Drugs commonly used in the symptomatic management of stiff-person syndrome (SPS), their mechanism of action and doses
Mechanism of action | Drug name | Dose | Other comments |
Centrally acting GABA-B receptor agonist | Baclofen | 5 mg three times per day to 30 mg three times per day | Often used as first line to minimise dependence potential of benzodiazepines |
GABA-A receptor modulation | Diazepam and clonazepam (alprazolam, lorazepam and temazepam—less commonly used) | Diazepam 5 mg two times to 10 mg three times per day (daily dose can be occasionally up to 100 mg/day) Clonazepam 0.5 mg to 5 mg/day | Most widely used drug for SPS. Diazepam given intravenously for status spasticus and rectally for prolonged spasms |
Enhanced GABA synthesis | Gabapentin | 300 mg three times a day can be increased as tolerated | Helps relieve painful spasms |
Centrally acting α2 adrenergic receptor agonist | Tizanidine | Start at 2 mg and increase by 2 mg every 3–4 days to 2 mg three times per day | Especially helpful for painful spasms |
Inhibit GABA reuptake | Tiagabine | 4–12 mg/day in divided doses | |
Potentiation of GABAergic transmission | Levetiracetam | 1000–2000 mg in two divided doses | |
Anti-spasmodic (likely GABA-A agonist) | Methocarbamol | 1000 mg four times per day | For painful spasms |
Anti-spasmodic (5-HT2 receptor antagonist) | Cyclobenzaprine | 10 mg three times per day | Paradoxical worsening possible |
Anti-spasmodic (decreases pre-synaptic acetylcholine release) | Botulinum toxin | Variable | For focal SPS |
Ryanodine receptor binding | Dantrolene | 100–200 mg in four divided doses | Useful in status spasticus |
GABA, gamma amino butyric acid.