26. 5. 2014. Focal dystonia - Wikipedia, the free encyclopedia

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Focal dystoniaFrom Wikipedia, the free encyclopedia

Focal dystonia is a neurological condition that affects a muscle or group of muscles in a specific part of thebody causing involuntary muscular contractions and abnormal postures. For example, in focal hand dystonia, thefingers either curl into the palm or extend outward without control. In musicians, the condition is referred to asMusician's Focal Dystonia, or simply musician's dystonia. In sports, it is commonly referred to as (the) yips. It is

believed that all adult-onset dystonias are a product of combined genetic factors and environmental modifiers.[1]

Contents

1 Causes

2 Notable cases

3 Treatment

4 References

4.1 Sources

Causes

The cause of dystonia is not precisely understood. Misfiring of neurons in the sensorimotor cortex, a thin layer ofneural tissue covering the brain, is thought to cause contractions. The source of this misfiring may be a result of

impaired inhibitory mechanisms during muscle contraction.[2] When the brain tells a given muscle to contract, itsimultaneously silences muscles that would oppose the intended movement. In dystonia, it appears that the

ability of the brain to inhibit the surrounding muscles is impaired, leading to loss of selectivity.[3]

The sensorimotor cortex is organized as discrete "maps" of the human body. Under normal conditions, eachbody part (such as individual fingers) occupies a distinct area on these cortical maps. In dystonia, these maps

lose their distinct borders and overlap occurs.[4] Exploration of this initially involved over-training particularfinger movements in non-human primates, which resulted in the development of focal hand dystonia. Examinationof the primary somatosensory cortex in the trained animals showed grossly distorted representations of the maps

pertaining to the fingers when compared to the untrained animals.[5] Additionally, these maps in the dystonicanimals had lost the distinct borders that were noted in the untrained animals.

Imaging studies in humans with focal dystonia have confirmed this finding.[6] Also, synchronous afferentstimulation of peripheral muscles induces organizational changes in motor representations, characterized both byan increase in map size of stimulated muscles and a reduction in map separation, as assessed using transcranial

magnetic stimulation.[7]

The cross-connectivity between areas that are normally segregated in the sensory cortex may prevent normalsensorimotor feedback and so contribute to the observed co-contraction of antagonist muscle groups, andinappropriately timed and sequenced movements that underlie the symptoms of focal dystonia. It is hypothesizedthat a deficit in inhibition caused by a genetically mediated loss of inhibitory interneurons may be the underlying

cause of the deficits observed in dystonia.[8]

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While usually painless, in some instances the sustained contraction and abnormal posturing in dystonia maycause pain. Focal dystonia most typically affects those who rely on fine motor skills (musicians, writers,surgeons, etc.). It is thought that the excessive motor training in these individuals may contribute to the

development of dystonia as their cortical maps become enlarged and begin to overlap.[9] Focal dystonia isgenerally "task specific," meaning that it is only problematic during certain activities.

Notable cases

Andrew Dawes, noted violinist and co-founder of the Orford String Quartet.[10]

Christian Münzner, lead guitar of progressive extreme metal band Obscura

Leon Fleisher, an international concert pianist, suffered from this affliction in his right hand beginning in the

1960s and switched to only left hand playing. In the 2000s, he regained use of his right hand and did

additional performances and recordings with two hands.

Alex Klein, formerly the first oboist of the Chicago Symphony

Ernestine Whitman, former member of the Atlanta Symphony and currently a professor of flute at

Lawrence University

Keith Emerson, pianist and keyboard player

Warren Deck, tubist and former member of the New York Philharmonic, was diagnosed with focal

dystonia in his upper lip, which ended his playing career.

Dominic Frasca, Guitarist

Gary Graffman, pianist, who changed to performing only with his left hand.

Billy McLaughlin, guitarist, switched to playing left-handed when afflicted with dystonia.

Liona Boyd, Canadian classical guitarist, known as the “First Lady of the Guitar”, retired from the

concert stage for six years in 2003, due to focal dystonia that affected her right hand. She worked to

retrain her right hand and since 2009 she has been performing again as a guitarist, singer and

songwriter.[11]

Andy Billups, bass guitarist with British rock group, The Hamsters, has made a partial recovery; he plays

by using modified guitar plectrums.

