Clinical features:
(1) ascending flaccid areflexic paraplegia
(2) spincter paralysis
(3) segmental loss of all sensory modalities
(4) low back pain and/or sciatic pain (pain radiating to the lower extremity)
(5) positive Babinski signs bilaterally
(6) progressive disease that responds poorly to therapy
where:
• Hyperreflexia may be present in the earliest stages.
The patient may have a history of a visceral (intra-abdominal) carcinoma. The onset of symptoms may precede diagnosis of the underlying malignancy.
Examination of the CSF shows an elevated protein concentration and a moderate pleocytosis.
MRI of the cord shows enlargement with abnormalities seen on T2-weighted images.
Histologic examination shows:
(1) patchy and symmetric destruction of the gray and white matter affecting segments of the spinal cord
(2) often most prominent in the thoracic spinal cord
(3) no or minimal inflammation
(4) no evidence of vasculitis
(5) some patients may have involvement of the brainstem, cerebellum, internal capsule and opic nerve
Differential diagnosis:
(1) Herpes simplex virus (HSV) with ascending necrotizing myelopathy
(2) vasculitis-associated myelopathy in Hodgkin's disease
(3) vertebral fracture with cord compression
