engleski

The treatment of Moersch-Woltman syndrome

Objectives: The authors report the efficacy of different therapeutic regimens in female patient with stiff person syndrome. It is rare disorder characterized by stiffness and spasms of truncal and extremity muscles. Materials and Methods: 44-year-old woman hospitalized because of symptoms of autonomic disfunction, paroxysmal muscle spasms and rigidity of the trunk and legs. Spasms are triggered by activity, passive movements, sensory stimuli and emotional stress. Examination eliminated the paraneoplastic variant of the illness. Anti-glutamic acid decarboxylase (GAD65) antibodies are negative. Treatment is symptomatic (diazepam, clonazepam, baclofen, nonsteroidal anti-inflammatory medications, physical terapy) and immunosupressive (immunoglobulin, prednisone). Results: Along with the applied therapy painful spasms become rarer, but rigidity persists and causes a joint deformities which fixes the patient in a permanent prostrate position. The most effective therapy are benzodiazepines (diazepam, clonazepam). These drugs produce symptomatic relief. Other drugs which modulate the function of GABA-ergic neurons provide mild to modest relief of clinical symptoms (baclofen). Because of strong autoimmune link, immunomodulating therapies have yet to produce consistent results (prednisone, immunoglobulin). Physical therapy should be used carefuly in those for whom passive motion may be trigger of spasm. Nonsteroidal anti-inflammatory medications can be used for pain managment. Conclusion: The disease progresses over the span of months and patient is bedridden now. Stiff person syndrome is a disabling nervous system disorder with no satisfactory treatment. Not one of available therapies can prevent the long-term progression of the illness or even bring a cure.

stiff person syndrome; treatment

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