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Focal dystonia, or focal task specific dystonia, is a neurological condition, a type of ''
dystonia Dystonia is a neurological hyperkinetic movement disorder in which sustained or repetitive muscle contractions result in twisting and repetitive movements or abnormal fixed postures. The movements may resemble a tremor. Dystonia is often inten ...
'', that affects a muscle or group of muscles in a specific part of the body during specific activities, causing involuntary muscular contractions and abnormal postures. There are many different types of focal dystonia, each affecting a different region of the body. For example, in ''focal hand dystonia'', or ''
writer's cramp Writer's cramp or focal hand dystonia (FHD) is an idiopathic movement disorder of adult onset, characterized by abnormal posturing and movement of the hand and/or forearm during tasks requiring skilled hand use, such as writing.Rana, AQ, Saeed, U. ...
'', the fingers either curl into the palm or extend outward without control. In musicians, the condition is called ''musician's focal dystonia'', or simply, ''musician's dystonia''. In sports, it may be involved in what is commonly referred to as the ''
yips In sports, the yips (in gymnastics, lost move syndrome or the twisties) are a sudden and unexplained loss of ability to execute certain skills in experienced athletes. Symptoms of the yips are losing fine motor skills and psychological issues that ...
''. The condition appears to be associated with over-training, and individualized treatment strategies may involve medications, retraining techniques, and procedures.


Signs and Symptoms

People with dystonia experience tightness, cramping, fatigue, involuntary sustained or repetitive muscle contractions that can be painless or painful and resulting in abnormal posturing, twisting motions, and even tremors. Focal dystonia typically presents in adults, more commonly in women ranging from ages 30 through 40. Major types of focal dystonia affect the limbs, face, mouth, neck, and larynx. These focal dystonias can be exclusive to specific tasks, like writing or playing an instrument. Focal dystonia will typically have a subtle and slow onset before slowly worsening over years. During the beginning stages, symptoms can be intermittent and without clear associations and provocation. The progression of this disease can vary from person to person. During the first couple years of onset, symptoms can partially or completely disappear for days to months, but return in other parts of the body.


Causes

Current medical science does not precisely describe the causes of dystonia, however genetic and environmental factors may play a large role. Misfiring of
neuron A neuron, neurone, or nerve cell is an electrically excitable cell that communicates with other cells via specialized connections called synapses. The neuron is the main component of nervous tissue in all animals except sponges and placozoa. N ...
s in the
sensorimotor cortex The motor cortex is the region of the cerebral cortex believed to be involved in the planning, control, and execution of voluntary movements. The motor cortex is an area of the frontal lobe located in the posterior precentral gyrus immediately ...
, a thin layer of neural tissue that covers the brain, is thought to cause contractions. This misfiring may result from impaired inhibitory mechanisms during muscle contraction. When the brain tells a given muscle to contract, it simultaneously silences muscles that would oppose the intended movement. It appears that dystonia interferes with the brain's ability to inhibit those surrounding muscles, leading to loss of selectivity. The sensorimotor cortex is organized as discrete "maps" of the human body. Under normal conditions, each body part (such as individual fingers) occupies a distinct area on these cortical maps. In dystonia, these maps lose their distinct borders and overlap occurs. Exploration of this initially involved over-training particular finger movements in non-human primates, which resulted in the development of focal hand dystonia. Examination of 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. Additionally, these maps in the dystonic animals had lost the distinct borders that were noted in the untrained animals. Imaging studies in humans with focal dystonia have confirmed this finding. Also, synchronous afferent stimulation of peripheral muscles induces organizational changes in motor representations, characterized both by an increase in map size of stimulated muscles and a reduction in map separation, as assessed using
transcranial magnetic stimulation Transcranial magnetic stimulation (TMS) is a noninvasive form of brain stimulation in which a changing magnetic field is used to induce an electric current at a specific area of the brain through electromagnetic induction. An electric pulse gener ...
. The cross-connectivity between areas that are normally segregated in the sensory cortex may prevent normal sensorimotor feedback and so contribute to the observed co-contraction of antagonist muscle groups, and inappropriately timed and sequenced movements that underlie the symptoms of focal dystonia. It is hypothesized that a deficit in inhibition caused by a genetically mediated loss of inhibitory
interneurons Interneurons (also called internuncial neurons, relay neurons, association neurons, connector neurons, intermediate neurons or local circuit neurons) are neurons that connect two brain regions, i.e. not direct motor neurons or sensory neurons. In ...
may be the underlying cause of the deficits observed in dystonia. While usually painless, in some instances the sustained contraction and abnormal posturing in dystonia cause pain. Focal dystonia most typically affects people who rely on fine motor skills—musicians, writers, surgeons, etc. It is thought that the excessive motor training those skills require may contribute to the development of dystonia as their cortical maps become enlarged and begin to overlap. Focal dystonia is generally "task-specific," meaning that it is only problematic during certain activities.


