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Stiff-Man Syndrome

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Neuromuscular Disorders in Clinical Practice

Abstract

Stiff-man syndrome is a central nervous system disorder, characterized by muscle rigidity and painful spasms, which involve mainly the muscles of the limbs, trunk, and neck. This chapter discusses the etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of this disorder.

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References

  1. Moersch FP, Woltman HW. Progressive fluctuating muscular rigidity and spasm (stiff-man syndrome): report of a case and some observations in 13 other cases. Mayo Clin Proc. 1956;31:421–7.

    CAS  Google Scholar 

  2. Gordon EE, Januszko DM, Kaufman L. A critical survey of stiff-man syndrome. Am J Med. 1967;42:582–99.

    Article  PubMed  CAS  Google Scholar 

  3. Ornsteen AM. Chronic generalized fibromyositis. Ann Surg. 1935;101:237–45.

    Article  PubMed  CAS  Google Scholar 

  4. Solimena M, Folli F, Aparisi R, Pozza G, De Camilli P. Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med. 1990;322:1555–60.

    Article  PubMed  CAS  Google Scholar 

  5. Meinck HM, Ricker K, Hulser PJ, Schmid E, Peiffer J, Solimena M. Stiff man syndrome: clinical and laboratory findings in eight patients. J Neurol. 1994;241:157–66.

    Article  PubMed  CAS  Google Scholar 

  6. Lorish TR, Thorsteinsson G, Howard FM. Stiff-man syndrome updated. Mayo Clin Proc. 1989;64:629–36.

    Article  PubMed  CAS  Google Scholar 

  7. Folli F, Solimena M, Cofiell R, Austoni M, Tallini G, Fassetta G, et al. Autoantibodies to a 128-kd protein in three women with the stiff-man syndrome and breast cancer. N Engl J Med. 1993;328:546–51.

    Article  PubMed  CAS  Google Scholar 

  8. Grimaldi LME, Martino G, Braghi S, Quattrini A, Furlan R, Bosi E, et al. Heterogeneity of autoantibodies in stiff-man syndrome. Ann Neurol. 1993;34:57–64.

    Article  PubMed  CAS  Google Scholar 

  9. Asher R. A woman with stiff-man syndrome. Br Med J. 1958;1:256–8.

    Google Scholar 

  10. O’Connor DCJ. Stiff-man syndrome. Br Med J. 1958;1:645.

    Article  Google Scholar 

  11. Price TML, Allott EN. The stiff-man syndrome. Preliminary report of a case. Br Med J. 1958;1:682–5.

    Article  PubMed  CAS  Google Scholar 

  12. Trethowan WH, Allsop JL, Turner B. The “stiff-man” syndrome. A report of two further cases. Arch Neurol. 1960;3:448–56.

    Article  PubMed  CAS  Google Scholar 

  13. Stuart FS, Henry M, Holley HL. The stiff-man syndrome: report of a case. Arthritis Rheum. 1960;3:229–32.

    Article  PubMed  CAS  Google Scholar 

  14. Brage D. Sindrome del hombre teiso (stiff-man). Rev Clin Esp. 1959;15:30–7.

    Google Scholar 

  15. Shukla KL, Sikand P. “Stiff-man” syndrome. J Indian Med Assoc. 1961;37:610.

    PubMed  CAS  Google Scholar 

  16. Werk EE, Sholiton LJ, Marnell RT. The “stiff-man” syndrome and hyperthyroidism. Am J Med. 1961;31:647–53.

    Article  PubMed  Google Scholar 

  17. Young W. The Stiff-Man syndrome. Br J Med Pract. 1966;20:507–10.

    CAS  Google Scholar 

  18. Drake Jr ME. Stiff-man syndrome and dementia. Am J Med. 1983;74:1085–7.

    Article  PubMed  Google Scholar 

  19. Ambrosetto C, Lugaresi E, Berti Ceroni G. Chronic cryptogenetic tetanus: “stiff-man” syndrome of Moersch and Woltman. G Psichiatr Neuropatol. 1966;94:511–20.

    PubMed  CAS  Google Scholar 

  20. Howard Jr FM. A new and effective drug in the treatment of stiff-man syndrome: preliminary report. Mayo Clin Proc. 1963;38:203–12.

