Differential Diagnosis in Neurology

Topic 4. Spinal Cord Disease

4.4. Vascular Anatomy of the Spinal Cord

  • General considerations:
    • The arterial blood supply to the spinal cord can be divided into a superior, intermediate and inferior zone
  • The superior territory:
    • The cervical cord (C1–T1–T2) receives its blood supply from branches of the vertebral arteries that derive from the subclavian arteries. At the level of the foramen magnum, the vertebral arteries give off medial branches that join to form the anterior spinal artery which lies ventrally in the midline.
      • The anterior portion of the anterior spinal artery supplies C1–C4 spinal segments
      • Two to four radicular arteries from the vertebral arteries in the transcervical canal or from cervical branches of the subclavian artery contribute to the blood supply of the mid and lower cervical segments.
      • C8–T1 is the most vulnerable level of the cervical cord for ischemic injury.
  • The intermediate territory (T3–T8):
    • Is supplied by the dorsal radicular artery that travels with the 5th, 6th or 7th thoracic anterior root.
  • The Inferior Arterial Territory
    • T8–S5 is supplied by the great radicular artery of Adamkiewicz.
      • Usually derives from radicular arteries from T1–L1–L2.

Intrinsic Vascular Anatomy of the spinal cord

  • Radicular arteries enter at each spinal level:
    • They divide and accompany the dorsal and ventral roots
    • Ventral root branches join along the midline to form the anterior spinal artery
    • Paired posterior spinal arteries form from anastomosis of the dorsal radicular branches
    • Circumflex arteries anastomose with the anterior and posterior circulations at the lateral margins of the cord
  • Anterior sulcal arteries
    • Arise from the anterior spinal artery and alternately supply:
      • The spinal grey matter
      • The anterior and lateral funiculi
      • Each anterior sulcal artery supplies one segment of the hemi chord
      • The greatest distance between sulcal arteries is in the thoracic cord
    • Posterior spinal artery penetrating branches supply:
      • The dorsal columns
      • Upper most Rexed layers of the dorsal horn (I–IV)
    • Circumflex anastomotic vessels (the anastomosing plexus of small arteries between the anterior and posterior vessels) supply the superficial white matter

Venous Drainage of the Spinal Cord

  • Radial veins drain the inner cord to form a coronal plexus
  • Deep parenchymal veins drain into central sulcal veins in the median fissure
  • Parenchymal veins drain both sides of the cord
  • Sulcal veins have intrasegmental anastomosis
  • Anterior median spinal vein:
    • Drains the sulcal vein
    • There may be two veins instead of one
  • Extramedullary venous channels:
    • Common on the dorsal cord
  • Eight-twelve anterior radicular veins arise from the anterior to median vein:
    • Drain the anterolateral anastomosis from the coronal venous plexus
    • The junction of the anterior radicular vein and the coronal venous plexus is at the nerve roots prior to their exit through the dura
  • The lumbar enlargement is drained by the vena radicularis magna
  • Posterior radicular veins are most prominent in the cervical cord
  • Spinal cord venous blood drains into:
    • Epidural and paravertebral plexus
      • This is a valveless system
    • It extend from the occipital bone to the sacrum

Causes of Spinal Cord Ischemia

  • Transient ischemic attacks of the cord occur (weakness and numbness that lasts 2–15 minutes)
    • Cholesterol emboli
    • Fibrocartilaginous emboli
  • Venous occlusion (secondary to severe cervical stenosis or spondylosis) is posited to cause progressive myelopathy
  • Spinal cord infarction:
    • Paresis within minutes of the ictus
    • Recovery of function is rare if total paralysis lasts for 24 hours
  • Watershed infarction with hypoperfusion is usually overshadowed by cerebral signs and symptoms. The spinal cord is perfused at a much lower pressure than the brain.

