Main treatment options in stiff person syndrome
| Agent | Action | Daily doses | Side effects |
|---|---|---|---|
| Drugs treating symptoms | |||
| Benzodiazepines, eg, diazepam, clonazepam | GABA-A agonist | Diazepam 5–100 mg (divided doses) Clonazepam 1–6 mg (divided doses) (though often doses are far higher) | Drowsiness, vertigo, dysarthria, respiratory depression |
| Baclofen | GABA-B agonist | Oral 5–60 mg (divided doses) Intrathecal 50–800 μg/day | Drowsiness, vertigo |
| Antiepileptics, eg, levetiracetam, gabapentin | GABA-ergic and other actions | Levetiracetam 2000 mg Gabapentin 3600 mg | Variable |
| Other options: tizanidine, dantrolene, botulinum toxin | |||
| Drugs that modulate the immune process | |||
| IVIG | Incompletely understood | 2 g/kg | Infusion reactions including anaphylaxis, thrombotic events, headaches, aseptic meningitis |
| Plasma exchange | Incompletely understood | 5–6 Exchanges | Hypotension, bleeding, allergic reaction, severe immune suppression |
| Rituximab | B cell depletion | 2 g (Divided doses) | Breathing problems, arrhythmia and rarely skin reactions (Stevens–Johnson syndrome) and progressive multifocal leucoencephalopathy |
Other options: corticosteroids, mycophenolate mofetil, cyclophosphamide, cyclosporine, tacrolimus, sirolimus.
Other treatments: analgesics, physiotherapy, occupational therapy.
GABA, γ-aminobutyric acid; IVIG, intravenous immunoglobulin.