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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1984 Mar;47(3):280–287. doi: 10.1136/jnnp.47.3.280

The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine.

H M Meinck, K Ricker, B Conrad
PMCID: PMC1027727  PMID: 6707674

Abstract

Neurophysiological investigations of a patient suffering from the stiff-man syndrome revealed that exteroceptive reflexes, in particular those elicited from the skin, were excessively enhanced. In contrast, no abnormalities were found within the monosynaptic reflex arc. Clomipramine injection severely aggravated the clinical symptoms whereas diazepam, clonidine, and tizanidine decreased both muscular stiffness and abnormal exteroceptive reflexes. The hypothesis is put forward that the stiff-man syndrome is a disorder of descending brain-stem systems which exert a net inhibitory control on axial and limb girdle muscle tone as well as on exteroceptive reflex transmission. Detection of abnormal exteroceptive reflex activity in conjunction with neuropharmacological testing might help in the diagnosis of this rare disease.

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Selected References

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  1. Alberca R., Romero M., Chaparro J. Jerking stiff-man syndrome. J Neurol Neurosurg Psychiatry. 1982 Dec;45(12):1159–1160. doi: 10.1136/jnnp.45.12.1159. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. 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(2):127–133. doi: 10.1007/BF00313175. [DOI] [PubMed] [Google Scholar]
  3. Caccia M. R., McComas A. J., Upton A. R., Blogg T. Cutaneous reflexes in small muscles of the hand. J Neurol Neurosurg Psychiatry. 1973 Dec;36(6):960–977. doi: 10.1136/jnnp.36.6.960. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Cobb J. Stiff man syndrome: is the lesion at spinal cord or brain stem level. Proc R Soc Med. 1974 Oct;67(10):1065–1066. [PMC free article] [PubMed] [Google Scholar]
  5. Conrad B., Aschoff J. C. Effects of voluntary isometric and isotonic activity on late transcortical reflex components in normal subjects and hemiparetic patients. Electroencephalogr Clin Neurophysiol. 1977 Jan;42(1):107–116. doi: 10.1016/0013-4694(77)90155-9. [DOI] [PubMed] [Google Scholar]
  6. Corrodi H., Fuxe K., Lidbrink P., Olson L. Minor tranquillizers, stress and central catecholamine neurons. Brain Res. 1971 Jun 4;29(1):1–16. doi: 10.1016/0006-8993(71)90413-6. [DOI] [PubMed] [Google Scholar]
  7. Davis S. M., Murray N. M., Diengdoh J. V., Galea-Debono A., Kocen R. S. Stimulus-sensitive spinal myoclonus. J Neurol Neurosurg Psychiatry. 1981 Oct;44(10):884–888. doi: 10.1136/jnnp.44.10.884. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Delwaide P. J., Juprelle M. The effects of caloric stimulation of the labyrinth on the soleus motor pool in man. Acta Neurol Scand. 1977 Apr;55(4):310–322. doi: 10.1111/j.1600-0404.1977.tb05650.x. [DOI] [PubMed] [Google Scholar]
  9. FOGARTY J. P. The stiff man syndrome. J Ir Med Assoc. 1959 Aug;45:44–46. [PubMed] [Google Scholar]
  10. Fisher M. A., Shahani B. T., Young R. R. Electrophysiologic analysis of the motor system after stroke: the flexor reflex. Arch Phys Med Rehabil. 1979 Jan;60(1):7–11. [PubMed] [Google Scholar]
  11. Franck G., Cornette M., Grisar T., Moonen G., Gerebtzoff M. A. Le syndrome de l'homme raide. Etude clinique, polygraphique et histoenzymologique. Acta Neurol Belg. 1974 Jul-Aug;74(4):221–240. [PubMed] [Google Scholar]
  12. Franz D. N., Perry R. S. Mechanisms for differential block among single myelinated and non-myelinated axons by procaine. J Physiol. 1974 Jan;236(1):193–210. doi: 10.1113/jphysiol.1974.sp010430. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Garnett R., Stephens J. A. The reflex responses of single motor units in human first dorsal interosseous muscle following cutaneous afferent stimulation. J Physiol. 1980 Jun;303:351–364. doi: 10.1113/jphysiol.1980.sp013290. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Gassel M. M., Ott K. H. Mononeuron excitability in man: a novel method of evaluation by modulation of tonic muscle activity. Electroencephalogr Clin Neurophysiol. 1970 Aug;29(2):190–195. doi: 10.1016/0013-4694(70)90122-7. [DOI] [PubMed] [Google Scholar]
