Skip to content

Dystonia

DIAGNOSIS: Dystonia ICD-10: G24.9 (Dystonia, unspecified); G24.1 (Genetic torsion dystonia); G24.2 (Idiopathic nonfamilial dystonia); G24.3 (Spasmodic torticollis); G24.5 (Blepharospasm); G24.8 (Other dystonia)

CPT CODES: 85025 (CBC with differential), 80048 (BMP), 80076 (Hepatic panel (AST, ALT, Alk Phos, bilirubin, albumin)), 82390 (Ceruloplasmin), 84443 (TSH), 82525 (24-hour urine copper), 82607 (Vitamin B12), 82728 (Ferritin), 84550 (Uric acid), 83605 (Lactate), 82310 (Calcium), 82550 (CK), 81479 (ATP7B gene sequencing), 83519 (CSF neurotransmitter metabolites (HVA, 5-HIAA, pterins)), 85060 (Acanthocyte smear), 82657 (Lysosomal enzyme panel), 86255 (Anti-neuronal antibodies (NMDAR, LGI1, CASPR2)), 86235 (Paraneoplastic antibody panel), 70553 (MRI Brain without and with contrast), 92012 (Slit lamp examination by ophthalmology), 95907-95913 (EMG with dystonia protocol), 78607 (DaTscan (ioflupane I-123)), 72156 (MRI Spine (cervical/thoracic)), 78816 (FDG-PET Brain) SYNONYMS: Torsion dystonia, spasmodic torticollis, cervical dystonia, blepharospasm, writer's cramp, Meige syndrome, DYT1 dystonia, dopa-responsive dystonia, Segawa disease, focal dystonia, generalized dystonia, segmental dystonia, hemidystonia, task-specific dystonia, musician's dystonia SCOPE: Diagnosis, classification, workup for secondary causes, and symptomatic management of dystonia. Covers focal (cervical, blepharospasm, writer's cramp), segmental, and generalized forms. Includes evaluation for Wilson's disease, dopa-responsive dystonia, and drug-induced dystonia.

VERSION: 1.1 CREATED: January 27, 2026 REVISED: January 30, 2026 STATUS: Approved

PRIORITY KEY: STAT = Immediate | URGENT = Within hours | ROUTINE = Standard | EXT = Extended/atypical cases | - = Not applicable to this setting

SECTION A: ACTION ITEMS

1. LABORATORY WORKUP

1A. Essential/Core Labs

Test ED HOSP OPD ICU Rationale Target Finding
CBC with differential (CPT 85025) URGENT ROUTINE ROUTINE STAT Baseline; exclude systemic illness Normal
BMP (CPT 80048) STAT ROUTINE ROUTINE STAT Electrolyte abnormalities can exacerbate movement disorders Normal
Hepatic panel (AST, ALT, Alk Phos, bilirubin, albumin) (CPT 80076) URGENT ROUTINE ROUTINE - Screen for hepatic dysfunction suggesting Wilson's disease Normal
Ceruloplasmin (CPT 82390) - ROUTINE ROUTINE - Wilson's disease screen; essential for age <50 with dystonia >20 mg/dL (low suggests Wilson's)
TSH (CPT 84443) URGENT ROUTINE ROUTINE - Hyperthyroidism can cause movement disorders Normal (0.4-4.0 mIU/L)

1B. Extended Workup (Second-line)

Test ED HOSP OPD ICU Rationale Target Finding
24-hour urine copper (CPT 82525) - ROUTINE ROUTINE - Wilson's disease confirmation if ceruloplasmin low/borderline <40 mcg/24hr (elevated in Wilson's)
Serum copper (CPT 82525) - ROUTINE ROUTINE - Interpret with ceruloplasmin (low free copper ratio in Wilson's) 75-145 mcg/dL
Vitamin B12 (CPT 82607) - ROUTINE ROUTINE - Deficiency causes myelopathy and movement disorders >300 pg/mL
Ferritin (CPT 82728), serum iron (CPT 83540), TIBC (CPT 83550) - ROUTINE ROUTINE - Neurodegeneration with brain iron accumulation (NBIA) Normal
Uric acid (CPT 84550) - - ROUTINE - Low in ataxia-telangiectasia, Lesch-Nyhan Normal
Lactate (CPT 83605), pyruvate (CPT 84210) - ROUTINE ROUTINE - Mitochondrial disorders Normal
Calcium (CPT 82310), magnesium (CPT 83735) STAT ROUTINE ROUTINE STAT Hypocalcemia, hypomagnesemia can cause movement disorders Normal
CK (CPT 82550) STAT ROUTINE - STAT Elevated in dystonia crises, NMS, prolonged dystonic posturing Normal or mildly elevated

