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April 1966

Kugelberg-Welander Syndrome With Dominant Inheritance

Author Affiliations

From University of Tokyo, Faculty of Medicine, Institute of Brain Research (Drs. Tsukagoshi and Sugita) and Third Department of Internal Medicine (Dr. Furukawa); University of Niigata, Institute of Brain Research (Dr. Tsubaki); and University of Keio, Faculty of Medicine, Department of Psychiatry (Dr. Ono).

Arch Neurol. 1966;14(4):378-381. doi:10.1001/archneur.1966.00470100034004

Many CASES have been reported since Kugelberg and Welander1,2 in 1954 and 1956 and Wohlfart et al3 in 1955 described "hereditary proximal neurogenic muscular atrophy simulating muscular dystrophy" as a clinical entity. Most of these cases were transmitted as an autosomal recessive trait. A family with dominant inheritance is reported in this paper.

Description of the Pedigree  Family A descended from II-6 in Fig 1. In four generations eight members are affected, five males and three females. As indicated in the following description, nine members of the family were examined; information about the others was provided by surviving individuals.CASE II-6.—This patient was not examined. He was unable to work at age 40 years because of muscular atrophy of the four extremities and lordotic gait. He died at the age of 65. His parents and six siblings were said to have been healthy.CASE III-2.—This patient was born