Hostname: page-component-848d4c4894-r5zm4 Total loading time: 0 Render date: 2024-06-17T16:22:42.019Z Has data issue: false hasContentIssue false
  • English
  • Français

The Physiology of Idiopathic Dystonia

Published online by Cambridge University Press:  05 January 2016

C.D. Marsden  and
John C. Rothwell
C.D. Marsden*
Affiliation:
MRC Movement Disorders Research Group, University Department of Neurology, and Parkinson's Disease Society Research Centre, Institute of Psychiatry and King's College Hospital Medical School, London, U.K.
John C. Rothwell
Affiliation:
MRC Movement Disorders Research Group, University Department of Neurology, and Parkinson's Disease Society Research Centre, Institute of Psychiatry and King's College Hospital Medical School, London, U.K.
*
MRC Movement Disorders Research Group, University Department of NeurologyInstitute of Psychiatry and King's College Hospital Medical School, London, U.K. SE5 8AF
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

Dystonia is mysterious and its pathophysiology is uncertain. The fundamental motor abnormality is an abnormality of muscle command signals, such that the wrong agonists may be activated for too long, there is abnormal co-contraction of agonist and antagonists, and there is excessive and misdirected action of synergists and postural fixators. The result is action dystonia. In addition, muscle spasms may occur spontaneously. The peripheral motor apparatus, the corticomotoneurone pathway, and (as far as is known) the proprioceptive feedback machinery, are all intact in primary dystonia. However, a defect of interneuronal machinery has been identified in both the brainstem and spinal cord. In blepharospasm there is hyperexcitability of the lower brainstem interneurones responsible for the R2 component of the blink reflex. In the dystonic arm there is loss of the later phases of la reciprocal inhibition from extensors to flexors. Both deficits may be due to loss of normal basal ganglia inputs onto interneurones. The known sites of focal lesions that may cause symptomatic dystonia all impair basal ganglia output. However, whether such abnormal interneuronal function is sufficient to explain dystonia is not known. Among many unanswered questions are 1) are the cortical instructions for movement specified correctly, and 2) what is responsible for the spontaneous dystonic spasms?

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue S3 , August 1987 , pp. 521 - 527
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

References

REFERENCES

1.Oppenheim, H.Uber eine ergenartige Krampfkrankheit des Kindlichen und jugendlichen Alters (Dystonia tardotica prograssiva. Dystonia musculorum deformans). Neurologishes Zentralblalt 1911: 30: 1090–1107.Google Scholar
2.Foerster, O.Zur analyse and pathophysiologic der striaren Bewegungstorungen Zeitschrift fur de Gesamte Neurologie und Psychiatrie 1921:73: 1–169.CrossRefGoogle Scholar
3.Rothwell, JC, Obeso, JA.Marsden, CD.Pathophysiology of dystonias.In: Advances in Neurology, vol 39, Desmedt, JE. ed. Raven Press. New York, 1983: 851–863.Google Scholar
4.Merton, PA, Morton, HB.Stimulation of the cerebral cortex in the intact human subject. Nature 1980; 285: 227.CrossRefGoogle ScholarPubMed
5.Herz, E.Dystonia, I.Historical review: analysis of dystonic symp-toms and physiological mechanisms. Arch Neurol Psychiat 1944: 51: 305–318.CrossRefGoogle Scholar
6.Yanagisawa, N.Goto, A.Dystonia musculorum deformans. J Neurol Sci 1971; 13: 39–65.CrossRefGoogle ScholarPubMed
7.Berardelli, A.Rothwell, JC.Day, BL, Marsden, CD.Pathophysiol-ogy of blepharospasm and oromandibulardystonia. Brain 1985; 108: 593–608.CrossRefGoogle Scholar
8.Obeso, JA, Rothwell, JC, Lang, AE, Marsden, CD.Myoclonic dystonia. Neurology 1983: 33: 825–830.CrossRefGoogle ScholarPubMed
9.Marsden, CD.The problem of adult-onset idiopathic torsion dysto-nia and other isolated dyskinesias in adult life (including blepharospasm, oromandibular dystonia, dystonic writer’s cramp, torticollis and axial dystonia. Advances in Neurology 1976: 14: 259–276.Google ScholarPubMed
10.Tatton, WG, Redingham, W, Verrier, MC.Blair, RDG. Characteris-tic alterations in responses to imposed wrist displacements in Parkinsonian rigidity and dystonia musculorum deformans. Canadian Journal of the Neurological Sciences 1984: 11: 281–287.CrossRefGoogle Scholar
11.Berardelli, A, Hallett, M.Shortening reaction of tibialis anterior. Neurology 1984; 34: 242–246.CrossRefGoogle ScholarPubMed
12.Matsuoka, S.Waltz, JM.Terada, C, Ikeda, T.Cooper, IS.A computer technique for evaluation of recovery cycle of the ?-reflex in abnormal movement disorders. Electroencephalography and Clinical Neurophysiology 1966; 21: 496–500.CrossRefGoogle ScholarPubMed
13.Delwaide, PJ.Contribution of human reflex studies to the under-standing and management of the pyramidal syndrome. In: Electromyograghy in CNS disorders, Shahani, BT. ed. Butter-worths, London, 1984:77–110.CrossRefGoogle Scholar
14.Day, BL.Marsden, CD.Obeso, JA.Rothwell, JC.Reciprocal inhibi-tion between the muscles of the human forearm. Journal of Physiology 1984; 349: 519–534.CrossRefGoogle ScholarPubMed
15.Berardelli, A, Day, BL, Marsden, CD, Rothwell, JC.Observations on the mechanism of long-lasting reciprocal inhibition in the human forearm. Journal of Physiology 1985: 365: 24P.Google Scholar
16.Hallett, M, Shahani, BR, Young, RR.EMG analysis of stereotyped voluntary movements in man. Journal of Neurology Neurosurgery and Psychiatry 1975: 38: 1154–1162.CrossRefGoogle ScholarPubMed
17.Rothwell, JC.Traub, MM, Day, BL.et al. Manual motor performance in a deafferented man. Brain 1982; 105: 515–542.CrossRefGoogle Scholar
18.Narbona, J, Obeso, JA, Tunon, T, et al. Hemi-dystonia secondary to a localised basal ganglia tumour. J Neurology Neurosurgery Psychiatry 1984; 47: 707–709.CrossRefGoogle ScholarPubMed
19.Burton, K, Farrell, J, Li, D, Calne, DB.Lesions of the putamen and dystonia: CTand magnetic resonance imaging. Neurology 1984; 34: 962–965.CrossRefGoogle Scholar
20.Marsden, CD, Obeso, JA, Zarranz, JJ, Lang, AE.The anatomical basis of symptomatic hemidystonia. Brain 1985; 108: 463–483.CrossRefGoogle ScholarPubMed
21.Pettigrew, LC, Jankovic, J.Hemidystonia: a report on 22 patients and a review of the literature. J Neurology Neurosurg Psychiatry 1985; 48: 650–657.CrossRefGoogle Scholar
22.Dooling, EC, Adams, RD.The pathological anatomy of posthemiplegic athetosis. Brain 1975; 98: 29–48.CrossRefGoogle ScholarPubMed