen aneurysm with DAVM has been noted

Prognosis and Natural History

  • An aggressive course is correlated with
    • Drainage into subarachnoid and parenchymal veins
    • Retrograde flow away from the lesion
  • Spontaneous closure may occur
  • Minimal symptoms for prolonged periods
    • Headache
    • Pulsatile tinnitus

Spinal AVM

General Features

  • Subarachnoid bleeding may occur with cervical dural AVM
    • Vertebral artery blood supply
    • Cervicomedullary junction in location
  • Thoracic, lumbar and sacral malformations rarely bleed; epidural hemorrhage is presentation if they rupture
    • Some dural fistulas are located in the in spine or paravertebrally.
  • Clinical signs and symptoms:
    • Pain occurs in approximately 40% of patients; may be radicular
    • Symptoms may worsen after exercise
    • May present with Brown-Sequard distribution
    • Usual course is progressive weakness with bladder involvement
    • Local back pain
    • Headache (intracranial meningeal irritation)
    • Those in spine or paravertebral location
      • Compressive myelopathy
  • Hemorrhage may be initiated by coagulopathy or anticoagulation
  • Blood may be seen:
    • Intraventricularly
    • Basal cisterns
    • Re bleeds occur in 50% of patient
    • 90% of patients have neurologic deficits

Type I Malformation Dural Form of Spinal AVM

  • Dural location most frequent
  • Blood supply derives from arteries located in the dural sleeves of spinal roots
  • Arteriovenous malformation fed by dural branches of a radicular artery
  • Fistula drained intradurally by:
    • Enlarged tortuous vein (seen partially on the dorsal cord)
    • Usually above the arterial feeder
  • Men > women 4:1
  • Age at onset of symptoms between 40–70
  • Usually located at lower thoracic and lumbosacral levels
  • Not associated with arterial or venous aneurysms
    • Low flow

Cavernous Angiomas

General Features

  • No entrapped brain parenchyma (well encapsulated)
  • Hyalinization and thickening of peripheral vessels
  • Often multiple
  • Familial (Mexican-American)
  • Zonula – Bannayan syndrome
  • Higher incidence of associated cavernous hemangioma
  • Cavernous hemangiomas are located in the posterior fossa in this circumstance
  • Associated with dural AVM
    • Most common location in this instance is the posterior fossa
  • May appear anywhere in brain parenchyma
  • Often periventricular location
  • May occur:
    • Spinal cord
    • Subarachnoid space
    • Dura
    • Multiple locations simultaneously
  • Compact sinusoidal vessels

Clinical Signs and Symptoms

  • Recurrent bleeding with focal neurologic deficit (parenchymal)
  • Seizures
  • SAH
  • Usually no correlation with vascular type headache
  • Rare occurrences:
    • Visual loss from chiasmatic lesions
    • Simultaneous bleeding from two lesions
    • Hemigeusia from pontine lesions
    • Associated with hippocampal sclerosis
  • May bleed intraventricularly

MRI Imaging

  • Multiple lesions
  • "Pop corn" appearance of the lesion
    • Mixed signal on T1; old blood (10–14 days) and new blood (<4 days)
    • Hemosiderin ring around the lesion with low intensity signal on T1 sequences

Venous Angioma

General Features

  • Most common type of vascular malformation
  • Composed of anomalous veins; no direct arterial input
  • Venous varix:
    • Often linear tubular structure in the cerebellum with connection to the IVth ventricle
    • May demonstrate a "caput medusa"
    • Cortical infarction if operated (occurs in the territory drained by the malformation)
    • There is normal brain parenchyma around the malformation
  • Imaging characteristics of venous angioma
    • Linear on MRI
    • Frequently near a ventricle
    • Large prominent central draining vein
      • May be dilated into a varix
      • Hyalinized wall

Clinical Features

  • Usually asymptomatic
  • Surgical removal may be associated with:
    • Infarction of the territory they drain
    • Venous angiomas should be preserved
  • Rare associations with:
    • Hydrocephalus (midbrain)
    • Spontaneous hemorrhage
      • Thrombosis of central draining vein
    • Developmental venous anomalies
    • Cerebral hemiatrophy
    • Aqueductal compression

Telangiectasia

  • Comprised of capillaries separated by normal brain parenchyma
  • Most common locations:
    • Pons
    • Cortex
  • Sudden VIth or VIIth nerve palsy
  • Rarely symptomatic
  • Often multiple

Non-Hypertensive Causes of Parenchymal and Subarachnoid Hemorrhage

There are multiple causes of parenchymal and subarachnoid hemorrhage that are not caused by idiopathic hypertension. Many are caused by induced hypertension in patients with underlying cystic aneurysms and vascular malformations. They frequently have very specific clinical profiles that are diagnostic.

Idiopathic perimesencephalic hemorrhage may account for approximately 10% of all SAH. It rarely occurs with congenital aneurysms and seems more likely to be caused by leakage of blood from the prepontine venous plexus. Most patients have no focal neurologic deficits on admission. The headache associated with this bleed reaches maximum intensity within minutes rather than seconds. There is no loss of consciousness with the ictus and no rebleeding or delayed ischemia.

Mycolic aneurysms most often occur in the distal branches of the MCA and cause both intraparenchymal and SAH. They may be the initial presentation of SBE.

Drug abuse with sympathomimetic agents and cocaine induce hypertension and cause hemorrhage as well as ischemic stroke. Cocaine in particular is associated with approximately 20% of ICH from an underlying aneurysm or vascular malformation. Drug abuse and SBE are coextensive.

Elderly nonhypertensive patients with superficial lobar hemorrhages should be suspected of having congophilic angiopathy. These hemorrhages are often recurrent which can be demonstrated by gradient ECHO MRI (demonstrates hemosiderin from prior bleeds).

Traumatic intracranial hemorrhages frequently are delayed to 4–7 days after ictus. They occur in the originally damaged brain issue.

Venous thrombosis with hemorrhage should be suspected in pregnant patients and those in a hypercoagulable state. Preceding sense headache lethargy and seizures are common.

Vasculopathy or vasculitis may also cause nonhypertensive hemorrhage.

