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December 1958

Primary Familial Amyloidosis

Author Affiliations

Bethesda, Md.
From the Ophthalmology Branch, National Institute of Neurological Diseases and Blindness, National Institutes of Health, U. S. Department Public Health Service.

AMA Arch Ophthalmol. 1958;60(6):1036-1043. doi:10.1001/archopht.1958.00940081056009

Primary familial amyloidosis is a rare systemic disease of protean manifestations.1-3 Ocular symptoms of amyloidosis result from (1) neuropathy secondary to deposition of the abnormal protein in the nerves, (2) vascular fragility and obstruction from amyloid deposition in the vessel wall, (3) functional impairment and structural distortion consequent to the mass effect of amyloid tumors, and (4) opacification of the vitreous.4-23

In Case 1 ocular symptoms are the earliest manifestation of the systemic disease. It is the first instance in which the vitreous opacities, seen to occur with the disease, are shown to be comprised of amyloid, and illustrates that these characteristic opacities may appear cellular as well as membranous and veil-like. Glaucoma is now documented in amyloidosis, though, in other cases, eye pain and blindness have occurred.59-60

Case 1.  —A 25-year-old single male stable groom, first noted dark spots, small flashing lights, cobweb-appearing opacities, and decreasing

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