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H. TSUKAGOSHI, MD; T. NAKANISHI, MD; K. KONDO, MD; T. TSUBAKI, MD

Arch Neurol. 1965;12(6):597-603.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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.

These . . . [Full Text PDF of this Article]

Author Affiliations
TOKYO

From the Third Department of Internal Medicine and the Institute of Brain Research, Faculty of Medicine, University of Tokyo.

Footnotes
Submitted for publication Sept 4, 1964; accepted Nov 30.

Reprint requests to University of Tokyo, Hongo, Tokyo (Dr. Tsukagoshi).

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