Familial oculoleptomeningeal amyloidosis: Report of a new family with unusual features

R. J. Uitti, J. R. Donat, B. Rozdilsky, R. J. Schneider, A. H. Koeppen

Research output: Contribution to journalArticle

58 Citations (Scopus)

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
StatePublished - 1988
Externally publishedYes

Fingerprint

Familial Amyloidosis
Prealbumin
Vitreous Body
Amyloid
Meninges
Retinal Vessels
Plasmapheresis
Spinal Cord Diseases
Vision Disorders
Cerebral Hemorrhage
Deafness
Peripheral Nervous System Diseases
Migraine Disorders
Peripheral Nerves
Psychotic Disorders
Retina
Spinal Cord
Seizures
Brain
Familial

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

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.

Familial oculoleptomeningeal amyloidosis : Report of a new family with unusual features. / Uitti, R. J.; Donat, J. R.; Rozdilsky, B.; Schneider, R. J.; Koeppen, A. H.

In: Archives of Neurology, Vol. 45, No. 10, 1988, p. 1118-1122.

Research output: Contribution to journalArticle

Uitti, RJ, Donat, JR, Rozdilsky, B, Schneider, RJ & Koeppen, AH 1988, 'Familial oculoleptomeningeal amyloidosis: Report of a new family with unusual features', Archives of Neurology, vol. 45, no. 10, pp. 1118-1122.
Uitti RJ, Donat JR, Rozdilsky B, Schneider RJ, Koeppen AH. Familial oculoleptomeningeal amyloidosis: Report of a new family with unusual features. Archives of Neurology. 1988;45(10):1118-1122.
Uitti, R. J. ; Donat, J. R. ; Rozdilsky, B. ; Schneider, R. J. ; Koeppen, A. H. / Familial oculoleptomeningeal amyloidosis : Report of a new family with unusual features. In: Archives of Neurology. 1988 ; Vol. 45, No. 10. pp. 1118-1122.
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