Primary Systemic Amyloidosis

Secondary Glaucoma Accompanied with Primary Familial Amyloidosis

“…Rukavina et al [13] reported that the signs of ocular involvement were present in 8% of his cases with systemic primary fa milial amyloidosis. Comparing with this figure, the incidences of ocular symptoms indicated 60% of our systemic primary fa milial amyloidosis.…”

“…The former is less common and has three forms according to the main organ of amyloid deposition; namely, the cardiopathy, nephropathy, and neuropathy type [11]. Moreover, the neuro pathy type consists of a further, three types, namely, Rukavina [13], Andrade [12], and Van Allen type [14] following the classi fication of the international symposium on primary familial amyloidosis [3], The symp toms and signs in our patients reported here are in accordance with those generally des cribed in patients of the Andrade type which has equal sex distribution, the age of onset being between 25 and 35 years with progres sion to death, peripheral neuropathy having onset in the lower extremities, with impair ment of thermal and painful sensation, auto nomic disturbances following with impotence in the male, and the inheritance is autosomal dominant [12].…”

“…Since this symptom was first reported by Kantarjian and Dejong [16] in 1953, Kauf man and Thomas [17], Rukavina et al [13], Talon and Duke [5], and Falls [18] have all extensively discussed this subject. Generally speaking, vitreous opacities were thought to be uncommon in the amyloid neuropathy of the Andrade type according to the international amyloid symposium [3], However, this symptom is not rare in our cases similar to the Andrade type and one of the main causes of loss of visual acuity.…”

Selective Massive Amyloidosis of Small Intestine

“…Amyloidosis is classically divided into the primary, sec¬ ondary, tumorous, or localized form, due to multiple mye¬ loma, and rare types like the hereditary type of familial Mediterranean fever, of Andrade7 and Rukavina et al 8 Heller et al3 classified the amyloidotic cases into perireticulin and pericollagenous types, according to the topo¬ graphic situation of the amyloid in the wall of the blood vessels. Localized amyloidosis was categorized as peri¬ collagenous.…”

Primary Familial Amyloidosis

“…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) 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.\p=m-\A 25-year-old single male stable groom, first noted dark spots, small flashing lights, cobweb-appearing opacities, and decreasing vision in the right eye in February 1954, but had no pain or photophobia at this time.…”