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HUNTINGTON'S CHOREA IN A CHILD : REPORT OF AN AUTOPSY CASE Maekawa, K. 1 , Nakajima, M. 2 , Nihei, K. 3 , Kobayashi, M. 4 , Yoshikura, N. 5 1Pediatrics, Ohkura National Hospital 2Pediatrics, Sagamihara National Hospital 3Pediatrics, Kanto-Teishin Hospital 42nd Dept. of Pathology, Nihon University School of Medicine 5Divison of Neurology, Nihon University Surugadai Hospital pp.699-704
Published Date 1974/7/1
DOI https://doi.org/10.11477/mf.1406203570
  • Abstract
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A 12-year-old Japanese girl was admitted to our hospital with the complaints of muscle rigidity, clumsy rigid gait, akinetic seizure, slurred speech and mental retardation. Her brother, 17 years old, and mother, 45 years old, are healthy, but father has been suffering from Huntington's chorea. The pa-tient was born at full term of uncomplicated labor following a normal pregnancy, weighing 2850 gm at birth. Despite the head trauma got at the age of 8 months, her growth and development were normal, and had been in good health until the age of 6 years, when she was noted to be clumsy and to stumble frequently. At the age of 7 years, she entered primary school. During the following 3 years her school performance became gradually worse and she was transferred to a special class at the age of 9 years.

Since then she had had generalized convulsions 2 to 3 times a month in spite of anticonvulsive medi-cation. She was transferred to our hospital for further evaluation at the age of 12 years.

Pneumoencephalogram revealed an atrophic head of the caudate nucleus. Bilateral 3-5 c/s spike & wave complex were found on EEG examination. During the following one year, she often fell into prolonged generalized convulsions, and muscle rigidity, mental deterioration and motor disabilites became more marked. She died with bron-chopneumonia at the age of 13 years and one month old.

The brain weighed 960 gm. Convolutions ap-pared somewhat atrophic. The ventricular system mildly dilated and the caudate nuclei were reduced in size. Changes and loss of nerve cells in the putamen and caudate nucleus, especially of smaller cells ; conspicuous change and reduction of number of neurons with marked gliosis in the Sommer sector ; and severe degeneration and loss of nerve cells in the cerebral cortex, especially in layers 3 to 5 of the parietal and occipital lobes, charact-erize the pathological features which roughly tally with the symptomatology of the case.


Copyright © 1974, 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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