Stiff man syndrome and related conditions

Movement Disorders

Volumn 17, Issue 5, 2002, Pages 853-866

  Meinck, Hans Michael ^a   Thompson, Philip D ^b  

^a Department of Neurology Im Neuenheimerfeld ^*  (Germany)

^b UNIVERSITY OF ADELAIDE   (Australia)

Author keywords

Encephalomyelitis; Rigidity; Spasms; Stiff man syndrome

Indexed keywords

4 AMINOBUTYRIC ACID; 4 AMINOBUTYRIC ACID RECEPTOR BLOCKING AGENT; ANTICONVULSIVE AGENT; AUTOANTIBODY; BACLOFEN; BENZODIAZEPINE DERIVATIVE; CLONIDINE; CORTICOSTEROID; DIAZEPAM; GLUTAMATE DECARBOXYLASE ANTIBODY; IMMUNOGLOBULIN; METHYLPREDNISOLONE; MUSCLE RELAXANT AGENT; SPASMOLYTIC AGENT; TIZANIDINE;

ATAXIA; CLINICAL FEATURE; DEPRESSION; DIFFERENTIAL DIAGNOSIS; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE COURSE; DYSARTHRIA; ELECTROMYOGRAPHY; ENCEPHALOMYELITIS; HUMAN; IMMUNOMODULATION; MUSCLE CONTRACTION; MUSCLE REFLEX; MUSCLE SPASM; MYOCLONUS; PATHOGENESIS; PHYSIOTHERAPY; PLASMAPHERESIS; PRIORITY JOURNAL; REVIEW; RIGIDITY; SEDATION; SIDE EFFECT; STIFF MAN SYNDROME; SYMPTOMATOLOGY; VERTIGO;

EID: 0036764836     PISSN: 08853185     EISSN: None     Source Type: Journal    
DOI: 10.1002/mds.10279     Document Type: Review

References (114)

