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August 1951

CLINICAL AND PATHOLOGICAL FINDINGS IN A CASE OF DYSTROPHIA MYOTONICA

Author Affiliations

AMSTERDAM, NETHERLANDS

From the Neurological Department of the Wilhelminagasthuis, University of Amsterdam; director, Prof. Dr. A. Biemond, and the Psychiatrische Inrichting at Franeker; director, Dr. J. C. van Andel.

AMA Arch NeurPsych. 1951;66(2):178-190. doi:10.1001/archneurpsyc.1951.02320080062006
Abstract

IN 1944 I1 described a typical case of dystrophia myotonica in a man aged 44. Death occurred in 1948, and autopsy was performed. An anatomical examination was made of the endocrine glands, the muscles and the central and peripheral nervous systems.

CLINICAL FINDINGS  The patient was extremely bald; the left eye was artificial, and the right eye revealed the remains of iridectomy and an operation for cataract. He presented the characteristic aspect of the facies myopathica, and the general appearance was that of typical Jammergestalt. Speech was unintelligible; the thyroid gland was not palpable. There was fairly general adiposity. The left testis was very small; the right testis was still smaller, being about the size of a marble. Both were soft. There was male distribution of hair. The extremities were thin and showed atrophy of the muscles. The left leg was thinner than the right. The calves and the

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