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Matti Iivanainen, MD; Pauli Leinikki, MD; Eero Taskinen, MD; Isabel C. Shekarchi, PhD; David Madden, DVM, PhD; John Sever, MD, PhD

Arch Neurol. 1981;38(4):206-208.

Abstract
• We studied CSF and serum samples from 16 patients with progressive myoclonus epilepsy (PME). These patients had juvenile-onset PME with evidence of autosomal recessive inheritance and no Lafora bodies. Twelve of the 16 patients with PME had immunologic abnormalities. Oligoclonal bands were seen in six of the eight patients from whom sufficient CSF was available. The CSF albumin and serum/CSF albumin ratios were normal in all 16 patients, indicating the presence of intact blood-brain barriers. Six of the 16 patients showed increased CSF IgG levels and five had an increased CNS IgG synthesis. All patients had normal serum and CSF IgM and IgA levels. Three patients, all with bands, had reduced measles and/ or vaccinia serum/CSF antibody ratios. The findings suggest altered immune response of the CNS of some patients with PME apparently caused by nonspecific immunostimulation.

Author Affiliations
From the Infectious Diseases Branch, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, Md (Drs Iivanainen, Leinikki, Shekarchi, Madden, and Sever); and the Cerebrospinal Fluid Laboratory, University Central Hospital, Helsinki (Dr Taskinen).

Footnotes
Accepted for publication July 13, 1980.

Reprint requests to Infectious Diseases Branch, IRP, NINCDS, National Institutes of Health, Bldg 36, Room 5D-06, Bethesda, MD 20205 (Dr Iivanainen).