    Google Scholar 

  21. Whelam JL. Baclofen in treatment of the “stiff-man syndrome”. Arch Neurol. 1980;37:600–1.

    Article  Google Scholar 

  22. George TM, Burke JM, Sobotka PA, Greenberg HS, Vinik AI. Resolution of stiff-man syndrome with cortisol replacement in a patient with deficiencies of ACTH, growth hormone and prolactin. N Engl J Med. 1984;47:280–7.

    Google Scholar 

  23. McQuillen MP, Tucker K, Pellegrino ED. Syndrome of acute generalized muscular stiffness and spasm. Arch Neurol. 1967;16:165–74.

    Article  PubMed  CAS  Google Scholar 

  24. Jog MS, Lambert CD, Lang AE. Stiff-person syndrome. Can J Neurol Sci. 1992;19:383–8.

    PubMed  CAS  Google Scholar 

  25. Solimena M, Folli F, Denis-Donini S, Comi GC, Pozza G, De Camilli P, et al. Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. N Engl J Med. 1988;318:1012–20.

    Article  PubMed  CAS  Google Scholar 

  26. Martinelli P, Pazzaglia P, Montagna P, Coccagna G, Rizzuto N, Simonati S, et al. Stiff-man syndrome associated with nocturnal myoclonus and epilepsy. J Neurol Neurosurg Psychiatry. 1978;41:458–62.

    Article  PubMed  CAS  Google Scholar 

  27. Cohen L. Stiff-man syndrome: two patients treated with diazepam. JAMA. 1966;195:222–4.

    Article  PubMed  CAS  Google Scholar 

  28. Saiz A, Minguez A, Graus F, Marin C, Tolosa E, Cruz-Sanchez F. Stiff-man syndrome with vacuolar degeneration of anterior horn motor neurons. J Neurol. 1999;246:858–60.

    Article  PubMed  CAS  Google Scholar 

  29. Ishizawa K, Komori T, Okayama K, Qin X, Kaneko K, Sasaki S, et al. Large motor neuron involvement in stiff-man syndrome: a qualitative and quantitative study. Acta Neuropathol. 1999;97:63–70.

    Article  PubMed  CAS  Google Scholar 

  30. Warich-Kirches M, Von Bossanyi P, Treuheit T, Kirches E, Dietzmann K, Feistner H, et al. Stiff-man syndrome: possible autoimmune etiology targeted against GABA-ergic cells. Clin Neuropathol. 1997;16:214–9.

    PubMed  CAS  Google Scholar 

  31. Westblom U. Stiff-man syndrome and clonazepam. JAMA. 1977;237:1930.

    Article  PubMed  CAS  Google Scholar 

  32. Spehlamann R, Norcross K, Rasmus SC, Schlageter NL. Improvement of stiff-man syndrome with sodium valproate. Neurology. 1981;31:162–3.

    Google Scholar 

  33. Meinck H-M, Conrad B. Neuropharmacological investigations in the stiff-man syndrome. J Neurol. 1986;233:340–7.

    Article  PubMed  CAS  Google Scholar 

  34. Boiardi A, Crenna P, Bussone G, Negri S, Merati B. Neurological and pharmacological evaluation of a case of stiff-man syndrome. J Neurol. 1980;223:127–33.

    Article  PubMed  CAS  Google Scholar 

  35. Henningsen P, Clement U, Kuchenhoff J, Simon F, Meinck HM. Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome. Neurology. 1996;47:38–42.

    Article  PubMed  CAS  Google Scholar 

  36. Black JL, Barth EM, Williams DE, Tinsley JA. Stiff-man syndrome. Results of interviews and psychologic testing. Psychosomatics. 1998;39:38–44.

    Article  PubMed  CAS  Google Scholar 

  37. Tinsley JA, Barth EM, Black JL, Williams DE. Psychiatric consultations in stiff-man syndrome. J Clin Psychiatry. 1997;58:444–9.

    Article  PubMed  CAS  Google Scholar 

  38. Mitsumoto H, Schwartzman MJ, Estes ML, Chou SM, La Franchise EF, De Camilli P, et al. Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome. J Neurol. 1991;238:91–6.

    Article  PubMed  CAS  Google Scholar 

  39. Floeter MK, Valls-Solè J, Toro C, Jacobowitz D, Hallett M. Physiologic studies of spinal inhibitory circuits in patients with stiff-person syndrome. Neurology. 1998;51:85–93.