Differential Diagnosis of Perimesencephalic Hemorrhage

  • Venous or capillary leak
  • Caudate hemorrhage that extends into the subarachnoid space
  • Subependymal AVM or malformations
  • Trauma
  • Blood dyscrasias
  • Leakage from small nonaneurysmal arteries on the brain surface
  • Ruptured aneurysms; site of rupture is thrombosed and not identified
  • Dural AVM
  • Cocaine
  • Sympathomimetic drugs
  • Pituitary apoplexy
  • Spinal AVM
  • Traumatic LP

Parenchymal Trauma With SAH

  • SAH often located in the superficial areas of the cortical sulci
  • Adjacent to a fracture site or area of intracerebral contusion
  • Basal frontal contusion
    • hemorrhage may mimic rupture of AcoA aneurysm
  • Direct neck trauma
    • tear of vertebral arteries or their feeders in the transcervical canals
    • basilar hemorrhage
  • May be delayed
    • occur 4–7 days in the contused area

Mycotic Aneurysm

  • A common cause of nonaneurysmal SAH and intraparenchymal hemorrhage
    • infective endocarditis
    • aspergillosis
  • Most common location
    • distal branches of the MCA
    • 10% have a proximal location
  • Most patients suffer ICH at the site of rupture; some present with typical SAH
  • May re bleed
  • May be the initial presentation of bacterial endocarditis
  • Mycotic aneurysms secondary to aspergillosis:
    • Proximal carotid location
    • Proximal vertebrobasilar system
    • Rupture is frequently fatal

Drug Abuse

  • General characteristics
    • 25% of drug related strokes are ICH
    • 6% related to recreational drug abuse
  • Location
    • Subcortical
  • Hemorrhage in a first time user
    • Occurs in minutes to hours
  • ICH responsible for approximately 50% of cocaine related stroke

Cocaine Abuse

  • Crack cocaine
    • Aqueous cocaine HCL with NH3 and baking soda
    • Smoked or inhaled
      • Aseptic necrosis of the nasal septum
    • Reaches the brain 10 seconds after it is smoked (1 circulatory pass)
    • Most rapid "high"
  • Effects of cocaine
    • Blood pressure increase
    • Increases temperature
    • Increases metabolism
      • Severe weight loss in chronic abusers
  • Signs and symptoms immediately after use:
    • Loss of consciousness
    • Headache
    • Focal neurological signs (vasospasm of specific blood vessels)
    • Frequent signs and symptoms due to concurrent alcohol use
    • Hemorrhages
    • Strokes occur shortly after use
    • Predilection for the brainstem
    • Bowel and myocardial ischemia may be associated
  • Approximately 20% of patient with ICH after cocaine use have an underlying vascular lesion: AVM or aneurysm
    • SAH occurs in approximately 48–80% of patients with an underlying aneurysm after cocaine use
    • Rebleed may occur even with normal angiography
    • Vessels do not show signs of vasculitis
    • Deep hemorrhage is less likely to be caused by an underlying vascular lesion
    • Some patients present with hypertensive encephalopathy
      • Usual pressure is 110–140 mmHg; 210–240 mmHg
      • Multiple hemorrhages
      • Cerebral edema

Heroine Abuse

  • General characteristics:
    • Cerebral or spinal cord ischemic stroke
    • FCH from SBE (IV route)
  • Clinical features:
    • Stroke occurs with use after a period of abstention
    • Ischemia of cerebral vessels:
      • Immediately after use
      • 6–24 hour delay
    • Pulmonary edema and transverse myelitis
    • If hemorrhage occurs:
      • Heroine altered or used concomitantly with:
        • Cocaine or methamphetamine
  • Laboratory evaluation:
    • Eosinophilia
    • Comb's positive hemolytic anemia
    • Increased immune globulins
    • False positive serology
    • Antibodies to hepatitis C

Amphetamine Abuse

  • General Features:
    • Hemorrhages occur a few minutes after drug use
    • Associated abuse of alcohol and other drugs
    • Focal ICH may be associated with:
      • Diffuse vasculopathy
      • Brain edema
      • Ischemic lesions
    • Clinical presentation:
      • Thin patients
      • Characteristics of adult attention disorder (chronic abusers)
      • Negative presentation:
        • Hypertension
        • Fever
        • Tachycardia
    • Fibrinoid necrosis of the media and intima of small and medium sized arteries
    • Arteriographic evidence of segmental constriction and "beading" of intracranial arteries (superficial cortical arteries)
    • Amphetamine and methamphetamine more likely to cause hemorrhage than dextroamphetamine
    • Hemorrhage may occur with doses as small as 20 mg
    • Solid form of D-methamphetamine base that can be smoked
      • Street name is "ice"
      • May reach the brain in <20 seconds in one CBF pass
  • Clinical Signs and Symptoms
    • Clinical presentation:
      • Symptoms occur a few minutes after use
      • Headache
      • Seizures
      • Focal neurological signs from ICH
      • Average age of patients 25.4 years
      • Intracerebral lobar hemorrhage most common
        • <5% have underlying vascular lesion
  • Laboratory Evaluation:
    • Comb's positive hemolytic anemia
    • Increased immune globulins
    • False positive serology
    • Antibodies to Hepatitis C

Phencyclidine (PCP)

  • Angel dust
  • Signs and symptoms
    • ICH
    • Hypertensive encephalopathy
    • Self destructive behavior
    • Dramatic nystagmus

LSD

  • Hypertension
  • Hallucinations
  • Vasoconstriction but no ICH

Phenylpropanolamine (PPA)

  • May be combined with caffeine or antihistamine
  • Partial α-adrenergic agonist
  • Clinical signs and symptoms
    • <30 years of age; female > male
    • SAH may occur
    • ICH
    • Blood pressure on admission
      • 160/104–210/110 mgHg
    • Segmental angiographic beading of intracranial vessels (similar to amphetamines)
    • Location: lobar > basal ganglia > thalamic
    • Reactions to PPA are often idiosyncratic
    • Risk of hemorrhage with PPA use occurs with:
      • Post partum use
      • Caffeine
      • Higher than recommended doses
      • Alcohol
      • Caffeine
      • Prior hypertension
      • Monoamine oxidase inhibitors1
        • Multiple simultaneous hemorrhages

Ethanol

  • Mechanisms predisposing to hemorrhage
    • Platelet abnormalities
    • Impaired production of clotting factors with concomitant liver disease
    • Hypertension associated with chronic alcoholism
    • Binge drinking
      • Acute elevations of blood pressure
    • Concomitant use of cocaine in face of liver dysfunction
      • May potentiate vasoconstrictor effects

Pentazocine and Pyribenzamine ("T's" and "blues")

  • IV use: associated with: starch, methylcellulose crystals, take pulmonary emboli
  • Pulmonary hypertension:
    • Destruction of pulmonary arterioles with right sided hypertension
    • Embolic material reaches the cerebral circulation
    • Damaged cerebral vessels result in ICH
  • crystals and talc may be noted in cerebral vessels

