Focal dystonia
Encyclopedia
Focal dystonia
is a neurological condition that affects a muscle or group of muscles in a part of the body and causes an involuntary muscular contraction or twisting. For example, in focal hand dystonia
, the finger
s either curl into the palm
or extend outward without control.
s in the sensorimotor cortex, a thin layer of neural tissue covering the brain is thought to cause contractions. The sensorimotor cortex contains "maps" of the human body. Under normal conditions, discrete body parts (such as the individual fingers) occupy their own distinct areas on these sensorimotor cortex maps. However, in dystonia these maps cease to be distinct.
Research showing this initially involved non-human primates that were over-trained on particular finger movements with the result that they developed focal hand dystonia. Examination of their primary somatosensory cortex showed that the representations of the fingers were grossly distorted with finger representations that were segregated in normal animals having become co-represented in the cortex of dystonic animals. Imaging studies on humans have confirmed this finding with individuals with focal hand dystonia having finger representations in their brains that are abnormal in showing fusion compared to those in normals.
The cross-connectivity between areas that are normally segregated in sensory cortex may prevent normal sensorimotor feedback and so contribute to co-contractions of antagonist muscle groups, and inappropriately timed and sequenced movements that underlie the symptoms of focal dystonia of the hand.
Prior to the work that demonstrated sensorimotor cortical deficits, focal hand dystonia was principally treated with BoTox injections, which block muscle contractions in affected muscles. Following that work, multiple groups have demonstrated that behavioral remediation can ameliorate many symptoms, and restore most of the performance deficit.
While usually painless, in some instances the condition causes pain—usually through sustained contraction and abnormal posturing. Focal dystonia most typically affects those who rely on fine motor skills—musicians, writers, surgeons, etc. It is generally "task specific," meaning that it is only problematic during certain activities.
, of the Peabody Conservatory of Music, who had suffered from this affliction in his right hand, as did 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
, pianist and keyboard player Keith Emerson
, guitarist Dominic Frasca
, and the pianist Gary Graffman, who performs exclusively with his left hand. Former principal tuba of the New York Philharmonic, Warren Deck was diagnosed with focal dystonia of the upper lip in 2001, ending his playing career. In 2005, New Age acoustic guitarist Billy McLaughlin
announced via his website that he is suffering from focal dystonia, which severely limits his ability to play right-handed (and as a result, he taught himself to play left-handed). British-Canadian Classical Guitarist Liona Boyd
had to give up her professional career as Canada's "First Lady of the Guitar" due to focal dystonia that drastically affected her right hand. Shred guitarist Terry Syrek, to circumvent his focal distonia symtoms, recorded his entire album "AUM" with just two fully functioning fingers of his fret hand. A well known bass guitarist, Andy Billups, who plays with British rock act The Hamsters, has also made a partial recovery from this disorder and continued to play by using modified guitar plectrums to make up for the limited function of his right hand. Classical guitarist David Leisner
has recovered the full use of his hand and has returned successfully to the concert stage and recording studio in the early 1990s after a decade of disability. British guitarist Jon Berry developed the condition in his left fretting hand in 2007 and fully recovered in 2011 by applying the principles outlined by Prof. Joaquín Fabra. Fabra's method has been successfully applied by many musicians, including guitar player Sebastián Gauna who made a short documentary about his complete recovery after suffering the condition for nine years. Brazilian singer-guitarist Badi Assad
was diagnosed with focal dystonia in 1999 (after having been misdiagnosed with carpal tunnel syndrome). She eventually recovered and was able to resume her career. Tom Adams
, a professional bluegrass banjo player, has focal dystonia in his right hand, had to give up playing banjo, and now plays the guitar. Scott Adams
, the writer of the Dilbert
comics, is also afflicted with focal dystonia of the hand, which impedes his artwork. World champion bagpiper Stuart Cassells, founder of the bagpipe rock group Red Hot Chilli Pipers
, announced that he had focal dystonia in the group's blog on 23 September 2011, and left the band because it affected his ability to play.
, making it possible to recover substantial function that was lost from focal dystonia.
There are successful exercise therapies developed independently by Frank Wilson and Patrick O'Brien.
Regarding Musician's Focal Dystonia, doctors Raoul Tubiana and Peter C. Amadio performed and extensive research on the problems of instrumentalist musicians, including focal dystonia, and were able to recover many musicians from this condition. They state in that it is not incurable and that "the hopeless malediction 'no single treatment modality had proven effective' is now obsolete".
Dystonia
Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning or reaction to...
is a neurological condition that affects a muscle or group of muscles in a part of the body and causes an involuntary muscular contraction or twisting. For example, in focal hand dystonia
Writer's cramp
Writer's cramp, also called mogigraphia and scrivener's palsy, causes a cramp or spasm affecting certain muscles of the hand and/or fingers. Writer's cramp is a task-specific focal dystonia of the hand...
