I Huntington. Huntington Huntington s disease HD HD HD. Huntington 1. HD polyq disease Qn. homozygotes heterozygotes. HD Huntington.

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1 Huntington 5 Huntington * ** Key Words : Huntington disease, polyglutamine disease Huntington Huntington s disease HD HD Huntington HD Huntington HD 1872 George Huntington 1 HD HD Huntingtin HTTCAG HD I Huntington , /10 Fig HD HD HD 5 II Huntingtin HTT HTT I CAG interme diate allele HD 40 HDCAG Q HD polyq disease HTT Qn Qn HD Qn homozygotes heterozygotes Qn anticipation Qn Qn Huntington s Disease Epidemiology, Diagnosis, Treatment. Kazuko HASEGAWA : National Hospital Organization, Sagamihara National Hospital Vol. 32 No

2 Huntington HD Fig. 1 Prevalence of Huntington s disease in Japan huntingtin Huntingtin Huntingtin post translational modification huntingtin III Table 1 3, 4 1. HD HDTable 1 Fig HD motor impersistance initial saccade ocular motor apraxia grimacing 3. 4 HD Vol. 32 No

3 Huntington Table 1 Motor & psychic symptoms in Huntington s disease HD Shannon Fig. 2 Clinical progression of Huntington s disease HD Vol. 32 No

4 Huntington HD 3 7 HD HD HD HD HD 4, 5 6, 7 4. Huntington HD HD westphal variant HD Choreopathy /3 2 3 IV MMSE FAB Vol. 32 No

5 Huntington V 1. tetrabenazine 8 tetrabenazine 9, 10 tetrabenazine sulpiride tiapride tetrabenazine reserpine benzodiazepine tetrabenazine clonazepam tetrabenazine amantadine 11, selective serotonin re uptake inhibitors SSRI benzodiazepine carbamazepine lamotrigine topiramate 14 SSRI SSRI clomipramine serotonin norepinephrine re uptake inhibi tors SNRIbenzodiazepine HD HD VI 15 VII Wilson neuronal ceroid lipo fucinosis DRPLA SCA17 SLE Schonlein Henoch HD ips HD HD 16 COI Vol. 32 No

6 Huntington 1 Michael R Hayden : Huntington s Chorea. Springer Verlag, Berlin, Heidelberg, New York, Huntington s Disease Collaborative Research Group : A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntingotn s disease chromosomes. The Hunting ton s Disease Collaborative Research Group. Cell 72 : , Shannon KM : Huntington s disease clinical signs, symptoms, presymptomatic diagnosis, and diagnosis. Handbook of Clini cal Neurology Weinerm WJ, Tolosa E ed, Vol 100 Chap 1, Elsevier science publishers, Anderson KE : Huntington s disease. Handbook of Clinical Neurology Weinerm WJ, Tolosa E ed, Vol 100 Chap 2, Elsevi er science publishers, Mohr E, Brouwers P, Claus JJ et al : Visuospatial cognition in Huntington s disease. Mov Disord 6 : , Stout JC, Rodawalt WC, Siemers ER : Risky decision making in Huntington s disease. J Int Neuropsychol Soc 7 : , Ribai P, Nguyen K, Hahn Barma V et al : Psychiatric and cog nitive difficulties as indicators of Juvenile Huntington disease onset in 29 patients. Arch Neurol 64 : , Jankovic J : Treatment of hyperkinetic movement disorders. Lancet Neurol 18 : , Huntington study group : A randomized, double blind, place bo controlled trial of tetrabenazine as antichorea therapy in Huntington s disease. Neurology 66 : , Burgunder JM, Guttman M, Periman S et al : An international survey based algorithm for the pharmacologic treatment of chorea in Huntington s disease. PLos Curr 2011 Aug 30;3 RRN1260. Doi: /currents.RRN O Suilleabhain P, Dewey RB : A randomized trial of amanta dine in Huntington s disease. Arch Neurol 60 : , Lucetti C, Del Dotto P, Gambaccini G et al : IV amantadine im proved chorea in Huntington s disease. An acute randomized, controlled study. Neurology 60 : , Groves M, van Duijin E, Anderson K : An international survey based algorithm for the pharmacologic treatment of irritabili ty in Huntington s disease. PLoS Curr 2011 Aug 30:RRN1259. doi; /currents.rrn Anderson K, Craufurd D, Edmondson MC et al : An interna tional survey based algorithm for the pharmacologic treat ment of obsessive compulsive behaviors in Huntington s dis ease. PLos Curr.2011 Sept 20;3 RRN1261. Doi: currents.rrn www.nanbyou.or.jp/ Vol. 32 No