Methylphenidate

  • Associated with ICH

Cerebral Congophilic Angiopathy as Cause of Non-Hypertensive Hemorrhage

  • General Features
    • Elderly non-hypertensive patients
    • Parietoccipital lobe subcortical location most frequent
    • 2–9% of ICH
    • 5% occurs 7th decade; 43% in 8th decade; 57% after 90 years of age
  • Location of CAA lobar hematoma
    • Frontal 35%
    • Parietal 26%
    • Occipital 19%
    • Deep central grey 4%
    • Cerebellum 2%
  • Clinical Characteristics
    • Large and may be multiple
    • Recurrent ICH or SAH
  • Gradient ECHO MRI
    • Multiple old bleeds (hemosiderin noted); old slit like hemorrhages
  • Rarer clinical presentation:
    • Insidious; similar to anticoagulant hemorrhage
    • Nausea and vomiting
    • Headache

Traumatic ICH

  • Concomitant contusions of the frontal and temporal lobes
    • Gyral contusions near bony prominences
  • Patients are frequently amnestic
  • Late hemorrhage
    • 4–7 days after trauma
      • Hemorrhage into damaged brain tissue

Metastatic Brain Tumors that Bleed

  • Most common hemorrhagic metastases:
    • Hypernephroma
    • Melanoma
    • Papillary cancer of the thyroid
    • Lung and breast
      • No specific proclivity to bleed but very common
    • Choriocarcinoma
      • Often metastatic to the dura
  • Many metastatic tumors have petechial hemorrhages; rarely bleed
    • New onset headache or seizures

Vasculopathy or Vasculitis as a Cause of Non-Hypertensive Hemorrhage

  • Rupture of affected vessels

Sinus Thrombosis

  • Post partum
  • Sagittal sinus most common:
    • Ill patient
    • Seizure in the contralateral leg
    • Bloody CSF
    • Cerebral edema
  • Dural AVM
    • Form as a consequence of sinus thrombosis; collateral venous pathways

Subarachnoid Hemorrhage

Subarachnoid hemorrhage is a devastating neurologic illness that carries a mortality of 30% with each bleed and very significant mortality in survivors.

The ictus is frequently characteristic as patients suffer a severe headache that reaches maximum intensity instantly. The headache is frequently basioccipital and then shifts to frontal areas. It is associated with vomiting in a significant number of patients with attendant loss of consciousness in approximately 30–50%. The neck is stiff, patients have positive Kearnig's and Brudzinski's signs and focal deficits depending on the location of the bleed. The patients' eyes are sore and photophobia is present. Seizures occur in 20% of patients. A sentinel bleed prior to the rupture of an aneurysm occurs in a significant proportion of patients. There is controversy whether enlargement of the aneurysm with stretch of the dura can cause a sentinel headache. Clearly at surgery there are often signs of hemosiderin and a prior bleed.

Familial aneurysm, polycystic kidney disease, Turner's syndrome and associated AVM are clear clinical settings for aneurysm. Acquired aneurysms have been noted both with trauma, atrial myxoma and SBE (mycotic) on peripheral arteries. Cigarette smoking and vasculitis may predispose at the circle of Willis. The role of hypertension in causation is controversial, but not in regard to rupture. Some autopsy series have demonstrated 0.9% of patients with a congenital aneurysm. An aneurysm of 7–10 mm is the most likely to bleed. Those less than 3 mm in general do not. An infundibulum at the take-off of cerebral arteries is usually less than 2 mm and do not bleed.

The use of coiling and interventional neuro-radioquaphic techniques has made aneurysm care much safer and successful for many patients. Unfortunately, vasospasm is still a major problem. The differential diagnosis for subarachnoid hemorrhage is wide and includes vascular malformations as well as trauma, congophilic angiopathy, hemostasis dysfunction and diseases of the blood vessel wall.

  • General characteristics
    • Headache
      • Severe: 10/10 on a visual analogue scale
      • Reaches maximal intensity immediately ("worst headache of my life")
      • Cervical-occipital location with frontal radiation; primarily C2 distribution
      • Lasts 4–6 weeks
      • Associations:
        • Vomiting
        • Loss of consciousness 30–50%
        • pain and soreness of the neck, shoulders and upper back
          • If there is never head pain suggests spinal AVM
        • Light sensitivity
        • Sore eyes
        • Focal motor or sensory deficits depending upon the etiology of the bleed
        • Seizures in 20%
    • Decreased level of consciousness
    • Vomiting: 30–50% of patients
    • Restlessness

Differential Diagnosis of SAH

  • Ruptured aneurysm
  • Arteriovenous malformation
  • Cavernous angioma
  • Congophilic angiopathy
  • Capillary, telangiectasia (very rare)
  • Dural arteriovenous malformation
  • Venous infarctions with hemorrhage
  • Sinus thrombosis
  • Head injury
  • Hemorrhagic metastasis
  • ICH with subarachnoid extension
  • Coagulopathies
  • Hemophilia
  • Amphetamines; cocaine; other sympathomimetic drugs
  • Migraine (extremely rare)
  • Intracranial arterial dissection

Specific Vascular Malformations

Congenital Aneurysms

General manifestations:
  • 0.9–6% of the general populations in autopsy series
  • 3.6% prospective autopsy series
  • 3.7% in retrospective angiography series
  • 6.0% in prospective angiography series in those undergoing arteriography
Incidentally discovered aneurysms by artery
  • Internal carotid – post communicating artery – 64%
    • internal carotid ophthalmic artery 19.9%
    • IC – anterior choroidal – 12.8%
    • IC – cavernous portion – 10%
  • Anterior communicating artery – 12.2%
  • Middle cerebral artery – 6.3%
  • Basilar artery (rare)
  • PICA – vertebral (rare)
  • AICA – basilar (rare)
Meta analysis of 23 studies of prevalence
  • 0.4% in retrospective autopsy series

Incidence of Unruptured Aneurysms by Age

  • 1.1%: 0–29 years of age
  • 3/5%: 40–49 years of age
  • 5.7%: 50–59 years of age
  • 7.4%: 60–69 years of age
  • 6.1%: 70–79 years of age
  • 9.3%: 80–89 years of age
  • 37.5%: >90 years of age

Natural History of Unruptured Aneurysms

  • 1.4% annual rate of rupture; other studies 3% bleed/year (untreated)
  • 10% have ruptured at 10 years; 20% at 20 years and 32% at 30 years
  • Multiple aneurysms
    • risk of rupture universally proportional to age
  • Aneurysmal growth is not associated with:
    • Sex
    • Blood pressure
    • Patient's age
    • Initial size
  • Associations with Hereditary Diseases of Collagen
    • Ehlers–Danlos (type IV; COL 1A gene, type III collagen)
    • Pseudoxanthoma elasticum (COL2A gene)
    • Marfan's syndrome
    • Osteogenesis imperfecta
    • Polycystic kidney disease (PKD gene chromosome 12)
    • Tuberous sclerosis
    • Neurofibromatosis I (chromosome 17)
    • Fibromuscular dysplasia (FMD)

Associated Other Diseases

  • Coarctation of the aorta
  • Congenital anomalous blood vessels
  • Arteriovenous malformation (arteries feeding the malformation)
  • Moyamoya syndrome
  • Takayasu syndrome