David Leisner, classical guitarist, has recovered the full use of his hand after a decade of disability.

Badi Assad, Brazilian singer-guitarist, was diagnosed with focal dystonia in 1999; she eventually

recovered and resumed her career.[12]

Tom Adams, bluegrass banjo player, has focal dystonia in his right hand, and has switched to the guitar.

Scott Adams, the writer of the Dilbert comics, has focal dystonia of the right hand, which impedes his

artwork.[13]

Stuart Cassells, founder of the bagpipe rock group Red Hot Chilli Pipers, announced focal dystonia in

September 2011; he has left the band.[14]

Apostolos Paraskevas, Greek-American classical guitarist-composer, was struck by focal dystonia to his

right hand in 2009. He fully recovered in 2013 after 7,000 hours of personal work in re-constructing his

technique. He was able to decode the condition as an unconscious behavioral habit and returned to

performing professionally again. His rehabilitation was based on reducing tension in his hand and

26. 5. 2014. Focal dystonia - Wikipedia, the free encyclopedia

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retraining his brain through proper, relaxed hand movements, practiced extensively. His article A

Classical Guitarist's Story of Recovery from Focal Dystonia will be published in London on the

Classical Guitar Magazine (http://www.classicalguitarmagazine.com).

Steve Dilling, bluegrass banjo player and founding member of the group, IIIrd Tyme Out. He was forced

to retire from the group due to focal dystonia.

Treatment

This condition is often treated with injections of botox, a commercially prepared form of botulinum toxin. Botoxreduces the symptoms of the disorder but it is not a cure for dystonia. Since the root of the problem isneurological, doctors have explored sensorimotor retraining activities to enable the brain to "rewire" itself andeliminate dystonic movements. The work of several doctors such as Nancy Byl and Joaquin Farias has shownthat sensorimotor retraining activities and proprioceptive stimulation can induce neuroplasticity, making it

possible for patients to recover substantial function that was lost from focal dystonia.[15][16][17]

References

1. ^ Hallet, Mark (2011). "Neurophysiology of dystonia: The role of inhibition". Neurobiology of Disease 42: 177–

184. doi:10.1016/j.nbd.2010.08.025 (http://dx.doi.org/10.1016%2Fj.nbd.2010.08.025).

2. ^ Hallet, Mark (2011). "Neurophysiology of dystonia: The role of inhibition". Neurobiology of Disease 42: 177–

184. doi:10.1016/j.nbd.2010.08.025 (http://dx.doi.org/10.1016%2Fj.nbd.2010.08.025).

3. ^ Hallet, Mark (2011). "Neurophysiology of dystonia: The role of inhibition". Neurobiology of Disease 42: 177–

184. doi:10.1016/j.nbd.2010.08.025 (http://dx.doi.org/10.1016%2Fj.nbd.2010.08.025).

4. ^ Byl, NN; Merzenich, MM; Jenkins, WM. (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 (2): 508–20. doi:10.1212/wnl.47.2.508

(http://dx.doi.org/10.1212%2Fwnl.47.2.508). PMID 8757029

(https://www.ncbi.nlm.nih.gov/pubmed/8757029).

5. ^ Byl, NN; Merzenich, MM; Jenkins, WM. (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 (2): 508–20. doi:10.1212/wnl.47.2.508

(http://dx.doi.org/10.1212%2Fwnl.47.2.508). PMID 8757029

(https://www.ncbi.nlm.nih.gov/pubmed/8757029).

6. ^ Bara-Jimenez, W; Catalan, MJ; Hallett, M; Gerloff, C (1998). "Abnormal somatosensory homunculus in

dystonia of the hand". Annals of neurology 44 (5): 828–831. doi:10.1002/ana.410440520

(http://dx.doi.org/10.1002%2Fana.410440520). PMID 9818942

(https://www.ncbi.nlm.nih.gov/pubmed/9818942).

7. ^ Schabrun SM, & Ridding MC (2007). “The influence of correlated afferent input on motor cortical

representations in humans”. Experimental Brain Research, 183(1): 41—49, doi: 10.1007/s00221-007-1019-8

8. ^ Hallet, Mark (2011). "Neurophysiology of dystonia: The role of inhibition". Neurobiology of Disease 42: 177–

184. doi:10.1016/j.nbd.2010.08.025 (http://dx.doi.org/10.1016%2Fj.nbd.2010.08.025).