    Article  PubMed  CAS  Google Scholar 

  40. Levy LM, Dalakas MC, Floeter MK. The stiff-person syndrome: an autoimmune disorder affecting neurotransmission of gamma-aminobutyric acid. Ann Intern Med. 1999;13:522–30.

    Article  Google Scholar 

  41. Geis C, Weishaupt A, Grünewald B, Wultsch T, Reif A, Gerlach M, et al. Human stiff-person syndrome IgG induces anxious behavior in rats. PLoS One. 2011;6:e16775.

    Article  PubMed  CAS  Google Scholar 

  42. Bosi E, Vicari A, Comi G, Comola M, Canal N, Pozza G, et al. Association of stiff-man syndrome and type I diabetes with islet cell and other autoantibodies. Arch Neurol. 1988;45:246–7.

    Article  PubMed  CAS  Google Scholar 

  43. Oertel WH, Schmechel DE, Mugnaini E, Tappaz ML, Kopin IJ. Immunocytochemical localization of glutamate decarboxylase in rat cerebellum with a new antiserum. Neuroscience. 1981;6:2715–35.

    Article  PubMed  CAS  Google Scholar 

  44. Mugnaini E, Oertel WH. An atlas of the distribution of GABAergic neurons and terminals in the rat CNS as revealed by GAD immunohistochemistry. In: Bjorklund A, Hokfelt T, editors. Handbook of chemical neuroanatomy, GABA and neuropeptides in the CNS, Part I, vol. 4. Amsterdam: Elsevier Science Publishers; 1985. p. 436–608.

    Google Scholar 

  45. Palay SL, Chan-Palay V. Cerebellar cortex: cytology and organization. New York: Springer; 1974.

    Book  Google Scholar 

  46. Kaufman DL, McGinnis JF, Krieger NR, Tobin AJ. Brain glutamate decarboxilase cloned in labda γt-11: fusion protein produces gamma-aminobutyric acid. Science. 1986;232:1138–40.

    Article  PubMed  CAS  Google Scholar 

  47. Erlander MG, Tillakatne NJK, Feldblum S, Patel N, Tobin AJ. Two genes encode distinct glutamate decarboxilases. Neuron. 1991;7:91–100.

    Article  PubMed  CAS  Google Scholar 

  48. Reetz A, Solimena M, Matteoli M, Folli F, Takei K, De Camilli P. GABA and pancreatic β-cells: colocalization of glutamic acid decarboxilase (GAD) and GABA with synaptic-like microvescicles suggests their role in GABA storage and secretion. EMBO J. 1991;10:1275–84.

    PubMed  CAS  Google Scholar 

  49. Vincent SR, Hokfelt T, Wu J-Y, Elde RP, Morgan LM, Kimmel JR. Immunohistochemical studies of the GABA system in the pancreas. Neuroendocrinology. 1983;36:197–204.

    Article  PubMed  CAS  Google Scholar 

  50. Persson H, Pelto-Huikko M, Metsis M, Soder O, Brene S, Skog S, et al. Expression of the neurotransmitter-synthesizing enzyme glutamic acid decarboxylase in male germ cells. Mol Cell Biol. 1990;10:4701–11.

    PubMed  CAS  Google Scholar 

  51. Erdo SL, Joo F, Wolff JR. Immunohistochemical localization of glutamate decarboxylase in the rat oviduct and ovary: further evidence for non-neural GABA systems. Cell Tissue Res. 1989;255:431–4.

    Article  PubMed  CAS  Google Scholar 

  52. McEvoy KM. Stiff-man syndrome. Semin Neurol. 1991;11:197–205.

    Article  PubMed  CAS  Google Scholar 

  53. Toro C, Jacobowitz DM, Hallett M. Stiff-man syndrome. Semin Neurol. 1994;14:154–8.

    Article  PubMed  CAS  Google Scholar 

  54. Brown P, Marsden CD. The stiff man and stiff man plus syndrome. J Neurol. 1999;246:648–52.

    Article  PubMed  CAS  Google Scholar 

  55. Gorin F, Baldwin B, Tait R, Pathak R, Seyal M, Mugnaini E. Stiff-man syndrome: a GABAergic autoimmune disorder with autoantigenic heterogeneity. Ann Neurol. 1990;28:71–4.

    Article  Google Scholar 

  56. Harding AE, Thompson PD, Kocen RS, Batchelor JR, Davey N, Marsden CD. Plasma exchange and immunosuppression in the stiff man syndrome. Lancet. 1989;2:915.

    Article  PubMed  CAS  Google Scholar 

  57. Baekkeskov S, Aanstoot H-J, Christgau S, Reetz A, Solimena M, Cascalho M, et al. Identification of the 64K autoantigen in insulin-dependent diabetes as the GABA-synthesizing enzyme glutamic acid decarboxylase. Nature. 1990;347:151–6.

    Article  PubMed  CAS  Google Scholar 

  58. Pugliese A, Gianani R, Eisenbarth GS, De Camilli P, Solimena M. Genetics of susceptibility and resistance to insulin-dependent diabetes in stiff-man syndrome. Lancet. 1994;344:1027–8.

    Article  PubMed  CAS  Google Scholar 

  59. Williams AC, Nutt JG, Hare T. Autoimmunity in stiff-man syndrome. Lancet. 1988;2:222.

    Article  PubMed  CAS  Google Scholar 

  60. Lernmark A. Glutamic acid decarboxylase – Gene to antigen to disease. J Intern Med. 1996;240:259–77.

    Article  PubMed  CAS  Google Scholar 

  61. Lohmann T, Hawa M, Leslie RDG, Lane R, Picard J, Londei M. Immune reactivity to glutamic acid decarboxylase 65 in stiff-man syndrome and type 1 diabetes mellitus. Lancet. 2000;356:31–5.

    Article  PubMed  CAS  Google Scholar 

  62. Saiz A, Arpa J, Sagasta A, Casamitjana R, Zarranz JJ, Tolosa E, et al. Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late-onset insulin dependent diabetes mellitus, and polyendocrine autoimmunity. Neurology. 1997;49:1026–30.

    Article  PubMed  CAS  Google Scholar 

  63. Nemni R, Braghi S, Natali-Sora MG, Lampasona V, Bonifacio E, Comi G, et al. Autoantibodies to glutamic acid decarboxylase in palatal myoclonus and epilepsy. Ann Neurol. 1994;36:665–7.

    Article  PubMed  CAS  Google Scholar 

  64. Levy LM, Levy-Reis I, Fujii M, Dalakas MC. Brain gamma-­aminobutyric acid changes in stiff-person syndrome. Arch Neurol. 2005;62:970–4.

    Article  PubMed  Google Scholar 

  65. Perani D, Garibotto V, Moresco RM, Ortelli P, Corbo M, Fazio F, et al. PET evidence of central GABAergic changes in stiff-person syndrome. Mov Disord. 2007;22:1030–3.

    Article  PubMed  Google Scholar 

  66. Galldiks N, Thiel A, Haense C, Fink GR, Hilker R. 11C-flumazenil positron emission tomography demonstrates reduction of both global and local cerebral benzodiazepine receptor binding in a patient with Stiff Person Syndrome. J Neurol. 2008;255:1361–4.

    Article  PubMed  Google Scholar 

  67. Masson C, Prier S, Benoit C, Henin D, Masson M, Cambier J. Amnesie, syndrome de l’ homme raide: Manifestations revelatrices d’une encephalomyelite paraneoplastique. Ann Med Interne. 1987;138:502–5.

    CAS  Google Scholar 

  68. Piccolo G, Cosi V, Zandrini C, Moglia A. Steroid-responsive and dependent stiff-man syndrome: a clinical and electrophysiological study of two cases. Ital J Neurol Sci. 1988;9:559–66.

    Article  PubMed  CAS  Google Scholar 

  69. Piccolo G, Cosi V. Stiff man syndrome, dysimmune disorder, and cancer. Ann Neurol. 1989;26:105.

    Article  PubMed  CAS  Google Scholar 

  70. Bateman DE, Weller RO, Kennedy P. Stiffman syndrome: a rare paraneoplastic disorder? J Neurol Neurosurg Psychiatry. 1990;53:695–6.

    Article  PubMed  CAS  Google Scholar 

  71. Silverman IE. Paraneoplastic stiff limb syndrome. J Neurol Neurosurg Psychiatry. 1999;67:126–7.

    Article  PubMed  CAS  Google Scholar 

  72. Valldeoriola F. Movement disorders of autoimmune origin. J Neurol. 1999;246:423–31.

    Article  PubMed  CAS  Google Scholar 

  73. De Camilli P, Cameron R, Greengard P. Synapsin I (protein I), a nerve terminal-specific phosphoprotein. I. Its general distribution in synapses of the central and peripheral nervous system demonstrated by immunofluorescence in frozen and plastic sections. J Cell Biol. 1983;96:1337–54.

