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Primary familial amyloidosis with vitreous opacities
Authors:Jun Ogata  Masahiro Okayama  Ikuo Goto  Hajime Inomata  Itaru Yoshida  Teruo Omae
Affiliation:(1) Second Department of Medicine Faculty of Medicine, Kyushu University, Maidashi 3-1-1, 812 Fukuoka City, Japan;(2) Second Department of Neurology Faculty of Medicine, Kyushu University, Maidashi 3-1-1, 812 Fukuoka City, Japan;(3) Second Department of Ophthalmology Faculty of Medicine, Kyushu University, Maidashi 3-1-1, 812 Fukuoka City, Japan;(4) Second Department of Pathology Faculty of Medicine, Kyushu University, Maidashi 3-1-1, 812 Fukuoka City, Japan
Abstract:Summary A 41-year-old Japanese male with a new type of primary familial amyloidosis was reported. The patient developed vitreous opacities, and later, disturbances in the gastrointestinal and nervous systems. At autopsy, amyloid was observed in the vitreous and the retinal vessels. There were extensive cerebral infarcts and heavy meningo-vascular amyloid deposition. Although the postmortem study revealed slight peripheral nerve degeneration in the lower extremities secondary to amyloid deposition, there was no clinical evidence of polyneuropathy.
Keywords:Primary familial amyloidosis  Vitreous opacities  Meningovascular deposition  Extensive cerebral infarction
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