1C. Rare/Specialized (Refractory or Atypical)

Test ED HOSP OPD ICU Rationale Target Finding
ATP7B gene sequencing (CPT 81479) - - EXT - Definitive Wilson's disease diagnosis No pathogenic variants
GCH1 gene sequencing (CPT 81479) - - EXT - Dopa-responsive dystonia (DYT5a) No pathogenic variants
TOR1A gene sequencing (CPT 81479) - - EXT - DYT1 dystonia (early-onset generalized) No GAG deletion
THAP1 gene sequencing (CPT 81479) - - EXT - DYT6 dystonia No pathogenic variants
Dystonia gene panel (multi-gene NGS) (CPT 81479) - - EXT - Atypical presentations; family history; early onset No pathogenic variants
CSF neurotransmitter metabolites (HVA, 5-HIAA, pterins) (CPT 83519) - - EXT - Dopa-responsive dystonia; neurotransmitter disorders Normal ratios
Acanthocyte smear (CPT 85060) - - EXT - Chorea-acanthocytosis, McLeod syndrome No acanthocytes
Lysosomal enzyme panel (CPT 82657) - - EXT - Lysosomal storage disorders (GM1, GM2 gangliosidosis) Normal enzyme activity
Anti-neuronal antibodies (NMDAR, LGI1, CASPR2) (CPT 86255) - EXT EXT - Autoimmune dystonia (rare) Negative
Paraneoplastic antibody panel (CPT 86235) - - EXT - Paraneoplastic dystonia Negative

2. DIAGNOSTIC IMAGING & STUDIES

2A. Essential/First-line

Study ED HOSP OPD ICU Timing Target Finding Contraindications
MRI Brain without and with contrast (CPT 70553) URGENT ROUTINE ROUTINE - All new dystonia cases; rule out structural lesion Normal; no basal ganglia lesions MRI-incompatible devices

2B. Extended

Study ED HOSP OPD ICU Timing Target Finding Contraindications
MRI Brain with SWI/iron sequences (CPT 70553) - ROUTINE ROUTINE - Suspected Wilson's disease or NBIA No T2 hypointensity in basal ganglia MRI contraindications
Slit lamp examination by ophthalmology (CPT 92012) - ROUTINE ROUTINE - Wilson's disease evaluation; Kayser-Fleischer rings No KF rings None
EMG with dystonia protocol (CPT 95907-95913) - - ROUTINE - Confirm diagnosis; characterize pattern; guide botulinum toxin Co-contraction of agonist/antagonist; overflow None
DaTscan (ioflupane I-123) (CPT 78607) - - ROUTINE - Differentiate dystonic tremor from PD; combined dystonia-parkinsonism Normal in primary dystonia; abnormal if parkinsonism Iodine allergy

2C. Rare/Specialized

Study ED HOSP OPD ICU Timing Target Finding Contraindications
MRI Spine (cervical/thoracic) (CPT 72156) - ROUTINE ROUTINE - Cervical dystonia with radiculopathy symptoms Normal; no cord compression MRI contraindications
FDG-PET Brain (CPT 78816) - - EXT - Atypical parkinsonism with dystonia Pattern interpretation varies None significant
Liver MRI or ultrasound (CPT 74183/76700) - ROUTINE ROUTINE - Wilson's disease staging if diagnosed Assess hepatic involvement None significant

