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Clinicopathologic Study of 8 Patients

Kevin J. Felice, DO; Charles H. Whitaker, MD; Margaret L. Grunnet, MD

Arch Neurol. 2003;60:1415-1420.

Background  The benign focal amyotrophy disorders have been described since 1959 for the upper limbs and since 1981 for the lower limbs. The clinicopathologic features have pointed to a restricted and self-limiting form of motor neuron disease.

Objective  To describe the clinical, electromyographic, and muscle histopathologic features in 8 patients with benign calf amyotrophy.

Design  Retrospective review of patient charts, electromyograms, and muscle histopathology.

Patients and Results  Eight patients, aged 37 to 88 years, developed insidiously progressive calf muscle weakness and wasting during 1 to 5 years. The gastrocnemius weakness and wasting were bilateral in 4 patients. Initial progression of symptoms was followed by disease stabilization. None had a history of poliomyelitis or family history of neuromuscular disease. Creatine kinase values were mildly elevated in 5 patients. The electromyographic and muscle histopathologic findings were consistent with a chronic neuropathic disorder. Despite the restricted calf muscle involvement clinically, the electromyographic abnormalities suggested more diffuse lower limb involvement. Further studies, including DNA tests and muscle-based protein studies, excluded several types of inherited neuromuscular disorders.

Conclusions  Benign calf amyotrophy is a variant of the benign focal amyotrophy disorders. The etiology for these disorders is unknown. Studies to exclude other causes of calf amyotrophy and careful follow-up examinations to document disease stabilization are necessary to diagnose this uncommon disorder.

From the Departments of Neurology (Drs Felice, Whitaker, and Grunnet) and Pathology (Neuropathology) (Dr Grunnet), University of Connecticut Health Center, University of Connecticut School of Medicine, Farmington.

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