Clinical Features of Spinal Cord Ischemia

  • Weakness:
    • May be gradual or maximal at onset
    • Paraparesis > quadriparesis (midthoracic T4–T6 watershed)
  • Sensory loss:
    • Numbness or paresthesias will parallel the weakness; may precede it
    • Aching back pain with radicular features is common
    • Thoracic band like sensation or abdominal radiations are common
    • Some form of sensory loss is invariant
    • Suspended sensory loss with sulcal artery occlusions
  • Urinary and bowel dysfunction:
    • Urinary retention > than incontinence
    • Bowel incontinence later in the course
  • Flaccid paralysis noted early:
    • Loss of superficial reflexes (cremasteric and abdominal)
    • Areflexia below the lesion is usual:
      • Schiff–Sherrington response may occur above the lesion. Slightly increased reflexes (release of ascending inhibition) above the level of the lesion.
      • Hyperactive reflexes with Babinski's response may occur if only a few spinal segments are ischemic.

Arterial Syndromes

Anterior Spinal Artery Ischemia

  • Origin of the anterior spinal from the vertebral artery
    • Atherosclerosis of the vertebral artery
    • Medial medullary syndrome
      • Ipsilateral XII nerve paralysis
      • Contralateral proprioceptive and vibratory deficit
      • Contralateral weakness of arm and leg (above the decussation of the pyramidal tract at C2)
      • Crural plegia (arm ipsilateral and leg contralateral)
  • Low anterior spinal artery infarction:
    • Involvement of the ventral 2/3 of the spinal cord
    • Weakness of extremities below the lesion
    • Loss of temperature and pain below the lesion
    • Bladder involved in 50% of patients
    • Intact dorsal column sensory function

Causes of Anterior Spinal Artery Syndrome

  • Atherosclerosis of the artery or its feeding vessels
  • Vertebral artery dissection
  • Syphilis (Erb's spastic paraparesis)
  • Compression from extruded disc or bone fragment
  • Sarcoid (meningeal involvement or vasculitis)
  • Vasculitis (SLE, Wegener's granulomatosis, collagen vascular disease)
  • Embolus (heart, atrial myxoma, atrial fibrillation); cholesterol emboli
  • Idiopathic pachymeningitis
  • Aortic dissection
  • Giant cell arteritis
  • Takayasu's disease
  • Bends (nitrogen bubbles)
  • Ischemic atherosclerotic emboli less frequent than asymptomatic emboli
  • Hypoperfusion:
    • Lumbosacral > cervical > cervicothoracic
  • Fibrocartilaginous emboli:
    • Women > men
    • Mid to low thoracic levels

Ischemia of Lower Thoracic Cord and the Lumbar Enlargement by Involvement of the artery of Adamkiewicz

  • Renal arteriogram
  • Surgical procedures (cross clamping the aorta)
  • Aortic dissection
  • Atherosclerosis
  • Emboli: heart; cholesterol; air; nitrogen bubbles, fibrocartilaginous
  • Aortic dissection

Watershed Infarction of T4–T6

  • General considerations:
    • The spinal cord is perfused at a much lower pressure 60–70 mmHg than the brain
    • Infarction of the spinal cord is frequently overshadowed by cerebral signs and symptoms
    • Dropped sensory levels to T4–T6 frequently occur from cervical cord disease (lamination of the spinothalamic tract)
    • Internal watershed zones in the spinal cord are the:
      • Corticospinal pathways
      • Rexed layers V–VI
    • Selective grey matter necrosis does occur
    • Most susceptible region to low flow states is T4–T6
    • Causes of thoracic watershed infarction:
      • Systemic hypotension
      • Prolonged surgical procedures
      • Cardiac arrest
      • Anoxia
      • Vertebral artery occlusion (primary involvement may be C8–T1)
      • Aortic dissection
      • Carbon monoxide poisoning