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;31:421-427.
2. Solimena M, Folli F, Aparisi R, Pozza G, De Camilli P. Autoantibodies to GABAergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med 1990;322:1555-1560.
3. Kasperek S, Zebrowski S. Stiff man syndrome and encephalomyelitis. Arch Neurol 1971;24:22-31.
4. 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;241:157-166.
5. Dalakas MC, Fujii, Li M, McElroy B. The clinical spectrum of antiGAD antibody positive patients with stiff person syndrome. Neurology 2000;55:1531-1535.
6. Asher R. A woman with the stiff-man syndrome. BMJ 1958;i: 265-266.
7. O'Connor DCJ. Stiff man syndrome. Br Med J 1958;i:645.
8. Cohen L. Stiff man syndrome. Two patients treated with diazepam. JAMA 1966;195:160-162.
9. Gordon EE, Januszko DM, Kaufman L. A critical survey of stiff-man syndrome. Am J Med 1967;42:582-599.
10. Rosin L, De Camilli P, Butler M, et al. Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome. Neurology 1998;50:94-98.
11. Goetz CG, Klawans HL. On the mechanism of sudden death in Moersch-Woltman syndrome. Neurology 1983;33:930-932.
12. Mitsumoto H, Schwartzman MJ, Estes ML, et al. Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome. J Neurol 1991;238:91-96.
13. Martinelli P, Pazzaglia P, Montagna P, Coccagna G, Rizzuto N, Simonati S, Lugaresi E. Stiff-man syndrome associated with nocturnal myoclonus and epilepsy. J Neurol Neurosurg Psychiatry 1978;41:458-462.
14. Henningsen P, Clement U, Kuchenhoff J, Simon F, Meinck HM. Psychological factors in the diagnoses and pathogenesis of stiff-man syndrome. Neurology 1996;47:38-42.
15. Mamoli B, Heiss WD, Maida E, Podreka I. Electrophysiological studies on the stiff-man syndrome. J Neurol 1977;217:111-121.
16. Boiardi A, Crenna P, Negri S, Merati B. Neurological and pharmacological evaluation of a case of Stiff-man syndrome. J Neurol 1980;23:127-33.
17. Meinck HM, Ricker K, Conrad B. The stiff man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine. J Neurol Neurosurg Psychiatry 1984;47:280-287.
18. Rossi B, Massetani R, Guidi M, Mondelli M, Rossi A. Electro-physiological findings in a case of stiff man syndrome. Electro-myogr Clin Neurophysiol 1988;28:137-140.
19. Floeter MK, Valls-SoIé J, Toro C, Jacobowitz D, Hallett M. Physiological studies of spinal inhibitory circuits in patients with stiff-person syndrome. Neurology 1998;51:85-93.
20. Struppler A, Struppler E, Adams RD. Local tetanus in man. Arch Neurol 1963;8:62-78.
21. Isaacs H. Stiff man in a black girl. J Neurol Neurosurg Psychiatry 1979;42:988-994.
22. Meinck HM, Ricker K, Hulser PJ, Solimena M. Stiff man syndrome: neurophysiological findings in eight patients. J Neurol 1995;242:134-142.
23. Martinelli P, Nassetti S, Minardi C, Macri S, Ippoliti M. Electrophysiological evaluation of the stiff man syndrome: further data. J Neurol 1996;243:551-553.
24. Franck G, Cornette M, Grisar T, Moonen G, Gerebtzoff MA. Le syndrome de l'homme raide. Acta Neurol Belg 1974;74:221-240.
25. Stöhr M, Heckl R. Das Stiff man Syndrom. Klinische, elektromyographische und pharmakologische Befunde bei einem eigenen Fall. Arch Psychiatr Nervenkr 1977;223:171-180.
26. Meinck HM, Conrad B. Neuropharmacological investigations in the stiff-man syndrome. J Neurol 1986;233:340-347.
27. Sandbrink F, Syed NA, Fujii MD, Dalakas MC, Floeter MK. Motor cortex excitability in stiff person syndrome. Brain 2000; 123:2231-2239.
28. Koerner C, Weiland B, Meinck H-M. Motor cortical excitability in stiff man syndrome. Mov Disord 2000;15(Suppl. 3):76-77.
29. Leigh PN, Rothwell JC, Traub M, Marsden CD. A patient with reflex myoclonus and muscle rigidity: "the jerking stiff man syndrome." J Neurol Neurosurg Psychiatry 1980;43:1125-1131.
30. Alberca R, Remero M, Chaparro J. Jerking stiff-man syndrome. J Neurol Neurosurg Psychiatry 1982;45:1159-1160.
31. Brown P, Rothwell JC, Thompson PD, Britton TC, Day BL, Marsden CD. The hyperekplexias and their relationship to the normal startle reflex. Brain 1991;114:1903-1928.
32. Guilleminault C, Sigwald J, Castaigne P. Sleep studies and therapeutic trial with L-Dopa in a case of stiff-man syndrome. Eur Neurol 1973;10:89-96.
