Adult-onset rapidly progressive spinal muscular atrophy of shoulder girdle with gammopathy

I. Szirmai, M. Antalicz, K. Trombitas, L. Kuntár, I. Gáti

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

The paper reports on clinical and pathologic findings in a 51-year-old man who presented with rapidly progressive spinal muscular atrophy associated with relatively early respiratory paralysis. Clinical diagnosis was based on typical EMG, and bioptic and enzyme findings. In the CSF protein and cell content were normal, the IgG concentration was high, the IgG/Alb quotient increased, intrathecal synthesis of IgG was evident, and oligoclonal gammopathy was found. All of these findings generally appear in neuroallergic diseases. Total loss of anterior-horn cells, demyelination of fasciculus cuneatus and spinothalamic pathways, as well as round Lafora-body like inclusions, preponderantly in the white matter, were observed with light microscope. Electron microscopic examination revealed reoviruses in the glia cells of the damaged motor zone.

Original languageEnglish
Pages (from-to)128-133
Number of pages6
JournalClinical Neuropathology
Volume2
Issue number3
Publication statusPublished - Dec 1 1983

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology

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    Szirmai, I., Antalicz, M., Trombitas, K., Kuntár, L., & Gáti, I. (1983). Adult-onset rapidly progressive spinal muscular atrophy of shoulder girdle with gammopathy. Clinical Neuropathology, 2(3), 128-133.