3. TREATMENT

3A. Acute/Emergent

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Diphenhydramine IV/IM Acute dystonic reaction (drug-induced) 25 mg :: IV/IM :: PRN :: 25-50 mg IV/IM over 2-5 min; may repeat in 30 min if needed; max 100 mg Narrow-angle glaucoma; urinary retention Sedation, hypotension STAT STAT - STAT
Benztropine IV/IM Acute dystonic reaction (drug-induced) 1 mg :: IV/IM :: PRN :: 1-2 mg IV/IM; may repeat in 30 min; follow with oral 1-2 mg BID x 2-3 days to prevent recurrence Narrow-angle glaucoma; GI obstruction; myasthenia gravis Anticholinergic effects, confusion (elderly) STAT STAT - STAT
Lorazepam IV/PO Status dystonicus; severe dystonic storm 1 mg :: IV/PO :: PRN :: 1-2 mg IV slow push or 2-4 mg PO; may repeat q4-6h PRN; monitor respiratory status Severe respiratory depression; acute narrow-angle glaucoma Respiratory depression, sedation STAT STAT - STAT
Diazepam IV/PO Status dystonicus; muscle spasm relief 5 mg :: IV/PO :: PRN :: 5-10 mg IV slow push (2 mg/min) or PO; may repeat q4h PRN Severe respiratory depression; acute narrow-angle glaucoma Respiratory depression, sedation STAT STAT - STAT

3B. Symptomatic Treatments (First-line for Focal Dystonia)

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
OnabotulinumtoxinA (Botox) - Cervical dystonia IM First-line for cervical dystonia/torticollis 100 units :: IM :: q12wk :: Start 100-200 units divided among affected muscles (SCM, splenius, trapezius, levator scapulae); adjust based on response; max 300 units/session; repeat q12wk Infection at site; myasthenia gravis; ALS Dysphagia (especially bilateral SCM); neck weakness; antibody formation - - ROUTINE -
OnabotulinumtoxinA (Botox) - Blepharospasm IM First-line for blepharospasm 25 units :: IM :: q12wk :: 1.25-5 units per site; inject orbicularis oculi at multiple points; total 25-50 units per eye; repeat q12wk Infection at site; myasthenia gravis; ALS Ptosis, diplopia, dry eyes, lagophthalmos - - ROUTINE -
OnabotulinumtoxinA (Botox) - Writer's cramp IM Focal hand dystonia refractory to therapy modification 50 units :: IM :: q12wk :: 10-50 units per muscle; EMG guidance required; target FCR, FCU, FDS as needed; repeat q12wk Infection at site; myasthenia gravis; ALS Finger weakness (may impair function) - - ROUTINE -
AbobotulinumtoxinA (Dysport) - Cervical dystonia IM Alternative to onabotulinum; different potency 500 units :: IM :: q12wk :: Start 500 units divided among affected muscles; max 1000 units/session; repeat q12wk Infection at site; myasthenia gravis; ALS Dysphagia (especially bilateral SCM); neck weakness; antibody formation - - ROUTINE -
IncobotulinumtoxinA (Xeomin) - Cervical dystonia IM Alternative; no complexing proteins 120 units :: IM :: q12wk :: Start 120-200 units divided; max 300 units/session; repeat q12wk; may have lower immunogenicity Infection at site; myasthenia gravis; ALS Dysphagia (especially bilateral SCM); neck weakness; antibody formation - - ROUTINE -
RimabotulinumtoxinB (Myobloc) - Cervical dystonia IM Type A toxin non-responders; secondary non-response 2500 units :: IM :: q12wk :: Start 2500-5000 units divided; max 10000 units; repeat q12wk; higher dry mouth rate Infection at site; myasthenia gravis; ALS; type B protein allergy Dry mouth; dysphagia; neck weakness; antibody formation - - ROUTINE -