    Article  PubMed  Google Scholar 

  74. Navone F, Jahn R, Di Gioia G, Stukenbrok H, Greengard P, De Camilli P. Protein p38: an integral membrane protein specific for small vesicles of neurons and neuroendocrine cells. J Cell Biol. 1986;103:2511–27.

    Article  PubMed  CAS  Google Scholar 

  75. De Camilli P, Thomas A, Cofiell R, Folli F, Lichte B, Piccolo G, et al. The synaptic vesicle-associated protein amphiphysin is the 128-kD autoantigen of Stiff-Man Syndrome with breast cancer. J Exp Med. 1993;178:2219–23.

    Article  PubMed  Google Scholar 

  76. Dropcho EJ. Antiamphiphysin antibodies with small-cell lung ­carcinoma and paraneoplastic encephalomyelitis. Ann Neurol. 1996;43:659–67.

    Article  Google Scholar 

  77. Perego L, Previtali SC, Nemni R, Longhi R, Carandente O, Saibene A, et al. Autoantibodies to amphiphysin II and amphiphysin II in a patient with sensory-motor neuropathy. Eur Neurol. 2002;47:196–200.

    Article  PubMed  CAS  Google Scholar 

  78. Saiz A, Dalmau J, Butler MH, Chen Q, Delattre JY, De Camilli P, et al. Anti-amphiphysin II antibodies in patients with paraneoplastic neurological disorders associated with small cell lung carcinoma. J Neurol Neurosurg Psychiatry. 1999;66:214–7.

    Article  PubMed  CAS  Google Scholar 

  79. Antoine JC, Absi L, Honnorat J, Boulesteix JM, de Brouker T, Vial C, et al. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. Arch Neurol. 1999;56:172–7.

    Article  PubMed  CAS  Google Scholar 

  80. Henningsen P, Meinck HM. Specific phobia is a frequent non-­motor feature in stiff man syndrome. J Neurol Neurosurg Psychiatry. 2003;74:462–5.

    Article  PubMed  CAS  Google Scholar 

  81. Nutt DJ, Malizia AL. New insights into the role of the GABA(A)-benzodiazepine receptor in psychiatric disorder. Br J Psychiatry. 2001;179:390–6.

    Article  PubMed  CAS  Google Scholar 

  82. Kalueff AV, Nutt DJ. Role of GABA in anxiety and depression. Depress Anxiety. 2007;24:495–517.

    Article  PubMed  CAS  Google Scholar 

  83. Thompson PD. Stiff muscles. J Neurol Neurosurg Psychiatry. 1993;56:121–4.

    Article  PubMed  CAS  Google Scholar 

  84. Klein R, Haddow JE, DeLuca C. Familial congenital disorder resembling stiff-man syndrome. Am J Dis Child. 1972;124:730–1.

    PubMed  CAS  Google Scholar 

  85. Sander JE, Layzer RB, Goldsobel AB. Congenital stiff-man ­syndrome. Ann Neurol. 1980;8:195–7.

    Article  PubMed  CAS  Google Scholar 

  86. Sigwald J, Guilleminault C. Syndrome de contracture permanente. Rev Neurol. 1971;124:191–212.

    PubMed  CAS  Google Scholar 

  87. Rowland LP. Cramps, spasms and muscle stiffness. Rev Neurol. 1985;141:271–3.

    Google Scholar 

  88. Blum P, Jankovic J. Stiff-person syndrome: an autoimmune disease. Mov Disord. 1991;6:12–20. Review.

    Article  PubMed  CAS  Google Scholar 

  89. Fogan L. Progressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression. Ann Neurol. 1996;40:451–3.

    Article  PubMed  CAS  Google Scholar 

  90. Burn DJ, Ball J, Lees AJ, Behan PO, Morgan-Hughes JA. A case of progressive encephalomyelitis with rigidity and positive antiglutamic acid dehydrogenase antibodies. J Neurol Neurosurg Psychiatry. 1991;54:449–51.

