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Successful treatment of stiff person syndrome with sequential use of tacrolimus
  1. Shunya Nakane1,2,
  2. Koji Fujita1,
  3. Yoshiko Shibuta1,
  4. Naoko Matsui1,
  5. Masafumi Harada3,
  6. Ryo Urushihara1,4,
  7. Yoshihiko Nishida5,
  8. Yuishin Izumi1,
  9. Ryuji Kaji1
  1. 1 Department of Clinical Neuroscience, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, Japan
  2. 2 Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan
  3. 3 Department of Medical Imaging, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, Japan
  4. 4 College of Social Sciences, Ritsumeikan University, Kyoto, Japan
  5. 5 Department of Neurology, Itsuki Hospital, Tokushima, Japan
  1. Correspondence to Dr Shunya Nakane, Department of Neurology, Nagasaki Kawatana Medical Center, 2005-1 Shimogumigo, Kawatanacho, Higashisonogigun, Nagasaki 859-3615, Japan; nakaneshunya{at}gmail.com

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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.

Methods

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 …

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