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) 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: Draft - Pending Review

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. J Neurosurg 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)