Amyloid neuropathy simulating lower motor neuron disease.
We report a 57-year-old man with progressive symmetric weakness and fasciculation affecting the legs. Electromyography revealed fibrillations and neurogenic motor unit potentials in the leg muscles. Biopsy of a motor branch of the obturator nerve revealed axonal degeneration, loss of myelinated nerve fibers, and amyloidosis with deposits of lambda light chains. At 6-month follow-up, the patient manifested sensory and autonomic symptoms, and lambda light chains were first detected in the serum. In this case, diagnosis of amyloidosis remained elusive until motor nerve biopsy.
Quattrini
A
A
Department of Neurology, San Raffaele Scientific Institute, University of Milan, Italy.
Nemni
R
R
Sferrazza
B
B
Ricevuti
G
G
Dell'Antonio
G
G
Lazzerini
A
A
Iannaccone
S
S
D017772:Amyloid Neuropathies / Q000175:diagnosis* / Q000503:physiopathology; D001706:Biopsy; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology