G24.8 - Other dystonia

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Acquired torsion dystonia
• Autosomal dominant idiopathic familial dystonia
• Autosomal dominant idiopathic familial dystonia
• Autosomal recessive idiopathic familial dystonia
• Brain dopamine-serotonin vesicular transport disease
• Cranial dystonia
• Cranio-cervical dystonia with laryngeal and upper limb involvement
• Diurnal dystonia
• Dopa responsive dystonia due to sepiapterin reductase deficiency
• Dystonia 16
• Dystonia caused by toxin
• Dystonia due to Leigh syndrome
• Dystonia lenticularis
• Facial myokymia
• Familial dyskinesia and facial myokymia
• Focal dystonia
• Fragments of torsion dystonia
• Generalized dystonia
• Hemidystonia
• Hemidystonia hemiatrophy syndrome
• Idiopathic familial dystonia
• Idiopathic familial dystonia
• Idiopathic familial dystonia
• Infantile-onset generalized dyskinesia with orofacial involvement
• Isolated cervical dystonia
• Laryngeal dystonia
• Myoclonic dystonia
• Occupational dystonia
• Orofacial dyskinesia
• Paroxysmal dystonia
• Paroxysmal exertion-induced dyskinesia
• Posthemiplegic dystonia
• Primary dystonia 21
• Primary dystonia type 13
• Primary dystonia type 2
• Segmental dystonia
• Sleep-related dystonia

Clinical Information

• Dystonia-. an attitude or posture due to the co-contraction of agonists and antagonist muscles in one region of the body. it most often affects the large axial muscles of the trunk and limb girdles. conditions which feature persistent or recurrent episodes of dystonia as a primary manifestation of disease are referred to as dystonic disorders. (adams et al., principles of neurology, 6th ed, p77)
• Dystonia Musculorum Deformans-. a condition characterized by focal dystonia that progresses to involuntary spasmodic contractions of the muscles of the legs, trunk, arms, and face. the hands are often spared, however, sustained axial and limb contractions may lead to a state where the body is grossly contorted. onset is usually in the first or second decade. familial patterns of inheritance, primarily autosomal dominant with incomplete penetrance, have been identified. (adams et al., principles of neurology, 6th ed, p1078)
• Dystonic Disorders-. acquired and inherited conditions that feature dystonia as a primary manifestation of disease. these disorders are generally divided into generalized dystonias (e.g., dystonia musculorum deformans) and focal dystonias (e.g., writer's cramp). they are also classified by patterns of inheritance and by age of onset.
• Meige Syndrome-. a syndrome characterized by orofacial dystonia; including blepharospasm; forceful jaw opening; lip retraction; platysma muscle spasm; and tongue protrusion. it primarily affects older adults, with an incidence peak in the seventh decade of life. (from adams et al., principles of neurology, 6th ed, p108)
• Nocturnal Paroxysmal Dystonia-. a parasomnia characterized by paroxysmal episodes of choreoathetotic, ballistic, dystonic movements, and semipurposeful activity. the episodes occur during non-rapid eye movement sleep and typically recur several times per night. (neurology 1992 jul;42(7 suppl 6):61-67; adams et al., principles of neurology, 6th ed, p391)
• Tardive Dyskinesia-. drug-related movement disorder characterized by uncontrollable movements in certain muscles. it is associated with a long-term exposure to certain neuroleptic medications (e.g., metoclopramide).
• Torticollis-. a symptom, not a disease, of a twisted neck. in most instances, the head is tipped toward one side and the chin rotated toward the other. the involuntary muscle contractions in the neck region of patients with torticollis can be due to congenital defects, trauma, inflammation, tumors, and neurological or other factors.
• Metoclopramide-. a dopamine d2 antagonist that is used as an antiemetic.
• ATP1A3 wt Allele|AHC2|ATP1A1|ATPase Na+/K+ Transporting Subunit Alpha 3 wt Allele|ATPase, Na(+)/K(+), Alpha III Gene|ATPase, Na+/K+ Transporting, Alpha 3 Polypeptide Gene|ATPase, Na+/K+ Transporting, Alpha-3 Polypeptide Gene|CAPOS|DYT12|Dystonia 12 Gene|Na+, K+ Activated Adenosine Triphosphatase Alpha Subunit 3 Gene|Na+, K+ Activated Adenosine Triphosphatase Alpha Subunit Gene|Na+/K+ ATPase 3 Gene|RDP|Sodium-Potassium-ATPase, Alpha 3 Polypeptide Gene|Sodium-Potassium-ATPase, Alpha-3 Polypeptide Gene-. human atp1a3 wild-type allele is located in the vicinity of 19q13.2 and is approximately 31 kb in length. this allele, which encodes sodium/potassium-transporting atpase subunit alpha-3 protein, plays a role in the exchange of sodium and potassium ions across the plasma membrane of neurons, resulting in a electrochemical gradient that promotes nutrient transport. mutation of the gene is associated with alternating hemiplegia of childhood 2, cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (capos) syndrome and dystonia type 12.
• Autosomal Dominant Torsion Dystonia 1|DYT1-. an autosomal dominant inherited disorder caused by mutations in the tor1a gene. it usually begins in childhood or adolescence and is characterized by involuntary muscle contractions in the arms, legs, trunk, and neck.
• Autosomal Recessive Torsion Dystonia 2|DYT2|Dystonia Musculorum Deformans 2-. an autosomal recessive inherited disorder caused by mutation in the hpca gene. it begins in childhood or adolescence and is characterized by involuntary, sustained muscle contractions and torsions affecting initially distal limbs and later the neck, orofacial, and craniocervical regions.
• Dopamine Transporter Deficiency Syndrome|PKDYS|Parkinsonism-Dystonia, Infantile-. an autosomal recessive condition caused by mutation(s) in the slc6a3 gene, encoding sodium-dependent dopamine transporter. it is characterized by parkinsonian features and has an onset in early infancy.
• Dopa-Responsive Dystonia|DYT5 Dystonia|Segawa's Disease-. a genetic disorder in females that presents in early childhood and is responsive to dopamine. it is characterized by clubfeet and parkinsonian symptoms that may progress from lower to upper extremities witha diurnal pattern, and involuntary muscle contractions and other uncontrolled movements in the lower limbs that worsen with excercise and improve with rest.
• Dystonia-. a movement disorder characterized by sustained or intermittent muscle contractions, resulting in abnormal movements and/or postures.
• Dystonia 12|DYT12|Rapid-Onset Dystonia-Parkinsonism-. an autosomal dominant condition caused by mutation(s) in the atp1a3 gene, encoding sodium/potassium-transporting atpase subunit alpha-3. it is characterized by abrupt onset of dystonia and parkinsonism in young adulthood, often triggered by physical or psychological stress.
• Dystonia 16|DYT16-. an extremely rare autosomal recessive dystonia-parkinsonism condition caused by mutation(s) in the prkra gene encoding interferon-inducible double-stranded rna-dependent protein kinase activator a.
• Dystonia Evaluation|DYSTONIA|Dystonia|Dystonia-. an evaluation of dystonia.
• Dystonin|230 kDa Bullous Pemphigoid Antigen|230/240 kDa Bullous Pemphigoid Antigen|Bullous Pemphigoid Antigen|Bullous Pemphigoid Antigen 1|Bullous Pemphigoid Antigen 1, 230/240kDa|DST|Dystonia Musculorum Protein|Hemidesmosomal Plaque Protein|Trabeculin-Beta-. dystonin (7570 aa, ~860 kda) is encoded by the human dst gene. this protein plays a structural role in cytoskeleton networks.
• Early Onset Primary Dystonia|Early Onset Torsion Dystonia|Early Onset Torsion Dystonia|Oppenheim's Dystonia|Oppenheim's Dystonia-. a genetic disorder that usually presents in early childhood and is characterized by muscle contractions in a foot, leg, or arm that gradually spreads to other body regions.
• Episodic Kinesigenic Dyskinesia-1|DYT10|Dystonia 10|EKD1|PKD Dystonia|Paroxysmal Kinesigenic Dyskinesia-. an autosomal dominant condition caused by mutation(s) in the prrt2 gene, encoding proline-rich transmembrane protein 2. it is characterized by dyskinesia triggered by sudden movement. it shares features with infantile convulsions and paroxysmal choreoathetosis, familial. it is an allelic disorder.
• ESRS-A - CGI-S Dystonia|ESRSA1-CGI-S Dystonia|ESRSA1-CGI-S Dystonia|ESRSA126-. extrapyramidal symptom rating scale-abbreviated (esrs-a) clinical global impression (cgi-s): dystonia.
• ESRS-A - Dystonia: Eyes/Upper and Lower Face/Larynx|ESRSA1-Dystonia: Eyes/Face/Larynx|ESRSA1-Dystonia: Eyes/Face/Larynx|ESRSA113-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: eyes, upper face, lower face, larynx.
• ESRS-A - Dystonia: Hips/Lower Limbs/Feet|ESRSA1-Dystonia: Hips/Lower Limbs/Feet|ESRSA1-Dystonia: Hips/Lower Limbs/Feet|ESRSA115-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: hips, lower limbs, feet.
• ESRS-A - Dystonia: Jaw|ESRSA1-Dystonia: Jaw|ESRSA1-Dystonia: Jaw|ESRSA112-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: jaw.
• ESRS-A - Dystonia: Shoulder/Upper Limbs/Hands|ESRSA1-Dystonia: Shoulder/Up Limb/Hand|ESRSA1-Dystonia: Shoulder/Up Limb/Hand|ESRSA114-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: shoulders, upper limbs, hands.
• ESRS-A - Dystonia: Tongue|ESRSA1-Dystonia: Tongue|ESRSA1-Dystonia: Tongue|ESRSA111-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: tongue.
• ESRS-A - Dystonia: Trunk/Neck|ESRSA1-Dystonia: Trunk/Neck|ESRSA1-Dystonia: Trunk/Neck|ESRSA116-. extrapyramidal symptom rating scale-abbreviated (esrs-a) dystonia: trunk, neck.
• Idiopathic Torsion Dystonia-. torsion dystonia for which no underlying cause has been identified.
• MDS-UPDRS - Painful Off-state Dystonia|UPD2-Painful Off-state Dystonia|UPD2-Painful Off-state Dystonia|UPD2406-. the movement disorder society version of the unified parkinson's disease rating scale (mds-updrs) painful off-state dystonia.
• Spasmodic Torticollis|Cervical Dystonia-. a rare movement disorder of unknown etiology, characterized by painful, involuntary turns of the head to the right, left, upwards, or downwards.
• TH wt Allele|DYT14|DYT5b|Dystonia 14 Gene|TYH|Tyrosine Hydroxylase wt Allele-. human th wild-type allele is located in the vicinity of 11p15.5 and is approximately 8 kb in length. this allele, which encodes tyrosine 3-monooxygenase protein, is involved in tyrosine metabolism. mutation of the gene is associated with autosomal recessive segawa syndrome.
• TOR1A wt Allele|DQ2|DYT1|Dystonia 1, Torsion (Autosomal Dominant; Torsin A) Gene|TA|TORA|Torsin A Gene|Torsin Family 1 Member A wt Allele|Torsin Family 1, Member A (Torsin A) Gene-. human tor1a wild-type allele is located in the vicinity of 9q34.11 and is approximately 11 kb in length. this allele, which encodes torsin-1a protein, is involved in chaperone activity. mutation of the gene is associated with autosomal dominant torsion dystonia 1.
• Torsin-1A|Dystonia 1 Protein|EC 3.6.4.-|TOR1A|Torsin 1A|Torsin A|Torsin ATPase 1|Torsin ATPase-1A|Torsin Family 1 Member A-. torsin-1a (332 aa, ~38 kda) is encoded by the human tor1a gene. this protein plays a role in protein folding, maturation and localization.
• Torsion Dystonia 6|DYT6-. an autosomal dominant condition caused by mutation(s) in the thap1 gene, encoding thap domain-containing protein 1. it is characterized by dystonic craniofacial movements, dysarthria, and dysphagia. limb involvement is common.
• TUBB4A wt Allele|Class IVa Beta-Tubulin Gene|DYT4|Dystonia 4, Torsion (Autosomal Dominant) Gene|TUBB4|TUBB5|Tubulin Beta 4A Class IVa wt Allele|Tubulin, Beta 4 Class IVa Gene|Tubulin, Beta 4 Gene|Tubulin, Beta, 5 Gene|Tubulin, Beta, Class IVa Gene|Tubulin, Beta-4A Gene|beta-5-. human tubb4a wild-type allele is located in the vicinity of 19p13.3 and is approximately 9 kb in length. this allele, which encodes tubulin beta-4a chain protein, plays a role in microtubule structure and function. mutations in the gene are associated with hypomyelinating leukodystrophy-6 and autosomal dominant torsion dystonia-4.
• Tyrosine Hydroxylase Deficiency|Dystonia, Dopa-Responsive, Autosomal Recessive|Parkinsonism, Infantile, Autosomal Recessive|Segawa Syndrome, Autosomal Recessive-. an autosomal recessive condition caused by mutation(s) in the th gene, encoding tyrosine 3-monooxygenase. it is characterized by onset in infancy of dopa-responsive dystonia.
• UHDRS 1999 Version - Maximal Dystonia: LLE|UHDR1-Maximal Dystonia: LLE|UHDR1-Maximal Dystonia: LLE|UHDR111E-. unified huntington's disease rating scale 1999 version (uhdrs 1999 version) maximal dystonia-lle.
• UHDRS 1999 Version - Maximal Dystonia: LUE|UHDR1-Maximal Dystonia: LUE|UHDR1-Maximal Dystonia: LUE|UHDR111C-. unified huntington's disease rating scale 1999 version (uhdrs 1999 version) maximal dystonia-lue.
• UHDRS 1999 Version - Maximal Dystonia: RLE|UHDR1-Maximal Dystonia: RLE|UHDR1-Maximal Dystonia: RLE|UHDR111D-. unified huntington's disease rating scale 1999 version (uhdrs 1999 version) maximal dystonia-rle.
• UHDRS 1999 Version - Maximal Dystonia: RUE|UHDR1-Maximal Dystonia: RUE|UHDR1-Maximal Dystonia: RUE|UHDR111B-. unified huntington's disease rating scale 1999 version (uhdrs 1999 version) maximal dystonia-rue.
• UHDRS 1999 Version - Maximal Dystonia: Trunk|UHDR1-Maximal Dystonia: Trunk|UHDR1-Maximal Dystonia: Trunk|UHDR111A-. unified huntington's disease rating scale 1999 version (uhdrs 1999 version) maximal dystonia-trunk.
• UPDRS - Complications of Therapy: Dyskinesias - Presence of Early Morning Dystonia|UPD1-Complications:Dyskinesias Dystonia|UPD1-Complications:Dyskinesias Dystonia|UPD135-. unified parkinson's disease rating scale (updrs) complications of therapy (in the past week); dyskinesias, presence of early morning dystonia (historical information.).
• X-Linked Dystonia Parkinsonism|DYT3|Dystonia 3|X-Linked Dystonia-Parkinsonism|XDP-. an x-linked recessive inherited movement disorder caused by mutations in and near the taf1 gene. it is found only in people of filipino descent. it is characterized by parkinsonism and later in life the development of involuntary, sustained muscle contractions.
• X-Linked Dystonia-Parkinsonism|DYT3|Dystonia 3|XDP-. an x-linked recessive condition caused by mutation(s) in the taf1 gene, encoding transcription initiation factor tfiid subunit 1. it is characterized by torsion dystonia and parkinsonism.

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Patient Education

Dystonia

Dystonia is a movement disorder that causes involuntary contractions of your muscles. These contractions result in twisting and repetitive movements. Sometimes they are painful.

Dystonia can affect just one muscle, a group of muscles or all of your muscles. Symptoms can include tremors, voice problems or a dragging foot. Symptoms often start in childhood. They can also start in the late teens or early adulthood. Some cases worsen over time. Others are mild.

Some people inherit dystonia. Others have it because of another disease. Researchers think that dystonia may be due to a problem in the part of the brain that handles messages about muscle contractions. There is no cure. Doctors use medicines, Botox injections, surgery, physical therapy, and other treatments to reduce or eliminate muscle spasms and pain.

NIH: National Institute of Neurological Disorders and Stroke

Code History

• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)