3C. Second-line/Oral Medications

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Trihexyphenidyl PO First-line oral for generalized dystonia; young patients tolerate best 1 mg :: PO :: daily :: Start 1 mg daily; increase by 1-2 mg q3-5d; typical target 6-30 mg/day divided TID; some patients require up to 60-80 mg/day Narrow-angle glaucoma; GI obstruction; urinary retention; dementia Anticholinergic effects (dry mouth, constipation, urinary retention, confusion); cognitive effects in elderly - ROUTINE ROUTINE -
Benztropine PO Alternative anticholinergic; maintenance after acute dystonic reaction 0.5 mg :: PO :: BID :: Start 0.5 mg BID; increase by 0.5 mg q5-7d; typical 1-4 mg/day; max 6 mg/day Narrow-angle glaucoma; GI obstruction; urinary retention; dementia Anticholinergic effects (dry mouth, constipation, urinary retention, confusion); cognitive effects in elderly - ROUTINE ROUTINE -
Baclofen PO Muscle relaxant; useful adjunct for dystonia 5 mg :: PO :: TID :: Start 5 mg TID; increase by 5 mg/dose q3d; typical 30-80 mg/day divided TID; max 80-120 mg/day Severe renal impairment (dose adjust); abrupt withdrawal risk Sedation, weakness; taper slowly if discontinuing - ROUTINE ROUTINE -
Clonazepam PO Adjunct for dystonia; myoclonus-dystonia 0.25 mg :: PO :: BID :: Start 0.25 mg BID; increase by 0.25-0.5 mg q3-7d; typical 1-4 mg/day; max 6 mg/day Respiratory depression; severe hepatic impairment; substance abuse history Sedation, dependence, falls; taper if discontinuing - ROUTINE ROUTINE -
Diazepam PO Alternative benzodiazepine; muscle relaxant properties 2 mg :: PO :: TID :: Start 2 mg TID; increase by 2-5 mg/day q3-7d; typical 10-40 mg/day divided Respiratory depression; severe hepatic impairment; substance abuse history Sedation, dependence, falls; taper if discontinuing - ROUTINE ROUTINE -
Levodopa/Carbidopa PO Dopa-responsive dystonia (DRD); diagnostic trial for young-onset 25/100 mg :: PO :: TID :: Start 25/100 mg TID; if DRD, dramatic response at low dose; may titrate to 50/200 mg TID; response confirms diagnosis None absolute; use caution in psychosis Nausea, orthostatic hypotension; dramatic improvement suggests DRD - ROUTINE ROUTINE -
Tetrabenazine PO Hyperkinetic movements including some dystonias 12.5 mg :: PO :: daily :: Start 12.5 mg daily; increase by 12.5 mg q1wk; max 200 mg/day; CYP2D6 testing recommended Depression; suicidality; Parkinsonism Depression, sedation, parkinsonism, akathisia; Black Box: suicidality - - ROUTINE -
Deutetrabenazine (Austedo) PO Longer half-life alternative to tetrabenazine 6 mg :: PO :: BID :: Start 6 mg BID; increase by 6 mg/day weekly; max 48 mg/day Depression; suicidality; Parkinsonism Depression, sedation, parkinsonism, akathisia; Black Box: suicidality; may be better tolerated than tetrabenazine - - ROUTINE -
Clonidine PO Adjunct; may help in some patients 0.1 mg :: PO :: BID :: Start 0.1 mg BID; increase by 0.1 mg q1wk; max 0.6 mg/day Severe bradycardia; renal impairment (adjust) Hypotension, bradycardia, sedation; rebound hypertension if stopped abruptly - ROUTINE ROUTINE -
Carbamazepine PO Paroxysmal kinesigenic dystonia/dyskinesia 100 mg :: PO :: BID :: Start 100 mg BID; increase by 100-200 mg q1wk; target 400-800 mg/day; check HLA-B*1502 in at-risk populations Bone marrow suppression; AV block; SJS risk (HLA-B*1502) CBC, LFTs, sodium; drug levels (4-12 mcg/mL) - ROUTINE ROUTINE -