The Defect Underlying Aneurysm Formation

  • Occur at branch points of arteries of the Circle of Willis
  • Decrease of type III collagen
  • Defect of the arterial media and localized loss of muscular elements
  • Loss or breakage of the internal elastic membrane

Familial Aggregation of Congenital Aneurysms

  • 7–10% of patients with aneurysmal rupture have first or second degree relatives with an aneurysm
  • Among first degree relatives of those with ruptured aneurysms risk of SAH is four times that of the population at large
  • Risk highest among siblings of the index patient
  • Most families have 2–3 members that are affected if there is familial aggregation
  • Inheritance patterns are unclear; autosomal dominant most probable

Incidence of SAH with Aneurysms

  • 1/10,000 people
  • At risk for development of new aneurysm (after rupture of the first)
  • Each year: new aneurysms develop in 2% of patients with previously ruptured aneurysms, this group ruptures at an incidence of 6/10,000 in the general population
  • Morbidity and mortality
    • 12% die prior to medical attention
    • 40% die in the hospital within 1 month after the ictus
    • 1/3 of survivors suffer major neurologic deficits

Associated Clinical Features of Aneurysms with Underlying Collagen Defects

  • They are in typical locations
  • Bilateral
  • Associated with enlarged tortuous extracranial vessels
  • Occur at supra clinoid level
  • Associated with A-V fistulas
  • Associated with dural ectasia

Familial Aneurysms

  • Rupture at an earlier age than sporadic aneurysms
  • Smaller when they rupture
  • More often followed by the formation of new aneurysms

Multiple Aneurysms

  • Usually 2–3 in patients
  • Occur in 20–30% of patients
  • Rarely > 10
  • Occur with AVMs and other associated congenital anomalies of blood vessels

Factors Influencing Aneurysmal Rupture

  • Risk of rupture 1.9–3%/year
  • Aneurysms of <10 mm 0.7% rupture/year; usual size 7–10 mm at rupture
  • Aneurysms >10 mm 5.5% risk of rupture
  • Risk of rupture > in women than men
  • Incidentally found aneurysms lower risk of rupture than those found in addition to one that has ruptured
  • Aneurysmal SAH; male > female
    • Premenopausal female low risk of rupture
    • Postmenopausal female with hormone replacement (intermediate risk)
    • Postmenopausal female without hormone replacement (highest risk)
    • Alcohol consumption
  • Cigarette smoking; 3–10× greater risk than nonsmoker
    • Smoking increases chance of developing a new aneurysm
    • Decreases alpha antitrypsin (inhibits proteases)
    • Inherited decrease of alpha antitrypsin causes increased risk of aneurysms
  • ADPCK disease (autosomal dominant polycystic kidney disease) 4.4% (2.7–7.2%) chance of aneurysm
    • 9% risk in first degree relatives if 2 or more members have aneurysms
    • Life time risk for SAH in persons with an affected first degree relative is 1% at age 50; 2% at age 70
  • Familial predisposition 4.0% (2.7–6%)
  • Atherosclerosis 2.3%
  • 8% of aneurysms detected >8 mm in size
  • Incidence of rupture increases after the third decade; average age of rupture is fifty years of age, increases with age until 80
    • Female > male risk of rupture after 40
    • Male > female risk of rupture < 40
      • Anterior communicating aneurysms female > male
  • 75% of unruptured aneurysms <10 mm; 80% < than 6 mm
    • Median time from diagnosis of unruptured aneurysms to rupture <2 years
    • Linear association of risk of rupture with increasing size
  • Younger patients at greater risk of rupture in the setting of multiple aneurysms
  • Aneurysms that were followed and found to have ruptured had gown in size
  • Multiple aneurysms female > males
  • Setting of hypertension
    • Rupture more common in older patients
    • Hemiparesis, hemihypesthesia, and hemianopsia have been described in
    • 6–25% of unruptured aneurysms

Precipitating Factors Associated with Rupture

  • Valsalva maneuvers
  • Pregnancy (release of elastases)
  • Sleep (30% rupture during sleep)
  • extreme sudden hypertension (amphetamines; cocaine, PPA)
  • Weight lifting
  • Coitus
  • Defecation
  • 30% rupture during normal activity

The Clinical Spectrum of Unruptured Intracranial Aneurysms

  • Mean age at diagnosis 50 years (11–77 years)
  • Mean aneurysm diameter 2.1 cm

Group I – Acutely Symptomatic Patients

  • Headache
    • Sentinel hemorrhage
      • Possibly 30% of aneurysmal SAH
      • Minute tear of the aneurysm:
        • Sudden onset "thunderclap" headache
        • Maximum intensity instantly
        • Resolves in 48 hours, but may last weeks
        • Radiation to shoulder, neck and back; "flu-like" symptoms (with SAH)
        • Aneurysm thrombosis; meningeal inflammation
      • Ischemia (vasoconstriction of the parent vessel)
      • Seizure (rare)
      • Cranial neuropathy (III most common)

Group II – Chronic Symptoms (giant aneurysms)

  • Average size of giant aneurysms 2.1 cm
  • Headache (mass effect)
  • Visual loss (optic nerve or chiasmatic compression)
  • Pyramidal tract dysfunction (MCA giant aneurysm)
  • Facial pain (V1 involvement in cavernous sinus)
  • Diplopia
    • III and IV nerve – giant basilar tip aneurysm
    • P-COM; III nerve compression
    • AICA – VI nerve rare
    • Cavernous sinus VI nerve > III > IV nerve

Asymptomatic Patients

  • Aneurysm size 1.1 cm

Unruptured Aneurysm Major Clinical Presentations

  • Mass lesion
  • Stroke
  • Seizure
  • 77.4% at ICA and PCA sites
  • More common in women than men
  • MCA aneurysms; approximately 15% are symptomatic

Clinical Presentation of SAH From A Ruptured Aneurysm

  • General manifestations
    • Severe headache (noted above)
    • 50% of patients lose consciousness or are stunned
    • Photophobia
    • 30–50% have suffered a sentinel headache days to weeks prior to the rupture
    • Neck stiffness
      • Kernig's sign
      • Brudzinski's sign
    • Subhyaloid hemorrhage (Torsten's syndrome)
      • Side of the ruptured aneurysm (if there are multiple); sudden increased ICP with rupture of the preretinal veins
      • Shoulder, back or sciatic pain
      • Pain across the shoulders with "flu-like" symptoms
    • <5% pain is limited to the low back or neck without headache
    • Lethargy; inability to follow four part commands or perform visual praxis
    • Sore eyes to movement or palpation

Specific Clinical Presentation of Congenital Aneurysms

P-COM (posterior communicating carotid)