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Sources

Tubiana, Raoul, Amadio, Peter C.; Medical Problems of the Instrumentalist Musician

(http://books.google.com/books?

id=8_hKRWPfz1EC&printsec=frontcover&dq=1853176125&source=bl&ots=MLv43voFgg&sig=SWC7q9vKO

_-

toU8_oZOKEZmy2A0&hl=en&ei=xM8XTMr0JMP98Aa3tujoDA&sa=X&oi=book_result&ct=result&resnum=1

&ved=0CBQQ6AEwAA#v=onepage&q&f=false); UK; Martin Dunitz (2000); 295-397

Rich, Robert F.; Mackin, Evelyn; Callahan, Anne; A. Lee Osterman; Terri M. Skirven; Schneider, Lawrence J.

(2002). Hunter, Mackin & Callahan's Rehabilitation of the Hand and Upper Extremity (2 Volume Set). St.

Louis: Mosby. pp. 2053–2075 ("Focal Hand Dystonia"). ISBN 0-323-01094-6.

Farias, Joaquin. "Intertwined. How to induce neuroplasticity. A new approach to rehabilitating dystonias".

Galene Editions 2012.

Farias, Joaquin. "Rebellion of the body. Understanding musician's Focal dystonia". Galene Editions 2004.

Leisner, David (2007). "Curing Focal Dystonia or How to Play the Guitar with Large Muscles". Guitar Review

133: 10–15.

Pascual-Leone A (2001). "The brain that plays music and is changed by it"

(http://www.annalsnyas.org/cgi/pmidlookup?view=long&pmid=11458838). Ann. N. Y. Acad. Sci. 930: 315–

329. doi:10.1111/j.1749-6632.2001.tb05741.x (http://dx.doi.org/10.1111%2Fj.1749-6632.2001.tb05741.x).

PMID 11458838 (https://www.ncbi.nlm.nih.gov/pubmed/11458838).

Solomon, Jason W. (2007). "What Every Guitarist Should Know: A Guide to the Prevention and Rehabilitation

of Focal Dystonia" (http://www.dystonia-foundation.org/filebin/pdf/focal%20dystonia.pdf). Guitar Review 133:

2–9.

Farias, Joaquin "Focal dystonia rehabilitation" (http://www.focaldystonia.net)

Gorrie, Jon. "Musician's Focal Dystonia" (http://musicians-focal-dystonia.com)

9. ^ Rosenkranz, Karin; Katherine Butler; Aaron Williamon; John C. Rothwell (November 18, 2009). "Regaining

Motor Control in Musician’s Dystonia by Restoring Sensorimotor Organization". The Journal of Neuroscience

29 (46): 14627–14636. doi:10.1523/JNEUROSCI.2094-09.2009

(http://dx.doi.org/10.1523%2FJNEUROSCI.2094-09.2009).

10. ^ http://www.wevancouver.com/news/202340481.html

11. ^ http://www.classicalguitar.com/about/

12. ^ Badi Assad's web site (http://www.badiassad.com/)

13. ^ Sordyl, Samantha (2005-05-10). "Scott Adams, Drawing the Line" (http://www.washingtonpost.com/wp-

dyn/content/article/2005/05/09/AR2005050901066.html). The Washington Post. Retrieved 2010-05-02.

14. ^ http://www.redhotchillipipers.co.uk/spanbNewsbspan/tabid/73/Default.aspx?newsItem=98

15. ^ Farias J, Yoshie M. Treatment efficacy in an ecologically valid neuropsycological treatment program of 120

professional musicians with focal dystonia, Galene Editions. Amsterdam 2012. ISBN 978-84-615-5124-8.

16. ^ Farias, J., Sarti-Martínez, MA. Title: "Elite musicians treated by specific fingers motion program to stimulate

propiceptive sense", Congreso Nacional De La Sociedad Anatómica Española, Alicante (España), European

Journal of Anatomy, p. 110

17. ^ Open Your Eyes. Freedom from Blepharospasm. Documentary. Out of the Box Productions.

http://www.youtube.com/watch?v=BlWXcsjyswY

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