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NEUROLOGY 1983;33:593
© 1983 American Academy of Neurology

Aseptic meningoencephalitis in primary Sjogren's syndrome

Elaine L. Alexander, MD, PhD and Garrett E. Alexander, MD, PhD

Department of Medicine, Rheumatology Division (Dr. E. Alexander) and the Department of Neurology (Dr. G. Alexander), Johns Hopkins University School of Medicine, Baltimore, MD.

The (clinical features and CSF characteristics of five patients with primary Sjogren's syndrome (SS) and associated aseptic meningoencephalitis (AME) are described. Episodes of AME were recurrent in four patients. Viral, fungal, and bacterial cultures were uniformly negative. Plasma cells were observed in the CSF but not in the blood of three patients. The CSF 1gG:albumin index was elevated, suggesting intrathecal synthesis of IgG in each of the four patients tested; each patient had either one or two broad bands with the mobility of IgG on CSF agarose electrophoresis. These observations are consistent with current understanding of SS as a polyclonal gammopathy associated with the multifocal proliferation of B lymphocytes and plasma cells.

Address correspondence and reprint requests to Dr. G. E. Alexander, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore City Hospitals, 4940 Eastern Avenue, Building G Room 124, Baltimore, MD 21224.

This study was supported in part by NIH grant No. NS-00632.

Accepted for publication September 3, 1982.

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