Hostname: page-component-586b7cd67f-t8hqh Total loading time: 0 Render date: 2024-11-24T09:34:40.607Z Has data issue: false hasContentIssue false
  • English
  • Français

Stiff-person syndrome with acute recurrent peripheral vertigo: possible evidence of gamma aminobutyric acid as a neurotransmitter in the vestibular periphery

Published online by Cambridge University Press:  01 August 2007

R Teggi ,
L O Piccioni ,
G Martino ,
C Bellini  and
M Bussi
R Teggi*
Affiliation:
ENT Department, San Raffaele Hospital, Vita-Salute University, Milan, Italy
L O Piccioni
Affiliation:
ENT Department, San Raffaele Hospital, Vita-Salute University, Milan, Italy
G Martino
Affiliation:
Department of Neurology, San Raffaele Hospital, Vita-Salute University, Milan, Italy
C Bellini
Affiliation:
ENT Department, San Raffaele Hospital, Vita-Salute University, Milan, Italy
M Bussi
Affiliation:
ENT Department, San Raffaele Hospital, Vita-Salute University, Milan, Italy
*
Address for correspondence: Roberto Teggi, Department of ORL, San Raffaele Hospital, via Olgettina 60, 20132 Milan, Italy. Fax: 39 2 26433508 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

We report a case of a 58-year-old man suffering from stiff-person syndrome and recurrent peripheral vertigo.

Method:

A case report and a review of the recent literature on stiff-person syndrome are presented.

Results:

The patient presented with recurrent episodes of vertigo with a pure peripheral pattern and with concomitant episodes of burning muscle pain, muscle twitching, weight gain and fatigue, worsening with tension or stress that also occurred in periods without vertigo. Cochlear examinations only showed presbyacusis-like hearing loss. The diagnosis of stiff-person syndrome was made with electromyographic examination and from findings in the blood and cerebrospinal fluid of high titres of anti-glutamic acid decarboxylase (GAD67) autoantibodies. In a two-year follow-up period, therapy for stiff-person syndrome abolished episodes of both stiffness and vertigo.

Conclusion:

As far as we know, no other clinical case of acute vestibular damage with a possible correlation with anti-glutamic acid decarboxylase antibodies has been described. Peripheral vertigo possibly related to a lack of gamma aminobutyric acid underlines a possible role of gamma aminobutyric acid as a neurotransmitter in the peripheral vestibular system.

Keywords

Stiff-Person SyndromeGamma Aminobutyric AcidVestibular Disorders
Type
Clinical Record
Information
The Journal of Laryngology & Otology , Volume 122 , Issue 6 , June 2008 , pp. 636 - 638
Copyright
Copyright © JLO (1984) Limited 2007

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

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;31:421–7Google Scholar
2 Toro, C, Jacobowitz, DM, Hallett, M. Stiff-man syndrome. Semin Neurol 1994;14:54–8Google Scholar
3 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;65:633–40Google Scholar
4 Shaw, PJ. Stiff-man syndrome and its variants. Lancet 1999;353:86–7CrossRefGoogle ScholarPubMed
5 Lorish, TR, Thorsteinsson, G, Howard, FM Jr. Stiff-man syndrome updated. Mayo Clin Proc 1989;64:629–36Google Scholar
6 Solimena, M, De Camilli, P. Autoimmunity to glutamic acid decarboxylase (GAD) in Stiff-Man syndrome and insulin-dependent diabetes mellitus. Trends Neurosci 1991;14:452–7Google Scholar
7 Butler, M, Solimena, M, Dirkx, R Jr, Hayday, A, De Camilli, P. Identification of dominant epitope of glutamic acid decarboxylase (GAD-65) recognized by autoantibodies in stiff-man syndrome. J Exp Med 1993;178:2097–106Google Scholar
8 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;241:157–66CrossRefGoogle ScholarPubMed
9 Tinsley, JA, Barth, EM, Black, JL, Williams, DE. Psychiatric consultations in stiff-man syndrome. J Clin Psychiatry 1997;58:444–9Google Scholar
10 Giometto, B, Nicolao, P, Macucci, M, Tavolato, B, Foxon, R, Bottazzo, GF. Temporal-lobe epilepsy associated with glutamic-acid-decarboxylase autoantibodies. Lancet 1998;352:457Google Scholar
11 Horii, A, Kitahara, T, Smith, PF, Darlington, CL, Masumura, C, Kubo, T. Effects of unilateral labyrinthectomy on GAD, GAT1 and GABA receptor gene expression in the rat vestibular nucleus. NeuroReport 2003;14:2359–63CrossRefGoogle ScholarPubMed
12 Tilikete, C, Vighetto, A, Trouillas, P, Honnorat, J. Potential role of anti-GAD antibodies in abnormal eye movements. Ann N Y Acad Sci 2005;1039:446–54Google Scholar
13 Zivotofsky, AZ, Siman-Tov, T, Gadoth, N, Gordon, CR. A rare saccade velocity profile in Stiff Person Syndrome with cerebellar degeneration. Brain Res 2006;1093:135–40Google Scholar
14 Flock, A, Lam, D. Neurotransmitter synthesis in the inner ear and lateral line sense organs. Nature 1974;249:142–4Google Scholar
15 Lopez, I, Meza, G. Neurochemical evidence of afferent GABAergic and efferent cholinergic neurotransmission in the frog vestibule. Neuroscience 1988;25:13–18Google ScholarPubMed
16 Meza, G, Hinojosa, R. Ontogenic approach to cellular localization of neurotransmitters in the chick vestibule. Hear Res 1987;28:73–85Google Scholar
17 Iturbe, AG, Meza, G. Asymmetrical development of GABA and acetylcholine synthesis in guinea pig vestibule. Int J Neurosci 1986;4(suppl 1): s32Google Scholar
18 Meza, G, Lopez, I, Paredes, MA, Penaloza, Y, Poblano, A. Cellular target of streptomycin in the internal ear. Acta Otolaryngol (Stockh) 1989;107:406–11Google Scholar
19 Kong, WJ, Egg, G, Hussl, B, Spoendlin, H, Schrott-Fischer, A. Localization of ChAT-like immunoreactivity in the vestibular-end organs of the rat. Hear Res 1994;75:192–200Google Scholar
20 Kong, WJ, Hussl, B, Thumfart, WF, Schrott-Fischer, A. Ultrastructural localization of GABA-like immunoreactivity in the human utricular macula. Hear Res 1998;119:104–12CrossRefGoogle ScholarPubMed