While many patients with amyloid myopathy present with pseudo-muscular hypertrophy, some do not.
The patient is usually middle aged or elderly.
Clinical features:
(1) proximal muscle weakness (unable to hold up head, unable to raise the arms, unable to climb stairs, unable to rise from sitting)
(2) muscular atrophy
(3) absence of macroglossia
(4) variable neuropathy
(5) variable cardiac disease
(6) variable claudication
(7) variable renal disease
Laboratory findings:
(1) monoclonal protein or serum light chain
(2) amyloid on skeletal muscle biopsy
The diagnosis may be easily missed if the only findings are the muscle weakness and atrophy. A patient with these findings but no explanation should have serum testing for monocloncal progeins.
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