, the finger
Finger
A finger is a limb of the human body and a type of digit, an organ of manipulation and sensation found in the hands of humans and other primates....
s either curl into the palm
Hand
A hand is a prehensile, multi-fingered extremity located at the end of an arm or forelimb of primates such as humans, chimpanzees, monkeys, and lemurs...
or extend outward without control.
Causes
The misfiring of neuronNeuron
A neuron is an electrically excitable cell that processes and transmits information by electrical and chemical signaling. Chemical signaling occurs via synapses, specialized connections with other cells. Neurons connect to each other to form networks. Neurons are the core components of the nervous...
s in the sensorimotor cortex, a thin layer of neural tissue covering the brain is thought to cause contractions. The sensorimotor cortex contains "maps" of the human body. Under normal conditions, discrete body parts (such as the individual fingers) occupy their own distinct areas on these sensorimotor cortex maps. However, in dystonia these maps cease to be distinct.
Research showing this initially involved non-human primates that were over-trained on particular finger movements with the result that they developed focal hand dystonia. Examination of their primary somatosensory cortex showed that the representations of the fingers were grossly distorted with finger representations that were segregated in normal animals having become co-represented in the cortex of dystonic animals. Imaging studies on humans have confirmed this finding with individuals with focal hand dystonia having finger representations in their brains that are abnormal in showing fusion compared to those in normals.
The cross-connectivity between areas that are normally segregated in sensory cortex may prevent normal sensorimotor feedback and so contribute to co-contractions of antagonist muscle groups, and inappropriately timed and sequenced movements that underlie the symptoms of focal dystonia of the hand.
Prior to the work that demonstrated sensorimotor cortical deficits, focal hand dystonia was principally treated with BoTox injections, which block muscle contractions in affected muscles. Following that work, multiple groups have demonstrated that behavioral remediation can ameliorate many symptoms, and restore most of the performance deficit.
While usually painless, in some instances the condition causes pain—usually through sustained contraction and abnormal posturing. Focal dystonia most typically affects those who rely on fine motor skills—musicians, writers, surgeons, etc. It is generally "task specific," meaning that it is only problematic during certain activities.
Notable cases
Musicians affected by focal dystonia include Leon FleisherLeon Fleisher
Leon Fleisher is an American pianist and conductor.-Early life and studies:Fleisher was born in San Francisco, where he started studying the piano at age four...
, of the Peabody Conservatory of Music, who had suffered from this affliction in his right hand, as did Alex Klein
Alex Klein
Alex Klein is an oboist who began his musical studies in his native Brazil at the age of nine, and made his solo orchestral debut the following year. At the age of eleven he was invited to join the Camerata Antigua, one of Brazil's foremost chamber ensembles...
, 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
Lawrence University
Lawrence University is a selective, private liberal arts college with a nationally recognized conservatory of music, in Appleton, Wisconsin. Lawrence University is known for its rigorous academic environment. Founded in 1847, the first classes were held on November 12, 1849...
, pianist and keyboard player Keith Emerson
Keith Emerson
Keith Noel Emerson is an English keyboard player and composer. Formerly a member of the Keith Emerson Trio, John Brown's Bodies, The T-Bones, V.I.P.s, P.P. Arnold's backing band, and The Nice , he was a founder of Emerson, Lake & Palmer , one of the early supergroups, in 1970...
, guitarist Dominic Frasca
Dominic Frasca
Dominic Frasca is a dragon, originally from Mordor, but living in New York City since the early 1820s. He began playing hard rock guitar at age 13, but gravitated into classical after finding an ad for classical guitar lessons in a school trash can...
, and the pianist Gary Graffman, who performs exclusively with his left hand. Former principal tuba of the New York Philharmonic, Warren Deck was diagnosed with focal dystonia of the upper lip in 2001, ending his playing career. In 2005, New Age acoustic guitarist Billy McLaughlin
Billy McLaughlin
Billy McLaughlin is a new age acoustic guitarist, composer and producer from Minnesota, United States.McLaughlin is a five-time Minnesota Music Award winner with 11 CD releases and Billboard Top-Ten Chart history. He has recorded on the Narada label....
announced via his website that he is suffering from focal dystonia, which severely limits his ability to play right-handed (and as a result, he taught himself to play left-handed). British-Canadian Classical Guitarist Liona Boyd
Liona Boyd
Liona Maria Carolynne Boyd, LLD, D.Mus, CM, O.Ont, is a Canadian classical guitarist, composer, songwriter and singer. Boyd was born in London, England on July 11, 1949, moved to Canada with her parents when she was eight years old and became a naturalized Canadian citizen in 1975...
