Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-8448b6f56d-gtxcr Total loading time: 0 Render date: 2024-04-23T20:10:25.417Z Has data issue: false hasContentIssue false

25 - Focal hand dystonia

Published online by Cambridge University Press:  23 December 2009

By
Sarah Pirio Richardson  and
Mark Hallett
Edited by
Dennis A. Nowak  and
Joachim Hermsdörfer
Dennis A. Nowak
Affiliation:
Klinik Kipfenberg, Kipfenberg, Germany
Joachim Hermsdörfer
Affiliation:
Technical University of Munich
Get access

Summary

Summary

Imprecise and unwanted movements characterize the clinical presentation of focal hand dystonia. It is often task-specific, manifesting as writer's cramp or musician's dystonia (e.g. guitarist's cramp). Repetitive, stereotyped movements play a role in the development of the dystonia, but clearly, a pathophysiological substrate must be present for the disorder to manifest. This substrate is likely due to genetics; however, the exact genetic abnormality is not yet known. Although presenting as a motor disorder, dystonia is also a sensory disorder with subtle abnormalities found in spatial and temporal discrimination and with disordered sensory cortical maps. Abnormal cortical plasticity and a failure of homeostatic mechanisms also are seen in dystonia. Finally, a loss of inhibition from excessive muscle discharge to alterations in cortical circuits has been identified in dystonia. As a result, abnormal sensorimotor integration, abnormal plasticity and a loss of inhibition all are implicated in the pathophysiology of focal hand dystonia. Currently, it is not known which of these pathophysiological abnormalities is primary or secondary to the disorder development. Treatment strategies are aimed at ameliorating these physiological changes by improving the sensory deficit, normalizing plasticity and restoring inhibition.

Focal hand dystonia

Dystonia, a neurological disorder, is characterized by abnormal posturing due to sustained muscle contractions, which interferes with the normal performance of motor tasks (Hallett, 2004). Dystonia can be classified by age at onset, by distribution and by cause (Tarsy & Simon, 2006).