3D. Disease-Modifying/Specialized Therapies

Treatment Route Indication Dosing Pre-Treatment Requirements Contraindications Monitoring ED HOSP OPD ICU
Deep brain stimulation (GPi-DBS) Surgical Medication-refractory generalized or segmental dystonia; DYT1 positive Bilateral GPi stimulation :: Surgical :: per protocol :: Bilateral GPi electrode placement; programming over weeks-months; full effect may take 6-12 months MRI; neuropsych testing; multidisciplinary evaluation; genetic testing if indicated Cognitive impairment; coagulopathy; infection; unrealistic expectations Programming optimization; battery replacement q3-5yr; speech/swallowing - - ROUTINE -
Deep brain stimulation (GPi-DBS) Surgical Cervical dystonia refractory to botulinum toxin Bilateral GPi stimulation :: Surgical :: per protocol :: Bilateral GPi electrode placement; programming over weeks-months; full effect may take 6-12 months MRI; neuropsych testing; multidisciplinary evaluation; genetic testing if indicated Cognitive impairment; coagulopathy; infection; unrealistic expectations Programming optimization; battery replacement q3-5yr; speech/swallowing - - ROUTINE -
Intrathecal baclofen pump Surgical Severe generalized dystonia with spasticity component; CP-related 25 mcg :: IT :: test dose :: Test dose 25-100 mcg; maintenance typically 100-1000 mcg/day; titrate over months IT baclofen trial with >50% improvement Infection; CSF obstruction; baclofen hypersensitivity Pump refills q1-3mo; withdrawal syndrome if interruption; MRI conditional pumps - ROUTINE ROUTINE -
D-penicillamine PO Wilson's disease with neurological symptoms 250 mg :: PO :: daily :: Start 250 mg daily; increase by 250 mg q4-7d; target 1000-1500 mg/day divided BID (take 1hr before meals); lifelong treatment 24h urine copper, LFTs, CBC, urinalysis Penicillin allergy (not absolute but caution); nephrotoxicity CBC weekly x1mo then monthly x6mo then q3mo; urinalysis monthly; LFTs monthly; neurologic worsening first 6 months possible - ROUTINE ROUTINE -
Trientine (Syprine) PO Wilson's disease; D-penicillamine intolerant 250 mg :: PO :: TID :: Start 250 mg TID; target 750-1250 mg/day divided TID; take 1hr before or 2hr after meals 24h urine copper, LFTs, CBC, urinalysis Caution with iron supplements (separate by 2hr) CBC, LFTs, urinalysis monthly initially then q3mo; fewer side effects than D-penicillamine - ROUTINE ROUTINE -
Zinc acetate (Galzin) PO Wilson's disease maintenance; presymptomatic; adjunct 50 mg :: PO :: TID :: 50 mg TID (elemental zinc); take between meals; blocks intestinal copper absorption Take separately from copper chelators GI upset 24h urine copper; serum zinc; 24h urine zinc - ROUTINE ROUTINE -

4. OTHER RECOMMENDATIONS

4A. Referrals & Consults

Recommendation ED HOSP OPD ICU
Movement disorders neurology for diagnosis confirmation, classification, and treatment planning - ROUTINE ROUTINE -
Ophthalmology for slit lamp examination to evaluate for Kayser-Fleischer rings (Wilson's disease workup) - ROUTINE ROUTINE -
Hepatology if Wilson's disease diagnosed for hepatic management and transplant evaluation if severe - ROUTINE ROUTINE -
Genetic counseling if genetic dystonia confirmed or suspected (DYT1, DYT6, GCH1) - - ROUTINE -
Neurosurgery consultation for DBS evaluation if medication-refractory - - ROUTINE -
Physical therapy for posture correction, stretching, and range of motion exercises - ROUTINE ROUTINE -
Occupational therapy for adaptive techniques and assistive devices for ADLs - ROUTINE ROUTINE -
Speech therapy if oromandibular dystonia, laryngeal dystonia, or cervical dystonia affecting swallowing - ROUTINE ROUTINE -
Pain management if chronic pain from dystonic posturing not controlled with primary treatment - - ROUTINE -
Psychology/psychiatry for coping strategies, depression/anxiety management related to chronic dystonia - - ROUTINE -