33. Schmidt RT, Stahl SM, Spehlmann R. A pharmacological study of the stiff-man syndrome. Neurology 1975;25:622-626.
34. Schomburg ED. Spinal sensorimotor systems and their surpaspinal control. Neurosci Res 1990;7:265-340.
35. Meinck HM, Ricker K. Long-standing stiff man syndrome: a particular form of disseminated inflammatory CNS disease? J Neurol Neurosurg Psychiatry 1987;50:1556-1557.
36. Schmierer K, Valdueza JM, Bender A, DeCamilli P, David C, Solimena M, Zschenderlein R. Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression. Neurology 1998;51:250-252.
37. Stayer C, Meinck HM. Stiff-man syndrome: an overview. Neurologia 1998;13:83-88.
38. Schmidli RS, Colman PG, Bonifacio E. Disease sensitivity and specificity of 52 assays for glutamic acid decarboxylase antibodies. Diabetes 1995;44:636-640.
39. Dinkel K, Meinck HM, Jury KM, Karges W, Richter W. Inhibition of gamma-aminobutyric acid synthesis by glutamic acid decarboxylase autoantibodies in stiff man syndrome. Ann Neurol 1998;44:194-201.
40. 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;73:1161-1166.
41. Koerner C, Dinkel K, Richter W, Meinck H-M. Intrathecal synthesis of GAD autoantibodies in stiff man syndrome. Clin Neurophysiol 1999;110(Suppl. 1):S235.
42. Dalakas MC, Li M, Fujii M, Jacobowitz DM. Stiff person syndrome: quantification, specificity and intrathecal synthesis of GAD65 antibodies. Neurology 2001;57:780-784.
43. Thompson PD. Stiff muscles. J Neurol Neurosurg Psychiatry 1993;56:121-124.
44. Lorish TR, Thorsteinsson G, Howard FM. Stiff-man syndrome updated. Mayo Clin Proc 1989;64:629-636.
45. Ricker K, Seitz D, Trostdorf E. Myositis fibrosa generalisata and stiff man syndrome. Eur Neurol 1970;3:13-27.
46. Penry JK, Hoefnagel D, van den Noort S, Denny-Brown D. Muscle spasm and abnormal postures resulting from damage to interneurones in spinal cord. Arch Neurol 1960;3:500-512.
47. Suttenfield FD, Kost PF. The stiff man syndrome, report of a case. Am J Phys Med Rehabil 1964;43:214-216.
48. Tarlov IM. Rigidity in man due to spinal interneuron loss. Arch Neurol 1967;16:536-543.
49. Davis SM, Murray NMF, Diengdoh JV, Galea-Debono A, Kocen RS. Stimulus-sensitive spinal myoclonus. J Neurol Neurosurg Psychiatry 1981;44:884-888.
50. Rowland LP. Cramps, spasms and muscle stiffness. Rev Neurol 1985;141:261-273.
51. Martin R, Meinck HM, Schulte-Mattler W, Mertens HG. Borrelia burgdorferi myelitis presenting as a partial stiff man syndrome. J Neurol 1990;237:51-54.
52. Trethowan WH, Allsop JL, Turner B. The stiff-man syndrome. Arch Neurol 1960;3:14-22.
53. Sikes SZ. Stiff man syndrome (analysis and case report with spinal cord autopsy). Dis Nerv Syst 1959;20:254-258.
54. 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;246:858-860.
55. Warich-Kirches M, Von Bossanyi P, Treuheit T, et al. Stiff man syndrome: possible autoimmune aetiology targeted against GABA-ergic cells. Clin Neuropathol 1997;16:214-219.
56. Warren JD, Scott G, Blumbergs PC, Thompson PD. Pathological evidence of encephalomyelitis in the stiff man syndrome with anti GAD antibodies. J Clin Neurosci (in press).
57. Watanabe K, Shimizu T, Mannen T, Toyokura Y, Nagashima K. An autopsy case with unusual muscle rigidity resembling stiff man syndrome. Clin Neurol (Tokyo) 1984;24:839-847.
58. Ishizawa K, Komori T, Okayama K, et al. Large motor neuron involvement in stiff man syndrome: a quantitative and qualitative study. Acta Neuropathol (Berl) 1999;97:63-70.
59. Nakamura N, Fujiya S, Yahara O, Fujioka Y, Kawakami Y. Stiff-man syndrome with spinal cord lesion. Clin Neuropathol 1986; 5:40-46.
60. Armon C, Swanson JW, McLean JM, et al. Subacute encephalomyelitis presenting as stiff-person syndrome: clinical, polygraphic and pathological correlations. Mov Disord 1996;11:701-709.
61. Lhermitte F, Chain F, Escourolle R, Chedru F, Guilleminault C, Francoucal M. Un nouveau cas de contracture tetaniforme distinct du "stiff-man syndrome." Rev Neurol 1973;128:3-21.
62. Whiteley AM, Swash M, Urich H. Progressive encephalomyelitis with rigidity. Brain 1976;99:27-42.
63. Howell DA, Lees AJ, Toghill PJ. Spinal internuncial neurones in progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1979;42:773-785.
