W baclofen dystonia 1 Parkinson 10 5% botulinum toxin ; BTX deep brain stimulation ; DBS BTX DBS BTX DBS Oppenheim 1911 dystonia m

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せせらおおばま
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1 W baclofen dystonia 1 Parkinson 10 5% botulinum toxin ; BTX deep brain stimulation ; DBS BTX DBS BTX DBS Oppenheim 1911 dystonia musculorum deformans DMD DYT Bell Duchenne Faradic treatment 1960 DMD Laitinen Parkinson globus pallidus interna ; GPi Parkinson dyskinesia dystonia 1990 GPi 15

2 DYT1 generalized dystonia Non-DYT1 generalized dystonia Paroxysmal dystonia & dyskinesias Dopa-responsive dystonia Myoclonic dystonia Rapid-onset dystonia-parkinsonism X-linked dystonia-parkinsonism Blephalospasm Cervical dystonia Embouchure dystonia Oromandibular dystonia Laryngeal dystonia Hand dystonia writer s cramp Medications, trauma, toxins, infections Stroke Perinatal cerebral injury Viral encephalitis SSPE AIDS Creutzfeldt-Jakob disease Kernicterus Huntington s disease Parkinson s disease Spinocerebellar ataxias HARP syndrome Familial frontotemporal dementias Familial basal ganglia calcifications Wilson s disease Juvenile parkinsonism Ataxia-telangiectasia Triosephosphate isomerase def Vitamin E deficiency Biopterin deficiency Sphingolipidoses Niemann-Pick disease Ceroid lipofuscinosis Homocystinuria Hartnup disease Methylmalonic aciduria Tyrosinaemia Lesch-Nyhan syndrome Rett s syndrome Pelizaeus-Merzbacher disease Dystonia-deafness syndrome MERRF MELAS Leber s disease Leigh s syndrome Neuroacanthocytosis Intranuclear inclusion disease Haemochromatosis Progressive supranuclear palsy Multiple system atrophy Corticobasal degeneration Dentatorubropalidoluysian atrophy Glutaric academia Methylmalonic academia Homocystinuria Metachromatic leukodystrophy Neuronal ceroid lipofuscinosis Primary antiphospholipid antobidy Gangliosidoses Hallervorden-Spatz disease Multiple sclerosis Atlantoaxial sublaxation GPi pallidotomy DBS 1970 Benabid DBS 1 GPi pallidotomy Meige tardive dystonia GPi DBS DYT1 DYT1 GPi DBS 2 Meige pantothenate kinase-associated neurodegeneration PKAN Hallervorden-Spatz 1 Lesch-Nyhan 2 GPi DBS GPi DBS 16

3 W BTX BTX SPD BTX 3 4 SPD W W C1-C6 3 C3-4 C5-6 C1-3 C4 C1-7 2 cm

4 W MR LR ml SPD W GPi DBS Meige GPi DBS Vo cortico-basal ganglia-thalamic loop Vo MRI CT Parkinson Vo 18

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6 DBS ITB DBS 18 DBS 17 fixed dystonia 19 Parkinson 1 Timmermann L, Pauls KA, Wieland K, et al. Dystonia in neurodegeneration with brain iron accumulation :outcome of bilateral pallidal stimulation. Brain 2010 ;133 : Taira T, Kobayashi T, Hori T. Disappearance of self-mutilating behavior in a patient with Lesch-Nyhan syndrome after bilateral chronic stimulation of the globus pallidus internus. Case report. J Neurosurg 2003 ;98 : Albanese A, Barnes MP, Bhatia KP, et al. A systematic review on the diagnosis and treatment of primary idiopathic dystonia and dystonia plus syndromes :report of an EFNS/MDS-ES Task Force. Eur J Neurol 2006 ;13 : Taira T. Peripheral procedures for cervical dystonia. In :Lozano A, Gildenberg P, Tasker R, eds. Textbook of stereotactic and functional neurosurgery. Berlin :Springer, 2009 : Ostrem JL, Marks WJ Jr, Volz MM, Heath SL, Starr PA. Pallidal deep brain stimulation in patients with cranial-cervical dystonia Meige syndrome. Mov Disord 2007 ;22 : Taira T, Kobayashi T, Hori T. Selective peripheral denervation of the levator scapulae muscle for laterocollic cervical dystonia. J Clin Neurosci 2003 ;10 : Taira T, Hori T. Peripheral neurotomy for torticollis :a new approach. Stereotact Funct Neurosurg 2001 ;77 : Taira T, Kobayashi T, Takahashi K, Hori T. A new denervation procedure for idiopathic cervical dystonia. J Neurosurg 2002 ;97 suppl 2 : Sheehy MP, Marsden CD. Writers cramp a focal dystonia. Brain 1982 ;105 : Goto S, Tsuiki H, Soyama N, et al. Stereotactic selective Vocomplex thalamotomy in a patient with dystonic writer s cramp. Neurology 1997 ;49 : Mempel E, Kucinski L, Witkiewicz B. Writer s cramp syndrome treated successfully by thalamotomy. Neurol Neurochir Pol 1986 ; 20 : Taira T, Ochiai T, Goto S, Hori T. Multimodal neurosurgical strategies for the management of dystonias. Acta Neurochir Suppl 2006 ; 99 : Shibata T, Hirashima Y, Ikeda H, Asahi T, Hayashi N, Endo S. Stereotactic Voa-Vop complex thalamotomy for writer s cramp. Eur Neurol 2005 ;53 : Taira T, Hori T. Stereotactic ventrooralis thalamotomy for taskspecific focal hand dystonia writer s cramp. Stereotact Funct Neurosurg 2003 ;80 : Taira T, Harashima S, Hori T. Neurosurgical treatment for writer s cramp. Acta Neurochir Suppl 2003 ;87 : Taira T, Hori T. Intrathecal baclofen in the treatment of poststroke central pain, dystonia, and persistent vegetative state. Acta Neurochir Suppl 2007 ;97 : ;36 : Albright AL, Barry MJ, Shafton DH, Ferson SS. Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol 2001 ;43 : van Rijn MA, Munts AG, Marinus J, et al. Intrathecal baclofen for dystonia of complex regional pain syndrome. Pain 2009 ;143 :41-7. Update on Multimodal Neurosurgical Management of Dystonias Takaomi Taira, MD Department of Neurosurgery, Tokyo Women s Medical University, Tokyo The neurosurgical treatment of dystonia has progressed markedly since the introduction of deep brain stimulation of the globus pallidum interna. However, dystonia is not a single disorder but comprises various types and causes, and it is true that deep brain stimulation cannot cover the complex nature of dystonia. Depending on the distribution of symptoms and causes, we have to consider other surgical managements such as thalamotomy, peripheral denervation, and intrathecal baclofen. Such a multi-modal strategy has enabled us to treat and even cure many patients with dystonias. No treatment other than various neurosurgical approaches yields better results in the management of dystonias. In this sense, we are now at a stage where we should regard dystonia as a neurosurgical disorder in terms of treatment. No To Hattatsu 2011 ;43 :

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et al No To Shinkei Clin Neurol Neurology et al J Neurosurg et al Arch Neurol et al Angiology 37 Recommendations for the Management of Moyamoya Disease A Statement from Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) Research on intractable diseases of the