Familial Oculoleptomeningeal Amyloidosis: Report of a New Family With Unusual Features

Ryan J. Uitti, Jeffrey R. Donat, B. Rozdilsky, Ralph J. Schneider, Arnulf H. Koeppen

Research output: Contribution to journalArticle

58 Scopus citations

Abstract

A family had a dominantly inherited amyloid angiopathy that involved the meninges of the brain and spinal cord, retina, vitreous humor, peripheral nerves, and systemic organs. Clinical features included hemiplegic migraine, periodic obtundation, psychosis, seizures, intracerebral hemorrhage, myelopathy, visual impairment, deafness, and peripheral neuropathy. Pathological findings consisted of amyloid deposition in the leptomeningeal and retinal vessels, in the vitreous humor, and in perivascular tissue throughout the body. Evaluation of the amyloid showed it to be a transthyretin (prealbumin). A brief course of plasmapheresis produced a short-lived decreased concentration in circulating transthyretin.

Original languageEnglish (US)
Pages (from-to)1118-1122
Number of pages5
JournalArchives of neurology
Volume45
Issue number10
DOIs
StatePublished - Oct 1988

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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    Uitti, R. J., Donat, J. R., Rozdilsky, B., Schneider, R. J., & Koeppen, A. H. (1988). Familial Oculoleptomeningeal Amyloidosis: Report of a New Family With Unusual Features. Archives of neurology, 45(10), 1118-1122. https://doi.org/10.1001/archneur.1988.00520340072015