64. Rothwell JC, Obeso JA, Marsden CD. Electrophysiology of somatosensory reflex myoclonus. Adv Neurol 1986;43:385-398.
65. McCombe PA, Chalk JB, Searle JW, Tannenberg AEG, Smith JJ, Pender MP. Progressive encephalomyelitis with rigidity: a case report with magnetic resonance imaging findings. J Neurol Neurosurg Psychiatry 1989;52:1429-1431.
66. Casado JL, Gil-Peralta A, Graus F, Arenas C, Lopez JM, Alberca R. Anti-Ri antibodies associated with opsoclonus and progressive encephalomyelitis with rigidity. Neurology 1994;44:1521-1522.
67. Campbell AMG, Garland H. Subacute myoclonic spinal neuronitis. J Neurol Neurosurg Psychiatry 1956;19:268-274.
68. Thompson PD. Stiff people. In: Marsden CD, Fahn S, editors. Movement disorders 3. Oxford: Butterworth Heinemann; 1994. p 372-405.
69. Steffen H, Menger N, Richter W, et al. Immune-mediated retinopathy in a patient with stiff-man syndrome. Graefe's Arch Clin Exp Ophthalmol 1999;237:212-219.
70. Saiz A, Graus F, Valldeoriola F, Valls-Solé J, Tolosa E. Stiff-leg syndrome: a focal form of stiff-man syndrome. Ann Neurol 1998; 43:400-430.
71. Thompson PD. Stiff man syndrome and related disorders. Parkinsonism Relat Disord 2001;8:147-153.
72. Brown P, Rothwell JC, Marsden CD. The stiff leg syndrome. J Neurol Neurosurg Psychiatry 1997;62:31-37.
73. Burn DJ, Ball J, Lees AJ, Behan PO, Morgan-Hughes JA. A case of progressive encephalomyelitis with rigidity and positive anti-glutamic acid dehydrogenase antibodies. J Neurol Neurosurg Psychiatry 1991;54:449-451.
74. Roobol TH, Kazzaz BA, Vecht CJ. Segmental rigidity and spinal myoclonus as a paraneoplastic syndrome. J Neurol Neurosurg Psychiatry 1987;50:628-631.
75. Bateman DE, Weller RO, Kennedy P. Stiffman syndrome: a rare paraneoplastic disorder? J Neurol Neurosurg Psychiatry 1990;53: 695-696.
76. Ferrari P, Federico M, Grimaldi LM, Silingardi V. Stiff-man syndrome in a patient with Hodgkin's disease. An unusual paraneoplastic syndrome. Haematologica 1990;75:570-572.
77. De Camilli P, Thomas A, Cofiell R, et al. The synaptic vesicle-associated protein amphiphysin is the 128kD autoantigen of stiff-man syndrome with breast cancer. J Exp Med 1993;178:2219-2223.
78. Folli F, Solimena M, Cofiell R, Austoni M, Tallim G, Fassetta G, et al. Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer. N Engl J Med 1993;328:546-551.
79. Dropcho EJ. Antiamphiphysin antibodies with small-cell lung carcinoma and paraneoplastic encephalomyelitis. Ann Neurol 1996;39:659-667.
80. Stingele R, Koelmel HW, Hilligardt B, von Maravic M, De Camilli P, Meinck HM. Two patients with stiff man syndrome, sensory neuronopathy and cancer. Mov Disord 1998;13(Suppl. 2): 208.
81. Mugnaini E, Oertel WH. An atlas on 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, Vol. 4, Part 1. Amsterdam: Elsevier 1985. p 436-608.
82. Saiz A, Arpa J, Sagasta A, et al. Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late-onset insulin-dependent diabetes mellitus, and polyendocrine autoimmunity. Neurology 1997;49:1026-1030.
83. Meinck HM, Faber L, Morgenthaler N, et al. Antibodies against glutamic acid decarboxylase prevalence in neurological diseases. J Neurol Neurosurg Psychiatry 2001;71:100-103.
84. Baekkeskov S, Aanstoot H-J, Christgau S, et al. Identification of the 64K autoantigen in insulin dependent diabetes mellitus as the GABA-synthesising enzyme glutamic acid decarboxylase. Nature 1990;347:151-156.
85. Seissler J, Bieg S, Yassin N, Mauch L, Northemann W, Boehm BO, Scherbaum WA. Association between antibodies to the MR 67,000 isoform of glutamate decarboxylase (GAD) and type I (insulin-dependent) diabetes mellitus with coexisting autoimmune polyendocrine syndrome type II. Autoimmunity 1994;19: 231-238.
86. Tuomi T, Bjorses P, Falorni A, et al. Antibodies to glutamic acid decarboxylase and insulin dependent diabetes in patients with autoimmune polyendocrine syndrome type 1. J Clin Endocrinol Metabol 1996;81:1488-1494.
87. Schmidli RS, DeAizpurua HJ, Harrison LC, Colman PG. Antibodies to glutamic acid decarboxylase in at-risk and clinical insulin-dependent diabetic subjects: relationship to age, sex and islet cell antibody status, and temporal profile. J Autoimmun 1994;7:55-66.