    Article  PubMed  CAS  Google Scholar 

  91. Kullmann DM, Howard RS, Miller DH, Hirsch NP, Brown P, Marsden CD. Brainstem encephalopathy with stimulus-sensitive myoclonus leading to respiratory arrest, but with recovery: a description of two cases and review of the literature. Mov Disord. 1996;11:715–8.

    Article  PubMed  CAS  Google Scholar 

  92. Whiteley AM, Swash M, Urich H. Progressive encephalomyelitis with rigidity. Its relation to “subacute myoclonic spinal neuronitis” and to the “stiff-man syndrome”. Brain. 1976;99:27–42.

    Article  PubMed  CAS  Google Scholar 

  93. Campbell AG, Garland H. Subacute myoclonic spinal neuronitis. J Neurol Neurosurg Psychiatry. 1956;19:268–74.

    Article  PubMed  CAS  Google Scholar 

  94. Lhermitte F, Chain F, Escourolle R, Chedru F, Guilleminault C, Francoual M. Un nouveau cas de contracture tetaniforme distinct du “stiff man syndrome”. Etude pharmacologique et neuropathologiche d’un cas d’encephalomyelite a’ predominance medullaire. Rev Neurol. 1973;128:3–21.

    PubMed  CAS  Google Scholar 

  95. Howell DA, Lees AJ, Toghill PJ. Spinal internuncial neurones in progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry. 1979;42:773–85.

    Article  PubMed  CAS  Google Scholar 

  96. Kasperek S, Zebrowski S. Stiff-man syndrome and encephalomyelitis. Arch Neurol. 1971;24:22–30.

    Article  PubMed  CAS  Google Scholar 

  97. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by stiff-man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff-limb syndrome, and ­progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry. 1998;65:633–40.

    Article  PubMed  CAS  Google Scholar 

  98. Folli F. Stiff man syndrome, 40 years later. J Neurol Neurosurg Psychiatry. 1998;65:618.

    Article  PubMed  CAS  Google Scholar 

  99. Saiz A, Graus F, Valldeoriola F, Valls-Solè J, Tolosa E. Stiff-leg syndrome: a focal form of stiff-man syndrome. Ann Neurol. 1998;43:400–1.

    Article  PubMed  CAS  Google Scholar 

  100. Shaw PJ. Stiff-man syndrome and its variants. Lancet. 1999;353:86–7.

    Article  PubMed  CAS  Google Scholar 

  101. Nguyen-Huu BK, Urban PP, Schreckenberger M, Dieterich M, Werhahn KJ. Antiamphiphysin-positive stiff-person syndrome associated with small cell lung cancer. Mov Disord. 2006;21:1285–7.

    Article  PubMed  CAS  Google Scholar 

  102. McKeon A, Pittock SJ, Lennon VA. Stiff-person syndrome with amphiphysin antibodies: distinctive features of a rare disease. Neurology. 2009;73:2132.

    Article  PubMed  Google Scholar 

  103. Valli G, Barbieri G, Cappa Pellegrini G, Scarlato G. Syndromes of abnormal muscular activity: overlap between continuous muscle fiber activity and the stiff-man syndrome. J Neurol Neurosurg Psychiatry. 1983;46:241–7.

    Article  PubMed  CAS  Google Scholar 

  104. Isaacs H. A syndrome of continuous muscle-fibre activity. J Neurol Neurosurg Psychiatry. 1961;24:319–25.

    Article  PubMed  CAS  Google Scholar 

  105. Leigh PN, Rothwell JC, Traub M, Marsden CD. A patient with reflex myoclonus and muscle rigidity: jerking stiff-man syndrome. J Neurol Neurosurg Psychiatry. 1980;43:1125–31.

    Article  PubMed  CAS  Google Scholar 

  106. Kimura J. Electrodiagnosis in diseases of nerve and muscle: ­principles and practice. Philadelphia: F.A. Davis; 1983.

    Google Scholar 

  107. Kissel JT, Elble RJ. Stiff-person syndrome. Stiff opposition to a simple explanation. Neurology. 1998;51:11–4.

    Article  PubMed  CAS  Google Scholar 

  108. Weinberg DH, Logigian EL, Kelly JJ. Cervical astrocytoma with arm rigidity: clinical and electrophysiologic features. Neurology. 1988;38:1635–7.