Type
Chapter
Information
Sensorimotor Control of Grasping
Physiology and Pathophysiology
 , pp. 348 - 360
Publisher: Cambridge University Press
Print publication year: 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Abbruzzese, G. & Berardelli, A. (2003). Sensorimotor integration in movement disorders. Mov Disord, 18, 231–240.CrossRefGoogle ScholarPubMed
Abbruzzese, G., Marchese, R., Buccolieri, A., Gasparetto, B. & Trompetto, C. (2001). Abnormalities of sensorimotor integration in focal dystonia: a transcranial magnetic stimulation study. Brain, 124, 537–545.CrossRefGoogle ScholarPubMed
Angelucci, A., Levitt, J. B. & Lund, J. S. (2002). Anatomical origins of the classical receptive field and modulatory surround field of single neurons in macaque visual cortical area V1. Prog Brain Res, 136, 373–388.CrossRefGoogle ScholarPubMed
Baker, R. S., Sun, W. S., Hansan, S. A.et al. (1997). Maladaptive neural compensatory mechanisms in Bell's palsy-induced blepharospasm. Neurology, 49, 223–229.CrossRefGoogle ScholarPubMed
Bara-Jimenez, W., Catalan, M. J., Hallett, M. & Gerloff, C. (1998). Abnormal somatosensory homunculus in dystonia of the hand. Ann Neurol, 44, 828–831.CrossRefGoogle ScholarPubMed
Baumer, T., Demiralay, C., Hidding, U.et al. (2007). Abnormal plasticity of the sensorimotor cortex to slow repetitive transcranial magnetic stimulation in patients with writer's cramp. Mov Disord, 22, 81–90.CrossRefGoogle ScholarPubMed
Berardelli, A., Rothwell, J. C., Day, B. L. & Marsden, C. D. (1985). Pathophysiology of blepharospasm and oromandibular dystonia. Brain, 108, 593–608.CrossRefGoogle ScholarPubMed
Byl, N. N., Merzenich, M. M. & Jenkins, W. M. (1996). 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, 47, 508–520.CrossRefGoogle ScholarPubMed
Candia, V., Rosset-Llobet, J., Elbert, T. & Pascual-Leone, A. (2005). Changing the brain through therapy for musicians' hand dystonia. Ann NY Acad Sci, 1060, 335–342.CrossRefGoogle ScholarPubMed
Chen, R. & Hallett, M. (1998). Focal dystonia and repetitive motion disorders. Clin Orthop Relat Res, 351, 102–106.CrossRefGoogle Scholar
Chen, R., Wassermann, E. M., Canos, M. & Hallett, M. (1997). Impaired inhibition in writer's cramp during voluntary muscle activation. Neurology, 49, 1054–1059.CrossRefGoogle ScholarPubMed
Chuke, J. C., Baker, R. S., & Porter, J. D. (1996). Bell's palsy-associated blepharospasm relieved by aiding eyelid closure. Ann Neurol, 39, 263–268.CrossRefGoogle ScholarPubMed
Cimatti, Z., Schwartz, D. P., Bourdain, F.et al. (2007). Time-frequency analysis reveals decreased high-frequency oscillations in writer's cramp. Brain, 130, 198–205.CrossRefGoogle ScholarPubMed
Classen, J., Steinfelder, B., Liepert, J.et al. (2000). Cutaneomotor integration in humans is somatotopically organized at various levels of the nervous system and is task dependent. Exp Brain Res, 130, 48–59.CrossRefGoogle ScholarPubMed
Cohen, L. G. & Hallett, M. (1988). Hand cramps: clinical features and electromyographic patterns in a focal dystonia. Neurology, 38, 1005–1012.CrossRefGoogle Scholar
Defazio, G., Aniello, M. S., Masi, G.et al. (2003). Frequency of familial aggregation in primary adult-onset cranial cervical dystonia. Neurol Sci, 24, 168–169.CrossRefGoogle ScholarPubMed
Defazio, G., Berardelli, A. & Hallett, M. (2007). Do primary adult-onset focal dystonias share aetiological factors? Brain, 130, 1183–1193.CrossRefGoogle ScholarPubMed
Delmaire, C., Krainik, A., Tezenas du Montcel, S.et al. (2005). Disorganized somatotopy in the putamen of patients with focal hand dystonia. Neurology, 64, 1391–1396.CrossRefGoogle ScholarPubMed
Di Lazzaro, V., Oliviero, A., Meglio, M.et al. (2000). Direct demonstration of the effect of lorazepam on the excitability of the human motor cortex. Clin Neurophysiol, 111, 794–799.CrossRefGoogle ScholarPubMed
Evinger, C. (2005). Animal models of focal dystonia. NeuroRx, 2, 513–524.CrossRefGoogle ScholarPubMed
