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Das Stiff-Man-Syndrom und seine Varianten

Stiff man syndrome and variants

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Zusammenfassung

Das Stiff-Man-Syndrom und seine Varianten sind seltene neurologische Erkrankungen mit ungewöhnlicher, oft bizarr anmutender motorischer und psychischer Symptomatik. Fehldiagnosen sind häufig; vor allem die Abgrenzung gegen die psychogene Bewegungsstörung bereitet oft Schwierigkeiten. Die klinische Verdachtsdiagnose kann durch neurophysiologische und immunologische Untersuchungen gesichert werden. Pathogenetisch handelt es sich um eine chronische immunogene Enzephalomyelopathie mit enger Beziehung zu den autoimmunen Endokrinopathien. Die initial hochdosierte Methylprednisolon-Langzeittherapie ist nach retrospektiven Verlaufsanalysen die derzeit effektivste immunmodulierende Behandlungsform.

Summary

Stiff man syndrome (SMS) and its variants are rare neurological disorders with unusual, often awkward motor and psychological symptoms. Misdiagnoses are frequent and differentiation from psychogenic movement disorder may be difficult. Clinical suspicion can be substantiated by neurophysiological and immunological testing. Autoimmunity against certain proteins of inhibitory synapses appears to be a key feature that links SMS to other autoimmune encephalopathies and endocrinopathies. According to retrospective analyses a front-loaded long-term methylprednisolone treatment appears to be most effective.

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Literatur

  1. Alexopoulos H, Dalakas MC (2010) A critical update on the immunopathogenesis of stiff person syndrome. Eur J Clin Invest 40:1018–1025

    Article  PubMed  CAS  Google Scholar 

  2. Bardutzky J, Tronnier V, Schwab S, Meinck HM (2003) Intrathecal baclofen for stiff-person syndrome: life-threatening intermittent catheter leakage. Neurology 60:1976–1978

    Article  PubMed  CAS  Google Scholar 

  3. Berger C, Meinck HM (2003) Head retraction reflex in stiff-man syndrome and related disorders. Mov Disord 18:906–911

    Article  PubMed  Google Scholar 

  4. Butler MH, Hayashi A, Ohkoshi N et al (2000) Autoimmunity to gephyrin in stiff-man syndrome. Neuron 26:307–312

    Article  PubMed  CAS  Google Scholar 

  5. Dalakas M, Fujii M, Li M et al (2001) High-dose intravenous immune globulin for stiff person syndrome. N Engl J Med 345:1870–1876

    Article  PubMed  CAS  Google Scholar 

  6. Diener HC, Weimar C (Hrsg) (2012) Leitlinien für Diagnostik und Therapie in der Neurologie, 5. Aufl. Thieme, Stuttgart

  7. Dinkel K, Rickert M, Möller G et al (2002) Stiff-man syndrome: identification of 17 beta-hydroxysteroid dehydrogenase type 4 as a novel 80-kDa antineuronal antigen. J Neuroimmunol 130:184–193

    Article  PubMed  CAS  Google Scholar 

  8. Hansen N, Grünewald B, Weishaupt A et al (2012) Human stiff person syndrome IgG containing high-titer anti-GAD65 autoantibodies induces motor dysfunction in rats. Exp Neurol 22. pii: S0014-4886(12)00396-2. DOI 10.1016/j.expneurol.2012.10.013. [Epub ahead of print]

  9. Henningsen P, Meinck H-M (2003) Specific phobia is a frequent non-motor feature in stiff man syndrome. J Neurol Neurosurg Psychiatry 74:462–465

    Article  PubMed  CAS  Google Scholar 

  10. Hutchinson M, Waters P, McHugh J et al (2008) Progressive encephalomyelitis, rigidity, and myoclonus: a novel glycine receptor antibody. Neurology 71:1291–1292

    Article  PubMed  CAS  Google Scholar 

  11. Khasani S, Becker K, Meinck HM (2004) Hyperekplexia and stiff-man syndrome: abnormal brainstem reflexes suggest a physiological relationship. J Neurol Neurosurg Psychiatry 75:1265–1269

    Article  PubMed  CAS  Google Scholar 

  12. Koerner C, Wieland B, Richter W, Meinck HM (2004) Stiff-person syndromes: motor cortex hyperexcitability correlates with anti-GAD autoimmunity. Neurology 62:1357–1362

    Article  PubMed  Google Scholar 

  13. McCabe DJ, Turner NC, Chao D et al (2004) Paraneoplastic „stiff person syndrome“ with metastatic adenocarcinoma and anti-Ri antibodies. Neurology 62:1402–1404

    Article  PubMed  CAS  Google Scholar 

  14. Meinck HM, Ricker K, Hülser PJ, Solimena M (1995) Stiff man syndrome: neurophysiological findings in eight patients. J Neurol 242:134–142

    Article  PubMed  CAS  Google Scholar 

  15. Meinck HM, Thompson P (2002) Stiff man syndrome and related conditions. Mov Disord 17:853–866

    Article  PubMed  Google Scholar 

  16. Moersch FP, Woltman HW (1956) Progressive fluctuating muscular rigidity and spasm („stiff-man“ syndrome): report of a case and some observations in other 13 cases. Mayo Clin Proc 31:421–427

    CAS  Google Scholar 

  17. Murinson BB, Guarnaccia JB (2008) Stiff-person syndrome with amphiphysin antibodies: distinctive features of a rare disease. Neurology 71:1955–1958

    Article  PubMed  CAS  Google Scholar 

  18. Stayer C, Tronnier V, Dressnandt J et al (1997) Intrathecal baclofen for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus. Neurology 49:1591–1597

    Article  PubMed  CAS  Google Scholar 

  19. Steffen H, Menger N, Richter W et al (1999) Immune-mediated retinopathy in a patient with stiff-man syndrome. Graefes Arch Clin Exp Ophthalmol 237:212–219

    Article  PubMed  CAS  Google Scholar 

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Vortragshonorare von CSL Behring und Orion-Pharma.

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  1. Neurologische Universitätsklinik Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Deutschland

    H.-M. Meinck

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  1. H.-M. Meinck
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Correspondence to H.-M. Meinck.

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Meinck, HM. Das Stiff-Man-Syndrom und seine Varianten. Nervenarzt 84, 450–454 (2013). https://doi.org/10.1007/s00115-012-3604-8

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  • DOI: https://doi.org/10.1007/s00115-012-3604-8

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