MANAGEMENT OF DYSTONIA

Optimize your dystonia management with this comprehensive guide. Explore effective treatments for acute dystonia, status dystonia, tardive dystonia, and DOPA-responsive dystonia.

MANAGEMENT OF DYSTONIA

Acute Dystonia

Benztropine 1-2mg slow IV; May repeat if needed in 20 minutes. Then 1 mg po bid orally.

Alternative:

Benadryl 1mg / kg slow IV

Status Dystonia

Monitor for Myoglobinuria. Admit to ICU.

Versed drip:

Versed 0.2 mg / kg IV bolus followed by; Maintenance rate 0.05 mg / kg / hour infusion; Max rate 5 mg /kg / hour infusion

Tardive Dystonia

Trihexyphenidyl 1mg po bid. Escalate dose slowly over 4 weeks to upto 6mg po bid if needed

Alternatives: Clonazepam, Sinemet, Tetrabenazine

DOPA Responsive Dystonia. Starting dose of Levodopa 1 mg/kg/ day. Maintenance 4-5 mg/kg/day

Dystonia can be focal or generalized or multifocal or generalized.

EARLY ONSET GENERALIZED DYSTONIA

First type:

DYT1 related Early onset generalized dystonia

Rare. Caused by mutation of DYT1 gene

Posturing of a foot or arm provoked by action, such as walking or writing

Molecular genetic testing of TOR1A : 3 base pair deletion in most patients

Autosomal dominant with incomplete penetrance.

50% chance of a progency inheriting the gene, 30% chance of symptomatic disease

Second type:

Non-DYT1 related Early onset generalized dystonia

More likely to have cranial/cervical involvement compared to DYT1 type. Can be primary or secondary; Secondary means the dystonia is due to some other neurological condition.