4B. Patient Instructions

Recommendation ED HOSP OPD
After botulinum toxin injection: avoid rubbing injection sites; effects develop over 3-14 days and last 10-12 weeks - - ROUTINE
Report any swallowing difficulty or breathing problems after botulinum toxin injection (may indicate spread) URGENT ROUTINE ROUTINE
Use sensory tricks (geste antagoniste) such as touching chin or face which may temporarily reduce cervical dystonia - ROUTINE ROUTINE
Return to ED if severe muscle spasms with fever, confusion, or dark urine (possible dystonic storm or rhabdomyolysis) STAT ROUTINE ROUTINE
Continue physical therapy exercises at home to maintain range of motion and prevent contractures - ROUTINE ROUTINE
Avoid medications that worsen dystonia: metoclopramide, prochlorperazine, haloperidol, and other dopamine blockers ROUTINE ROUTINE ROUTINE
For Wilson's disease: strict adherence to copper chelation is lifelong; follow dietary copper restrictions - ROUTINE ROUTINE
Keep a symptom diary noting triggers, sensory tricks that help, and response to medications - - ROUTINE

4C. Lifestyle & Prevention

Recommendation ED HOSP OPD
Stress management with relaxation techniques as stress can exacerbate dystonia - ROUTINE ROUTINE
Adequate sleep as fatigue worsens dystonia symptoms - ROUTINE ROUTINE
Limit caffeine and stimulants which may worsen tremor/dystonia - ROUTINE ROUTINE
Regular gentle exercise (swimming, yoga) to maintain flexibility without overexertion - - ROUTINE
Ergonomic workstation modifications for task-specific dystonia (writer's cramp, musician's dystonia) - - ROUTINE
For Wilson's disease: avoid high-copper foods (liver, shellfish, chocolate, nuts, mushrooms) especially during initial treatment - ROUTINE ROUTINE
Avoid alcohol especially if on benzodiazepines or with hepatic involvement (Wilson's) - ROUTINE ROUTINE
Heat application to affected muscles may provide temporary relief - ROUTINE ROUTINE
Support groups for dystonia provide coping strategies and community connection - - ROUTINE

SECTION B: REFERENCE

5. DIFFERENTIAL DIAGNOSIS

Alternative Diagnosis Key Distinguishing Features Tests to Differentiate
Drug-induced dystonia Temporal relationship with dopamine blockers (antipsychotics, antiemetics); acute or tardive Medication history; resolves with withdrawal (acute) or persists (tardive)
Wilson's disease Age <50; hepatic dysfunction; psychiatric symptoms; Kayser-Fleischer rings Ceruloplasmin (low), 24h urine copper (high), slit lamp exam, ATP7B testing
Dopa-responsive dystonia (DYT5/Segawa) Childhood onset; diurnal fluctuation (worse evening); dramatic levodopa response Levodopa trial (dramatic improvement); GCH1 gene testing
Parkinson's disease with dystonia Rest tremor; bradykinesia; rigidity; asymmetric; sustained postures DaTscan (abnormal); clinical exam; levodopa response
Psychogenic/functional dystonia Inconsistent; distractible; fixed posture from onset; associated somatization Clinical features; psychiatric evaluation; resolution with distraction
Stiff-person syndrome Axial rigidity; episodic spasms triggered by startle; anti-GAD antibodies Anti-GAD65 antibodies; EMG (continuous motor unit activity)
Tardive dystonia Prolonged dopamine blocker exposure (months-years); may persist after withdrawal History of antipsychotic/antiemetic use; AIMS assessment
Cervical spondylotic myelopathy Neck pain; sensory symptoms; hyperreflexia; Hoffmann sign MRI C-spine (cord compression, myelopathy signal)
Torticollis from posterior fossa tumor Constant head tilt; headache; papilledema; cranial nerve signs MRI Brain with contrast (tumor, hydrocephalus)
Neuroacanthocytosis/chorea-acanthocytosis Chorea; orofacial dystonia; lip/tongue biting; elevated CK Acanthocyte smear; CK; VPS13A gene testing
NBIA (neurodegeneration with brain iron accumulation) Progressive; childhood/young adult onset; parkinsonism; retinopathy MRI (eye-of-the-tiger sign); genetic panels (PANK2, etc.)
Meige syndrome Combined blepharospasm + oromandibular dystonia Clinical diagnosis; rule out secondary causes
Myoclonus-dystonia (DYT11) Myoclonic jerks + dystonia; alcohol responsive; SGCE gene SGCE gene testing; alcohol response
Paroxysmal kinesigenic dyskinesia Brief attacks (<1 min); triggered by sudden movement; responds to carbamazepine Clinical history; PRRT2 gene testing

