Differential Diagnosis in Neurology

4.9. Tumors of the Spinal Cord

General considerations of Spinal Cord Tumors

• Patterns of involvement:
  • Epidural
  • Intradural extramedullary
  • Intraparenchymal

Epidural

• General considerations:
  • The overwhelming majority of tumors that are epidural are metastatic. Multiple myeloma and chordoma are the primary spine tumors that invade this space.
  • Epidural spinal cord compression occurs in approximately 5% of cancer patients
  • 50% of all epidural metastatic lesions are from the lung or breast. Others are:
    • Prostate
    • Melanoma
    • Lymphoma
    • GI tract
  • Tumors reach the epidural space by:
    • Hematogenous spread
    • Through Batson's venous plexus (perivertebral)
    • Through nerve root exit foramina (lymphoma in particular)
  • 70% involve the thoracic cord; 10% the cervical cord and 20% the lumbosacral cord. Lung and breast metastasize to the thoracic spine; colon, prostate and ovarian tumors to the lumbosacral spine and melanoma to unusual areas of the spine (in addition to digits, heart surface and intestinal wall).
• Clinical features:
  • Unrelenting boring back pain; made worse with recumbency (differential point from disc disease)
  • Exacerbated at night
  • Persists with bed rest
  • Gait ataxia prior to weakness (compression of the dorsal and ventral spinocerebellar tracts)
  • Weakness may precede sensory symptoms
  • Bowel and bladder dysfunction rarely the earliest symptoms
  • Sensory complaints are often painful
  • Rapid progression suggests:
    • Lung
    • Renal cell
    • Melanoma
• Laboratory evaluation:
  • CSF:
    • Malignant cell determination is directly related to the volume of fluid studied
    • Glucose of 30–40 mg%
    • Protein 80–150 mg%
    • If the protein is 800 mg to 1000 mg% is a spinal block (Freund's reaction)
• Imaging evaluation:
  • Pedicle involvement; erosion of the end plates and central vertebral body lesions noted on plain film suggest metastatic disease
  • Bone involvement is cancer; disc involvement is infection
  • MRI evaluation of metastatic disease to the vertebrae:
    • Multiple areas of T1 weighted low density areas in the vertebra
    • Pathologic fractures
    • Osteoblastic bone changes;
      • Myeloma; prostate; Hodgkin's, rarely breast cancer
    • Does not invade the disc space
    • Density or enhancement in the CSF space between cerebellar folia suggests associated carcinomatosis of the meninges

Division A: Primary Tumors of the Vertebral Column with Secondary Epidural Compression

• General considerations:
  • Tumors of the vertebral column are often malignant
  • Multiple myeloma is the major malignant bone tumor of adults:
    • Peak incidence is sixth–eighth decades
    • A higher incidence in African-Americans than Caucasians
    • Extradural compression occurs in 10% of patients
    • Plasmacytoma:
      • Evolves into multiple myeloma within 10 years
      • Male to female ratio is 3:1
      • Associated with neuropathy
  • Chordoma:
    • Arises form notochordal remnants
    • Clivus and sacrococcygeal in location
    • Peak incidence is 5th through 7th decade
    • Hematogenous metastasis occur in approximately 1/3 of patients

Intradural Extramedullary Tumors

Differential diagnosis includes:

• Neurofibroma
• Schwannoma
• Meningioma
• Dropped metastasis from medulloblastoma and pinealoma (from cells circulating in spinal fluid)
• Leptomeningeal metastasis
• Congenital neuropathy with enlarged nerve roots (HSMN III)

Nerve Sheath Tumors

• General considerations:
  • Neurofibroma is a mixture of Schwann cells, fibroblastic and collagen fibers that grow through and cannot be separated from the nerve
  • Schwannoma is composed of Schwann cells that cause an enlargement of the involved nerve root
  • They are evenly distributed along the neuraxis
  • Occur equally in men and women
  • Onset is usually between 30–60 years of age (average is 43 years)
  • Two thirds are intradural; they often are intradural and extradural (dumbbell)
  • Clinical features of Neurofibroma:
    • Specific painful radicular syndrome
    • Skin stigmata of NFI (chromosome 17)
    • Slow spinal cord compression (intradural component)
      • Weakness and spasticity below the level of the lesion
      • Sensory loss at two segments below the lesion if in the thoracic cord; dropped sensory level of many segments if the lateral cervical cord is compressed
      • Clear Babinski sign is present
      • Usually only mild bladder involvement
  • Imaging features:
    • Dumbbell tumor; widens the neural exit foramina; intra and extradural component
    • Scalloped vertebral body (long standing pressure)
    • Meniscus sign on myelography
    • Heterogeneity of Schwannoma, more homogeneity of neurofibroma on T2 weighted MRI images; enhancement is often a sign of cystic degeneration

Meningioma

• General considerations:
  • Arise from arachnoid cap cells
  • Greater than 90% are intradural; rare in the thoracic cord of men; peak incidence is between 40–70 years of age
  • Approximately 80% are in women and 80% are in the thoracic cord; <5% are intradural extramedullary and extradural
  • Meningiomas in men are in the cervical spine and may have an intradural extramedullary configuration.
• Clinical features:
  • Pain followed by paraparesis, sensory loss and a neurogenic bladder
  • Recurrence rate after surgery at 10 years is less than 15%
• Imaging evaluation:
  • Plain X-rays are often negative
  • MRI:
    • Isointense to spinal cord in T1 and T2 weighted images
    • They enhance homogenously.

