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A 59-year-old Woman with Personality Change and Abnormal Behavior Followed by Amyotrophy and Dementia Jiro Fukae 1 , Yumiko Motoi 1 , Yasuko Komatsuzaki 2 , Masashi Takanashi 1 , Hideki Mochizuki 1 , Yoshikuni Mizuno 1 1Department of Neurology, Juntendo University School of Medicine 2Department of Neurology, Hatsuishi Hospital Keyword: personality change , abnormal behavior , dementia , amyotrophy , ALS with dementia (ALS-D) , ALS of Mitsuyama type , Pick disease pp.810-820
Published Date 2005/9/1
DOI https://doi.org/10.11477/mf.1406100081
  • Abstract
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We report a 59-year-old woman with generalized amyotrophy and dementia. She showed personality change at 53 years of age. When she was 56 years old, she began to show abnormal and violent behaviors. At age 58, she developed dysphagia and amyotrophy of upper limbs. She was admitted to a hospital for the treatment of aspiration pneumonia. She was severely demented and showed pseudobulbar palsy, amyotrophy of tongues, weakness of upper limbs, and pyramidal signs. She was still able to walk by herself. Dementia, pseudobulbar palsy, and amyotrophy progressed rapidly. At age 59, she became bed ridden and required tube feeding. She died by aspiration pneumonia at age 59. The patient was discussed at a neurological CPC and the chief discussant arrived at the conclusion that the patient had ALS dementia. Other possibility discussed was Pick's disease with amyotrophy. Post-mortem examination revealed severe lower motor neuron degeneration. The upper motor neurons were unaffected. Neuronal loss was not observed in the cerebral cortex, but moderate gliosis was seen in the cerebral white matter. In addition, the substantia nigra was moderately degenerated. There were ubiquitin positive neuronal inclusions in the granular cells of the dentate gyrus. Also, Bunina bodies were seen in the neurons of spinal anterior horns. These findings were characteristic pathology for ALS with dementia.


Copyright © 2005, 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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