• Dale J Lange, Rajwinder Nijjar, Andrei Voustianiouk, Gregory Seidel, Janki Panchal, and Annabel K Wang.
• Department of Neurology, Division of Neuromuscular Disease, Mount Sinai School of Medicine, New York, New York, USA. langed@hss.edu
• Muscle Nerve. 2011 Apr 1;43(4):537-42.

IntroductionAre there electrophysiological findings that predict response to intravenous immunoglobulin (IVIg) in patients with lower motor neuron (LMN) syndromes without multifocal conduction block (MCB)?MethodsWe enrolled 9 patients with LMN syndromes without MCB to receive 18 weeks of IVIg therapy. Response was measured at weeks 2 and 18 using the Appel Amyotrophic Lateral Sclerosis (AALS) score (includes grip and pincer strength measures), ALS Functional Rating Scale (ALSFRS), and electrophysiological measures, including motor unit estimates (MUNEs).ResultsNo change occurred in AALS or ALSFRS scores posttreatment. Grip/pincer strength increased in 7 patients (P = 0.028) after initial treatment (responders); 2 showed no improvement (non-responders). No electrophysiological measure changed after treatment in either group but MUNEs trended higher (P = 0.055). "Abnormal A-waves" (complex, repetitive biphasic, or present in multiple nerves) occurred in pretreatment studies more often in responders (P = 0.028).Discussion"Abnormal A-waves" may signal IVIg-responsive LMN syndromes even if conduction block is absent.Copyright © 2011 Wiley Periodicals, Inc.

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