Van Allen's syndrome
A hereditary form of amyloidosis involving both sexes originally reported in Iowa in a family of Scottish-English-Irish extraction. Onset of symptoms is usually observed in the third or fourth decade with average onset at age 35 years. Neuropathy dominates at onset, involving all four extremities. It is usually followed by muscle weakness and atrophy, diminished deep-tendon reflexes, dysenthesia, shooting pain, and other neurological disorders of the extremities, lower limbs being more severely affected than the upper ones. Also severe peptic ulcer disease. Frequently, hearing loss and blurred vision (cataracts but no vitreous opacities). Commonly, impotence and sphincter disturbances. Average survival is 12 years from the onset. Inheritance is autosomal dominant.
- M. W. van Allen, J. A. Frohlich, J. R. Davis:
Inherited predisposition to generalized amyloidosis. Clinical and pathological study of a family with nephropathy and peptic ulcer.
Neurology, Cleveland, Ohio, 1969, 19: 10-25.