No abstract available.
Huntington Disease*

No abstract available.
Huntington Disease*

Huntington's Disease (HD), an autosomal dominant disorder of mid-life onset, is characterized by progressive involuntary choreiform movement, psychological change and dementia. We present here two cases of Huntington's disease. One case has family history, but the other has none.
Chorea ; Dementia ; Humans ; Huntington Disease*

Huntington's disease (HD), an autosomal dominant dominant disorder of mid-life onset, is characterized by progressive involuntary choreiform movement, psychological change and dementia. We present here one case of Huntington's disease, who was admitted at Seoul National University Hospital from 2nd Oct. to 16 Oct. 1985.
Chorea ; Dementia ; Huntington Disease ; Seoul

Huntington's disease(HD) is a neurcdegenerative disorder caused by a highly penetrant autosomal dominant genetic defect. It is characterized by adult onset progressive chorea, psychological change, and dementia with relentless deterioration. Until now the reported cases are very rare in Korea, so we presented here two cases of definite HD.
Adult ; Chorea ; Dementia ; Humans ; Huntington Disease* ; Korea

OBJECTIVE: Gait disturbance is the main factor contributing to a negative impact on quality of life in patients with Huntington’s disease (HD). Understanding gait features in patients with HD is essential for planning a successful gait strategy. The aim of this study was to investigate temporospatial gait parameters in patients with HD compared with healthy controls. METHODS: We investigated 7 patients with HD. Diagnosis was confirmed by genetic analysis, and patients were evaluated with the Unified Huntington’s Disease Rating Scale (UHDRS). Gait features were assessed with a gait analyzer. We compared the results of patients with HD to those of 7 age- and sex-matched normal controls. RESULTS: Step length and stride length were decreased and base of support was increased in the HD group compared to the control group. In addition, coefficients of variability for step and stride length were increased in the HD group. The HD group showed slower walking velocity, an increased stance/swing phase in the gait cycle and a decreased proportion of single support time compared to the control group. Cadence did not differ significantly between groups. Among the UHDRS subscores, total motor score and total behavior score were positively correlated with step length, and total behavior score was positively correlated with walking velocity in patients with HD. CONCLUSION: Increased variability in step and stride length, slower walking velocity, increased stance phase, and decreased swing phase and single support time with preserved cadence suggest that HD gait patterns are slow, ataxic and ineffective. This study suggests that quantitative gait analysis is needed to assess gait problems in HD.
Diagnosis ; Gait* ; Humans ; Huntington Disease* ; Quality of Life ; Walking

Huntington's disease (HD) is a late-onset and progressive neurodegenerative disorder that is caused by aggregation of mutant huntingtin protein which contains expanded-polyglutamine. The molecular chaperones modulate the aggregation in early stage and known for the most potent protector of neurodegeneration in animal models of HD. Over the past decades, a number of studies have demonstrated molecular chaperones alleviate the pathogenic symptoms by polyQ-mediated toxicity. Moreover, chaperone-inducible drugs and anti-aggregation drugs have beneficial effects on symptoms of disease. Here, we focus on the function of molecular chaperone in animal models of HD, and review the recent therapeutic approaches to modulate expression and turn-over of molecular chaperone and to develop anti-aggregation drugs.
Huntington Disease* ; Models, Animal ; Molecular Chaperones ; Neurodegenerative Diseases

OBJECTIVES: Much interest has recently been focused on the possibility of the involvement of unstable DNA in the etiology of schizophrenia following several publications that reported increases in frequency of large CAG repeats in affected individuals. Tardive dyskinesia(TD), an involuntary movement disorder following pharmacological treatment of schizophrenia, shares a great deal of common clinical and biological features with Huntington's disease, a representative movement disorder with CAG repeat expansions. The authors studied for a possible CAG repeat expansions in patients with schizophrenia and TD. METHODS: TD was diagnosed by the Abnormal Involuntary Movement Scale. Using repeat expansion detection(RED), a method in which a thermostable ligase is used to detect repeat expansions directly from genomic DNA, subjects with schizophrenia with/without TD(n=79/n=75) and normal controls (n=72) were studied for the presence of the CAG repeat expansions were analyzed. RESULTS: No significant size differences were detected in the(CTG)17 ligation products between schizophrenic cases and controls using RED(X(2)=2.907, df=2, p=0.234). CONCLUSIONS: This finding does not support the hypothesis that CAG repeat expansions contributes to the susceptibility for schizophrenia and TD.
DNA ; Dyskinesias ; Humans ; Huntington Disease ; Ligation ; Movement Disorders* ; Schizophrenia

Acupuncture Therapy ; Female ; Humans ; Huntington Disease ; therapy ; Middle Aged

Cellular autophagy is a major degradative pathway for clearance of aggregate-prone proteins and damaged organelles. It plays an important role in regulating cellular homeostasis, cell growth and development, and disease development. Dysfunctional autophagy contributes to the pathology of various neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease and Huntington's disease, in which specific pathological protein accumulation occurs. A growing body of evidence suggests that resveratrol plays a significantly role in the regulation of autophagy and clearance of pathological proteins. Resveratrol is a potential drug for neurodegenerative diseases therapy. This review focuses on the effects of resveratrol on cellular autophagy and clinical application in the control of neurodegenerative diseases.
Alzheimer Disease ; Autophagy ; Humans ; Huntington Disease ; Neurodegenerative Diseases ; drug therapy ; Parkinson Disease ; Stilbenes ; pharmacology