6. MONITORING PARAMETERS

Parameter Frequency Target/Threshold Action if Abnormal ED HOSP OPD ICU
Dystonia severity (Burke-Fahn-Marsden scale, TWSTRS for cervical) Each visit 30%+ improvement with treatment Adjust botulinum toxin dose/muscles; add oral agent; consider DBS - ROUTINE ROUTINE -
Functional status and disability Each visit Improved ADLs, work function OT/PT referral; adaptive devices; treatment escalation - ROUTINE ROUTINE -
Anticholinergic side effects (dry mouth, constipation, urinary retention, cognition) Each visit on anticholinergics Tolerable side effects Reduce dose; switch agents; add symptomatic treatment - ROUTINE ROUTINE -
Dysphagia screening (after cervical botulinum toxin) 2 weeks post-injection Normal swallowing Dietary modification; speech therapy; reduce dose next session - - ROUTINE -
CK level (if severe dystonia or dystonic storm) PRN <1000 U/L IV fluids; monitor renal function; treat underlying cause STAT STAT - STAT
24-hour urine copper (Wilson's on chelation) Every 3-6 months <100 mcg/24hr on maintenance Adjust chelator dose; assess compliance - - ROUTINE -
CBC, urinalysis (on D-penicillamine) Monthly initially then q3mo Normal Hold drug if cytopenias or proteinuria; switch to trientine - ROUTINE ROUTINE -
LFTs (Wilson's disease) Monthly initially then q3mo Stable or improving Hepatology consultation if worsening - ROUTINE ROUTINE -
Depression screening (on tetrabenazine) Each visit No suicidal ideation Discontinue tetrabenazine; psychiatric referral - ROUTINE ROUTINE -
DBS parameters and battery Every 6-12 months Optimal control; battery >30% Reprogram; schedule battery replacement if needed - - ROUTINE -

7. DISPOSITION CRITERIA

Disposition Criteria
Discharge home New diagnosis with workup initiated; mild symptoms; treatment plan in place; follow-up scheduled
Admit to floor Acute dystonic reaction requiring observation; new generalized dystonia requiring workup; Wilson's disease with hepatic decompensation
Admit to ICU Status dystonicus (dystonic storm); respiratory compromise; rhabdomyolysis; severe drug-induced dystonia with airway concern
Transfer to higher level DBS programming issues requiring movement disorders center; refractory status dystonicus requiring specialized center
Outpatient follow-up 2-4 weeks after new medication started; 12 weeks for botulinum toxin reassessment; 6-12 months when stable

8. EVIDENCE & REFERENCES

Recommendation Evidence Level Source
Botulinum toxin first-line for cervical dystonia Class I, Level A Simpson DM et al. Neurology 2008 (AAN Guideline)
Botulinum toxin for blepharospasm Class I, Level A Simpson DM et al. Neurology 2008
GPi-DBS effective for generalized dystonia Class I, Level A Vidailhet M et al. NEJM 2005
DBS superior effect in DYT1-positive patients Class II, Level B Coubes P et al. J Neurosurg 2004
Trihexyphenidyl for generalized dystonia Class II, Level C Burke RE et al. Neurology 1986
Levodopa trial for suspected dopa-responsive dystonia Class II, Level B Nygaard TG et al. Neurology 1991
Dystonia classification consensus Expert consensus Albanese A et al. Mov Disord 2013
Wilson's disease diagnosis and treatment Class I, Level A Roberts EA, Schilsky ML. Hepatology 2008 (AASLD Guideline)
D-penicillamine for Wilson's disease Class I, Level A Roberts EA, Schilsky ML. Hepatology 2008
Intrathecal baclofen for severe dystonia Class II, Level B Albright AL et al. Dev Med Child Neurol 2001
Benztropine/diphenhydramine for acute dystonic reaction Class III, Level C van Harten PN et al. J Clin Psychiatry 1999
Carbamazepine for paroxysmal kinesigenic dyskinesia Class II, Level B Bruno MK et al. Brain 2004
EFNS guidelines on dystonia diagnosis and treatment Expert consensus Albanese A et al. Eur J Neurol 2011
Tetrabenazine for hyperkinetic movement disorders Class II, Level B Jankovic J, Beach J. Neurology 1997

