The Neurophysiology of Dystonia | Genetics and Genomics | JAMA Neurology | JAMA Network
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1.
Byl  NMerzenich  MMJenkins  WM A primate genesis model of focal dystonia and repetitive strain injury, I: learning-induced dedifferentiation of the representation of the hand in the primary somatosensory cortex in adult monkeys.  Neurology. 1996;47508- 520Google ScholarCrossref
2.
Chuke  JCBaker  RSPorter  JD Bell's palsy–associated blepharospasm relieved by aiding eyelid closure.  Ann Neurol. 1996;39263- 268Google ScholarCrossref
3.
Rothwell  JCObeso  JADay  BLMarsden  CD Pathophysiology of dystonias.  Adv Neurol. 1983;39851- 863Google Scholar
4.
Ceballos-Baumann  AOPassingham  REWarner  TPlayford  EDMarsden  CDBrooks  DJ Overactive prefrontal and underactive motor cortical areas in idiopathic dystonia.  Ann Neurol. 1995;37363- 372Google ScholarCrossref
5.
Ibañez  VSadato  NKarp  BDieber  M-PHallett  M Investigation of cortical activity in writer's cramp patients: a PET study [abstract].  Neurology. 1996;46 (suppl 2) A260Google Scholar
6.
Ikoma  KSamii  AMercuri  BWassermann  EMHallett  M Abnormal cortical motor excitability in dystonia.  Neurology. 1996;461371- 1376Google ScholarCrossref
7.
Ridding  MCSheean  GRothwell  JCInzelberg  RKujirai  T Changes in the balance between motor cortical excitation and inhibition in focal, task specific dystonia.  J Neurol Neurosurg Psychiatry. 1995;59493- 498Google ScholarCrossref
8.
Chen  RWassermann  ECaños  MHallett  M Impaired inhibition in writer's cramp during voluntary muscle activation.  Neurology. 1997;491054- 1059Google ScholarCrossref
9.
Matsumura  MSawaguchi  TOishi  TUeki  KKubota  K Behavioral deficits induced by local injection of bicuculline and muscimol into the primate motor and premotor cortex.  J Neurophysiol. 1991;651542- 1553Google Scholar
10.
Hallett  M Is dystonia a sensory disorder?  Ann Neurol. 1995;38139- 140Google ScholarCrossref
11.
Hallett  M Physiology of dystonia.  Adv Neurol. In press.Google Scholar
Neurological Review
May 1998

The Neurophysiology of Dystonia

Author Affiliations

From the Office of the Clinical Director, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Md.

Arch Neurol. 1998;55(5):601-603. doi:10.1001/archneur.55.5.601
Abstract

Any model for the physiology of dystonia must be able to explain how dystonia can be produced in various circumstances. Brain lesions can cause dystonia; responsible sites include the basal ganglia, brainstem, and thalamus, but the most common site is the putamen. Dystonia can be hereditary, and genetic linkage has been found for both generalized and focal dystonia. The only genetic dystonia for which the gene product is known is Segawa disease, a hereditary progressive dystonia with marked diurnal fluctuation. The defect is in guanosine triphosphate cyclohydrolase I, a gene that makes a cofactor for the synthesis of dopamine, which explains why this form of dystonia should be amenable to treatment with levodopa. Another example of dystonia in which a disorder of dopamine pharmacology appears responsible is the dystonia occurring in Parkinson disease, either spontaneously or as a result of treatment. Curiously, the dystonia occurs at both peak and trough dopamine levels.

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