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Lubarsch-pick syndrome (primary amyloidosis; idiopathic amyloidosis; amyloidosis)


Lubarsch-Pick Syndrome (Primary Amyloidosis; Idiopathic Amyloidosis; Amyloidosis)

General: Rare condition of unknown etiology; inherited as a dominant trait, with male preponderance; c 333e49d haracterized by amyloid accumulation in muscles and in gastrointestinal and genitourinary tracts.

Ocular: Internal and external ophthalmoplegia; diminished lacrimation; amyloid deposits in conjunctival, episcleral, and ciliary vessels; vitreous opacities; amyloid deposits in the corneal stroma; retinal hemorrhages and perivascular exudates; paralysis of extraocular muscles; pseudopodia lentis; strabismus fixus convergens; keratoconus.

Clinical: Peripheral neuropathy (extremities); heart failure; defective hepatic and renal functions with hepatosplenomegaly; waxy skin lesions; muscular weakness (progressive); multiple myeloma; hoarseness; chronic gastrointestinal symptoms.

Biswas J, et al. Primary nonfamilial amyloidosis of the vitreous. A light microscopic and ultrastructural study. Retina 1992; 12:251-253.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Goebel MH, Friedman AM. Extraocular muscle in idiopathic primary amyloidosis. Am J Ophthalmol 1971; 71:1121.

Lubarsch O. Zur Kenntnis Ungewohnlicher Amyloidablagerungen. Virchows Arch Pathol Anat 1929; 271:867.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Sharma P, et al. Strabismus fixus convergens secondary to amyloidosis. J Pediatr Ophthalmol Strabismus 1991; 28: 2367.

Wong VG, McFarlin DE. A case of primary familial amyloidosis. Arch Ophthalmol 1967; 78:208.




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