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A CASE OF WOHLFART-KUGELBERG-WELANDER SYNDROME Hiroshi SHIBASAKI 1 , Shukuro ARAKI 1 1Department of Neurology, Kyushu University Faculty of Medicine pp.697-703
Published Date 1967/7/1
DOI https://doi.org/10.11477/mf.1406202243
  • Abstract
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We reported a case, 18-year-old Japanese male, whose onset began with muscular atrophy of the thighs and gait disturbance at the age of 4 years. The clinical course has been slowly progressive, and the neurological examination on admission revealed diffuse muscular atrophy with more involvement of the proximal portion than distal, fasciculations, decreased or absent deep reflexes, bilateral pes equinovarus, posi-tive Gowers' sign and waddling gait. The muscular atrophy was proven to be neurogenic by electromyo-graphy (EMG) and muscle biopsy. This case differs from the original cases reported by Kugelberg and Welander in that the muscular atrophy is distributed almost diffusely, flexors and extensors are involved in the same degree, gluteus and triceps muscles show low amplitude NMU potentials on EMG. Chromosome study revealed no abnormality. To our knowledge, this case seems to be the first case with Kugelberg-Welander syndrome in which chromosome study was done.

His elder brother, 23 years of age, has also been suffering from the same kind of muscular atrophy since the age of 4 years, reportedly.

We reviewed 26 cases from the literature in this country with special regards to the age of onset, du-ration, type of heredity and so forth. Further we dis-cussed about the differential diagnosis of Wohlfart-Kugelberg-Welander syndrome from Werdnig-Hoff-mann disease, particularly its benign form.


Copyright © 1967, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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