Intraparenchymal Tumors

• General considerations:
  • Extend over many segments; rarely involve the entire spinal cord (exception is in children); they grow asymmetrically so there is a C scoliosis from destruction of paraspinal neurons on one side of the cord.
  • Patterns of dissociated sensory loss
  • Severe pain if the dorsal root entry zone is involved
  • Anterior commissure involvement causes segmental loss of pain and temperature
  • Weakness and atrophy of involved segments
  • Pseudosyringomyelia is often the clinical picture
  • Patients may sleep sitting up in a chair (poor venous return; sensitized nerve roots compressed by draining veins). Disc disease also awakens patient with back pain early in the AM (4–5 AM).
  • Intramedullary tumors are 2–4% of adult spinal tumors
  • Glial tumors are approximately 22%
  • Astrocytomas and ependymomas are greater than 80% of intraspinal tumors at all ages

Astrocytoma

• General considerations:
  • Peak incidence 3rd to 5th decade; 30 years of age for high grade astrocytoma and 40 years of age for lower grades. Approximately 75% are low grade.
  • Malignant forms are more common in adults than children
  • Approximately 40% are thoracic
• Clinical features:
  • Localized back pain, progressive weakness; paresthesias > pain (ependymomas have pain)
  • Malignant astrocytomas may have leptomeningeal spread (similar to the extra phytic spread of cerebral gliomas)
• Imaging evaluation:
  • Approximately 40% have an associated syrinx
  • Usually enhance with gadolinium in a heterogeneous pattern
  • Asymmetrically expand the cord
  • In the presence of NF-2 they may be multiple

Pilocytic Astrocytoma

• General considerations:
  • May occur simultaneously or at different sites in the CNS (usually with NF-1)
  • Grow long distances throughout the brainstem with minimal dysfunction
  • No progression to malignant astrocytoma
  • Primary tumor of children and adolescents
  • Rare in the spinal cord
• Clinical features:
  • Depends on the level of involvement in the cord
• Imaging evaluation:
  • MRI:
    • Fusiform thickening of the cord
  • PET:
    • High rate of glucose utilization

Ependymoma

• General considerations:
  • Approximately 55% involve the cervical spinal cord or arise from the filum terminale
  • Male to female ratio is 2:1
  • Mean age of onset is 40 years of age
  • Most common neuroepithelial tumor of the spinal cord
• Clinical features:
  • Most common sites are: cervical > cervicothoracic > lumbosacral > cauda equina
  • Back pain is the most common complaint
  • Dysesthesia without sensory loss

Myxopapillary Ependymoma

• General considerations:
  • Age of onset 36 years (6–82 years); males > females 1.7:1
  • Compresses and envelops the cauda equina
  • Occasionally invades the nerve roots
  • Pain and numbness occur in a radicular pattern in the legs; the tumor is in the filum or conus medullaris; these are usually histologically separate from neural tissue.
• Clinical features:
  • Asymmetric loss of lower extremity reflexes with radicular pain
  • Large tumors may invade adjacent bone
  • Post-operative complications are: dysesthesias and loss of proprioception
• Imaging evaluation:
  • Homogeneous enhancement by MRI; sharply defined rostral and caudal borders
  • Approximately 30% have associated cysts
  • Pseudocapsule; intense enhancement
• Subcutaneous Sacrococcygeal Myxopapillary Ependymoma
  • Develop in the skin and subcutaneous tissue of the sacrococcygeal area
  • Often misdiagnosed as pilonidal cysts
  • May cause distant metastasis
  • Approximately 20% have local recurrence

Intramedullary Metastasis

• General considerations:
  • Approximately 5% of spinal tumors in cancer patients
  • Lung > breast > lymphoma > colorectal > head and neck > renal
  • Origin is arterial blood
  • Approximately 50% of spinal metastasis have concomitant brain metastasis
  • Conus medullaris is the most common site of metastases
  • Approximately 10% are multilevel
  • Occurs in up to 8% of patients with non-Hodgkin's lymphoma; 70% of patients with Leukemia
  • Most common solid tumors causing infiltration of the arachnoid and pia mater (leptomeninges) are:
    • Breast cancer
    • Lung carcinoma
    • Melanoma
    • Gastrointestinal cancers
  • Risk factors for leptomeningeal spread of non-Hodgkin's lymphoma are:
    • Bone marrow and testicular involvement
    • Extranodal spread
    • Epidural metastasis
    • Lymphoblastic histology
    • Burkitt's syndrome
  • General rule:
    • Primary lymphomas are parenchymal with leptomeningeal spread
    • Systemic lymphomas are meningeal with secondary parenchymal spread
• Clinical features:
  • Rapid progression of clinical signs
  • Ataxia, weakness, sensory level, urinary incontinence and pain
  • Weakness is asymmetrical

Benign Spinal Tumors (Embryonal)

• General considerations:
  • These tumors comprise <2% of primary spinal tumors
  • Usually found in the lumbar area associated with skeletal and skin stigmata:
    • Spina bifida
    • Posterior dermal sinuses
    • Dimple on or off the midline
    • Hypertrichosis (hair is thick)
    • Cutaneous angiomas
    • Pigmented skin
  • Intraparenchymal associations:
    • Diastematomyelia
    • Syringomyelia
  • The major tumors are:
    • Lipomas
    • Epidermoids
    • Dermoid cysts
    • Teratomas
• Clinical features:
  • Often due to underlying associated features
  • Lipomas may degenerate into sarcomas and infiltrate the cauda equina
• MRI evaluation:
  • Lipomas are high signal intensity on T1 weighted images and are low signal on T2 weighted images
  • Dermoids have mixed signal intensities
  • Epidermoids are bright on T1 weighted images