  • Pain in or behind the affected eye
  • Slowly reactive, oval or paralyzed pupil
    • Pressure sensitive parasympathetic fibers affected early or
    • Parasympathetic fibers localized to outer area 12-3 o'clock of the IIIrd nerve
  • Partial ptosis; lampshade ptosis secondary to infarction of the III nerve from medical diseases
  • Blood in the suprachiasmatic cisterns
    • Localized to the side of the bleed

A-COM Artery Complex (Anterior Communicating Artery Complex)

  • Mental status changes
    • Acutely: agitation (with no or minimal motor or sensory signs)
    • Several days following the ictus:
      • Abulia
      • Akinetic mutism
      • Amnesia
      • Decrease of executive function
    • Slight contralateral leg weakness and sensory loss
    • DH: Broca's type aphasia (SMA involvement)
  • CT/MRI evaluation
    • Blood in medial frontal lobe
    • Intra hemispheric fissure anteriorly and extending posteriorly
  • Anterior communicating artery syndrome
    • Amnesia
    • Confabulation
    • Personality change

Middle Cerebral Artery Trifurcation Aneurysm

  • Anterior temporal headache
  • Contralateral hemiparesis and hemisensory defect
    • Embolus from giant aneurysm
    • Vasoconstriction
    • Mass affect from hematoma following rupture
  • Rare seizure (compression or irritation of the cortex)
  • Aphasia (DH)
  • Weakness arm and face > leg
  • Aneurysm may be > 2 cm (giant aneurysm)
  • Large amount of blood in Sylvian fissure, vasoconstriction of MCA
  • Giant MCA aneurysm: seizures, dysphasia, hemiparesis

Internal Carotid Artery Aneurysm

  • Pain behind the eye
  • Ptosis (partial) and pupillary paresis
  • Rare complete IIIrd nerve palsy
  • Contralateral hemiparesis and hemisensory deficits
  • Nasal visual field deficit
    • Homonymous
    • Compression of the lateral chiasm

Ophthalmic artery aneurysm

  • Pain in or behind the eye
  • Painless loss of central vision
  • Rare bitemporal VF deficit
  • Chiasmatic syndrome (anterior)

Anterior choroidal artery aneurysm

  • Contralateral hemiparesis
  • Contralateral hemisensory defect
  • Central sector VF deficit
  • Transient focal ischemia of the internal capsule
  • Limb shaking (ischemia of internal capsule)
  • Tonic spasm (ischemia of internal capsule)
  • Rupture with:
    • Intracranial or intraventricular hemorrhage

Posterior Circulation Congenital Aneurysm

Vertebral Artery Aneurysm

  • PICA symptoms
  • Anterior spinal artery symptoms (VA-ASA)
    • Hemiparesis
    • Proprioception and vibration loss
  • Pain in the side of the neck
  • Rarely pain radiates to lateral eyebrow
  • Acute blood in the IV ventricle

PICA-Vertebral Artery Aneurysm

  • Dysphagia and hoarseness
  • Lateral pulsion (ipsilateral side)
  • Inability to stand
  • Nausea and vomiting
  • Pain in the basiocciput; lateral eyebrow or side of the face
  • Crossed hemisensory loss to pain and temperature
  • Oscillopsia
  • Acute rotary nystagmus (greatest ipsilaterally)
  • Ipsilateral Horner's syndrome
  • Blood in the IV ventricle and the posterior fossa

AICA-Basilar Artery Aneurysm

  • Sudden hearing loss or tinnitus
  • Peripheral ipsilateral facial weakness
  • Ipsilateral pain in the face
  • Contralateral pain and temperature loss (below the clavicle)
  • Contralateral hemiparesis
  • Blood in the posterior fossa

Superior Cerebellar-Basilar Artery Aneurysm

  • III nerve palsy
  • Ipsilateral dysmetria
  • Dysarthria
  • Blood in the perimesencephalic cistern

Top of the Basilar-PCA Bifurcation

  • Pain in the back of the neck or basiocciput
  • III nerve palsy (peduncular)
  • Visual field deficit (embolus to PCA)
  • Hemiparesis or quadriparesis (emboli or pressure on the cerebral peduncles)
  • Movement disorder (emboli or vasoconstriction of the thalamoperforate artery)
  • Thalamic sensory deficit (emboli or vasoconstriction of the thalamogeniculate artery)
  • Blood in the perimesencephalic cisterns; intra peduncular blood

PCA-P1 (posterior communicating artery)

  • Similar to top of the basilar–PCA aneurysm

Specific Clinical Signs Denoting Specific Aneurysms

  • Giant MCA aneurysm
    • Seizure (focal motor)
    • Dysphasia
    • Hemiparesis
  • IIIrd nerve compression
    • ICA (hemiparesis often associated)
    • P-COM
    • Superior cerebellar artery
  • Isolated VIth nerve following aneurysmal SAH
    • Blood
    • Increased intracranial pressure
  • Cavernous sinus aneurysm
    • VIth nerve first sign
    • Severe Vth nerve facial pain
    • Late: IIIrd, IVth, VIth
  • Basilar tip
    • Forward pointing
      • Destroys the pituitary (endocrine failure)
      • Visual field deficits
    • Vertical
      • IIIrd, IVth nerve
      • Compresses the IIIrd ventricle

Rare Clinical Signs of Aneurysms

  • Transient neurologic signs:
    • Ischemia of the parent vessel
    • Embolization:
      • Usually from giant aneurysms
      • Cause strokes in the affected territory
  • Torsten's syndrome
    • Subhyaloid hemorrhage (preretinal)
    • Hemorrhage is moveable with changing head positions
    • Due to sudden increased pressure
    • Occurs on the side of the ruptured aneurysm (20% of patients harbor two or more aneurysm)
    • Secondary to rupture of a preretinal vein
  • Prognosis of SAH from an aneurysm is better if no aneurysm is demonstrated

Radiological features of an aneurysm that has ruptured if more than one is present by arteriography

  • The largest aneurysm most often has bled
    • Most commonly between 7–10 mm
    • Giant aneurysm usually do not bleed
  • Aneurysm with the most excrescences
  • Local vasospasm
  • Local hemorrhage or hematoma

