Table 2

Drugs commonly used in the symptomatic management of stiff-person syndrome (SPS), their mechanism of action and doses

Mechanism of actionDrug nameDoseOther comments
Centrally acting GABA-B receptor agonistBaclofen5 mg three times per day to 30 mg three times per dayOften used as first line to minimise dependence potential of benzodiazepines
GABA-A receptor modulationDiazepam 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 synthesisGabapentin300 mg three times a day can be increased as toleratedHelps relieve painful spasms
Centrally acting α2 adrenergic receptor agonistTizanidineStart at 2 mg and increase by 2 mg every 3–4 days to 2 mg three times per dayEspecially helpful for painful spasms
Inhibit GABA reuptakeTiagabine4–12 mg/day in divided doses
Potentiation of GABAergic transmissionLevetiracetam1000–2000 mg in two divided doses
Anti-spasmodic (likely GABA-A agonist)Methocarbamol1000 mg four times per dayFor painful spasms
Anti-spasmodic (5-HT2 receptor antagonist)Cyclobenzaprine10 mg three times per dayParadoxical worsening possible
Anti-spasmodic (decreases pre-synaptic acetylcholine release)Botulinum toxinVariableFor focal SPS
Ryanodine receptor bindingDantrolene100–200 mg in four divided dosesUseful in status spasticus
  • GABA, gamma amino butyric acid.