Posterior Spinal Artery Ischemia

  • General considerations:
    • Extremely rare
    • Atherosclerosis
    • Dissection or occlusion of the vertebral arteries
  • Clinical features:
    • Severe sensory ataxia (particularly without vision)
    • Loss of vibration and position sense
  • Medical Causes of Ischemic Spinal Cord Disease
    • Atherosclerosis
    • Systemic hypotension
    • Collagen vascular disease (PAN, SLE)
    • Thromboangiitis obliterans (Buerger's Disease)
    • Aortic arch syndrome; coarctation of the aorta
    • Dissection of the aorta (infarction of grey matter; superficial white matter)
    • Neoplastic endarteritis (malignant angioendotheliosis)
    • Infective endarteritis:
      • Syphilis
      • Cryptococcus
      • HIV
    • Fungal invasion of blood vessels:
      • Mucormycosis
      • Nocardia
      • Aspergillosis
    • Illicit drugs:
      • Cocaine
      • Propalolamine
      • Amphetamine
    • Disseminated intravascular coagulation
    • Prothrombotic states
    • Aortic atherosclerosis (feeding vessels; emboli)
    • Aortic aneurysm
    • Aortic surgery (particularly cross clamping > 18 minutes)
      • Abdominal aortic aneurysms; cord and cauda equina may be infracted
    • Ligation of lower thoracic intercostal vessels
    • Nonpenetrating abdominal trauma (torsional occlusion of vessels supplying the cord)
    • X-RT (intraspinal vessels from proliferative endarteritis)

Embolic Disease of the Spinal Cord

  • Atheromatous aortic plaque
    • Blue toe syndrome (abdominal aorta)
    • Digital arteries of the toes are occluded
  • Cardiac source:
    • Atrial fibrillation
    • Embolus
    • Infective endocarditis (bacteria; fungi)
    • Mitral valve prolapse
    • Valvular clots
    • Atrial myxoma
  • Fibrocartilaginous debris (disc)
    • Anterior portion of the cervical cord
    • May occlude veins
    • Women > men
    • Rapidly evolving tetra or paraparesis
    • Rarely following trauma
    • Anterior spinal artery territory most often affected; mid to low thoracic level
  • Angiography:
    • Catheterization for dural AVM
  • Fat:
    • Broken bones (long bones) in young patients
    • Hip replacement (older patients)
    • Diabetic ketoacidosis
  • Tumor cells
  • Nitrogen bubble (Caisson's disease)
  • Air bubbles
    • Diving accidents (scuba)
    • Cardiac surgery
    • Surgical procedures in sitting position (ENT)
  • Therapeutic renal artery embolization

Venous Infarction of the Spinal Cord

  • General considerations:
    • Venous infarctions without hemorrhage are hard to distinguish from arterial infarction
  • Causes of venous infarction of the spinal cord:
    • Extension of thrombophlebitis of pelvic veins
    • Associated with chronic obstructive pulmonary disease
    • Polycythemia vera
    • Associated thrombophlebitis:
      • Propagates into cord via venous plexus
    • Subacute necrotizing myelitis:
      • Associated with spinal cord thrombophlebitis
      • No systemic foci
      • Association with chronic obstructive pulmonary disease
    • Associated with epidural abscess
    • Endoscopic sclerotherapy (reported for esophageal varices)
    • Coagulopathy:
      • Factor V Leiden with protein C resistance
      • Anti-thrombin III
      • Factor C deficiency
      • Factor S deficiency
      • Anti-phospholipid syndrome
      • Lupus anticoagulant
    • Cancer (prothrombotic states); breast and pancreas
    • Subacute necrotizing myelopathy (paraneoplastic)
    • Birth control pills
    • Pregnancy
    • Disseminated intravascular coagulation (LIC)
    • Dehydration