    Article  PubMed  CAS  Google Scholar 

  109. Sandbrink F, Syed NA, Fujii MD, Dalakas MC, Floeter MK. Motor cortex excitability in stiff-person syndrome. Brain. 2000;123:2231–9.

    Article  PubMed  Google Scholar 

  110. Walikonis JE, Lennon VA. Radioimmunoassay for glutamic acid decarboxylase (GAD65) autoantibodies as a diagnostic aid for stiff-man syndrome and a correlate of susceptibility to type 1 diabetes mellitus. Mayo Clin Proc. 1998;73:1161–6.

    Article  PubMed  CAS  Google Scholar 

  111. Rakocevic G, Raju R, Dalakas MC. Anti-glutamic acid decarboxylase antibodies in the serum and cerebrospinal fluid of patients with stiff-person syndrome: correlation with clinical severity. Arch Neurol. 2004;61:902–4.

    Article  PubMed  Google Scholar 

  112. Rehman HU. Stiff-man syndrome. Postgrad Med J. 1998;74:641–2.

    Article  PubMed  CAS  Google Scholar 

  113. Link H. Characteristics of the immune response within the CNS in neurologic disorders. Acta Neurol Scand Suppl. 1978;67:177–90.

    Article  PubMed  CAS  Google Scholar 

  114. Tourtellotte WW, Walsh MJ. Cerebrospinal fluid profile in multiple sclerosis. In: Poser CM, editor. The diagnosis of multiple sclerosis. New York: Thieme Stratton; 1984. p. 165–78.

    Google Scholar 

  115. Liguori R, Medori R, Marcello L, Grimaldi L, Lugaresi E, Montagna P. Vigabatrin improves rigidity in stiff-person syndrome (SPS). Neurology. 1993;43:311.

    Google Scholar 

  116. Vermiji FH, Doorn PAV, Busch HFM. Improvement of stiff-man syndrome with vigabatrin. Lancet. 1996;346:612.

    Article  Google Scholar 

  117. Prevett MC, Brown P, Duncan JS. Improvement of stiff-man syndrome with vigabatrin. Neurology. 1997;48:1133–4.

    Article  PubMed  CAS  Google Scholar 

  118. Stayer C, Tronnier V, Dressnandt J, Mauch E, Marquardt G, Rieke K, et al. Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus. Neurology. 1997;49:1591–7.

    Article  PubMed  CAS  Google Scholar 

  119. Maramattom BV. Intrathecal baclofen pump implantation in a case of stiff person syndrome. Neurol India. 2010;58:115–7.

    Article  PubMed  Google Scholar 

  120. Ho BL, Shih PY. Successful intrathecal baclofen therapy for seronegative stiff-person syndrome: a case report. Acta Neurol Taiwan. 2008;17:172–6.

    PubMed  Google Scholar 

  121. Vicari AM, Folli F, Pozza G, Comi GC, Comola M, Canal N, et al. Plasmapheresis in the treatment of Stiff-Man Syndrome. N Engl J Med. 1989;320:1499.

    PubMed  CAS  Google Scholar 

  122. Hummel M, Durinovic-Bello I, Bonifacio E, Lampasona V, Endl J, Fessele S, et al. Humoral and cellular immune parameters before and after immunosuppressive therapy of a patient with stiff-man syndrome and insulin dependent diabetes mellitus. J Neurol Neurosurgery Psychiatry. 1998;65:204–8.

    Article  CAS  Google Scholar 

  123. Hao W, Davis C, Hirsch IB, Eng LJ, Daniels T, Walsh D, et al. Plasmapheresis and immunosuppression in stiff-man syndrome with type 1 diabetes: a 2-year study. J Neurol. 1999;246:731–5.

    Article  PubMed  CAS  Google Scholar 

  124. Brashear HR, Phillips LH. Autoantibodies to GABAergic neurons and response to plasmapheresis in stiff-man syndrome. Neurology. 1991;41:1588–92.

    Article  PubMed  CAS  Google Scholar 

  125. Assessment of plasmapheresis. Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 1996;47:840–3.

    Article  Google Scholar 

  126. Eiger G, Khanlou H. Long-term remission of refractory stiff-man syndrome after treatment with intravenous immunoglobulin. Mayo Clin Proc. 1999;74:1231–2.

    Article  PubMed  Google Scholar 

  127. Karlson EW, Sudarsky L, Ruderman E, Pierson S, Scott M, Helfgott SM. Treatment of stiff-man syndrome with intravenous immune globulin. Arthritis Rheum. 1994;37:915–8.