Extremely Rare Tumors of the Spinal Cord

Oligodendroglioma

• 5% of intracranial neoplasms
• Majority of tumors in adults between the 4th and 5th decade
• Extremely rare in the spinal cord

Medulloblastoma

• Approximately 20–25% occur after age 20; male > female 1.6/1
• Origin is most frequently the roof of the IVth ventricle (superior medullary velum)
• Metastasize to the spinal cord via CSF pathways

Intravascular Malignant Lymphomatosis

• General considerations:
  • Rare fatal disease
  • Multicentric proliferation of intravascular malignant mononuclear cells
  • Systemic involvement:
    • Heart
    • Kidneys
    • Skin
    • Lymph nodes
  • Primary cerebral or spinal intravascular lymphomatosis is rare; a good clue is the triad of dementia, small strokes and meningeal signs
• Clinical features:
  • A disease of middle age and elderly patients (61 years is median; 12–87 years)
  • 34% of patients have myelopathy
  • Occludes and infarcts spinal vessels; dementia and meningeal signs are associated
• Laboratory evaluation:
  • Increased sed rate
  • Elevated serum lactic dehydrogenase
  • CSF reveals lymphocytic pleocytosis
• MRI evaluation:
  • Rare meningeal involvement demonstrated

Ganglioma

• General considerations:
  • Most tumors develop in the temporal lobe
  • 20–50% arise in the spinal cord:
    • Any site may be the origin including the conus medullaris
    • Usual sites:
      • Cervicothoracic cord > thoracolumbar > multiple spinal cord segments
• Clinical features:
  • Dependent on spinal cord location
  • Onset at age 20 (1–80 years)
  • Slight male preponderance at all sites 1:2
  • Not associated with phakomatosis or heritable disease, but with migrational defects
  • 6% of patients develop malignant anaplastic ganglioglioma

Gangliocytoma

• General considerations:
  • Increased number of abnormal neurons
  • Site of origin is cerebrum and cervicothoracic spinal cord
• Clinical features:
  • Clinically symptomatic by age 30 (20–60 years of age)
  • Slowly evolving long tract signs
  • Recurrence of the tumor is restricted to its original site

Central Neurocytoma

• General considerations:
  • Most often they are supratentorial around the lateral or III ventricle
  • Tumors occur in the cervical spinal cord

Central Neuroblastoma

• General considerations:
  • Ganglion cell differentiation occurs in 50% of patients
  • Age of onset is around 13 years (described into the 8th decade)
  • Rare tumor of the spinal cord and cauda equina

Differential Diagnosis of Spinal Cord Tumors

Meningiomas occur in the thoracic cord of women. They are extremely rare in men in this distribution. A common mistake in the past was making the diagnosis of metastatic disease in a breast cancer patient that had a thoracic meningioma. The lumbosacral cord is the region of most benign tumors that often have associated skin manifestations. A malignant subcutaneous myxopapillary ependymoma may grow into lumbar subcutaneous tissue. Ependymomas present with pain more frequently than paresthesias. Radicular complaints and weakness suggest a myxopapillary tumor. Asymmetric reflexes in the lower extremities support the diagnosis. As a general rule epidural tumors are painful and present with symmetrical weakness and bladder dysfunction. Ataxia due to cervical cord lesions is commonly overshadowed by weakness. Obstipation may occur prior to bladder dysfunction. In medullary cervical tumors one may be precipitate micturition with a valsalva maneuver. Most patients with intraparenchymal tumors have difficulty with recumbency that causes them to sit up in a chair to sleep. Astrocytomas, hemangioblastomas and ependymomas may have an associated syrinx. A combination of cerebral strokes, dementia and meningeal signs should suggest cerebral angiolymphomatosis.

Malignant Bone Tumors Involving the Spinal Cord

Chordoma

• General considerations:
  • Mean age at diagnosis is 48 years (8–83 years); mean age for sacrococcygeal ependymoma is 56 years
  • Male predominance of 2:1
  • Arises from vestiges of the embryonal notochord
  • Sacrococcygeal > sphenooccipital > greater than the vertebral column
  • Vertebral chordoma:
    • Lumbar > cervical > thoracic
    • Bulk of the tumor is usually anterior to the vertebral body
• Clinical features:
  • Pain
  • Rectal dysfunction
  • Urinary incontinence
  • Mass over the coccyx
  • Sciatic pain
• Clinical features of vertebral body chordoma:
  • Pain
  • Bladder dysfunction
  • Lower extremity weakness
• Differential Diagnosis
  • Metastatic carcinoma (particularly colorectal or prostate)
  • Chondroid tumors
  • Chondrosarcoma

Multiple Myelomatosis (Disseminated plasma cell tumors)

• General considerations:
  • Skull and vertebral columns most often affected
  • Predilection for bones of the thoracic cage (clavicle, ribs, vertebrae)
• Clinical features:
  • Vertebral body invasion with collapse and subsequent spinal cord compression
  • Necrotizing progressive myelopathy
  • Leptomeningeal spread may occur
  • Plasmacytomas and plasma cell myeloma are associated with HIV infection

Chondrosarcoma

• General considerations:
  • Occur in the first and second decades in the same areas as chordomas
  • May occur after excision; late metastases noted
  • Arise from the petroclival suture
  • Rarely arises from the nasopharynx, vomer and sphenoethmoidal region
• Clinical features:
  • Rare in the spinal cord; features of extradural compression
• Imaging evaluation:
  • CT and MRI demonstrate an erosive destructive lesion of the skull base that may involve the brainstem and cervical cord
  • May calcify