Diagnosis

The diagnosis of focal dystonia is highly dependent on the history of the patient, as physical exam is typically normal, and ruling out other causes of movement disorder. The main types of are
blepharospasm Blepharospasm is any abnormal contraction of the orbicularis oculi muscle. The condition should be distinguished from the more common, and milder, involuntary quivering of an eyelid, known as myokymia, or fasciculation. In most cases, blepharosp ...
,
oromandibular dystonia Oromandibular dystonia is a form of focal dystonia affecting the mouth, jaw and tongue, and in this disease it is hard to speak. It is associated with bruxism. Botulinum toxin has been used in treatment. Since the root of the problem is neurolo ...
,
spasmodic dysphonia Spasmodic dysphonia, also known as laryngeal dystonia, is a disorder in which the muscles that generate a person's voice go into periods of spasm. This results in breaks or interruptions in the voice, often every few sentences, which can make a pe ...
,
spasmodic torticollis Spasmodic torticollis is an extremely painful chronic neurological movement disorder causing the neck to involuntarily turn to the left, right, upwards, and/or downwards. The condition is also referred to as "cervical dystonia". Both agonist and ...
, and ''limb dystonia'', all affecting a different region of the body. Labs and imaging may be ordered as workup to evaluate for other causes of dystonia. Focal dystonias involving are commonly misdiagnosed A provider can consider to rule in or out Dopamine-responsive dystonia with a levodopa trial.


Treatment

Treatment options would be based on the type of focal dystonia the patient has. If possible, incorporating rest earlier to symptom onset has shown to have greater improvement in symptoms for
Writer's cramp Writer's cramp or focal hand dystonia (FHD) is an idiopathic movement disorder of adult onset, characterized by abnormal posturing and movement of the hand and/or forearm during tasks requiring skilled hand use, such as writing.Rana, AQ, Saeed, U. ...
.


Physical Therapy

Physical therapy is commonly used as an adjunct or main form of treatment for focal dystonia, however more studies for its benefit are needed. Bass guitarist and instructor Scott Devine said that he wears a glove while playing bass guitar because of the condition. He finds that the glove stops the involuntary finger movements. He says it works for him but does not suggest that it may work for everyone with the condition.


Medications

Anticholinergics Anticholinergics (anticholinergic agents) are substances that block the action of the neurotransmitter called acetylcholine (ACh) at synapses in the central and peripheral nervous system. These agents inhibit the parasympathetic nervous sy ...
such as Artane can be prescribed for
off-label Off-label use is the use of pharmaceutical drugs for an unapproved indication or in an unapproved age group, dosage, or route of administration. Both prescription drugs and over-the-counter drugs (OTCs) can be used in off-label ways, although m ...
use, as some patients have had success.
Benzodiazepine Benzodiazepines (BZD, BDZ, BZs), sometimes called "benzos", are a class of depressant drugs whose core chemical structure is the fusion of a benzene ring and a diazepine ring. They are prescribed to treat conditions such as anxiety disorders, ...
s, such as
Clonazepam Clonazepam, sold under the brand names Klonopin and Rivotril, is a medication used to prevent and treat seizures, panic disorder, anxiety disorders, and the movement disorder known as akathisia. It is a tranquilizer of the benzodiazepine clas ...
, can be used as a muscle relaxant. Other oral medications have been studied for different types of focal dystonia, such as
baclofen Baclofen, sold under the brand name Lioresal among others, is a medication used to treat muscle spasticity such as from a spinal cord injury or multiple sclerosis. It may also be used for hiccups and muscle spasms near the end of life. It is ta ...
,
tetrabenazine Tetrabenazine is a drug for the symptomatic treatment of hyperkinetic movement disorders. It is sold under the brand names Nitoman and Xenazine among others. On August 15, 2008, the U.S. Food and Drug Administration approved the use of tetrabena ...
,
amantadine Amantadine, sold under the brand name Gocovri among others, is a medication used to treat dyskinesia associated with parkinsonism and influenza caused by type A influenzavirus, though its use for the latter is no longer recommended due to wid ...
,
antipsychotic Antipsychotics, also known as neuroleptics, are a class of Psychiatric medication, psychotropic medication primarily used to manage psychosis (including delusions, hallucinations, paranoia or disordered thought), principally in schizophrenia but ...
s, and
amphetamine Amphetamine (contracted from alpha- methylphenethylamine) is a strong central nervous system (CNS) stimulant that is used in the treatment of attention deficit hyperactivity disorder (ADHD), narcolepsy, and obesity. It is also commonly used ...