    Article  PubMed  CAS  Google Scholar 

  128. Barker RA, Marsden CD. Successful treatment of stiff-man syndrome with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1997;62:426–7.

    Article  PubMed  CAS  Google Scholar 

  129. Mueller-Schoop JW, Meyer M. Total alopecia, diabetes mellitus and falls. Lancet. 1996;348:1420.

    Article  PubMed  CAS  Google Scholar 

  130. Dinkel K, Meinck H-M, Jury KM, Karges W, Richter W. Inhibition of γ-aminobutyric synthesis by glutamic decarboxylase autoantibodies in stiff-man syndrome. Ann Neurol. 1998;44:194–201.

    Article  PubMed  CAS  Google Scholar 

  131. Dalakas MC, Fujii M, Li M, Lutfi B, Kyhos J, McElroy B. High-dose intravenous immune globulin for stiffperson syndrome. N Engl J Med. 2001;345:1870–6.

    Article  PubMed  CAS  Google Scholar 

  132. Gerschlager W, Brown P. Effect of treatment with intravenous immunoglobulin on quality of life in patients with stiff-person syndrome. Mov Disord. 2002;17:590–3.

    Article  PubMed  Google Scholar 

  133. Amato AA, Corman EW, Kissel JT. Treatment of stiff-man syndrome with intravenous immunoglobulin. Neurology. 1994;44:1652–4.

    Article  PubMed  CAS  Google Scholar 

  134. Gurcan HM, Ahmed AR. Efficacy of various intravenous immunoglobulin therapy protocols in autoimmune and chronic inflammatory disorders. Ann Pharmacother. 2007;41:812–23.

    Article  PubMed  CAS  Google Scholar 

  135. Elovaara I, Apostolski S, van Doorn P, Gilhus NE, Hietaharju A, Honkaniemi J, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. 2008;15:893–908.

    Article  PubMed  CAS  Google Scholar 

  136. Ishida K, Mitoma H, Song S-Y, Uchicara T, Inaba A, Eguchi S, et al. Selective suppression of cerebellar GABAergic transmission by an autoantibody to glutamic acid decarboxylase. Ann Neurol. 1999;46:263–7.

    Article  PubMed  CAS  Google Scholar 

  137. Butler MH, Solimena M, Dirkx R, Hayday A, De Camilli P. Identification of a dominant epitope of glutamic acid decarboxylase (GAD-65) recognized by autoantibodies in Stiff-Man Syndrome. J Exp Med. 1993;178:2097–106.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

This chapter is dedicated to my beloved cousins, Maria Cristina Folli MD and Marcello Folli.

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Authors and Affiliations

  1. Diabetes Division, Department of Medicine, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX, 78229, USA

    Franco Folli MD, Phd

  2. Diabetes Center, ACISMOM Associazione Dei Cavalieri Italiani, Sovrano Militare Ordine di Malta, Piazza del Grillo 1, Rome, Italy

    Annamaria Prioletta MD

  3. Division of Neuroscience – INSPE, San Raffaele Scientific Institute, Via Olgettina 60, Milan, Italy

    Angelo Quattrini MD

  4. Department of Rehabilitation and Neurophysiology, Fondazione Istituto San Raffaele – Giglio, Via Giardina, Cefalù – Palermo, Italy

    Giuseppe Galardi MD

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  1. Franco Folli MD, Phd
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  2. Annamaria Prioletta MD
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  3. Angelo Quattrini MD
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  4. Giuseppe Galardi MD
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Correspondence to Franco Folli MD, Phd .

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Editors and Affiliations

  1. School of Medicine Department of Neurology, University Hospitals Case Medical Center Case Western Reserve University, Cleveland, Ohio, USA

    Bashar Katirji

  2. Department of Neurology, George Washington University, Washington, District of Columbia, USA

    Henry J. Kaminski

  3. University School of Medicine Department of Neurology, Louis Stokes Cleveland VA Medical Center Case Western Reserve, Cleveland, Ohio, USA

    Robert L. Ruff

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Folli, F., Prioletta, A., Quattrini, A., Galardi, G. (2014). Stiff-Man Syndrome. In: Katirji, B., Kaminski, H., Ruff, R. (eds) Neuromuscular Disorders in Clinical Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6567-6_72

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