Hemorrhagic Spinal Cord Disease

Angiomas

  • General considerations:
    • Spinal vascular malformations
  • Tortuous group of dilated arteries and veins:
    • No intervening capillary network (cavernous hemangioma)
      • May involve spinal cord, leptomeninges or both
    • Veins predominance (venous angioma)
    • Arterial predominance (arterial venous malformation)
    • Telangiectasis (capillaries)
  • Epidemiology of angiomas:
    • Incidence is not known:
      • Occur in 3–11% of spinal cord masses
      • Men > women 2:1
      • Clinical presentation between 30–70 years of age
      • Lower thoracic and lumbar angiomas are the most frequent
      • Posterior or posterior lateral intraspinal location
    • Clinical signs and symptoms:
      • Onset with pain, weakness and sensory symptoms
      • Later bowel and bladder dysfunction
      • Cervical lesions produce symptoms earlier
      • Onset associated often with:
        • Exercise
        • Trauma
        • Pregnancy
        • Menstruation
        • May bleed during sleep
        • Angiomas may cause:
          • Increased local venous pressure
          • Ischemia of the cord
      • Onset of symptoms to diagnosis may be delayed
      • Severe weakness in 20% of patients by six months after presentation; 50% by three years
      • After onset of leg weakness further disability is rapidly progressive
      • Pain may be local or radicular
      • Spinal bruit (extremely rare)
      • Upper and lower motor neuron weakness

Cobb's Syndrome

  • General considerations:
    • Cutaneomeningospinal angiomatosis
    • Angiomas on large portions of the trunk
    • Dural angioma with cutaneous angioma in the corresponding dermatome or over many dermatomes
  • Clinical features:
    • Spastic paraparesis at the appropriate level
    • Usually at thoracic level

Foix Alajouanine syndrome (Angiodysgenetic Necrotizing Myelopathy)

  • General considerations:
    • Men of middle to late adulthood
    • Clusters or masses of enlarged engorged subarachnoid veins; anterior predominance
    • Lower thoracic and lumbosacral segments are most commonly involved
    • Proliferation of small blood vessels (veins or capillaries)
    • Associated with a dural arteriovenous shunt generally within or near a root sleeve; arterialized venous shunts that drain into the venous system of the cord
  • Clinical features:
    • Intermittent and progressive course
    • Asymmetrical clinical findings
    • Pain and sensory loss of the lower limbs
    • Bowel, bladder and sexual dysfunction
    • Spastic and amyotrophic lower extremities

Bannayan–Zonana Syndrome

  • General considerations:
    • AD spinal AVM
    • Most common in Mexican Americans
    • May hemorrhage
  • Clinical features:
    • Spastic paraparesis at the appropriate spinal level
    • Associated with intracranial lesions

Klippel–Weber–Trenaunay

  • General considerations:
    • AD
  • Clinical features:
    • Enlarged extremity with dilated veins and telangiectasias
    • Corresponding AVM at the cervical or lumbosacral level with spastic paraparesis or radiculopathy

Differential Diagnosis of Spinal Angioma

  • Multiple sclerosis
  • Neoplasm
  • Herniated disc
  • Spondylolisthesis
  • Intracranial SAH (5% present with neck and back pain)
  • Subacute combined degeneration (B12 deficiency)
  • Transverse myelitis (all causes)
  • Meningovascular syphilis

Spinal Hemorrhage

Differential diagnosis by location:

  • SAH
  • Intramedullary
  • Subdural
  • Epidural

Subarachnoid Hemorrhage in the Spinal Cord

  • General considerations:
    • Spinal SAH < 1% of all SAH
    • Most common cause is rupture of a spinal angioma (10%)
  • Clinical features:
    • Acute onset
    • Severe back pain at the level of the hemorrhage
    • Pain quickly becomes diffuse with signs of meningeal irritation
    • Radiculopathy at the site of the lesion
    • If blood diffuses above the foramen magnum:
      • Headache
      • Cranial neuropathy
      • Depressed level of consciousness
      • Decreased ankle jerks with increased knee and upper extremity reflexes
      • Increased intracranial pressure with papilledema (rare)
    • CSF:
      • Hemorrhage (1,000,000 RBCs/mm3)
      • Within days may have hypoglycorrhachia (severely depressed glucose <20 mg%); may occur with all forms of SAH

