Annals of Indian Academy of Neurology (Jan 2006)
A rare presentation of primary (AL) amyloidosis
Abstract
Primary amyloidosis is a multi-system disease, involving renal, cardiovascular, gastrointestinal, articular and nervous systems. Peripheral neuropathy occurs in 10-35% of patients and is the presenting feature in 10% of cases. Clinical involvement of the autonomic nervous system is well recognized, although uncommon. Most patients develop features of autonomic dysfunction including postural hypotension, impotence, gastrointestinal disturbances, impaired sweating and loss of bladder control. Nearly 25% develop a superimposed carpal tunnel syndrome caused by amyloid infiltration of the flexor retinaculum at the wrist. We report a case of a 53-year-old man, who presented with autonomic dysfunction, who on investigation was found to have systemic AL amyloidosis. The constellation of painful dysesthesia, autonomic dysfunction and a history of carpal tunnel syndrome should alert the physician of the possibility of amyloidosis.
