There is no evidence-based criterion for treating<unk> , and there have been no large controlled trials of treatments for the condition. The rarity of the disease complicates efforts to establish guidelines. GABAA agonists, usually diazepam but sometimes other benzodiazepines, are the primary treatment for<unk> . Drugs that increase GABA activity alleviate muscle stiffness caused by a lack of GABAergic tone. They increase pathways that are dependent upon GABA and have muscle relaxant and anticonvulsant effects, often providing symptom relief. Because the condition worsens over time, patients generally require increased dosages, leading to more side effects. For this reason, gradual increase in dosage of benzodiazepines is indicated. Baclofen, a GABAB agonist, is generally used when individuals taking high doses of benzodiazepines have high side effects. In some cases it has shown improvements in electrophysiological and muscle stiffness when administered intravenously. Intrathecal baclofen administration may not have long-term benefits though, and there are potential serious side effects. Treatments that target the autoimmune response are also used. Intravenous immunoglobin is the best second-line treatment for<unk> . It often decreases stiffness and improves quality of life and startle reflex. It is generally safe, but there are possible serious side effects and it is expensive. The European Federation of Neurological Societies suggests it be used when disabled patients do not respond well to diazepam and baclofen. Steroids, rituximab, and plasma exchange have been used to suppress the immune system in<unk>  patients, but the efficacy of these treatments is unclear. Botulinum toxin has been used to treat<unk> , but it does not appear to have long-term benefits and has potential serious side effects. In paraneoplastic cases, tumors must be managed for the condition to be contained. Opiates are sometimes used to treat severe pain, but in some cases they exacerbate symptoms. Hematopoietic stem cell transplantation (HSCT) with high intensity conditioning protocol has been performed in a few cases with severe anti-GAD positive<unk> , resulting in clinical remission. In carefully selected cases of severe, treatment refractory<unk> , HSCT may be an effective therapeutic option.
Stiff-person syndrome
Parkinson-plus syndrome
Dejerine–Roussy syndrome
Mauriac syndrome
Stiff skin syndrome
Dopamine-responsive dystonia
Huntington's disease-like syndrome
Benign fasciculation syndrome