Title

Paraneoplastic opsoclonus-myoclonus syndrome revealing T-cell lymphoma

Author

Ducrocq X; Petit J; Taillandier L; Dorvaux V; Anxionnat R; Pl´enat F; Vespignani H

Address

Service de Neurologie, H^opital Central, Nancy

Source

Presse Med, 28(7):330-3 1999 Feb 20

Abstract

BACKGROUND: Nonrhythmic involuntary ocular oscillations and axial and segmentary myoclonia are associated in the opsoclonus-myoclonus syndrome. In adults, a paraneoplastic origin is generally found. We report the first of opsoclonus-myoclonus associated with non-Hodgkin's lymphoma. CASE REPORT: A 66-year-old woman rapidly developed a typical opsoclonus-myoclonus syndrome within a few hours, presenting vertigo, cerebellous ataxia, multidirectional involuntary ocular movements and non-rhythmic axial and segmentary myoclonia. Brain computed tomography and magnetic resonance imaging demonstrated discrete diffuse anomalies of the white substance predominating in the pons. The cerebrospinal fluid showed discrete lymphocytosis. Antineuron antibodies were negative. No cause could be identified until the development 11 months later of pleomorphic T-cell mediastino-cervical lymphoma. The patient responded moderately to a CHOP regimen which had no effect on the opsoclonus-myoclonus syndrome. Death occurred after a 16-month course due to pulmonary complications. DISCUSSION: Neuroblastoma and infectious causes predominate in opsoclonus-myoclonus syndromes observed in children; in adults, the predominant cause is cancer. Antineuron, anti-Ri and anti-Hu antibodies can be evidenced in some cases, arguing in favor of a paraneoplastic mechanism. Recent reports have evidenced MRI anomalies in the pons and the cerebellum, anatomically well correlated with the opsoclonus-myoclonus syndrome. Besides small-cell bronchogenic anaplastic cancer, the possibility of cancer of the breast and uterus, and both non-Hodgkin and Hodgkin lymphoma should be explored, knowing the cancer develops several month after the opsoclonus-myoclonus syndrome.

Language

French

Unique Identifier

99183620

MESH Headings

Aged ; Case Report ; English Abstract ; Female ; Human ; Lymphoma, T-Cell CF/*CO/DI ; Magnetic Resonance Imaging ; Mediastinal Neoplasms CF/*CO/DI ; Myoclonus CF/*ET ; Neuroblastoma *CO/DI ; Ocular Motility Disorders CF/*ET ; Paraneoplastic Syndromes

Publication Type

JOURNAL  ARTICLE

ISSN

0755-4982

Country of Publication

FRANCE