Clinical, CSF, and MRI findings in Devic's neuromyelitis optica.

OBJECTIVES: Since Devic's original description of neuromyelitis optica in 1894 there has been much debate regarding its aetiology. A specific cause has been identified in a minority of cases but in most the question has arisen whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This study was undertaken to help clarify this issue. METHODS: Neuromyelitis optica was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody status was documented. Patients underwent CSF examination and MRI of brain and spinal cord. RESULTS: Twelve patients, with a mean age of presentation of 35.1 years, were seen. Eleven were women; vision was reduced to counting fingers or worse in 10 patients and seven became confined to a wheelchair. Examination of CSF showed local synthesis of oligoclonal bands in only two patients and a neutrophil pleocytosis in two. A possible aetiology was identified in five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and possible acute disseminated encephalomyelitis (two). Six had non-specific increases in various autoantibodies. Eleven patients underwent MRI of the brain and spinal cord. In 10 there were diffuse abnormalities involving cervical and thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in five; in five there were multiple deep white matter lesions, and one patient had minor age related changes. CONCLUSION: It is proposed that Devic's neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI features different from those found in classic multiple sclerosis. In most cases a specific aetiology is not identified, but an immunological mechanism of tissue damage seems likely

The clinical use of cerebrospinal fluid studies in demyelinating neurological diseases.

The clinical diagnosis of definite multiple sclerosis is supported by abnormalities in the cerebrospinal fluid: variable mild pleocytosis and elevation of total protein, moderately elevated total IgG in most patients, and the almost invariable presence of discrete immunoglobulins after electrophoresis, the oligoclonal bands. The oligoclonal bands are non-specific, and are seen in most diseases of the nervous system, but their temporal uniformity in each patient with multiple sclerosis is characteristic. Prognostically, patients with a single episode of optic neuritis or paraesthesia who have oligoclonal bands are more likely to develop multiple sclerosis than if the spinal fluid were normal. In the Guillain-Barré syndrome, the spinal fluid total protein is transiently elevated, with no pleocytosis. Oligoclonal bands are usually found in the acute phase and only persist in those patients with chronic or relapsing polyneuropathy