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June 1965

Hereditary Proximal Neurogenic Muscular Atrophy in Adult

Arch Neurol. 1965;12(6):597-603. doi:10.1001/archneur.1965.00460300045005

IN 1955 Wohlfart et al1 separated hereditary proximal muscular atrophy from muscular dystrophy as a new clinical entity. Kugelberg and Welander2 in 1956 gave a detailed clinical description of this syndrome, based on 12 patients, which might be summarized as follows: (1) onset of the disease from childhood to adolescence, (2) proximal muscular atrophy resembling muscular dystrophy, (3) fasciculations, (4) absence of the syndrome of bulbar palsy, (5) apparently non-sex-linked recessive form of inheritance, (6) very slow progression, and (7) neurogenic nature of the disorder according to electromyography and muscle biopsy.

Similar cases have been reported by Magee and Dejong,3 and Hausmanowa-Petrusewicz et al.4

We have recently seen five patients with muscular atrophy resembling this syndrome, except for the following two points: (1) onset of the disorder in adults; and (2) presence of the syndrome of bulbar palsy in three of the five cases.


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