Neurogenic skeletal myopathy in patients with primary cardiomyopathy.

Abstract

Eleven patients with hypertrophic obstructive cardiomyopathy and eight patients with idiopathic congestive cardiomyopathy underwent extensive neuromuscular studies to determine if a skeletal myopathy is associated with uncomplicated primary cardiomyopathy. The clinical examination revealed peripheral neuropathies in six patients, but no evidence of muscle weakness or atrophy. Nerve conduction studies demonstrated a neuropathy in five of these six and in one other patient: three were in the hypertrophic group and three in the congestive group. Seven patients had abnormal electromyography, but none had characteristic myopathic changes. Of these seven patients, muscle biopsies showed denervation in two patients in the congestive group and type II atrophy in two patients in the hypertrophic group. We found no evidence of primary skeletal muscle involvement; however, neuropathic features and biopsy changes of denervation were present in both groups.

Hypergonadotropic hypogonadism with congestive cardiomyopathy: An autosomal‐recessive disorder?

“…Congestive cardiomyopathy per se rarely is familial. The familial forms are associated with various myopathic or neurogenic conditions [Darsee et al, 1979;Chakrabarti and Pearce, 19811. Najjar et a1 [1973, 19841 reported a condition with genital anomaly, mental retardation, and cardiomyopathy in five male children of two sibships.…”