Ewing's Sarcoma

• General considerations:
  • Primarily a tumor of adolescence
  • Involves the cervical spinal cord
• Clinical features:
  • Extradural compression
• Imaging evaluation:
  • Onion peel periosteal reaction

Osteogenic Sarcoma

• General considerations:
  • 10% occur in the third decade; osteosarcoma in the fifth and sixth decades are due to transformation from a benign condition
• Clinical features:
  • May affect any vertebrae
  • Extradural clinical features
  • Destructive moth eaten lesion; speculated periosteal reaction (sunburst appearance)

Division B: Differential Diagnosis of Benign Spinal Cord Tumors

• Meningioma (see intradural extramedullary tumor)
• Neurofibroma (see intradural extramedullary tumor)
• Schwannoma (see peripheral nerve sheath tumor)
• Ganglioneuroma
• Hemangioblastoma
• Hemangioma
• Central neurocytoma
• Dermoid
• Teratoma
• Hibernoma (vestigial remnants of brown adipose tissue)

Ganglioneuroma

• General considerations:
  • Circumscribed ganglionic cell tumor
    • 56–70% of tumors are located within the posterior mediastinum or retroperitoneum in association with the sympathetic neuraxis
    • 20–30% involve the adrenal gland
    • May arise anywhere along the paramidline
    • Onset second to third decade (later onset if arise in unusual positions)
    • Female predominance 1.3–3.5
• Clinical features:
  • Compress the spinal cord (intradural extramedullary component)
  • Grow through the intervertebral foramina
  • Dumbbell shaped tumor
  • Rare active hormone secretion (vasointestinal peptide VIP; catecholamines; testosterone may also be secreted)
  • Ganglioneuromatosis occurs concomitantly with NFI; gastrointestinal tract may have diffuse ganglioneurofibromatosis
  • Associated with:
    • MEN type 2B syndrome
    • Familial neural crest tumors
    • Cowden's syndrome
    • Juvenile polyposis
• Imaging evaluation:
  • CT-calcification 20–30%
  • Thoracic tumors are associated with rib deformities
  • Paraspinal lesions; dumbbell shaped tumors

Hemangioblastoma (Capillary Hemangioblastoma)

• General considerations:
  • Benign; highly vascular often cystic
  • Single or multiple
  • Seen often in associated with Van Hippel–Lindau's disease
  • Peak incidence between 35–45 years of age
  • Male predominance 2:1
  • Site of origin:
    • Posterior fossa (94%)
    • Spinal cord (3%)
    • Supratentorial (3%)
    • Very rare at the cervicomedullary junction
  • Capillary hemangioblastoma occur with:
    • Gliomas
    • Subependymomas
    • AV malformations
• Clinical features:
  • Spinal cord hemorrhage
  • Slow progression of long tract signs
• Pathology:
  • Formed by blood vessels:
    • Lined by single layer of epithelium
    • Cysts occur with tumors in the spinal cord

Lipoma

• General considerations:
  • Probable aberrant differentiation of the tail bud (multipotential cells)
  • Low lumbar sacral origin
  • Histology:
    • Simple lipoma
    • Complex forms: ectodermal, mesodermal or endodermal origin
• Clinical features:
  • Radicular pain and motor loss
  • Congenital lumbosacral lipomas with tethered cord symptomatology
    • Conus medullaris lipoma
    • Lipomyeloschisis
  • Tethered cord intradural lipoma:
    • Cervical to lumbar cord in location
    • Dysesthetic pain (non-radicular)
    • Mixed with nerve roots

Dermoid Cysts

• General considerations:
  • Lumbosacral site; extra or intramedullary
    • Dermoid cysts connect to dermal sinuses that may penetrate the dura
    • Dermis, hair follicles, sebaceous and sweat glands are present; bone and cartilage is rare
    • Slow growing benign lesion; rare malignant transformation to squamous cell cancer
• Clinical features:
  • Pyogenic infection (if extends through the dura) with unusual organisms
  • Rupture of the cyst into CSF pathways that causes severe granulomatous meningitis
  • Associated midline skin dimple or pit:
    • Below the inguinal crease; they end blindly and never extend intraspinally
    • Dimples above the inguinal fold may connect intradurally
    • Dermal sinuses may lead to spinal cord tethering
  • Pes cavus may be associated
  • Asymmetry of motor and sensory loss
  • Bladder dysfunction

Epidermoid

• General considerations:
  • Extremely rare in the spinal cord:
    • May extend into the cervical cord from the cerebellopontine angle
    • Intramedullary cysts have been described
  • Epidermoid cysts originate from the ectoderm
  • Benign; slowly growing; encapsulated; whitish capsule (pearly tumor)
  • Usually associated with dysraphism
• Clinical features:
  • Sites:
    • Intramedullary
    • Intradural
  • Re-tethering of the cord after repair of lipomyelome-ningocele (delayed >10 years)
  • Associated with LP early in neonatal life
  • Intradural extramedullary types:
    • Bone or skin malformation (spina bifida or dermal sinus)
    • Pathogenic from LP (epidermal cells pushed in by the needle)
    • Lumbar pain
    • Urinary dysfunction
  • Thoracic intradural epidermoid:
    • Sudden paraplegia

