2005, Number 1
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Rev Mex Neuroci 2005; 6 (1)
Cogan’s syndrome: Neurological manifestations. Case report and review of literature
Rivera-Olmos VM, Lozano JA
Language: Spanish
References: 19
Page: 97-101
PDF size: 206.53 Kb.
ABSTRACT
Cogan’s syndrome is a rare entity of inflammatory and autoimmune origin apparently triggered through molecular mimetism by infectious agent. Immunodominant peptides similar to autoantigens SSA/Ro and Lambda protein1 of Reovirus III as well as sequences of tyrosin phosphatase-1 (DEP-1/CO 148) that are capable as well to transfect laboratory animals, are expressed in endothelial cells, sensory epithelium, neurons and glia. The clinical result is a multi-system complex with constitutional symptoms, uveitis, interstitial keratitis, acute deafness and vasculitic manifestation in central and peripheral nervous system. We describe here the first observations of brain lesions by MR. The syndrome responds to therapy with steroids and limited immunosuppression.
REFERENCES
Clair EW, McCallum S. Cogan’s syndrome. Current Opin Rheumatol 1999; 11: 47-52.
Mogan RF, Baumgartner CJ. Meniere’s disease complicated by recurrent interstitial queratitis: excellent results following cervical ganglionectomy. West J Surg 1934; 42: 628-31.
Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestíbulo-auditory symptoms. Arch Ophtal 1945; 33: 149.
Hayes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies on thirteen patients, long term follow up and review of the literature. Medicine 1980; 59: 426-41.
Vollersten R. Vasculitis and Cogan’s syndrome. Rheum Dis Clin North Am 1990; 16: 433-8.
Hayes BF, Picus A, Kaiser-Kupfer MI, Fauci AS. Successful treatment of sudden hearing loss in Cogan’s syndrome with corticosteroids. Artritis Rheum 1981; 24: 501-3.
Vollertsen RS, McDonald B, Younge B, et al. Cogan’s syndrome: 18 cases and a review of literature. Mayo Clin Proc 1986; 61: 344-61.
Lunardi C, Bason C, Leandro M, et al. Autoantibodies to inner ear and endotelial antigens in Cogan’s syndrome. Lancet 2002; 360: 915-21.
Takahashi T, Takahashi K, Mernaugh R, et al. Endotelial localization of receptor tyrosine phosphatase, ECRTP/DEP1, in developing and mature renal vasculature. J Am Soc Nephrol 1999; 10: 2135-45.
Kruger RP, Goodyear RJ, Legan PK, et al. The supporting-cell antigen: A receptor-like protein tyrosine phosphatase expressed in the sensory epithelia of the avian inner ear. J Neurosci 1999; 19: 4815-27.
Estivill X, Fortina P, Surrey S, et al. Connexin-26 mutations in sporadic and inherited sensoryneural deafness. Lancet 1998; 351: 394-8.
Lefebvre PP, Van De Water TR. Connexins, hearing and deafness: clinical aspects of mutations in the connexin 26 gene. Brain Res Rev 2000; 32: 159-62.
Autschbach F, Palou E, Mechtersheimer G, et al. Expression of the membrane protein tyrosine phosphatase CD148 in human tissues. Tissue Antigens 1999; 54: 485-98.
Vinceneux P. Cogan syndrome. Orphanet Enciclopedia. October 2003: http://www.orpha.net/data/patho/GB/uk-cogan.pdf
Clay B, Behnia AS, Brown M, et al. Mitochondrial disease: A pulmonary and critical-care medicine perspective. Chest 2001; 120: 634-48.
Rivera VM. Neuroimagen de la esclerosis múltiple. Rev Neurol 2001; 32: 283-5.
Rivera-Olmos VM, Hutton GJG, Breitbach WT. Discrepancia entre clínica e imagen: esclerosis múltiple en fase intermedia. Rev Neuroci 2004; 5(2): 158-62.
Vinceneux P, Puchot J. Le syndrome de Cogan. In: Khan MF, Peltier AP, Meyer O, Piette JC eds. Les Maladies systémiques. Paris: Flammarion; 1991 p. 753-62.
Bicknell JM, Holland JV. Neurologic manifestations of Cogan síndrome. Neurology 1978; 28: 278-328.