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Rituximab induces remission in stiff person syndrome

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The first report of successful treatment with a monoclonal anti-B cell antibody may offer new hope for patients with stiff person syndrome, a rare but ultimately fatal autoimmune disease of the CNS.

Monoclonal antibody specific for B cells expressing CD20 antigen (rituximab) alleviated severe symptoms when other treatments failed. It abolished intrathecal autoantibody against glutamic acid decarboxylase (GAD), suggesting that the syndrome is a B cell mediated autoimmune disease.

The 41 year old woman was an emergency admission in January 2004 with prolonged painful muscle spasms in her neck and back and arms and legs, rendering her bedridden and dependent on carers for months previously. She was taking baclofen and dantrolene sodium daily, fentanyl patches twice weekly and parenteral diazepam up to 80 mg and diamorphine up to 25 mg daily, providing subjective benefit.

The syndrome had been diagnosed in 2001. Various antispasmodic agents and disease modifying treatments, including seven courses of intravenous immunoglobulin and courses of cytotoxic drugs, tried since then had had no lasting success. Eventually, intrathecal infusions of hydrocortisone produced transient improvement, in December 2003.

However, after just over two weeks of rituximab at 375 mg/m3, in January 2004, muscle spasms started to subside, and the woman was able to sit up and shower herself for the first time in two years. Testing in November 2003 showed intrathecal autoantibody to GAD, but at 17 days’ treatment none was evident. One month after discharge her condition was stable and she needed only small doses of oral benzodiazepine and analgesia until symptoms recurred, at six weeks, when she was given further rituximab, with mycophenolate mofetil. She improved again after 14 days and was discharged, remaining well after five months.

Symptomatic treatment relies on γ-amino butyric acid (GABA) enhancing agents, but previous treatments modifying immune response by reducing antibody to GAD, a rate limiting enzyme in GABA synthesis, has had variable results.

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