Aneurysmal Rebleeding

  • Clinical manifestations
    • Sudden abrupt severe headache (described above)
    • Meningismus
    • Focal signs secondary to early hemorrhage
    • Late
      • Vasospasm of the parent vessel with focal expected signs and symptoms
    • Associated ICH leads to rapid coma
    • Aneurysms that cause neurologic symptoms rupture at a higher rate
      • 15% of rebleeding within 6 months
    • Fewer re bleeds in patients with Hunt grades I and II
  • Time of aneurysmal rebleeding
    • 0.8–4% re bleed the first 24 hours
    • 20% re bleed the first 2 weeks
    • 30% re bleed by the end of the first month
    • 40% by the end of 6 months
    • >6 months 3%/year
  • Vasospasm concurrent with rebleeding
    • Occurs from day 3–5
    • Peaks between days 5–9
    • Resolves after the second week
    • Directly related to the amount of blood surrounding the artery
    • Configuration of the arteries
      • Focal vasoconstriction approximate to area of the bleed
      • Diffuse vasoconstriction of the artery
    • Lumen of the affected artery <0.5 mm
    • May occur at a distance from the rupture
  • Early clinical signs of vasospasm
    • Tachycardia
    • EKG changes
      • Inverted T waves leads V3–V6
      • ST depression or elevation without q waves
      • Atrial and ventricular arrhythmia
    • Decreased level of consciousness
    • Deficits corresponding to the affected artery
  • Early clinical signs of hydrocephalus
    • Decreased spontaneity
    • Constant headache (often frontal)
    • Lethargy
    • Incontinence
    • Increased reflexes legs > arms

Medical Complications of Aneurysmal Subarachnoid Hemorrhage

  • General manifestations
    • Seen at 3 months
      • Symptomatic vasospasm – 46%
      • Rebleeding 7%
      • Total mortality 19%
    • Frequency of at least one severe (life threatening) medical complication
      • 40%
      • Death from medical complications 23% (of those that die)
        • Rebleeding 22% of these deaths
      • Vasospasm with stroke 23%

Cardiac Arrhythmia

  • 50% have an abnormal admission EKG
  • 25% have ST segment or T wave abnormalities
  • 15% have prolonged QT interval associated with at least one episode of moderate or severe arrhythmia
  • 35% of all aneurysms have one episode of cardiac arrhythmia on day 0–14; 66% of these are mild; 29% moderate and 5% are severe
  • Onset of arrhythmias usually first 7 days (peak day 2–3)
  • 17% of patients suffer moderate or severe arrhythmia on the day of rupture
  • Cardiac arrhythmia on the day after surgery
    • Life threatening 5% of patients
    • Less serious 30% of patients
  • Moderate arrhythmias
    • Sinus bradycardia
    • Sinus tachycardia
    • Atrial extrasystole
    • Atrial fibrillation
  • Severe arrhythmia
    • Asystole
    • Ventricular tachycardia
    • Atrial fibrillation with hypotension
    • AV block
    • Supraventricular tachycardia
    • Ventricular extrasystole
  • EKG changes with SAH
    • ST depression or elevation without q waves
    • Deeply caved T waves (Pardee T waves) V3–V6
    • u waves
    • Hyperpolarized T waves
    • Pathogenesis
      • Myocytolysis
        • Contraction band necrosis
        • Myofibrillar degeneration
        • Effects of increased sympathetic discharge

Pulmonary Edema

  • Occurs in 6% of patients (day 3 to 7)
  • Probable sudden increase in ICP
  • Water and electrolyte disturbance
    • Particularly with anterior communicating artery aneurysms
  • Increase of atrial natruretic factor
  • Discharge of substance P containing afferents in lung capillaries

Hepatic Dysfunction

  • Occurs in 7% of patients
  • Usually 24% of patients have mild enzyme abnormalities
  • 4% severe hepatic impairment

Renal Dysfunction

  • Occurs in 7% of patients
  • 1.2% severe life threatening renal failure

Thrombocytopenia

  • Occurs in 4% of patients
    • Usually associated with sepsis

Sensitivity of Computed Tomography in Detecting SAH

  • Sensitivity of new generation CT scans for patients screened < 24 hours from the ictus is 93%
  • Sensitivity of new generation CT scans in patients after 24 hours from the ictus is 90%
  • L.P. remains the gold standard to rule out SAH

Grading Scale for Subarachnoid Hemorrhage

  Glasgow Coma Scale
Grade I15headache only
Grade II13 or 14no motor deficit, may have cranial nerve deficit
Grade III12 or 13motor deficit, cranial nerve deficit
Grade IV7–12motor deficit, cranial nerve deficit, obtunded
Grade V3–6cranial nerve and motor deficit, comatose

Giant Aneurysms (greater than 2 Centimeters)

  • General characteristics
    • Do bleed but rarely
    • Symptoms are caused by:
      • Pressure on local structures
      • Emboli distally
      • Seizure activity
      • Endocrine dysfunction (erosion into the pituitary)

Giant Carotid Aneurysm

  • Pain behind or around the eye
  • May erode into the pituitary with endocrine failure
  • Compression of the optic chiasm with nasal VF deficit or chiasmal syndrome
  • III nerve palsy

Middle Cerebral Artery Giant Aneurysm

  • Anterior temporal or retroauricular headache
  • Seizure activity
  • Contralateral hemiparesis

Top of the Basilar Giant Aneurysm

  • Occipital headache
  • III, IV nerve palsy (if extends dorsally)
  • Compression or invasion of the III ventricle
  • May cause obstructive hydrocephalus
  • Associated with emboli to the posterior cerebral artery territories with VF deficits
  • Hemiparesis (compression of the cerebral peduncle)

Acquired Aneurysms

  • General manifestations
    • Peripheral arterial location (not at the Circle of Willis)
    • May be multiple on superficial conducting vessels
    • Cause of peripheral lobar hemorrhage
  • Differential diagnosis
    • Fusiform (secondary to hypertension; basilar artery)
    • Subacute bacterial endocarditis
    • Trauma
    • Atrial myxoma
    • Collagen vascular disease
    • Cigarette smoking (women > men)

Basilar Fusiform Aneurysm

  • Occurs in elderly hypertensive patients with associated atherosclerosis
  • Dysarthria (pontine compression)
  • Hydrocephalus (blocks ventral CSF pathways)
  • Spastic gait (pressure on ventral pontine motor fibers)
  • XII nerve palsy (dysarthria)
  • Spastic quadriparesis (ventral pontine compression)

Closed Head Injury

  • Aneurysms of superficial subarachnoid conducting vessels
  • Rarely basilar artery is traumatized after cervical or basilar skull fracture with aneurysmal formation
  • May be multiple

Atrial Myxoma

  • Multiple peripheral aneurysms
  • MCA territory most common
  • Myxomatous emboli may grow through the wall of the blood vessel

Traumatic Peripheral Carotid Artery Aneurysm (Pharyngeal Portion)

  • Knife or gunshot wound to the neck
  • Sympathetic ocular paralysis (complete Horner's syndrome)
  • Abnormal neck pulsation
  • Embolization in carotid territory
  • Pain in the side of the face
  • Carotodynia (pain in the artery itself)
  • Associated connective tissue disease:
    • Ehlers Danlos type IV and VI
    • Marfan's disease
    • COLIA gene mutations
    • Fibromuscular dysplasia
    • May be bilateral

