Recurrent Optic Neuromyelitis with Endocrinopathies
- 1 January 1997
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 48 (1), 58-64
- https://doi.org/10.1212/wnl.48.1.58
Abstract
We report a new syndrome that we call "recurrent optic neuromyelitis with endocrinopathies" in eight Antillean women from Martinique and Guadeloupe.Ocular involvement was either monocular or binocular, whereas myelopathy was acute or subacute. In seven patients, myelopathic symptoms recurred, and in six patients, visual problems recurred. Spinal cord involvement was a consistent band-like pseudo-syringomyelic dissociated sensory loss. All eight patients had endocrinopathies consisting of amenorrhea, galactorrhea, diabetes insipidus, hypothyroidism, or hyperphagia. Spinal cord MRI revealed cavitation-like images. Various immunosuppressant treatments had little effect on the uniformly deteriorating course, ending in blindness and paraplegia. Six patients died within 5 years of onset, and an autopsy in one patient showed multiple demyelinizing lesions of the spinal cord with thickened blood vessel walls without evidence of inflammation. These cases appear to constitute a syndrome distinct from MS and from classic Devic's syndrome, not only because of the association with endocrinopathies but because of the stereotypy of the recurrences, the absence of MRI lesions in the cerebral white matter, and the unusual image of cavitation of the spinal cord. The syndrome is also distinct from HTLV-I-associated paraparesis, which is endemic in the West Indies. NEUROLOGY 1997;48: 58-64Keywords
This publication has 7 references indexed in Scilit:
- The epidemiology of multiple sclerosis: A general overviewAnnals of Neurology, 1994
- FOREWORDJournal of Neurology, Neurosurgery & Psychiatry, 1994
- Geographic variations in Wilson's diseaseJournal of the Neurological Sciences, 1993
- Thanks to reviewersAnnals of Neurology, 1993
- Oral session I: TumoursZeitschrift für Neurologie, 1990
- The role of NMR imaging in the assessment of multiple sclerosisClinical Neurology and Neurosurgery, 1988
- Cortical auditory disorders: clinical and psychoacoustic features.Journal of Neurology, Neurosurgery & Psychiatry, 1987