Spinal Teratoma

• General considerations:
  • Site of origin:
    • Sacrococcygeal area
  • Associated with:
    • Dysraphic states
    • Diastematomyelia
    • Lipomyelomeningocele
• Clinical features:
  • Urinary retention
  • Weakness of the legs
  • May be seen at different level than an associated malformation
  • Scoliosis
  • Rarely may be extradural and compress the cord having extended through intervertebral foramen

Metastatic Spinal Cord Tumors

• General considerations:
  • Destroy the vertebra:
    • Mid vertebral body destruction
    • Pedicles and posterior elements are affected
    • Effects the cord by epidural compression from the intervertebral canal
    • Does not involve the disc space
    • Pathologic fractures are common
• Clinical features:
  • Boring nocturnal pain
  • May have radicular component
  • Ataxia prior to weakness; usually overshadowed by the weakness
  • Early bladder involvement
  • Obstipation may be noted prior to bladder symptoms
  • Dropped sensory levels (particularly from the cervical cord)
  • Parenchymal metastases:
    • Blood borne to the spinal cord
    • Lung
    • Malignant melanoma
    • Less bladder and bowel involvement
    • Sacral sparing (if Intramedullary)
    • Local atrophy at the affected level
  • Rare involvement of sympathetic thoracic ganglion with segmental hyperhidrosis; usually from lung tumors

Specific tumor metastatic patterns

• Lung:
  • Multiple metastatic sites
  • Most common in males
  • Blood borne to center of the cord
  • Hemorrhagic Intramedullary necrosis
  • Direct extension (Pancoast); sulcal tumor from apex of the lung
  • Almost always concomitant cerebral metastases
• GI tract:
  • May destroy the sacrum
  • Colorectal cancer
• Prostate:
  • Local lumbosacral invasion
  • Osteoblastic and osteoclastic components
  • Spinal cord and radicular components T10–L1 to L3 roots as opposed to L4–S1 roots from mechanical cause
  • Slowly progressive
  • Has been described in the petrous apex of the posterior fossa
• Renal cell:
  • Radicular component
  • Usually only one cerebral metastasis
• Leukemia
  • Marble vertebra (dense on spine film; marrow change on MRI)
  • Involvement of nerve roots and spinal compression
  • 70% have leptomeningeal spread
• Lymphoma:
  • May infiltrate vertebral body (dense vertebrae)
  • Envelops the lumbosacral dura and cauda equina
  • Cord compression and nerve root involvement
  • Leptomeningeal spread
• Drop metastasis:
  • Medulloblastoma
  • Ependymoma

Differential Diagnosis of intrinsic vertebral body bone disease (with spinal cord compression)

• Paget's disease
• Fibrous dysplasia of bone (Albright's disease)
• Brown bone cyst
• Articular bone cyst
• Osteosarcoma
• Ewing's sarcoma (12% vertebral body)
• Chondrosarcoma
• Osteosclerotic myeloma (POEM's syndrome)
• Multiple myeloma
• Osteoid osteoma
• Hemangioma of bone
• Eosinophilic granuloma
• Osteochondroma
• Osteoporosis
• Hyperparathyroidism
• Ankylosing spondylitis
• Vitamin deficiency
• Osteomalacia (syndromes)
• Vertebral collapse (prolonged IV heparin)
• Chordoma

Osteosarcoma

• General considerations:
  • Young patients
  • Long bones preferred sites; distal femur most common
• Clinical features:
  • Rare in vertebral body <5%
• Imaging evaluation:
  • Star burst pattern on plain film
  • Rapid growth; soft tissue mass

Osteochondrosarcoma

• General considerations:
  • Young patients
• Clinical features:
  • Painful
  • Rapid progression
  • Brainstem compression
  • Cervical vertebra
  • Petroclival suture
• Imaging evaluation:
  • Cauliflower appearance (plain X-ray)

Multiple Myeloma

• General considerations:
  • Multiple vertebrae involved
  • Anemia (severe)
• Clinical features:
  • Associated small fiber amyloid neuropathy
  • Vertebral collapse with spinal cord compression
• Imaging evaluation:
  • Multiple levels involved
  • Osteoblastic bone lesions
  • MRI:
    • Moth eaten low density lesions on T1 weighted images

Extramedullary Hematopoiesis with Epidural Cord Compression

• General considerations:
  • Spinal Paget's when the reparative processes is predominant
  • Convex masses of partially calcified osteoid tissue project from vertebral bodies to paravertebral regions
  • Pedicles, laminae are thickened; vertebral body is flattened
  • Weakened vertebrae may suddenly collapse with involvement:
    • Lumbar > thoracic levels
    • With spinal cord compression 3–5 vertebrae are involved
• Clinical features:
  • Slow progression (6–12 months)
  • Sensory loss and weakness early; then spasticity, bowel and bladder involvement
  • Lumbar involvement usually single vertebrae:
    • Radiculopathy (single)
    • Neurogenic claudication
    • Polyradiculopathy
  • Cervical:
    • Extremely rare
    • Quadriplegia reported

Osteoclastic Monostatic Myeloma

• General considerations:
  • One vertebral body involved
  • May be any bone in the body or any vertebral level
  • Motor sensory neuropathy
• Clinical features:
  • POEMS syndrome:
    • Polyneuropathy
    • Organomegaly
    • Endocrinopathy
    • Myeloma
    • Skin lesions

Eosinophilic Granuloma

• General considerations:
  • Young patients (10–20 years of age)
  • Involvement of proximal humerus, skull and thoracic spine
• Clinical features:
  • Sudden vertebral collapse with spinal cord compression
• Imaging evaluation:
  • Clean punched out oval lesions

