Abstract
Objective: To investigate systematically the rate and type of phobia in stiff man syndrome and its variants, and to compare patients with stiff man syndrome with and without phobia for sociodemographic and neurological characteristics.
Methods: 43 consecutive patients with stiff man syndrome referred to a university department of neurology were assessed using the anxiety disorders interview schedule, revised (ADIS-R), a structured diagnostic interview for anxiety disorders, in addition to a full clinical neurological and psychiatric assessment.
Results: 19 patients (44.2%) developed task specific phobia—that is, fear and avoidance of situations difficult to master owing to the motor symptoms of stiff man syndrome (such as crossing streets). Three further patients (7%) had subthreshold phobia—that is, phobic anxiety without avoidance. There were no significant differences between patients with and without phobia in terms of age, illness duration, type of stiff man syndrome, antibody status, or frequency of falls. Patients with phobia were more likely to present with exaggerated startle responses and to have an initial misdiagnosis of psychogenic movement disorder.
Conclusions: Specific phobia is a frequent non-motor symptom of stiff man syndrome. Early recognition is an important aid to correct diagnosis. The aetiology of phobia in stiff man syndrome is unknown. There is no evidence of a direct pathogenic role of autoantibodies directed against glutamic acid decarboxylase in the development of phobia.
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Selected References
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- Barker R. A., Revesz T., Thom M., Marsden C. D., 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 Nov;65(5):633–640. doi: 10.1136/jnnp.65.5.633. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brown P., Marsden C. D. The stiff man and stiff man plus syndromes. J Neurol. 1999 Aug;246(8):648–652. doi: 10.1007/s004150050425. [DOI] [PubMed] [Google Scholar]
- Curtis G. C., Magee W. J., Eaton W. W., Wittchen H. U., Kessler R. C. Specific fears and phobias. Epidemiology and classification. Br J Psychiatry. 1998 Sep;173:212–217. [PubMed] [Google Scholar]
- Dalakas M. C., Fujii M., Li M., McElroy B. The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology. 2000 Nov 28;55(10):1531–1535. doi: 10.1212/wnl.55.10.1531. [DOI] [PubMed] [Google Scholar]
- Dalakas M. C., Li M., Fujii M., Jacobowitz D. M. Stiff person syndrome: quantification, specificity, and intrathecal synthesis of GAD65 antibodies. Neurology. 2001 Sep 11;57(5):780–784. doi: 10.1212/wnl.57.5.780. [DOI] [PubMed] [Google Scholar]
- Davies Robert D., Gabbert Sherri L., Riggs Paula D. Anxiety Disorders in Neurologic Illness. Curr Treat Options Neurol. 2001 Jul;3(4):333–346. doi: 10.1007/s11940-001-0038-1. [DOI] [PubMed] [Google Scholar]
- Di Nardo P., Moras K., Barlow D. H., Rapee R. M., Brown T. A. Reliability of DSM-III-R anxiety disorder categories. Using the Anxiety Disorders Interview Schedule-Revised (ADIS-R). Arch Gen Psychiatry. 1993 Apr;50(4):251–256. doi: 10.1001/archpsyc.1993.01820160009001. [DOI] [PubMed] [Google Scholar]
- Dinkel K., Meinck H. M., Jury K. M., Karges W., Richter W. Inhibition of gamma-aminobutyric acid synthesis by glutamic acid decarboxylase autoantibodies in stiff-man syndrome. Ann Neurol. 1998 Aug;44(2):194–201. doi: 10.1002/ana.410440209. [DOI] [PubMed] [Google Scholar]