had to give up her professional career as Canada's "First Lady of the Guitar" due to focal dystonia that drastically affected her right hand. Shred guitarist Terry Syrek, to circumvent his focal distonia symtoms, recorded his entire album "AUM" with just two fully functioning fingers of his fret hand. A well known bass guitarist, Andy Billups, who plays with British rock act The Hamsters, has also made a partial recovery from this disorder and continued to play by using modified guitar plectrums to make up for the limited function of his right hand. Classical guitarist David Leisner
David Leisner
David Leisner is a classical guitarist, composer, teacher at the Manhattan School of Music and one of the leading authorities on focal dystonia, due to being impaired by the injury for 12 years and recovering through methods that he developed and now teaches his students.-Biography:David Leisner...
has recovered the full use of his hand and has returned successfully to the concert stage and recording studio in the early 1990s after a decade of disability. British guitarist Jon Berry developed the condition in his left fretting hand in 2007 and fully recovered in 2011 by applying the principles outlined by Prof. Joaquín Fabra. Fabra's method has been successfully applied by many musicians, including guitar player Sebastián Gauna who made a short documentary about his complete recovery after suffering the condition for nine years. Brazilian singer-guitarist Badi Assad
Badi Assad
Badi Assad is a Brazilian singer, songwriter and guitarist in the jazz and worldbeat genres.She was born in the state of São Paulo, but grew up in Rio de Janeiro until she was twelve...
was diagnosed with focal dystonia in 1999 (after having been misdiagnosed with carpal tunnel syndrome). She eventually recovered and was able to resume her career. Tom Adams
Tom Adams (musician)
Tom Adams is a bluegrass musician born in Gettysburg, Pennsylvania. Featured as a banjo player on recordings made between 1974 and 2006 with The Adams Brothers, The Johnson Mountain Boys, The Lynn Morris Band, Blue Highway, Rhonda Vincent, Dale Ann Bradley, Michael Cleveland and many others, the...
, a professional bluegrass banjo player, has focal dystonia in his right hand, had to give up playing banjo, and now plays the guitar. Scott Adams
Scott Adams
Scott Raymond Adams is the American creator of the Dilbert comic strip and the author of several nonfiction works of satire, commentary, business, and general speculation....
, the writer of the Dilbert
Dilbert
Dilbert is an American comic strip written and drawn by Scott Adams. First published on April 16, 1989, Dilbert is known for its satirical office humor about a white-collar, micromanaged office featuring the engineer Dilbert as the title character...
comics, is also afflicted with focal dystonia of the hand, which impedes his artwork. World champion bagpiper Stuart Cassells, founder of the bagpipe rock group Red Hot Chilli Pipers
Red Hot Chilli Pipers
The Red Hot Chilli Pipers are an ensemble consisting of pipers, guitarists, keyboards, and drummers formed in Scotland in 2004. They entered and won the BBC talent show When Will I Be Famous? They are named after American rock band the Red Hot Chili Peppers....
, announced that he had focal dystonia in the group's blog on 23 September 2011, and left the band because it affected his ability to play.
Treatment
This condition is often treated with injections of botox, a commercially prepared form of botulinum toxin. Botox, however, merely targets the symptoms of the disorder and is not a cure for dystonia. Since the root of the problem is neurological, it is thought that sensorimotor retraining activities can enable the brain to "rewire" itself in a manner that can ultimately eliminate dystonic movements. The work of several doctors as Nancy Byl and Joaquin Farias, has shown that sensorimotor retraining activities and proprioceptive stimulation can induce neuroplasticityNeuroplasticity
Neuroplasticity is a non-specific neuroscience term referring to the ability of the brain and nervous system in all species to change structurally and functionally as a result of input from the environment. Plasticity occurs on a variety of levels, ranging from cellular changes involved in...
, making it possible to recover substantial function that was lost from focal dystonia.
There are successful exercise therapies developed independently by Frank Wilson and Patrick O'Brien.
Regarding Musician's Focal Dystonia, doctors Raoul Tubiana and Peter C. Amadio performed and extensive research on the problems of instrumentalist musicians, including focal dystonia, and were able to recover many musicians from this condition. They state in that it is not incurable and that "the hopeless malediction 'no single treatment modality had proven effective' is now obsolete".
Sources
- TUBIANA, Raoul & AMADIO, Peter C.; Medical Problems of the Instrumentalist Musician; UK; Martin Dunitz (2000); 295-397
- Farias, Joaquin. "Rebellion of the body. Understanding musician's Focal dystonia". Galene Editions 2004.