Osteochondroma

• General considerations:
  • Long bones are involved
  • Most often asymptomatic
• Clinical features:
  • Associated with syndromes that may affect the spinal cord
    • Bannayan–Zonana syndrome (AVMs of the cord)
    • Gardner's syndrome

Paget's Disease

• General considerations:
  • Increased bone resorption followed by increased bone formation
  • Long bones of lower extremities, pelvic bones, skull, upper extremity bones, ribs, scapulae, vertebrae; one vertebrae (ivory vertebrae) are affected
  • Slowly progressive
• Clinical features:
  • Malignant (sarcomatous transformation)
    • Occurs in pagetic bone of skull or spine
    • <1% of patients
    • Malignant degeneration of vertebral body distinctly rare
  • Paget's disease may develop:
    • Benign giant cell tumor (osteoclastoma)
  • Atlantoaxial fracture dislocation
  • Epidural calcification
  • Extramedullary hematopiesis

Giant Articular Bone Cyst

• General considerations:
  • Often asymptomatic
  • Occurs in thoracic vertebrae
• Clinical features:
  • Collapse with radicular symptoms or rarely spinal cord compression

Hemangioma of Bone

• General considerations:
  • Often multiple; more common in thoracic vertebral bodies
  • Extremely vascular tumors
• Clinical features:
  • Compress the spinal cord by vertebral fracture and hematoma
  • May become symptomatic with minor trauma
• Imaging evaluation:
  • Reticulated pattern by CT

Osteoid Osteoma

• General considerations:
  • Most common in long bones; wrist is frequently involved
• Clinical features:
  • Locally painful
  • Affects posterior elements (pedicle and facets)
  • Pain responds more favorably to aspirin than narcotics
  • Rare to have vertebral collapse
• Imaging evaluation:
  • Destruction of posterior elements

Hyperparathyroidism

• General considerations:
  • Prominent in renal failure (secondary form)
• Clinical features:
  • Muscle symptoms of proximal weakness, aches and pain are predominant
  • Rare vertebral collapse
  • Diffuse involvement and weakening of vertebral column

Ankylosing Spondylitis

• General considerations:
  • Asymmetrical large joint arthritis
  • Plantar fasciitis
  • Sacroiliitis; often first joint affected
  • HLA-B27 associated
  • Worse in the morning
  • Anterior uveitis
• Clinical features:
  • Severe restriction of spinal movement
  • Falls and fractures with quadra and paraparesis
• Image evaluation:
  • Dural ectasia
  • Cysts of lumbosacral nerve roots
• Differential diagnosis of spondyloarthropathy
  • Cervical and lumbar spondylosis
  • Reiter's syndrome (reactive arthritis)
  • Psoriasis
  • Inflammatory bowel disease (regional enteritis and ulcerative colitis)
  • Idiopathic ankylosing spondylitis

Forester's Disease (Diffuse Idiopathic Hyperostosis) DISH

• General considerations:
  • Bridging osteophytes between vertebrae
  • Osteoporotic vertebral bodies
• Clinical features:
  • Restriction of spinal movement
  • Spinal cord and root compression

Osteoporosis

• General considerations:
  • Decrease in mineralized bone matrix without abnormality of bone cell populations or mineral metabolism
  • Vertebral compression fracture (anterior wedge) with minimal trauma
  • Fractures of proximal femur and distal radius
• Clinical features:
  • Vertebral compression fractures:
    • Local pain for 2–3 months
    • Thoracic spine anterior wedging causes dorsal kyphosis
    • Lumbar pain induced from compensatory lumbar hyperlordosis
    • Neurological deficits rare (r/o underlying metastatic lesion)
    • Back pain without radiographic fracture are probable microfractures

Scoliosis

• General considerations:
  • Kyphosis and kyphoscoliosis more likely to cause spinal cord compression than scoliosis
  • 72% of neurological complications are from scoliosis
  • Male > female
  • Pattern of involvement is:
    • 60–70% kyphoscoliosis; 17% kyphosis; 15% scoliosis (pure)
  • Slowly progressive thoracic myelopathy
• Clinical features:
  • Pain locally at area of spinal deformity
  • Acute precipitation by trauma (rare)
  • Bladder relatively spared
  • Spasticity with paraparesis
  • Radiculopathy

Thoracic Spine Kyphoscoliosis

• General considerations:
  • Earlier causes tbc and poliomyelitis
  • Myelomeningocele common cause at present:
    • Associated anomalies:
      • Hemivertebrae
      • Absence of ribs
      • Diastematomyelia
      • Klippel–Feil deformity
      • Sprengel's deformity
      • Arnold Chiari malformation
    • Kyphoscoliosis occurs in patients with hydrocephalus that is unrelated to congenital spine defects
    • Acquired kyphoscoliosis of the thoracic spine is from:
      • Infection of vertebral body, lamina, pedicles
      • After laminectomy for tumor
      • Syringomyelia
  • Complications of therapy:
    • Halo femoral or halo pelvic traction
      • Cranial nerve palsies involving VI, IX, X, XI (cervical kyphoscoliosis)
      • Brachial plexus traction injury
      • Horner's syndrome
      • Myelopathy with motor and sensory deficits
    • Harrington rod placement:
      • Paraplegia
      • Anterior spinal cord syndrome
      • Urinary tract dysfunction
    • Postlaminectomy kyphosis:
      • Follows spinal operations for: trauma, tumor, myelomeningocele

Vitamin D Deficiency:

• General considerations:
  • Dilantin use
  • Kidney disease
  • Multiple bone fracture
• Clinical features:
  • Severe bone pain affecting the pelvic girdle
  • Associated proximal muscle weakness pelvic girdle > shoulder girdle

I.V. Heparin Therapy

• General considerations:
  • Thoracic vertebrae deformity (fish mouth)
• Clinical features:
  • Pain locally
  • Heparin use greater than two weeks
  • Rare vertebral body collapse

Differential Diagnosis of Intrinsic Disc Disease with Spinal Cord Involvement

• Extruded disc (centrally)
• Free fragment disc (may migrate)
• Relapsing polychondritis
• Ochronosis (homogentisic aciduria)
• Collagen gene defects (COLA 1–5)
• Ehlers Danlos syndrome (type IV)
• Marfan's syndrome
• Tbc
• SBE (staph aureus)
• Gram negative infection following surgery
• Charcot joint (disc destruction)
  • Syphilis
  • Diabetes mellitus
  • Amyloid
  • Syrinx
• Trauma (exacerbates pre-existing disc dysfunction; does not cause degenerative disc disease)
• Fungal infection:
  • Nocardia
  • Cryptococcus
  • Blastomycosis
  • Coccidioidomycosis (not uncommon from contiguous sacroiliac infection)

Disc Disease

• General considerations:
  • Central disc protrusion may compress the spinal cord acutely or chronically at cervical, thoracic, and high lumbar levels
  • Most dangerous area is thoracic due to the small diameter of the spinal canal at this level
  • A disc fragment may erode through the posterior longitudinal ligament and compress the cord; it may migrate rostrally and caudally
  • Radicular symptoms may be noted with lateral or contralateral disc protrusion (loss of bilateral foraminal integrity) there may or may not be associated cord compression
  • Initial thoracic disc protrusion symptoms:
    • Patients legs collapse during heavy lifting
    • Severe thoracic radicular pain
  • Central cervical disc rupture most common at C5–C6; C6–C7 (greatest flexion and extension movement segments)
• Clinical features:
  • Incomplete spinal cord injury
  • Brown-Sequard syndrome
  • Anterior spinal artery syndrome
  • May cause pseudo peripheral nerve sensory loss of the lower extremities (stocking like)

Relapsing Polychondritis

• General considerations:
  • Autoimmune disease of cartilage
  • Associated cartilage involvement of trachea, pinna and nose
  • Cause of relapsing meningitis
  • Cause of optic neuritis
• Clinical features:
  • Affects disc at any level with cord compression

Ochronosis

• General considerations:
  • Defect of homogentisic acid metabolism
  • Affects thoracic discs
  • Dark urine
• Clinical features:
  • Rare clinical symptoms
• Imaging evaluation:
  • Calcification of thoracic discs ("rugger Jersey")
  • Calcified ear lobes

Charcot Joints and Disc Destruction of the Thoracic Spine

• General considerations:
  • Loss of pain and proprioceptive input to the joint
  • Occurs with:
    • Diabetes mellitus
    • Amyloidosis
    • Syphilis (tabes dorsalis)
    • Thoracic or cervical syrinx
• Clinical features:
  • Proprioceptive loss at the affected level
  • Abnormal motion segment; vertebral body collapse
  • Cord compression, radicular motor or sensory loss
• Collagen defects all have disc capsule defects with extrusion of the nucleus pulposus
  • Marfan's disease
  • COLA 1–5 gene defects
  • Ehlers Danlos (type IV)
  • Osteogenesis imperfecta

Infections of the Disc Space

• General considerations:
  • Exquisite pain with movement or vibrating the bed
  • Frequent radicular component
  • High sedimentation rate
  • Local pain at the level of involvement
  • Eburnated end plates of the vertebral bodies are characteristic of tuberculosis
  • Involvement of the pre and post vertebral fascia is common; may extend over several segments
  • Usually only one disc level is involved
  • Arteries that supply the disc are end arteries
  • Paravertebral muscle involvement:
    • Common in the iliopsoas (cold abscess) at the L1–L3 levels in American patients with tuberculosis
    • Asian patients tend to have more cervical involvement

Clinical Characteristics of Specific Infections of the Disc Space

• Staphylococcus aureus:
  • Common with IV drug abuse and in HIV infected patients
  • Rapid symptomatology
  • Can be at any spinal level but most often at thoracic levels
  • Concomitant with SBE
• Gram negative infections:
  • Post disc surgery
  • Pseudomonas; E coli; enterococcus are the most common
  • Organisms may have delayed onset; 2–3 weeks after post-surgical procedure; rarely 6–8 weeks after surgery
• Nocardia:
  • Noted in patients with lung abscess
  • May have cervical predilection
  • Associated with immunosuppressed patients
• Cryptococcus
  • Low cervical and thoracic disc spaces
  • Lung origin
  • HIV and immunocompromised patients
  • Associated meningitis and cranial nerve II involvement
• Coccidiomycosis
  • Lumbosacral disc spaces; origin of infection is contiguous bone osteomyelitis
  • Associated arteritis
  • South West USA (San Joaquin valley fever)
• Actinomycosis
  • Thoracic and low cervical disc spaces
  • Concomitant lung infection
  • Draining sinuses with sulfur granules

Division C: Concomitant Spinal Cord Compression from Pathology of Ligaments/Arachnoid Cysts and Dural Processes