Procedures

This condition is often treated with injections of
botulinum neurotoxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium '' Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neuromus ...
type A (BoNT/A). BoNT/A reduces the symptoms of the disorder but it is not a cure for dystonia. Since the root of the problem is neurological, doctors have explored sensorimotor retraining activities to enable the brain to "rewire" itself and eliminate dystonic movements. The work of several doctors such as Nancy Byl and Joaquin Farias has shown that sensorimotor retraining activities and proprioceptive stimulation can induce
neuroplasticity Neuroplasticity, also known as neural plasticity, or brain plasticity, is the ability of Neural circuit, neural networks in the brain to change through growth and reorganization. It is when the brain is rewired to function in some way that diffe ...
, making it possible for patients to recover substantial function that was lost to focal dystonia. For some dystonias,
Deep brain stimulation Deep brain stimulation (DBS) is a neurosurgical procedure involving the placement of a medical device called a neurostimulator, which sends electrical impulses, through implanted electrodes, to specific targets in the brain (the brain nucleu ...
may be considered where BoNT treatment fails. If the dystonia is cause by an underlying
nerve entrapment Nerve compression syndrome, or compression neuropathy, or nerve entrapment syndrome, is a medical condition caused by direct pressure on a nerve. It is known colloquially as a ''trapped nerve'', though this may also refer to nerve root compress ...
,
surgical decompression In medicine, decompression refers to the removal or repositioning of any structure compressing any other structure. Common examples include decompressive craniectomy (removal of part of the skull to relieve pressure on the brain), and spinal decomp ...
has shown to have improvement in some cases.


Prognosis

Recovery from focal dystonia has varying results, ranging from minimal improvement in function to complete recovery and ranging from weeks to years.


Prevention

Though studies on prevention of focal dystonia is limited, it is believed for musician's dystonia that mindfulness of muscle use and proper rest can help prevent focal dystonia and injury.


Performing Arts Medicine


Musician's Dystonia

Musician's dystonia treatment studies have yielded varying results. This condition can often be misdiagnosed as psychogenic, as this was previously thought to be its cause. In this population, men are affected more than women from 20 to 60 years of age. Evaluation of musician's dystonia should include history before and after symptom onset and observation of playing the instrument, as deficits can be subtle and considered minor to the general population. Treatment modalities available, such as BoNT/A, leave many musicians unsatisfied due to muscle weakness and lack of improvement in muscle control. In musicians, this is commonly associated with overuse or high volume of practicing. Though musician's dystonia presents initially as task-specific, one study found that the dystonia can progress outside of playing their instrument. Treatments such as splinting and sensorimotor retraining for focal hand dystonia have shown to be beneficial with varying results. It is important to consider the mental health and social aspect of this condition as a musician's self-identity and career can be affected considerably. The approach to treating musician's dystonia requires an understanding and collaboration with the musician to obtain a complete history and tailor treatment and education effectively. The most successful treatment for musician's dystonia has been a multidisciplinary approach, involving physicians, physical therapists, physiotherapists, psychologists, and music instructors, focusing on rehabilitation that can involve retraining the brain to play their instrument again and even learning to play other instruments.


