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Ann Neurol. 1993 Aug;34(2):162-8.

Devic's neuromyelitis optica: a clinicopathological study of 8 patients.

Author information

1
Department of Neurology, University of New Mexico School of Medicine, Albuquerque 87131.

Abstract

We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to benefit the patients, 5 of whom died. Autopsies of these 5 patients demonstrated a severe necrotizing myelopathy with thickening of blood vessel walls and no lymphocyte infiltrates. In the appropriate clinical setting, the lack of white matter abnormalities demonstrated by magnetic resonance imaging of the head facilitates the recognition of Devic's syndrome during life. Inasmuch as Devic's myelopathy is necrotizing, rather than demyelinating, the prognosis of this syndrome is poor.

PMID:
8338340
DOI:
10.1002/ana.410340211
[Indexed for MEDLINE]

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