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January 1969

Scapuloperoneal Amytrophy

Arch Neurol. 1969;20(1):9-12. doi:10.1001/archneur.1969.00480070019002

DAVIDENKOW was the first to describe in detail the syndrome of scapuloperoneal amyotrophy which he considered to be a variant of Charcot-Marie-Tooth disease. He described patients with distal weakness in the lower limbs and peripheral sensory abnormalities associated with proximal weakness in the upper limbs affecting predominantly the scapular muscles. Electrodiagnostic facilities were not available at that time and motor nerve conduction velocities were, therefore, not measured.

More recently, Kaeser2 described a large family with dominantly inherited weakness of similar distribution, in whom sensory signs were absent and motor nerve conduction velocities were normal. Autopsy in one such case, in which bulbar involvement was present, revealed degeneration of motor nerve nuclei, thus establishing a diagnosis of bulbospinal muscular atrophy.

A diagnosis of spinal muscular atrophy seems inappropriate in Davidenkow's cases, in whom the typical distal sensory abnormalities suggest a relationship with Charcot-Marie-Tooth disease. These differences led Kaeser to