Noted patients

*
Scott Adams Scott Raymond Adams (born June 8, 1957) is an American author and cartoonist. He is the creator of the syndicated ''Dilbert'' comic strip, and the author of several nonfiction works of satire, commentary, and business. ''Dilbert'' gained nation ...
, the writer of the ''
Dilbert ''Dilbert'' is an American comic strip written and illustrated by Scott Adams, first published on April 16, 1989. It is known for its satirical office humor about a white-collar, micromanaged office with engineer Dilbert as the title characte ...
'' comics, has focal dystonia of the right hand, which impedes his artwork. * Tom Adams, bluegrass banjo player, has focal dystonia in his right hand, and has switched to the guitar. *
Badi Assad Badi Assad (born 23 December 1966) is a Brazilian singer, composer and guitarist in the jazz and worldbeat genres. Early life and education Assad was born in São João da Boa Vista in the state of São Paulo, but lived in Rio de Janeiro unt ...
, Brazilian singer-guitarist, was diagnosed with focal dystonia in 1999; she eventually recovered and resumed her career. *
Andy Billups The Hamsters were a British band from Southend-on-Sea, Essex, England. They performed their first live show at the Cliffs Pavilion, Southend-on-Sea, on 1 April 1987, and their last at The Half Moon, Putney on 1 April 2012, exactly 25 years l ...
, bass guitarist with British rock group, The Hamsters, has made a partial recovery; he plays by using modified guitar plectrums. *
Liona Boyd Liona Maria Carolynne Boyd, (born 11 July 1949) is a classical guitarist often referred to as the First Lady of the Guitar. Music career Early years Boyd was born in London and grew up in Toronto. Her father grew up in Bilbao, Spain, and her ...
, Canadian classical guitarist, publicized 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 has been performing again as a guitarist, singer, and songwriter. * Berkley Breathed, American Pulitzer Prize winning comic strip artist and book writer/illustrator. Known for ''
Bloom County ''Bloom County'' is an American comic strip by Berkeley Breathed which originally ran from December 8, 1980, until August 6, 1989. It examined events in politics and culture through the viewpoint of a fanciful small town in Middle America, wh ...
'', ''
Opus ''Opus'' (pl. ''opera'') is a Latin word meaning "work". Italian equivalents are ''opera'' (singular) and ''opere'' (pl.). Opus or OPUS may refer to: Arts and entertainment Music * Opus number, (abbr. Op.) specifying order of (usually) publicatio ...
'', and others. His work was featured as that of the main character, as an adult, of the film '' Second Hand Lions''. * Stuart Cassells, founder of the bagpipe rock group
Red Hot Chilli Pipers Red Hot Chilli Pipers are a Celtic rock band from Scotland. Formed in 2002, they became popular internationally in 2007 after winning the BBC talent show ''When Will I Be Famous?'' The band's lineup features three highland bagpipers and trad ...
, announced focal dystonia in September 2011; he has left the band. *
Andrew Dawes Andrew Dawes (February 7, 1940 – October 30, 2022) was a Canadian violinist. He was known for his performances with the Orford String Quartet. Early life and education Dawes was born in High River, Alberta.Curtin Call: A Photographer's Candi ...
, noted violinist and co-founder of the
Orford String Quartet The Orford String Quartet was a Canadian string quartet active from 1965 through 1991. They came to be the leading string quartet in Canada, and were well-known internationally. Founding In 1951, Gilles Lefebvre launched a summer music camp for L ...
. * Warren Deck, former tubist of the New York Philharmonic. *
Keith Emerson Keith Noel Emerson (2 November 1944 – 11 March 2016) was an English keyboardist, songwriter, and record producer. He played keyboards in a number of bands before finding his first commercial success with the Nice in the late 1960s. He became ...
, pianist and keyboard player *
Leon Fleisher Leon Fleisher (July 23, 1928 – August 2, 2020) was an American classical pianist, conductor and pedagogue. He was one of the most renowned pianists and pedagogues in the world. Music correspondent Elijah Ho called him "one of the most re ...
, an international concert pianist, dealt with this condition 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 recommenced performing and recording with two hands. *
Dominic Frasca Dominic Frasca (born 5 April 1967) is a guitarist, originally from Akron, Ohio, USA, but living in New York City since the early 1990s. He began playing hard rock guitar at age 13, but gravitated into classical after finding an ad for classical g ...