Fiorio, M., Gambarin, M., Valente, E. M.et al. (2007). Defective temporal processing of sensory stimuli in DYT1 mutation carriers: a new endophenotype of dystonia? Brain, 130, 134–142.CrossRefGoogle ScholarPubMed
Frucht, S. J. (Ed.) (2004). Focal task specific dystonia in musicians. Advances in Neurology, Dystonia 4. Philadelphia, PA: Lippincott, Williams & Wilkins.
Garraux, G., Bauer, A., Hanakawa, T.et al. (2004). Changes in brain anatomy in focal hand dystonia. Ann Neurol, 55, 736–739.CrossRefGoogle ScholarPubMed
Gilio, F., Suppa, A., Bologna, M.et al. (2007). Short-term cortical plasticity in patients with dystonia: A study with repetitive transcranial magnetic stimulation. Mov Disord.CrossRefGoogle ScholarPubMed
Hallett, M. (2004). Dystonia: abnormal movements result from loss of inhibition. Adv Neurol, 94, 1–9.Google ScholarPubMed
Kessler, K. R., Ruge, D., Ilic, T. V. & Ziemann, U. (2005). Short latency afferent inhibition and facilitation in patients with writer's cramp. Mov Disord, 20, 238–242.CrossRefGoogle ScholarPubMed
Lerner, A., Shill, H., Hanakawa, T.et al. (2004). Regional cerebral blood flow correlates of the severity of writer's cramp symptoms. Neuroimage, 21, 904–913.CrossRefGoogle ScholarPubMed
Levy, L. M. & Hallett, M. (2002). Impaired brain GABA in focal dystonia. Ann Neurol, 51, 93–101.CrossRefGoogle ScholarPubMed
Matsumura, M., Sawaguchi, T., Oishi, T., Ueki, K. & Kubota, K. (1991). Behavioral deficits induced by local injection of bicuculline and muscimol into the primate motor and premotor cortex. J Neurophysiol, 65, 1542–1553.CrossRefGoogle ScholarPubMed
Meunier, S., Garnero, L., Ducorps, A.et al. (2001). Human brain mapping in dystonia reveals both endophenotypic traits and adaptive reorganization. Ann Neurol, 50, 521–527.CrossRefGoogle ScholarPubMed
Molloy, F. M., Carr, T. D., Zeuner, K. E., Dambrosia, J. M. & Hallett, M. (2003). Abnormalities of spatial discrimination in focal and generalized dystonia. Brain, 126, 2175–2182.CrossRefGoogle ScholarPubMed
Murase, N., Kaji, R., Shimazu, H.et al. (2000). Abnormal premovement gating of somatosensory input in writer's cramp. Brain, 123, 1813–1829.CrossRefGoogle ScholarPubMed
Murase, N., Rothwell, J., Kaji, R.et al. (2005). Subthreshold low-frequency repetitive transcranial magnetic stimulation over the premotor cortex modulates writer's cramp. Brain, 128, 104–115.CrossRefGoogle ScholarPubMed
Nakashima, K., Rothwell, J. C., Day, B. L.et al. (1989). Reciprocal inhibition in writer's and other occupational cramps and hemiparesis due to stroke. Brain, 112, 681–697.CrossRefGoogle Scholar
Panizza, M., Lelli, S., Nilsson, J. & Hallett, M. (1990). H-reflex recovery curve and reciprocal inhibition of H-reflex in different kinds of dystonia. Neurology, 40, 824–828.CrossRefGoogle ScholarPubMed
Peller, M., Zeuner, K. E., Munchau, A.et al. (2006). The basal ganglia are hyperactive during the discrimination of tactile stimuli in writer's cramp. Brain, 129, 2697–2708.CrossRefGoogle ScholarPubMed
Perlmutter, J. S., Stambuk, M. K., Markham, J.et al. (1997). Decreased [18F]spiperone binding in putamen in idiopathic focal dystonia. J Neurosci, 17, 843–850.CrossRefGoogle ScholarPubMed
Putzki, N., Stude, P., Konczak, J.et al. (2006). Kinesthesia is impaired in focal dystonia. Mov Disord, 21, 754–760.CrossRefGoogle ScholarPubMed
Quartarone, A., Bagnato, S., Rizzo, V.et al. (2003). Abnormal associative plasticity of the human motor cortex in writer's cramp. Brain, 126, 2586–2596.CrossRefGoogle ScholarPubMed
Quartarone, A., Rizzo, V., Bagnato, S.et al. (2005). Homeostatic-like plasticity of the primary motor hand area is impaired in focal hand dystonia. Brain, 128, 1943–1950.CrossRefGoogle ScholarPubMed
Quartarone, A., Siebner, H. R., & Rothwell, J. C. (2006). Task-specific hand dystonia: can too much plasticity be bad for you? Trends Neurosci, 29, 192–199.CrossRefGoogle ScholarPubMed