Hematomyelia

  • General considerations:
    • Trauma
      • Direct injury to the spine (penetrating and non-penetrating)
      • Hyperextension of the cervical spine
    • Bleeding of a vascular malformation
    • Hemorrhage into a tumor
    • Hemorrhage into a syrinx
    • Coagulopathy
    • Anticoagulation (particularly coumadin)
    • Venous infarction
    • Syphilis
  • Clinical features:
    • Presents as spinal shock associated with severe back pain
    • Autonomic instability
    • Gray matter more affected than white matter
    • Spasticity develops below the level of the lesion
    • Atrophy and fasciculations of affected segments occurs with time
    • Babinski's sign is prominent
    • Sensory level:
      • Two segments lower than the lesion (if thoracic)
      • Higher in back than front (follows patterns of intercostal nerves)
      • Dropped sensory level (T4–T6) following cervical lesions is common (lamination of spinothalamic tract)
      • Rare suspended sensory loss (interruption of segmental dorsal horn at the level of injury with preserved long tract sensory function)
      • Mass reflex late in the course of the illness

Spinal Epidural and Subdural Hemorrhage

  • General considerations:
    • Epidural hemorrhage occurs with greater frequency than subdural hemorrhage
    • Peak occurrence during childhood and fifth to sixth decade
    • Cervical lesions more common in childhood; thoracic and lumbar lesions in adults
    • Frequently occur with trivial trauma or exertion
  • Clinical features:
    • Similar for both subdural and epidural hematoma
    • Severe back pain at the level of the bleed
    • Myelopathy (compressive)
    • Cauda equina syndrome
    • Radicular pain (involved segment)
    • Progresses over hours to days
    • Acute transverse myelitis (presentation of epidural hematoma)
  • MRI imaging:
    • T1 weighted image
      • Signal intensity of the epidural hematoma is primarily isointense to the spinal cord
    • T2 weighted images of epidural hemorrhage
      • Heterogenous hyperintensity as compared to the cord
      • Focal hypointensity (acute blood; deoxyhemoglobin)
    • Capping of epidural fat
    • Direct continuity with adjacent osseous structures
    • Compression of epidural fat, subarachnoid sac and spinal cord
    • Posterolateral location in the spinal cord
    • Spontaneous spinal epidural hematomas located in the ventral space
      • Premembranous hematoma
      • Posterior longitudinal ligament hematoma
  • Differential Diagnosis:
    • Epidural anesthesia
    • Lumbar puncture
    • Blood dyscrasia
    • Anticoagulation
    • Neoplasm
    • Vascular malformations
    • Thrombocytopenia
    • Following heparinization after lumbar surgery

Differential Diagnosis of Specific Spinal Cord Malformations

  • Hemangioblastoma (Von Hippel–Lindau Syndrome)
  • Arteriovenous malformation
  • Dural arteriovenous fistula
  • Intramedullary spinal cord cavernous malformations
  • Bannayan–Zonana Syndrome
  • Occult vascular malformation of the spinal cord

Hemangioblastoma

  • General considerations:
    • May arise in isolation
    • Major manifestation of Von Hippel–Lindau disease (VHL)
      • Prevalence 1/36,000 population

Von Hipple–Lindau Syndrome (VHL)

  • General considerations:
    • AD: chromosome 3p25–3p26 deletions or mutations
    • Associated central nervous system hemangioblastomas:
      • Cerebellum
      • Retinal
    • Associated systemic manifestations:
      • Renal cysts
      • Renal carcinoma
      • Pheochromocytoma (specific alleles of VHL disease)
      • Increased levels of vascular endothelial neurotrophic factor (VENGF)
  • Clinical features:
    • 80% in cerebellum
    • 20% in spinal cord
    • 35% of patients with hemangiomas have VHL disease; 60% of those patients who are symptomatic are less than 30 years of age
    • Spinal hemangioblastoma:
      • Relationship to VHL is greater when in an infratentorial location
    • VHL patients are younger than sporadic cases
    • VHL patients develop endolymphatic sac tumors (tinnitus or deafness as clinical manifestations)
    • Spinal hemangioblastoma:
      • Often associated with cerebellar hemangioblastoma
      • May be multiple
      • May be extramedullary in a spinal root location
      • Renal cell carcinoma may metastasize to the hemangioblastoma