  15. Gordon E. E., Januszko D. M., Kaufman L. A critical survey of stiff-man syndrome. Am J Med. 1967 Apr;42(4):582–599. doi: 10.1016/0002-9343(67)90057-5. [DOI] [PubMed] [Google Scholar]
  16. Guilleminault C., Sigwald J., Castaigne P. Sleep studies and therepeutic trial with L-dopa in a case of Stiffman syndrome. Eur Neurol. 1973;10(2):89–96. doi: 10.1159/000114266. [DOI] [PubMed] [Google Scholar]
  17. HOWARD F. M., Jr A new and effective drug in the treatment of the stiff-man syndrome: preliminary report. Proc Staff Meet Mayo Clin. 1963 May 22;38:203–212. [PubMed] [Google Scholar]
  18. Haefely W., Kulcsár A., Möhler H., Pieri L., Polc P., Schaffner R. Possible involvement of GABA in the central actions of benzodiazepines. Adv Biochem Psychopharmacol. 1975;(14):131–151. [PubMed] [Google Scholar]
  19. Heiligman R., Paulson M. J. The stiff man syndrome: a psychiatric disease? Int J Psychiatry Med. 1976;7(4):363–371. doi: 10.2190/40re-8dku-rg2u-lj0c. [DOI] [PubMed] [Google Scholar]
  20. Howell D. A., Lees A. J., Toghill P. J. Spinal internuncial neurones in progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry. 1979 Sep;42(9):773–785. doi: 10.1136/jnnp.42.9.773. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Ishikawa K., Ott K., Porter R. W., Stuart D. Low frequency depression of the H wave in normal and spinal man. Exp Neurol. 1966 May;15(1):140–156. doi: 10.1016/0014-4886(66)90039-2. [DOI] [PubMed] [Google Scholar]
  22. Klein R., Haddow J. E., DeLuca C. Familial congenital disorder resembling stiff-man syndrome. Am J Dis Child. 1972 Nov;124(5):730–731. doi: 10.1001/archpedi.1972.02110170108018. [DOI] [PubMed] [Google Scholar]
  23. Lawrence D. G., Kuypers H. G. The functional organization of the motor system in the monkey. II. The effects of lesions of the descending brain-stem pathways. Brain. 1968 Mar;91(1):15–36. doi: 10.1093/brain/91.1.15. [DOI] [PubMed] [Google Scholar]
  24. Leigh P. N., Rothwell J. C., Traub M., Marsden C. D. A patient with reflex myoclonus and muscle rigidity: "jerking stiff-man syndrome". J Neurol Neurosurg Psychiatry. 1980 Dec;43(12):1125–1131. doi: 10.1136/jnnp.43.12.1125. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. MOERSCH F. P., WOLTMAN H. W. Progressive fluctuating muscular rigidity and spasm ("stiff-man" syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin. 1956 Jul 25;31(15):421–427. [PubMed] [Google Scholar]
  26. Maida E., Reisner T., Summer K., Sandor-Eggerth H. Stiff-man syndrome with abnormalities in CSF and computerized tomography findings. Report of a case. Arch Neurol. 1980 Mar;37(3):182–183. doi: 10.1001/archneur.1980.00500520080018. [DOI] [PubMed] [Google Scholar]
  27. Mamoli B., Heiss W. D., Maida E., Podreka I. Electrophysiological studies on the "stiff-man" syndrome. J Neurol. 1977 Dec 13;217(2):111–121. doi: 10.1007/BF00312924. [DOI] [PubMed] [Google Scholar]
  28. McQuillen M. P., Tucker K., Pellegrino E. D. Syndrome of subacute generalized muscular stiffness and spasm. Arch Neurol. 1967 Feb;16(2):165–174. doi: 10.1001/archneur.1967.00470200053004. [DOI] [PubMed] [Google Scholar]
  29. Meier-Ewert K., Gleitsmann K., Reiter F. Acoustic jaw reflex in man: its relationship to other brain-stem and microreflexes. Electroencephalogr Clin Neurophysiol. 1974 Jun;36(6):629–637. doi: 10.1016/0013-4694(74)90229-6. [DOI] [PubMed] [Google Scholar]
  30. Meinck H. M., Benecke R., Küster S., Conrad B. Cutaneomuscular (flexor) reflex organization in normal man and in patients with motor disorders. Adv Neurol. 1983;39:787–796. [PubMed] [Google Scholar]
