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NEUROLOGY 1998;51:600-602
© 1998 American Academy of Neurology

Amyloid neuropathy simulating lower motor neuron disease

A. Quattrini, MD, R. Nemni, MD, B. Sferrazza, MD, G. Ricevuti, MD, G. Dell'Antonio, MD, A. Lazzerini, MD and S. Iannaccone, MD

From the Departments of Neurology (Drs. Quattrini, Nemni, Sferrazza, and Iannaccone), Pathology (Dr. Dell'Antonio), and Orthopedics (Dr. Lazzerini), San Raffaele Scientific Institute, University of Milan; and the Department of Clinical Medicine and Clinical Therapeutics (Dr. Ricevuti), IRCCS San Matteo, University of Pavia, Italy.

Address correspondence and reprint requests to Dr. Angelo Quattrini, Department of Neurology, San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milano, Italy.

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.

Supported by "Centro per la diagnosi e la terapia dei tumori cerebrali Margherita Magenes," and a grant from Telethon, Italy (no. 613, A.Q. and R.N.).

Received December 8, 1997. Accepted in final form March 11, 1998.




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