Arteriovenous Malformations

  • General considerations:
    • Males more than females
    • Thoracolumbar location (dorsal)
    • Associated with:
      • Cobb's syndrome
      • Wyburn Mason syndrome
      • Kartagener's syndrome

Spinal Dural Arteriovenous Malformations

  • General considerations:
    • Most frequent spinal arteriovenous malformation
    • Posterior and posterolateral spinal cord is the most common location
  • Clinical features:
    • Diagnosis established after months of intermittent symptoms
    • Progressive myelopathy
    • Cauda equina and conus medullaris dysfunction
    • Low back pain (pseudo radicular pain)
    • Lower extremity weakness
    • Sphincter disturbance (early involvement of micturition; later involvement with disc disease)
    • Combined upper and lower motor neuron signs
    • Symptoms may vary with posture; menses and exercise
  • Evaluation of dural arteriovenous fistula (DAVF)
    • Patients may demonstrate enlarged spinal perimedullary veins with intracranial DAVF images by MRI
    • Angiographic evaluation of spinal dural arteriovenous fistula (SDAVF)
      • Anterior spinal vein draining SDAVF (spinal dural AV fistula)
      • Opacification of other medullary veins of the ASV (anterior spinal venous system)
      • Distortion of the hairpin turn of the artery of Adamkiewicz
    • MRI evaluation of spinal dural arteriovenous fistula:
      • Intramedullary high intensity signal changes on T2 weighted images (after hemorrhage)
      • High intrinsic area of T2 weighted intensity decreases within 1–4 months after treatment
    • CSF:
      • Abnormal in > 75–90% of patients
    • Some angiographic evidence for vascular steal phenomenon as a cause of symptoms
    • Dural arteriovenous fistula at the craniocervical junction:
      • MRI evaluation: dilated perimedullary veins around the spinal cord C1–C2
      • High intensity T2 weighted signal in the spinal cord at appropriate levels on T2 weighted images
  • Clinical presentations:
    • Present with SAH
    • Progressive myelopathy that may interfere with respiration
    • Intramedullary arteriovenous malformation may occur with lipomyelomeningocele
      • MRI:
        • Hypervascular intradural lesion; fatty component extradurally

Intramedullary Spinal Cord Cavernous Malformation (IMSCM)

  • General considerations:
    • Cervical and thoracic cord most common level; rare in the conus medullaris
    • Intramedullary spinal cavernous malformations are associated with cryptic venous malformations
    • May develop tethering of the cord following surgery
    • Episodic, acute or subacute spinal cord dysfunction
    • Appropriately 50% of patients, may have multiple cavernous malformations in the neuraxis
  • Clinical features:
    • Myelopathy
    • Radicular pain
    • Conus medullaris syndrome

Bannayan–Zonana Syndrome

  • General considerations:
    • AD
    • Hamartomatous disorder
    • Macrocephaly
    • Multiple lipomas
    • Male > female
    • Rare extradural spinal hemangiomas occur

Angiographically Occult Vascular Malformations of the Spinal Cord (AOVMS)

  • Cavernous malformations are the most common type of AOVM of the spinal cord
  • Insidious onset with limb asymmetry has been noted
  • Deterioration putatively from repeated hemorrhages around vessels or from intrahumeral thrombosis

