Opsoclonus as a dominant sign in primary Sjögren’s syndrome

Autor: D. Lubec, W. Müllbacher, B. Mamoli, Josef Finsterer, V. Weiler
Rok vydání: 1999
Předmět:
Zdroj: Neuro-Ophthalmology. 22:135-138
ISSN: 1744-506X
0165-8107
DOI: 10.1076/noph.22.3.135.3725
Popis: Primary Sjogren’s syndrome (PSS) is often associated with nervous system abnormalities, although opsoclonus as the dominant sign has not been reported before. In a 69-year-old woman with acute-onset opsoclonus as the dominant sign, ataxia, dysarthria, vertigo, depression, anxiety, and limb jerking, PSS was diagnosed based on xerophthalmia, xerostomia, increased anti-nuclear antibodies, a positive Schirmer’s test, and a pathologic salivary gland scintigraphy. Cerebral MRI revealed multiple, hyperintense basal ganglia and brainstem lesions. Cerebral HMPAO-SPECT showed unilateral cerebellar hypometabolism. Muscle/nerve biopsy was compatible with axonal polyneuropathy. Corticosteroids improved the patient’s condition considerably. In conclusion, opsoclonus may be the dominant sign in PSS and responds well to corticosteroids.
Databáze: OpenAIRE