|
|
|
|
|
Click Here to return To Results
|
|
|
|
The distinguishing clinical findings, together indicative of destruction of gray matter elements of the cord, were limb atrophy, persistent areflexia, and flaccidity. The concentration of cerebrospinal fluid protein was typically elevated between 500 g/L and 1000 g/L, without oligoclonal bands, accompanied infrequently by pleocytosis. Magnetic resonance imaging showed features suggesting cord necrosis, specifically swelling, T2-weighted hyperintensity, and gadolinium enhancement over seve ral spinal cord segments, succeeded month later by atrophy in the same regions. Necrosis of the cord was found in biopsy material from one patient and postmortem pathology in another case, but inflammation and blood vessel abnormalities were absent. Onl y 2 patients had prolonged visual evoked responses. The disease progressed despite immune-modulating treatments although several patients had brief epochs of limited improvement. The saltatory course, prolonged visual evoked responses in 2 patients, and a cranial abnormality on magnetic resonance imaging in another, raised the possibility of a link to multiple sclerosis. However, then normal cranial magnetic resonance imaging scans in 6 other patients, uniformly absent oligoclonal bands, and poor respo nse to treatment were atypical for multiple sclerosis. On the basis of shared clinical and laboratory features, idiopathic progressive necrotic myelopathy is indistinguishable from a limited form of Devic disease. |
|
(click to filter results - removes previous filter)
cerebrospinal fluid,elevated protein of
MRI
MRI,abnormal
MRI,spinal cord
MRI,spinal cord,increased intramedullary cord signal
myelomalacia
myelopathy
myelopathy,chronic progressive
myelopathy,necrotizing
pain
spinal cord,enlargement
spinal cord,pathologic exam of
|
Click Here to return To Results
|
|