88. Felgenhauer K, Reiber H. The diagnostic significance of antibody specificity indices in multiple sclerosis and herpes virus induced diseases of the nervous system. Clin Invest 1992;70:28-37.
89. Kitze B, Usuku K, Isumo S, Nakamura M, Shiraki H, Ijichi S, Yashiki S, et al. Density of antibody synthesis against HTLV-1 and its relation to HTLV-1 associated myelopathy. J Neurol 1996; 243:393-400.
90. Nemni R, Braghi S, Natali-Sora MG, Lampasona V, Bonifacio E, Comi G, Canal N. Autoantibodies to glutamic acid decarboxylase in palatal myoclonus and epilepsy. Ann Neurol 1994;36:665-667.
91. Honnorat L, Trouillas P, Thivolet C, Aguera M, Belin MF. Autoantibodies to glutamate decarboxylase in a patient with cerebellar cortical atrophy, peripheral neuropathy and slow eye movements. Arch Neurol 1995;52:462-468.
92. Abele M, Weller M, Mescheriakov S, Bork K, Dichgans J, Klockgecher T. Cerebellar ataxia with glutamic acid decarboxylase autoantibodies. Neurology 1999;52:857-859.
93. Ikeda J, Harada T, Kamei H, Nakamura S. Autoantibody to glutamate decarboxylase in a patient with spinocerebellar degeneration and Sjorgen's syndrome. No To Shinkei 1998;50:177-180.
94. Giometto B, Nicolao P, Macucci M, Tavolato B, Foxon R, Bottazzo GF. Temporal lobe epilepsy associated with glutamic acid decarboxylase autoantibodies. Lancet 1998;352:457.
95. Peltola J, Kulmala P, Isojarvi J, Saiz A, Latvala K, Savola K, Knip M, et al. Autoantibodies to glutamic acid decarboxylase in patients with therapy resistant epilepsy. Neurology 2000;55: 46-50.
96. Trivedi R, Mundanthanam G, Amyes E, Lang B, Vincent A. Autoantibody screening in subacute cerebellar ataxia. Lancet 2000;2:565-566.
97. Butler HM, Hatashi A, Ohkoshi N, et al. Autoimmunity to gephyrin in stiff-man syndrome. Neuron 2000;26:307-312.
98. Darnell RB, Victor J, Rubin M, Clouston P, Plum F. A novel antineuronal antibody in stiff-man syndrome. Neurology 1993; 43:114-120.
99. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1998;65:633-640.
100. Penn RD, Mangieri EA. Stiff-man syndrome treated with intrathecal baclofen. Neurology 1993;43:241-242.
101. Stayer C, Tronnier V, Dressnandt J, et al. Intrathecal baclofen therapy for stiff-man syndrome and progressive encephalomyelopathy with rigidity and myoclonus. Neurology 1997;49:1519-1597.
102. Meinck HM, Tronnier V, Marquardt G. Surgical treatment of stiff-man syndrome with intrathecal baclofen. In: Krauss JK, Jankovic J, Grossman RG, editors. Movement disorders surgery. Philadelphia: Lippincott, William, and Wilkins; 2001. p 393-396.
103. Vicari AM, Folli F, Pozza G, et al. Plasmapheresis in the treatment of stiff-man syndrome. N Engl J Med 1989;320:1499-1499.
104. Brashear HR, Phillips LH. Autoantibodies to GABAergic neurons and response to plasmapheresis in stiff-man syndrome. Neurology 1991;41:1588-1592.
105. McEvoy KM, Ahslskog JE. Antibody positive stiff man syndrome responding to immunosuppression. Neurology 1991;41: 143-146.
106. Amato AA, Cornman EW, Kissel JT. Treatment of stiff-man syndrome with intravenous immunoglobulin. Neurology 1994;44: 1652-1654.
107. Karlson EW, Sudarsky L, Ruderman E, Pierson S, Scott M, Helfgott SM. Treatment of stiff-man syndrome with intravenous immune globulin. Arthritis Rheum 1994;37:915-918.
108. Fogan L. Progressive encephalomyelitis with rigidity responsive to plasmapheresis and immunosuppression. Ann Neurol 1996;40: 451-453.
109. Barker RA, Marsden CD. Successful treatment of stiff man syndrome with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 1997;62:426-427.
110. Hau W, Davis C, Hirsch IB, Eng LJ, Daniels T, Walsh D, Lernmark A. Plasmapheresis and immunosuppression in the stiff-man syndrome with type 1 diabetes: a 2-year study. J Neurol 1999;246:731-735.
111. Souza-Lima CF, Ferraz HB, Braz CA, Araujo AM, Manzano GM. Marked improvement in a stiff limb patient treated with intravenous immunoglobulin. Mov Disord 2000;15:358-359.
112. Dalakas MC, Fujii M, Li M, Lufti B, Kyhos J, McElroy B. High dose intravenous immunoglobulin for stiff person syndrome. N Engl J Med 2001;345:1870-1876.
113. 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.
114. Meinck H-M. Immunomodulation in the stiff man syndrome. Mov Disord 2000;15(Suppl. 3):252-253.