, guitarist *
Reinhard Goebel Reinhard Goebel (; born 31 July 1952 in Siegen, West Germany) is a German Conducting, conductor and baroque violin, violinist specialising in early music on Historically informed performance, authentic instruments and professor for historical perf ...
, Baroque violinist, switched to playing left-handed. *
Gary Graffman Gary Graffman (born October 14, 1928) is an American classical pianist, teacher and administrator. Early life Graffman was born in New York City to Russian-Jewish parents. Having started piano at age 3, Graffman entered the Curtis Institute of M ...
, pianist, who changed to performing only with his left hand. *
Jang Jae-in Jang Jae-in (Hangul: 장재인, born June 6, 1991), also known as Jang Jane, is a South Korean singer-songwriter known for her unique voice. Jang became well known after she finished third in the South Korean reality television series '' Superstar ...
, Korean singer-songwriter and guitarist diagnosed with dystonia in her left hand in 2012. In 2015, on
You Hee-yeol's Sketchbook ''You Hee-yeol's Sketchbook'' () is a South Korean talk show and live music television program airing from April 24 2009 until July 22nd 2022. It was hosted by You Hee-yeol, also known as his one-man band, Toy. The program has aired since April ...
, she announced that she quit playing guitar. *
Alex Klein Alex Klein (born 1964, Porto Alegre) 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 o ...
, principal oboist of the
Chicago Symphony Orchestra The Chicago Symphony Orchestra (CSO) was founded by Theodore Thomas in 1891. The ensemble makes its home at Orchestra Hall in Chicago and plays a summer season at the Ravinia Festival. The music director is Riccardo Muti, who began his tenure ...
* Alfred Koffler, guitarist of rock band
Pink Cream 69 Pink Cream 69 is a German hard rock/ heavy metal band founded in 1987 in Karlsruhe by Andi Deris, Dennis Ward, Kosta Zafiriou and Alfred Koffler. The band gained their first contract by winning music magazine ''Metal Hammer''s newcomer compe ...
, diagnosed in early 2000s. To help him with live shows, Uwe Reitenauer was hired as a second guitarist. Koffler continues to write and perform live on stage with the band. * David Leisner, classical guitarist, has recovered the full use of his hand after a decade of disability. * Billy McLaughlin, guitarist, switched to playing left-handed when affected by dystonia. *
Christian Münzner Christian Münzner (born 21 August 1981) is a German guitarist who plays for the bands Obscura and Alkaloid. He is also known for playing for the technical death metal band Necrophagist from 2002 until 2006, appearing on their 2004 release ''Epi ...
, lead guitarist of progressive extreme metal band Obscura *
Apostolos Paraskevas Apostolos Paraskevas is a Grammy nominated composer and guitarist. He was born in Volos, Greece. Parents of Apostolos were Panayiotis Paraskevas (1925-1990) and Chrysoula Paraskevas (1929-2002). Apostolos Paraskevas is a published recording ar ...
, Greek-American classical guitarist-composer, was struck by focal dystonia to his right hand in 2009. He fully recovered in 2013. *
Charlie Parr Charlie Parr is an American country blues musician. Born in Austin, Minnesota, he spent part of his childhood in Hollandale before starting his music career in Duluth. His influences include Charlie Patton, Bukka White, Reverend Gary Davis, Dave ...
, American country blues musician from Minnesota *
Alex Webster Alex Webster (born 1969) is an American bass player who is best known as a member of the death metal band Cannibal Corpse. He is one of two remaining members of the original lineup of the band, along with drummer Paul Mazurkiewicz. He is al ...
, bassist *
Victor Wooten Victor Lemonte Wooten (born September 11, 1964) is an American bassist, songwriter, and record producer. He has been the bassist for Béla Fleck and the Flecktones since the group's formation in 1988 and a member of the band SMV with two other ...
, bassist, has it on both hands


References


Sources

* Tubiana, Raoul, Amadio, Peter C.
Medical Problems of the Instrumentalist Musician
UK; Martin Dunitz (2000); 295-397 *
Farias, Joaquin
"Limitless. How your movements can heal your brain. An essay on the neurodynamics of dystonia". Galene Editions 2016.
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. * * *
Farias, Joaquin
- Farias Technique * Gorrie, Jon
"Musician's Focal Dystonia"
{{DEFAULTSORT:Focal Dystonia Dystonia Music psychology