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Accession number;00A0952830
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| Title;A Case of Huntington's Disease Which Was Difficult to Diagnose and Showed both Obsession-Compulsion and Severe Aggression. |
| Author;
KAWAMURA KOSUKE
(Kurume Univ.)
OTA TAKUO
(Kokuritsuhizenryoyosho Jodokodoshogaise)
MAEDA HISAO
(Kurume Univ.)
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Journal Title;Kyushu Neuropsychiatry
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Journal Code:Z0180B
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ISSN:0023-6144
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VOL.46;NO.2;PAGE.84-92(2000)
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| Figure&Table&Reference;FIG.1, REF.39 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;A clinically complex case of Huntington's disease (HD) with obsession-compulsion and severe aggression was examined. The patient was originally diagnosed to have schizophrenia at the time of onset, and later was also determined to have dentato-rubro-pallido-luysian atrophy (DRPLA) at the first admission. However, a genetic analysis revealed the patient to have HD. In the present case, treatment with bromazepam proved to be effective for controlling obsession-compulsion while the administration of sodium valproate (VPA) significantly improved the patient's aggression. The hypothesis that the mutant protein "huntingtin" encoded by the HD gene leads to a delayed but progressive selective neuronal death has been proposed at the striatum level, which is the major site of HD lesions. Furthermore, the recent introduction of neuronal transplantaion using brain stem cells and striatal tissue, in addition to improvements in the methodology to reduce polyglutamete-induced cytotoxicity when using transglutaminase inhibitors, caspase inhibitors and neurotrophic factors, has provided a new perspective for the treatment of neurodegenerative diseases. In this paper, the author also reviewed the literature regarding the pharmacotherapy of psychiatric symptoms while also analyzing various clinical perspectives in light of the involvement of a genetic abnormality at the onset of the disease based on the recent findings of HD in molecular biology as a triplet repeat disease. (author abst.) |
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