CHANGE LOG

v1.1 (January 30, 2026) - Reformatted lab tables (1A/1B/1C) to standard column order: Test | ED | HOSP | OPD | ICU | Rationale | Target Finding - Reformatted imaging tables (2A/2B/2C) to standard column order: Study | ED | HOSP | OPD | ICU | Timing | Target Finding | Contraindications - Added inline CPT codes to all lab tests and imaging studies - Fixed structured dosing format: starting dose only in first field across all treatment sections - Expanded all cross-references ("Same as lorazepam", "Same as onabotulinum", "Same as trihexyphenidyl", "Same as clonazepam", "Same as tetrabenazine", "Same as above", "Same as D-penicillamine") with actual content - Added clinical synonyms for searchability - Added VERSION/CREATED/REVISED header block

v1.0 (January 27, 2026) - Initial template creation - Comprehensive coverage of focal, segmental, and generalized dystonia - Detailed botulinum toxin dosing for cervical dystonia, blepharospasm, and focal hand dystonia - Oral medication options including anticholinergics, baclofen, benzodiazepines - Wilson's disease and dopa-responsive dystonia workup - DBS and intrathecal baclofen for refractory cases - Structured dosing format for order sentence generation

APPENDIX A: DYSTONIA CLASSIFICATION

By Body Distribution

Type Definition Examples
Focal Single body region Cervical (torticollis), blepharospasm, writer's cramp, laryngeal
Segmental Two or more contiguous regions Cranial-cervical (Meige + cervical), brachial
Multifocal Two or more non-contiguous regions Blepharospasm + writer's cramp
Hemidystonia Ipsilateral arm and leg Usually secondary (stroke, tumor)
Generalized Trunk + at least 2 other regions DYT1 dystonia, Wilson's disease

By Etiology (Axis 2)

Category Subtype Examples
Inherited Isolated dystonia DYT1 (TOR1A), DYT6 (THAP1)
Inherited Combined dystonia DYT5 (GCH1, dopa-responsive), myoclonus-dystonia (SGCE)
Inherited Complex dystonia Wilson's disease, NBIA, Huntington's disease
Acquired Perinatal injury Cerebral palsy with dystonia
Acquired Drugs Tardive dystonia, acute dystonic reaction
Acquired Structural Stroke, tumor, demyelination
Acquired Autoimmune Anti-NMDAR encephalitis, paraneoplastic
Idiopathic Sporadic Adult-onset focal dystonia (most cervical)
Idiopathic Familial Family history without identified gene

APPENDIX B: SENSORY TRICKS (GESTE ANTAGONISTE)

Sensory tricks are voluntary maneuvers that temporarily reduce dystonic posturing. Their presence supports the diagnosis of primary dystonia.

Common Sensory Tricks by Type

Dystonia Type Common Sensory Tricks
Cervical dystonia Light touch to chin, cheek, or back of head; leaning against wall; wearing cervical collar
Blepharospasm Touching eyelid or brow; wearing dark glasses; pulling eyelid skin; singing or humming
Writer's cramp Using thicker pen; changing grip; writing on different surface
Oromandibular Chewing gum; touching lips; biting on object

Clinical Significance

  • Present in ~70-80% of cervical dystonia patients
  • Absence does not exclude diagnosis
  • Diminish over time in some patients
  • May be used therapeutically (collar, glasses)