Amyloidosis primary

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Genetic testing









Fig. 8. Peripheral nerve amyloidosis. The biopsy shows a congo red stained section with evidence of apple green birefringence in amyloid deposits within endoneurial vessels

Primary amyloidosis (AL) is a multi-organ systemic disease affecting the peripheral and autonomic nervous systems. Axonal degeneration, particularly of small myelinated and unmyelinated fibers is present with diffuse amyloid deposits infiltrating epineurial and endoneurial connective tissue.


Initial neuropathic symptoms are most commonly burning pain and loss of sensation in the feet. These symptoms may precede development of multiorgan involvement by 1 year. With disease progression, patients experience distal muscle weakness and in advance cases autonomic symptoms of postural hypotension, syncope and impotence.


AL is a disorder of older men. Approximately 70% of affected patients are men with a median age of 65 who experience weight loss, hepatomegaly, macro-glossia, purpura and ankle edema. Early in the disease examination reveals a stocking/glove loss of all sensory modalities and depressed ankle reflexes. Approximately 25% of patients will have signs of a median mononeuropathy with paresthesias in the first 3 fingers with variable weakness of thenar muscles. As AL progresses, distal weakness, absent reflexes and autonomic signs are present, including orthostatic hypotension and abnormal sweating.

Clinical syndrome/ signs

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