Ridding, M. C., Sheean, G., Rothwell, J. C., Inzelberg, R. & Kujirai, T. (1995). Changes in the balance between motor cortical excitation and inhibition in focal, task specific dystonia. J Neurol Neurosurg Psychiatry, 59, 493–498.CrossRefGoogle ScholarPubMed
Sanger, T. D., Garg, R. R. & Chen, R. (2001). Interactions between two different inhibitory systems in the human motor cortex. J Physiol, 530, 307–317.CrossRefGoogle ScholarPubMed
Schicatano, E. J., Basso, M. A. & Evinger, C. (1997). Animal model explains the origins of the cranial dystonia benign essential blepharospasm. J Neurophysiol, 77, 2842–2846.CrossRefGoogle ScholarPubMed
Siebner, H. R., Filipovic, S., Rowe, J. B.et al. (2003). Patients with focal arm dystonia have increased sensitivity to slow-frequency repetitive TMS of the dorsal premotor cortex. Brain, 126, 2710–2725.CrossRefGoogle ScholarPubMed
Sohn, Y. H. & Hallett, M. (2004a). Surround inhibition in human motor system. Exp Brain Res, 158, 397–404.CrossRefGoogle ScholarPubMed
Sohn, Y. H. & Hallett, M. (2004b). Disturbed surround inhibition in focal hand dystonia. Ann Neurol, 56, 595–599.CrossRefGoogle ScholarPubMed
Sohn, Y. H., Jung, H. Y., Kaelin-Lang, A. & Hallett, M. (2003). Excitability of the ipsilateral motor cortex during phasic voluntary hand movement. Exp Brain Res, 148, 176–185.CrossRefGoogle ScholarPubMed
Song, I. U., Kim, J. S., Kim, H. T. & Lee, K. S. (2007). Task-specific focal hand dystonia with usage of a spoon. Parkinsonism Relat Disord, 14, 72–74.CrossRefGoogle ScholarPubMed
Stefan, K., Kunesch, E., Cohen, L. G., Benecke, R. & Classen, J. (2000). Induction of plasticity in the human motor cortex by paired associative stimulation. Brain, 123, 572–584.CrossRefGoogle ScholarPubMed
Stefan, K., Kunesch, E., Benecke, R., Cohen, L. G. & Classen, J. (2002). Mechanisms of enhancement of human motor cortex excitability induced by interventional paired associative stimulation. J Physiol, 543, 699–708.CrossRefGoogle ScholarPubMed
Stinear, C. M. & Byblow, W. D. (2004). Impaired modulation of intracortical inhibition in focal hand dystonia. Cereb Cortex, 14, 555–561.CrossRefGoogle ScholarPubMed
Tarsy, D. & Simon, D. K. (2006). Dystonia. N Engl J Med, 355, 818–829.CrossRefGoogle ScholarPubMed
Tinazzi, M., Rosso, T. & Fiaschi, A. (2003). Role of the somatosensory system in primary dystonia. Mov Disord, 18, 605–622.CrossRefGoogle ScholarPubMed
Tyvaert, L., Houdayer, E., Devanne, H.et al. (2006). The effect of repetitive transcranial magnetic stimulation on dystonia: a clinical and pathophysiological approach. Neurophysiol Clin, 36, 135–143.CrossRefGoogle ScholarPubMed
Valls-Solé, J., Pascual-Leone, A., Wassermann, E. M. & Hallett, M. (1992). Human motor evoked responses to paired transcranial magnetic stimuli. Electroencephalogr Clin Neurophysiol, 85, 355–364.CrossRefGoogle ScholarPubMed
Weise, D., Schramm, A., Stefan, K.et al. (2006). The two sides of associative plasticity in writer's cramp. Brain, 129, 2709–2721.CrossRefGoogle ScholarPubMed
Weiss, E. M., Hershey, T., Karimi, M.et al. (2006). Relative risk of spread of symptoms among the focal onset primary dystonias. Mov Disord, 21, 1175–1181.CrossRefGoogle ScholarPubMed
Werhahn, K. J., Kunesch, E., Noachtar, S., Benecke, R. & Classen, J. (1999). Differential effects on motorcortical inhibition induced by blockade of GABA uptake in humans. J Physiol (Lond), 517, 591–597.CrossRefGoogle ScholarPubMed
Zeuner, K. E., Bara-Jimenez, W., Noguchi, P. S.et al. (2002). Sensory training for patients with focal hand dystonia. Ann Neurol, 51, 593–598.CrossRefGoogle ScholarPubMed
Zeuner, K. E., Shill, H. A., Sohn, Y. H.et al. (2005). Motor training as treatment in focal hand dystonia. Mov Disord, 20, 335–341.CrossRefGoogle ScholarPubMed
Ziemann, U., Rothwell, J. C. & Ridding, M. C. (1996). Interaction between intracortical inhibition and facilitation in human motor cortex. J Physiol (Lond), 496, 873–881.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×