• Posterior longitudinal ligament calcification (cervical); Japanese patients primarily
• Ankylosing spondylitis (anterior and posterior longitudinal ligament calcification)
• Acromegaly (generalized swelling of all soft tissue)
• Mucopolysaccharidoses (Shie's disease)
• Idiopathic hypertrophic pachymeningitis
• Syphilitic cervical pachymeningitis
• Familial amyloid polyneuropathy – type I (meningeal involvement)
• Sarcoid dural infiltration
• Lymphomatous dural infiltration
• Tuberculous dural infiltration
• Low CSF pressure states (dural enhancement after LP); no pressure on the spinal cord
• Rheumatoid dural proliferation
• Ligamentous laxity syndromes:
  • Mongolism (C1–C2)
  • Ehlers Danlos type IV
  • Marfan's disease
  • Homocystinuria
  • Rheumatoid arthritis (pannus formation; destruction and erosion of atlanto axial ligaments at C1–C2)
  • Spinal arachnoid cysts
• Hirayama's Disease
  • Abnormal laxiff of ligaments and movement at C8–T1

Ossification of the Posterior Longitudinal Ligament

• General considerations:
  • Occurs in the cervical spine
  • Extends only two segments or the entire cervical spine; upper and midportion of the spine involved > lower segments
  • Occurs in patients > 40 years of age
  • Males > females
  • Most frequent in Japanese patients
• Clinical features:
  • Myelopathy > radiculopathy; both may occur concomitantly
  • Posterior cervical pain may be an isolated complaint
  • Usually a slowly progressive myelopathy
  • Trauma may precipitate acute cord compression in a congenitally narrow canal
  • Radiculopathy with segmental pain; weakness and sensory loss is frequent
  • Most frequently involved roots are C7, C8 and T1
  • May compress one half of the spinal cord

Acromegaly

• General considerations:
  • Enlargement of all ligaments
  • Associated soft tissue hypertrophy
  • Cardiac and joint involvement
• Clinical features:
  • Carpal and tarsal tunnel syndromes are the frequent presenting complaints
  • Spinal cord compression with myelopathy (all levels)

Mucopolysaccharidoses

• General considerations:
  • Cataracts; dysmorphisms (type V Scheie's)
  • Scheie patients may have normal intelligence
• Clinical features:
  • Carpal and tarsal tunnel syndromes are frequent presenting complaints
  • Spinal cord compression with myelopathy may occur at all levels

Idiopathic Hypertrophic Pachymeningitis

• General considerations:
  • Affects dura throughout the CNS
  • Cerebral and cranial nerve dura are involved (cranial nerve compressive palsies occur)
• Clinical features:
  • Slowly progressive myelopathy concomitant with cerebral and cranial nerve involvement
• Imaging evaluation:
  • Meninges thickened
  • Most often enhance with gadolinium
• Differential diagnosis:
  • Sarcoid
  • Lymphoma
  • Rheumatoid dural involvement
  • Syphilis (pachymeningitis cervicalis)

Sarcoid

• General considerations:
  • Midthoracic dural involvement
  • May involve dura throughout the CNS
• Clinical features:
  • Concomitant cranial nerve, pituitary, chiasmatic and parenchymal involvement
  • May present with concomitant sensorimotor or GBS syndrome
• CSF:
  • Glucose 30–40 mg /dL)
  • Protein 100–150 mg /dL)
  • Lymphocytes 5–30 mm³
• Imaging evaluation:
  • Dural enhancement with gadolinium
  • Lesions occur in the posterior pituitary, hypothalamus and all areas of the parenchyma

Ligamentous Laxity Syndrome

• General considerations:
  • Atlanto-axial hypermobility
    • Odontoid to axis should separate no more than 3 mm on flexion in an adult
    • Cruciate ligament (behind odontoid); alar ligament (tip of the odontoid to rim of foramen magnum); lateral ligaments (odontoid to lateral masses of C1) are loose
    • Atlanto axial subluxation > subaxial subluxation
    • Differential diagnosis of lax ligaments:
      • Mongolism
      • Ehlers Danlos type IV
      • Marfan's
      • Rheumatoid arthritis
      • COLA 1–5 gene defects
• Clinical features:
  • Paresthesias of the hands
  • Minimal trauma may cause quadriparesis (most common in spondylolisthesis of rheumatoid arthritis)
  • Neck flexion may cause drop attacks and increased reflexes

Unusual Causes of Spinal Cord Compression

• General considerations:
  • Cord compressed at thoracic level
    • Associated with:
      • Severe obesity
      • Chronic steroid treatment
• Clinical features:
  • Usually slowly progressive paraparesis
• Imaging evaluation:
  • Calcification of fat in Paget's disease
  • Increased fat on T1 weighted images

Extramedullary hematopoiesis

• General considerations:
  • Bone marrow failure
  • Hemolytic anemia
  • Male > female
  • Postpolycythemic myeloid metaplasia
  • Thalassemia
  • Sickle cell disease
  • Hereditary spherocytosis
• Clinical features:
  • Clinical level is usually midthoracic
  • Slowly progressive myelopathy
• Imaging evaluation:
  • Abnormal marrow signal over the vertebral column (loss of marrow fat)

Enlarged Nerve Root

• General considerations:
  • HSMN I, III, V are most common
• Clinical features:
  • Clinical signs of congenital neuropathy
  • Cauda equina compression
  • Radiculopathy
  • Widening of the spinal canal
  • Association with nerve root cysts
  • Rupture with chronic low pressure headache
• Imaging evaluation:
  • MRI demonstrates thickened roots compressing the spinal cord

Differential diagnosis of dural ectasia:

• Neurofibromatosis type I
• Marfan's syndrome
• Ehlers Danlos type IV
• Collagen gene defects (I–V)
• Ankylosing spondylitis