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Hemimasticatory Spasm Associated With Localized Scleroderma and Facial Hemiatrophy
Ho Jin Kim, MD;
Beom S. Jeon, MD, PhD;
Kwang-Woo Lee, MD, PhD
Arch Neurol. 2000;57:576-580.
Objectives To report a case and discuss the mechanism of hemimasticatory spasm.
Design Case report.
Patient A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms.
Conclusion On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.
From the Department of Neurology, College of Medicine, Seoul National University, Seoul, Korea.
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