Traumatic Vertebral Artery Peripheral Aneurysm

  • Trauma to the neck
    • Often minimal and unrecognized when it occurs
  • Pain in the posterior or side of the neck
    • Mastoid pain
    • Occipital headache
  • Chiropractic manipulation (horizontal C2 portion most vulnerable)
  • Congenital connective tissue disorders (particularly if bilateral)
  • Signs and symptoms:
    • Distribution of the vertebral or basilar artery
    • Due to emboli at the site of the vessel tear
    • Rarely flow limiting pathogenesis for signs and symptoms
  • Cancer or cancer treatment (surgical trauma)

Subacute Bacterial Endocarditis

  • May be multiple
  • MCA territory most frequent locations
  • Hemorrhage is common
    • Cause of peripheral ICH
    • May be delayed after successful antibiotic treatment (destruction of the vessel wall)
  • Seizures are common
  • Focal motor deficits: the symptomatology depends on location
  • Streptococcus viridans; gram negative organisms most common
  • Following dental work
  • In association with deformed values
    • Congenital bicuspid calcified valves
    • Rheumatic fever deformed valves
    • Rarely mitral valve prolapse

Differential diagnosis of subarachnoid hemorrhage without detectable aneurysm

General Features

  • 15–20% of spontaneous SAH no aneurysm is detected by 4 vessels arteriography
  • There is a better prognosis in these patients than in those in which an aneurysm is found

Diseases

  • Idiopathic perimesencephalic hemorrhage
  • Venous trauma
  • Diffuse or anteriorly located hemorrhage in the basal cisterns
  • Incomplete visualization of the posterior circulation
  • Occult aneurysm
  • Carotid artery dissection
  • Vertebral artery dissection
  • Dural arteriovenous malformation
  • Spinal AVM
  • Parenchymal trauma
  • Mycotic aneurysm
  • Cocaine abuse
  • Sickle cell disease
  • Coagulopathies
  • Subarachnoid blood in the basal cisterns
    • Pituitary apoplexy
    • Cervical tumor
    • Rupture of circumferential artery (penetrators) in the pontine cisterns
    • Trauma
    • Mycotic aneurysm
    • Coagulopathies (rare)
    • Cocaine or sympathomimetic drug abuse

Idiopathic Perimesencephalic Hemorrhage

  • Approximately 10% of all SAH
  • Comprise 2/3 of patients with normal arteriograms
  • Occur in older patients (>6th decade)
  • Few patients with HCVD
  • Rarely concomitant with congenital aneurysm
  • 1/3 have history of strenuous exercise prior to the bleed
  • Headache reaches maximum intensity within minutes rather than seconds
  • No loss of consciousness with the ictus
  • No focal neurological deficits
  • No rebleeding or delayed ischemia
  • Normal on admission other than headache
  • 20% have hydrocephalus with no other clinical deficits
  • Excellent prognosis
  • Some evidence of capillary bleed as cause

Venous Trauma

  • Shearing head injury
  • Blood most often seen in the posterior part of the ambient cistern (superior cerebellar-quadrigeminal cisterns) at the level of the tentorium
  • Vein is torn against the tentorium

Diffuse or Anteriorly Located Hemorrhage with Rupture into the Basal Cisterns

  • Patients are lethargic to stuporous
  • Focal neurologic deficits are present
  • Symptomatic hydrocephalus
  • Nidus of bleeding medial frontal lobe
    • Blood dissects into the inter hemispheric fissure
    • May re bleed
  • Deterioration from associated ischemia

Incomplete Visualization of the Posterior Circulation

  • Ruptured aneurysm of the posterior circulation is suspected from the early CT scan
  • 85% of patients demonstrate blood in the IV ventricle and to a lesser extent the IIIrd and lateral ventricles
  • IVH is the only abnormality on CT scan in 25% of posterior circulation aneurysms
  • Subarachnoid blood is noted in the midbrain cisterns with spread to the anterior basal cisterns

Occult Aneurysm

  • Approximately 20% of aneurysms are detected on the second arteriogram (after 2 weeks; vasospasm has cleared)
  • Aneurysmal rupture is likely if there is blood in the Sylvian or interhemispheric fissure

Carotid Artery Dissection

  • Rare cause of SAH
  • Clinical features:
    • Severe neck pain; subacute or acute onset
    • Facial pain
    • Oculosympathetic paralysis
    • MCA or carotid embolic stroke
    • May be associated with depressed level of consciousness
    • Rebleeding may occur within hours of the initial hemorrhage

Vertebral Artery Dissection

  • Autopsy series – comprises 4% of patients who died from SAH
  • Primarily middle aged patients
  • 1/3 have HCVD
  • Common after neck movement or minor trauma
    • Patient may be going to a chiropractor because he has neck pain from the dissection
  • Rarely associated with lower cranial nerve palsies; symptoms from occlusion or embolus of the PICA
  • May be bilateral if the cause is an underlying collagen defect
  • Rebleed occurs in 30% of patients; may occur within hours of the initial hemorrhage

Anticoagulation with Warfarin or Heparin

Risk factors for ICH or systemic bleeding

  • Prolongation of prothrombin time beyond the therapeutic range
    • hemorrhages occur occasionally in the therapeutic range
  • Large size of infarcted area (ischemic stroke)
  • Uncontrolled hypertension
  • Age related (relative risk); primarily for subdural hematoma

Clinical Features

  • Gradual and insidious onset
    • Hours to days
  • Predilection for the cerebellum and central lobes
  • High morbidity and mortality

Patients Requiring Long Term Anticoagulation after Bleed; Reinstitution of Rx

  • 10–14 days possibly safe; 2–3 weeks heparin or warfarin probably safe
  • First year of anticoagulation most dangerous

MRI Evaluation

  • Inhomogeneous infarction mixed signal on T2 weighted images suggest acute bleeding and clots

Primary Tumors that Hemorrhage

  • Glioblastoma multiforme
    • In general petechial hemorrhage
    • Bleeding and edema may be initiated by minimal trauma
  • Pituitary apoplexy
    • Most often MRI demonstrates blood confined to the pituitary
  • General Characteristics
    • Bleeding into tumors account for 5–10% of ICH
    • 1% of tumors bleed
    • Hemorrhagic metastatic tumors
      • Melanoma
      • Choriocarcinoma
      • Papillary Ca of the thyroid
      • Bronchogenic Ca
    • Tumors that bleed are overwhelmingly malignant

Clinical Characteristics Suggesting ICH is Secondary to a Tumor

  • Underlying systemic cancer
  • Focal symptoms prior to hemorrhage
  • Unusual site:
    • Corpus callosum (glioma)
  • Multiple simultaneous hemorrhages

