Successful treatment of stiff person syndrome with sequential use of tacrolimus
| Autor: | Shunya Nakane, Naoko Matsui, Koji Fujita, Yuishin Izumi, Ryuji Kaji, Ryo Urushihara, Yoshihiko Nishida, Masafumi Harada, Yoshiko Shibuta |
|---|---|
| Rok vydání: | 2013 |
| Předmět: |
Male
Magnetic Resonance Spectroscopy Gabapentin medicine.medical_treatment Stiff-Person Syndrome Drug Administration Schedule Tacrolimus chemistry.chemical_compound Humans Medicine gamma-Aminobutyric Acid Aged Autoantibodies Neurologic Examination Autoimmune disease Dose-Response Relationship Drug Glutamate Decarboxylase business.industry Motor Cortex Brain Middle Aged Muscle stiffness Evoked Potentials Motor equipment and supplies medicine.disease Transcranial Magnetic Stimulation Psychiatry and Mental health Baclofen chemistry Anesthesia Female Surgery Plasmapheresis Neurology (clinical) business Diazepam Immunosuppressive Agents Stiff person syndrome Follow-Up Studies medicine.drug |
| Zdroj: | Journal of Neurology, Neurosurgery & Psychiatry. 84:1177-1180 |
| ISSN: | 1468-330X 0022-3050 |
| DOI: | 10.1136/jnnp-2013-305425 |
| Popis: | Stiff person syndrome (SPS) is a rare neurological disease with features of an autoimmune disease. SPS is characterised by severe progressive muscle stiffness of the spine and lower extremities with superimposed muscle spasms triggered by external stimuli such as noise, touch and emotional distress.1 Patients with SPS respond to high doses of muscle relaxants, such as diazepam and baclofen, several anticonvulsants, and gabapentin. Previous studies have reported that several causal treatments with corticosteroids, plasmapheresis, intravenous immunoglobulin (Ig) and new immunomodulating agents can reduce stiffness and lower sensitivity to stimuli and stress in patients with SPS.2 ,3 Because SPS is a chronic disease, patients generally require long-term treatments, which may be effective but difficult to take for long periods. Tacrolimus (Prograf, FK506) is a macrolide molecule belonging to the same immunosuppressant class as cyclosporine. Tacrolimus has a lower molecular weight and is 100-fold more potent in inhibiting T cell proliferation than cyclosporine. It acts through inhibition of the calcium-calcineurin (CaN) pathway and exerts its immunosuppressive effect by reducing the proliferation of activated T cells.4 We now report the successful treatment of patients with SPS using tacrolimus as the immunosuppressive agent. ### Patients We assigned two patients with SPS who had incomplete responses to conventional therapies and fulfilled the defined clinical criteria (online supplementary table 1).3 ### Clinical evaluations At baseline and each month thereafter, a neurologist used the distribution of stiffness index, the most consistent indicator of stiffness among patients and within patients, to evaluate the patients with SPS.3 Scores on this index range from 0 to 6 and reflect the extent of stiffness. The same neurologist also assessed the patients for changes in frequency of spasms with use of the heightened-sensitivity scale, which has been a reproducible and consistent means of assessing the number of factors triggering spasms.3 … |
| Databáze: | OpenAIRE |
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