Differential Diagnosis of AOVMS

  • Primary hemorrhagic tumors of the spinal cord:
    • Ependymoma
    • Astrocytoma
    • Medulloblastoma
  • Hemorrhagic transverse myelitis:
    • Lung metastasis (small cell)
  • Paraneoplastic spinal cord hemorrhagic necrosis:
    • Small cell lung carcinoma
    • Anti-Hu antibody syndrome
      • Occurs at the thoracic level
  • Bleeding diathesis:
    • Coagulation defects
    • Anticoagulation (coumadin/heparin)
    • Platelet disorders
    • Leukemia/lymphoma
    • Hemorrhagic leukoencephalitis (acuteautoimmune disease)
    • Subdural hematoma
    • Parenchymatous bleed (trauma)
  • Systemic Conditions Affecting the Spinal Cord
    • Whipple's disease
    • Gluten sensitive enteropathy
    • Kohlmeier–Degos disease
    • Sjögren's disease
    • B12 deficiency
      • Acute following nitrous oxide anesthesia (cobalamin deficiency)
      • High serum B12 level noted with leukemia and lymphoma
    • Vitamin E deficiency
      • Dietary
      • Alpha tocopherol transporter defect
    • Pure spinal angitis

Rare Spinal Cord Abnormalities

Werner's syndrome (Progeria)

  • General considerations:
    • Childhood age of onset
    • Accelerated aging
    • AR
  • Clinical features:
    • Balding
    • Atherosclerosis of all circulations
    • Premature graying
    • Osteosclerosis
    • Diabetes mellitus
    • Juvenile cataracts
    • Hypogonadism
    • Rare spastic paraparesis
      • Many patients with increased reflexes

Spinal Pseudoathetosis

  • General considerations:
    • Occurs after protracted severe loss of proprioception with acute painful crisis
    • Secondary to:
      • Lesions of the dorsal columns or their nuclei
      • Lesions of the dorsal root ganglia
      • Lesions of the dorsal root entry zone
  • Clinical features:
    • Lateral and updrift with polyminimyoclonus
    • Rare flinging of the arms and legs (hemiballism)
    • Severe proprioceptive loss partially compensated
    • Pain may precede the pseudoathetosis
  • Differential diagnosis of spinal pseudoathetosis:
    • Tabes dorsalis
    • Spinal trauma (dorsal column destruction)
    • Demyelinating disease
    • Syringomyelia
    • SLE myelitis
    • Friedreich's ataxia

Stiff Leg Syndrome

  • General considerations:
    • Suspected autoimmune etiology
  • Clinical features:
    • Onset in middle age
    • Stiffness and painful spasms of the lower limbs
    • Spasms are spontaneous
    • Reduced by movement
    • Rigidity and abnormal postures of both legs occur
    • No brainstem pyramidal tract or sensory dysfunction
  • EMG abnormalities:
    • Repetitive grouped discharge of motor units
  • Differential diagnosis of stiff leg syndrome:
    • Stiff man syndrome
    • Idiopathic encephalomyelitis with rigidity
    • Spinal interneuronitis

Venoocclusive Disease

  • General considerations:
    • Associated with spinal cord vascular malformations
    • Associated with acute compressive epidural processes:
      • Hematoma
      • Epidural abscess

Hemorrhagic venous occlusive disease:

  • Back pain at the level of involvement
  • Rapidly progressive (days to weeks)
  • Multiple spinal segments involved

Non-hemorrhagic venous occlusive disease:

  • Course is over weeks to months
  • Less pain
  • Fewer spinal segments involved

Embolic venous infarction:

  • Fibrocartilaginous
  • Sudden onset
  • Pain at the level of involvement
  • Asymmetric deficit

Venous infarction in association with vascular malformations:

  • Subacute neurologic syndrome
  • Foix–Alajouanine syndrome
    • Step wise dysfunction due to thrombosis of supplying vessels
  • Intramedullary hypertension due to spinal dural arterial venous fistula

Sclerotherapy:

  • Direct penetration of epidural veins
  • Occlusion of the anterior spinal artery
  • Cirrhosis (acquired or congenital) is often the systemic defect
    • Related AV shunts
    • Inadvertent arterial access following esophageal vein injection
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