E. Lee Murray, MD

OVERVIEW

Differential diagnosis of myelopathy is broad and can include almost any of the cardinal mechanisms of disease. The following list presents common and important conditions that can cause myelopathy. Additional details are subsequently presented in this chapter for disorders not discussed elsewhere.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of myelopathy includes:

•Inflammatory disorders

◦Transverse myelitis: Rapidly progressive inflammatory myelopathy. Thoracic spine is most common site.

◦Neuromyelitis optica (NMO): Rapidly progressive inflammatory myelopathy associated with optic neuritis

◦Multiple sclerosis: Episode of myelopathy but usually incomplete, less severe than NMO; also history or magnetic resonance imaging (MRI) evidence of brain lesions

◦Acute disseminated encephalomyelitis (AEDM): Rapidly progressive myelopathy with other deficits including confusion, headache, neck pain, cranial nerve deficits

◦Paraneoplastic myelitis/Necrotizing myelopathy: Rapidly progressive myelopathy with inflammatory and hemorrhagic findings on MRI.

•Structural disorders

◦Cauda equina syndrome: Compression of the cauda equina with progressive asymmetric leg weakness, sensory disturbance, and sphincter disturbance, with back and radicular pain

◦Conus medullaris syndrome: Compression, infiltration, or inflammation of the conus produces rapidly progressive symmetric motor, sensory, and sphincter deficits

◦Syringomyelia: Fluid-filled cavity in the spinal cord, often associated with Chiari malformation; often produces central cord syndrome

◦Compressive myelopathy from vertebral disease: Compression from bony or disc elements

◦Spinal cord tumor: Primary or metastatic lesion at any level of the cord initially produces mainly local pain then progressive myelopathy

◦Spinal hematoma: Back pain followed rapidly by myelopathy or cauda equina syndrome

◦Epidural abscess: Rapidly progressive myelopathy, back pain, often with headache and malaise

•Degenerative disorders

◦Hereditary spastic paraparesis: Family of disorders of various inheritance with progressive myelopathy, but also can produce nonspinal neurologic manifestations—cerebral, brainstem, cerebellar

◦Primary lateral sclerosis: Progressive spastic paraparesis without sensory loss or lower motoneuron involvement

•Vascular disorders

◦Spinal cord infarction: Acute onset of paralysis, usually at the level of the lower thoracic spine

◦Spinal arteriovenous malformation (AVM): Widely variable but generally stepwise-progressive asymmetric myelopathy; usually but not invariably painless

•Metabolic/nutritional disorders

◦B₁₂ deficiency: Spinal manifestations are spastic paraparesis, sensory disturbance affecting especially vibration and position sense

◦Copper deficiency: Can present with weakness from myelopathy of neuropathy; can resemble and be combined with B₁₂ deficiency

•Cancer-related disorders (nonstructural)

◦Radiation myelopathy:

– Acute myelopathy: Quadriplegia or paraplegia with acute onset. There is a transient form.

– Chronic progressive radiation myelopathy: Progressive ascending deficit with sensory, motor, and sphincter disturbance. Spasticity is prominent.

◦Upper motor neuron degeneration: Spasticity as a component of encephalomyelitis, usually with small-cell lung cancer

◦Lower motor neuron degeneration: Progressive paralysis, often upper extremities, especially with lymphoma

CLINICAL DIAGNOSIS

Clues from clinical presentation can suggest a shorter list of differential diagnoses.

Acute myelopathy following fall:

•Intraspinal extra-medullary hematoma

•Intramedullary spinal hematoma

•Spinal cord contusion

•Acute herniated disc with compressive myelopathy

•Vertebral fracture with bony compression of the spinal cord

Subacute myelopathy:

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