  31. Meinck H. M., Piesiur-Strehlow B., Koehler W. Some principles of flexor reflex generation in human leg muscles. Electroencephalogr Clin Neurophysiol. 1981 Aug;52(2):140–150. doi: 10.1016/0013-4694(81)90161-9. [DOI] [PubMed] [Google Scholar]
  32. Mertens H. G., Ricker K. Ubererregbarkeit der gamma-Motoneurone beim "Stiff-man" Syndrom. Klin Wochenschr. 1968 Jan 1;46(1):33–42. doi: 10.1007/BF01725298. [DOI] [PubMed] [Google Scholar]
  33. OLAFSON R. A., MULDER D. W., HOWARD F. M. "STIFF-MAN" SYNDROME: A REVIEW OF THE LITERATURE, REPORT OF THREE ADDITIONAL CASES AND DISCUSSION OF PATHOPHYSIOLOGY AND THERAPY. Mayo Clin Proc. 1964 Feb;39:131–144. [PubMed] [Google Scholar]
  34. PRICE T. M., ALLOTT E. N. The stiffman syndrome; preliminary report of a case. Br Med J. 1958 Mar 22;1(5072):682–685. doi: 10.1136/bmj.1.5072.682. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Ricker K., Seitz D., Trostdorf E. Myositis fibrosa generalisata and 'stiff-man' syndrome. Eur Neurol. 1970;3(1):13–27. doi: 10.1159/000114002. [DOI] [PubMed] [Google Scholar]
  36. Sander J. E., Layzer R. B., Goldsobel A. B. Congenital stiff-man syndrome. Ann Neurol. 1980 Aug;8(2):195–197. doi: 10.1002/ana.410080212. [DOI] [PubMed] [Google Scholar]
  37. Scherrer J., Rondot P., Korn H., Métral S. Etude accélérométrique et EMG des mouvements anormaux et des réflexes pathologiques. Sem Hop. 1968 Mar 20;44(14):911–919. [PubMed] [Google Scholar]
  38. Schmidt R. T., Stahl S. M., Spehlmann R. A pharmacologic study of the stiff-man syndrome. Correlation of clinical symptoms with urinary 3-methoxy-4-hydroxy-phenyl glycol excretion. Neurology. 1975 Jul;25(7):622–626. doi: 10.1212/wnl.25.7.622. [DOI] [PubMed] [Google Scholar]
  39. Starke K. Regulation of noradrenaline release by presynaptic receptor systems. Rev Physiol Biochem Pharmacol. 1977;77:1–124. doi: 10.1007/BFb0050157. [DOI] [PubMed] [Google Scholar]
  40. Strassburg H. M., Oepen G., Thoden U. The late facilitation in H-reflex recovery cycles in different pyramidal lesions. Arch Psychiatr Nervenkr (1970) 1980;228(3):197–204. doi: 10.1007/BF00342345. [DOI] [PubMed] [Google Scholar]
  41. Stöhr M., Heckl R. Das Stuff-man-Syndrom. Klinische, elektromyographische und pharmakologische Befunde bei einem eigenen Fall. Arch Psychiatr Nervenkr (1970) 1977 Feb 23;223(2):171–180. doi: 10.1007/BF00345955. [DOI] [PubMed] [Google Scholar]
  42. VOSS H. Zahl und Anordnung der Muskelspindeln in den unteren Zungenbeinmuskeln, dem M. sternocleidomastoideus und den Bauch- und tiefen Nackeamuskeln. Anat Anz. 1958 Nov 29;105(10-16):265–275. [PubMed] [Google Scholar]
  43. Valli G., Barbieri S., Cappa S., Pellegrini G., Scarlato G. Syndromes of abnormal muscular activity: overlap between continuous muscle fibre activity and the stiff man syndrome. J Neurol Neurosurg Psychiatry. 1983 Mar;46(3):241–247. doi: 10.1136/jnnp.46.3.241. [DOI] [PMC free article] [PubMed] [Google Scholar]
  44. Vogel P., Goebel H. H., Seitz D. Rigid spine syndrome in a girl. J Neurol. 1982;228(4):259–265. doi: 10.1007/BF00313416. [DOI] [PubMed] [Google Scholar]
  45. WHALEN R. E., COMBS J. J., Jr, DEISS W. P., Jr "Stiff-man" syndrome. Am J Med. 1959 Oct;27:678–681. doi: 10.1016/0002-9343(59)90052-x. [DOI] [PubMed] [Google Scholar]
  46. Whiteley A. M., Swash M., Urich H. Progressive encephalomyelitis with rigidity. Brain. 1976 Mar;99(1):27–42. doi: 10.1093/brain/99.1.27. [DOI] [PubMed] [Google Scholar]
  47. Yarom R., Chaco J., Steigbuegel D. Ultrastructure of muscle in stiff-man syndrome. Virchows Arch A Pathol Anat Histol. 1974;362(3):207–221. doi: 10.1007/BF00432195. [DOI] [PubMed] [Google Scholar]
  48. van Zwieten P. A. Interaction between centrally acting hypotensive drugs and tricyclic antidepressants. Arch Int Pharmacodyn Ther. 1975 Mar;214(1):12–30. [PubMed] [Google Scholar]

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