TY - JOUR
T1 - Familial Oculoleptomeningeal Amyloidosis
T2 - Report of a New Family With Unusual Features
AU - Uitti, Ryan J.
AU - Donat, Jeffrey R.
AU - Rozdilsky, B.
AU - Schneider, Ralph J.
AU - Koeppen, Arnulf H.
PY - 1988/10
Y1 - 1988/10
N2 - 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.
AB - 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.
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U2 - 10.1001/archneur.1988.00520340072015
DO - 10.1001/archneur.1988.00520340072015
M3 - Article
C2 - 3178532
AN - SCOPUS:0023741439
SN - 0003-9942
VL - 45
SP - 1118
EP - 1122
JO - Archives of neurology
JF - Archives of neurology
IS - 10
ER -