- Fyer A. J. Current approaches to etiology and pathophysiology of specific phobia. Biol Psychiatry. 1998 Dec 15;44(12):1295–1304. doi: 10.1016/s0006-3223(98)00274-1. [DOI] [PubMed] [Google Scholar]
- Henningsen P., Clement U., Küchenhoff J., Simon F., Meinck H. M. Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome. Neurology. 1996 Jul;47(1):38–42. doi: 10.1212/wnl.47.1.38. [DOI] [PubMed] [Google Scholar]
- Jacob R. G., Furman J. M. Psychiatric consequences of vestibular dysfunction. Curr Opin Neurol. 2001 Feb;14(1):41–46. doi: 10.1097/00019052-200102000-00007. [DOI] [PubMed] [Google Scholar]
- Jacob R. G., Whitney S. L., Detweiler-Shostak G., Furman J. M. Vestibular rehabilitation for patients with agoraphobia and vestibular dysfunction: a pilot study. J Anxiety Disord. 2001 Jan-Apr;15(1-2):131–146. doi: 10.1016/s0887-6185(00)00047-5. [DOI] [PubMed] [Google Scholar]
- Kash S. F., Tecott L. H., Hodge C., Baekkeskov S. Increased anxiety and altered responses to anxiolytics in mice deficient in the 65-kDa isoform of glutamic acid decarboxylase. Proc Natl Acad Sci U S A. 1999 Feb 16;96(4):1698–1703. doi: 10.1073/pnas.96.4.1698. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kiriakos Carol R., Franco Kathleen N. Stiff-man syndrome: a case report and review of the literature. Psychosomatics. 2002 May-Jun;43(3):243–244. doi: 10.1176/appi.psy.43.3.243. [DOI] [PubMed] [Google Scholar]
- Lamberg L. Psychiatric symptoms common in neurological disorders. JAMA. 2001 Jul 11;286(2):154–156. [PubMed] [Google Scholar]
- Lorish T. R., Thorsteinsson G., Howard F. M., Jr Stiff-man syndrome updated. Mayo Clin Proc. 1989 Jun;64(6):629–636. doi: 10.1016/s0025-6196(12)65339-7. [DOI] [PubMed] [Google Scholar]
- 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]
- Magee W. J., Eaton W. W., Wittchen H. U., McGonagle K. A., Kessler R. C. Agoraphobia, simple phobia, and social phobia in the National Comorbidity Survey. Arch Gen Psychiatry. 1996 Feb;53(2):159–168. doi: 10.1001/archpsyc.1996.01830020077009. [DOI] [PubMed] [Google Scholar]
- Marks I. Space "phobia": a pseudo-agoraphobic syndrome. J Neurol Neurosurg Psychiatry. 1981 May;44(5):387–391. doi: 10.1136/jnnp.44.5.387. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mathew S. J., Coplan J. D., Gorman J. M. Neurobiological mechanisms of social anxiety disorder. Am J Psychiatry. 2001 Oct;158(10):1558–1567. doi: 10.1176/appi.ajp.158.10.1558. [DOI] [PubMed] [Google Scholar]
- Meinck Hans-Michael, Thompson Philip D. Stiff man syndrome and related conditions. Mov Disord. 2002 Sep;17(5):853–866. doi: 10.1002/mds.10279. [DOI] [PubMed] [Google Scholar]
- Saiz A., Graus F., Valldeoriola F., Valls-Solé J., Tolosa E. Stiff-leg syndrome: a focal form of stiff-man syndrome. Ann Neurol. 1998 Mar;43(3):400–403. doi: 10.1002/ana.410430322. [DOI] [PubMed] [Google Scholar]
- Shaw P. J. Stiff-man syndrome and its variants. Lancet. 1999 Jan 9;353(9147):86–87. doi: 10.1016/S0140-6736(05)76151-1. [DOI] [PubMed] [Google Scholar]
- 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 May 31;322(22):1555–1560. doi: 10.1056/NEJM199005313222202. [DOI] [PubMed] [Google Scholar]
- Tinsley J. A., Barth E. M., Black J. L., Williams D. E. Psychiatric consultations in stiff-man syndrome. J Clin Psychiatry. 1997 Oct;58(10):444–449. doi: 10.4088/jcp.v58n1007. [DOI] [PubMed] [Google Scholar]
- Wittchen H. U., Reed V., Kessler R. C. The relationship of agoraphobia and panic in a community sample of adolescents and young adults. Arch Gen Psychiatry. 1998 Nov;55(11):1017–1024. doi: 10.1001/archpsyc.55.11.1017. [DOI] [PubMed] [Google Scholar]