Bleeding Diathesis

  • General manifestations
    • Platelet abnormalities
      • Associated with petechial hemorrhage in the lower extremities
      • Bleeding from the gums
      • Vaginal bleeding prior to cerebral hemorrhage may occur
      • Hemorrhage starts at <30–40 thousand/mm3
    • Waldenström's macroglobulinemia
      • Associated abnormalities of platelets with lower extremity petechiae
      • Bleeding from mucous membranes
    • Disseminated intravascular coagulation
      • Associated bleeding from all former venous puncture sites
      • Acrocyanosis
    • Hemophilias
      • Joint involvement
      • Associated SDH
      • Internal bleeding
      • Bleeding may develop gradually
      • Recurrent ICH less likely after 14 days
    • Liver transplantation
  • Differential Diagnosis
    • Coagulopathies (inherited and acquired)
    • Anticoagulation (INR > 5)
    • Thrombolytic treatment (TPA; urokinase)
    • Connective tissue disease
    • Sickle cell disease

Specific Diseases of Blood Vessels That Hemorrhage

  • Cerebral amyloid angiopathy
    • AD (Icelandic; Dutch variants)
    • Sporadic; incidence increases with age
    • Concomitant Alzheimer's disease and variants
  • Granulomatous angitis of the CNS
  • Lymphomatous granulomatosis
  • Benign appearing immunoproliferative vasculitis
  • Angiocentric lymphoma
  • Periarteritis nodosa
  • SLE (with and without lupus anticoagulant)
  • Connective tissue diseases
  • Sickle cell disease
    • 30% of sickle cell disease SAH occur in children
    • Blood is in the superficial sulci
    • Mechanisms
      • Distal branch occlusions
      • Collateral leptomeningeal vessels
      • Moyamoya disease (bilateral carotid occlusion)
    • Adult patients may have:
      • Underlying aneurysm
      • Blood in the basal cisterns

Thrombolysis Related Intracranial Hemorrhage

  • Hemorrhages are large (approximate mean of 70 ml)
  • Solitary in 66%
  • Lobar 77%
  • Confluent 80%
  • Intraparenchymal 80%
  • Mortality 44–83%
  • Increased risk of subdural hematoma

Clinical Features

  • 2/3 of hemorrhages are supratentorial and solitary
  • 1/3 subtentorial or multifocal
  • Peri hemorrhage edema is minimal
    • Less in those hemorrhages with a blood fluid level
    • Mottling and a blood fluid level is indicative of continuing fibrinolysis
  • TPA > hemorrhage risk than from streptokinase

Thrombolysis Induced Hemorrhage Occur in 6% of Treated Patients

  • 7% may be asymptomatic; noted on follow-up CT scan
  • Main comorbidities associated with ICH thrombosis:
    • History of diabetes melitis and cardiac disease
    • Use of antiplatelet agents other than ASA prior to stroke related hemorrhage
    • Elevated pretreatment blood pressure
    • Age
  • Criteria that utilized CT findings and laboratory findings that predict ICH:
    • Ischemic changes on CT exceeding 1/3 of the MCA territory
    • diabetes
    • Elevated serum glucose
    • Low platelet counts
  • Pretreatment diffusion-weighted MRI parameters and laboratory values predictive of ICH after thrombolysis
    • High systolic blood pressure
    • Severity of the stroke
    • Elevated serum glucose
    • Volume of initial DWI lesion
    • Voxels with decreased ADC value
  • Predictive CT findings for thrombolytic hemorrhage:
    • Large arterial territory involved
    • Obscuration of caudate nucleus
    • Loss of the insular stripe
    • Sulcal effacement

Hemorrhage After an Acute Ischemic Stroke

  • All ischemic infarctions have petechial hemorrhages microscopically
  • Hemorrhagic transformation determinants:
    • Hypertension
    • Size of the ischemic area
    • Age of the patient
    • Embolic > thrombotic stroke
    • Endothelial cells of blood vessels are sensitive to hypoxia
  • Unfavorable prognostic signs
    • Intraparenchymal hemorrhage
    • Extraparenchymal bleeding (often occurs after streptokinase)
    • Cerebral edema
    • Size of the hematoma
      • Increased cerebral edema
    • Hemorrhagic transformation of an ischemic stroke has a better prognosis than a hematoma for ICH

Recurrent Intracerebral Hemorrhage

  • 1.8–5% of patients in Asia
  • 2–24% of European patients
  • 6% of ICH are recurrent in the USA; 2.4%/year for primary ICH
  • Mean age is 64.7 years
  • Interval between bleeds is 48 months
  • Recurrent hemorrhages occur at different locations
  • Patterns of recurrences
    • Basal ganglionic in hypertensive patients
    • Lobar in patients with congophilic angiopathy
  • Younger age and lobar hemorrhage increase the risk of recurrence of ICH
  • Recurrent ICH
    • Poor prognosis
    • Severe cognitive deficits
    • Risk is 4× higher in lobar hemorrhage

Post-Partum Cerebral Angiopathy

  • Cause of recurrent intracerebral hemorrhage in young women
  • Rsually a vasospastic process
  • Rsually benign and non-relapsing
  • Several intracranial vessels involved; often bilaterally
  • Similarities to benign isolated CNS angiitis:
    • Young women
    • Headache, seizures, neurologic deficits
    • Angiogram consistent with vasospasm

Mechanisms of Recurrence of Intracranial Hemorrhage

  • Young patients – hypertension
  • Older patients > 65 years – congophilic angiopathy
  • Approximately 50% are secondary to hypertension

Differential Diagnosis of Multiple ICH

  • Underlying malignancy
  • Hypertension
  • Coagulopathies
  • Subacute bacterial endocarditis
  • Vasculitis of collagen disease
  • Congophilic angiopathy
  • Venous sinus occlusion
  • Multiple cardiac emboli
  • Artery to artery emboli
  • Multiple hemorrhage from thrombolysis therapy (IV after myocardial infarction)

Multiple Hemorrhages From Thrombolysis Therapy (IV After Myocardial Infarction)

  • Multiple hemorrhages are unusual in spontaneous ICH
  • Multiple hemorrhages occur earlier than solitary hemorrhages after thrombolytic therapy
  • Multiple hemorrhages
    • 0.47% for streptokinase (CSK) with sc. heparin
    • 0.57% for SK with iv heparin
    • 0.7% accelerated TPA with iv heparin
    • 0.95% for SK with both accelerated TPA and intravenous heparin
  • Time from thrombolytic treatment to ICH
    • Streptokinase – 17.5 hours
    • TPA – 10 hours
    • TPA + SK – 13 hours
  • Factors associated with increased risk of hemorrhage after thrombolysis for MI
    • Age
    • Congophilic angiopathy
    • Facial and head trauma
      • Risk increases if syncope had occurred < 2 days prior to treatment
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