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Vogt-Koyanagi-Harada syndrome: Clinical and instrumental contribution

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Abstract

Vogt-Koyanagi-Harada syndrome is a rare disease, which probaly has a cell-mediated autoimmune pathogenesis, marked by ocular (anterior and/or posterior uveitis), dermatological (poliosis, canities, vitiligo) and neurological (meningo-encephalitis) disorders of variable severity in variable combinations. The clinical pattern in the case reported here showed severe neurological involvement (headache, ataxia and confusional state) followed by anteroposterior uveitis. Instrumental investigations (cerebrospinal fluid, VEPs, BAEPs, EEG, CT and MRI brainscans) confirmed the diagnosis. The response to cortisone therapy was excellent. We emphasize the importance of the neuroradiological investigations, because of their peculiarities, and review the reports of cases with marked meningo-encephalitic impairment, given the dearth of reports in the neurological journals.

Sommario

La sindrome di Vogt-Koyanagi-Harada è una rara malattia a verosimile patogenesi autoimmunitaria cellulo-mediata, caratterizzata da disturbi oculari (uveite anteriore e/o posteriore), dermatologici (poliosi, canizie, vitiligo) e neurologici (meningo-encefalite) di grado variabile e variamente associati fra loro.

Il caso descritto presenta clinicamente un severo interessamento neurologico (cefalea, atassia, stato confusionale) con successiva comparsa di uveite antero-posteriore; gli esami strumentali (studio del liquor, PEV-BAERS, EEG, TAC e RMN encefaliche) hanno confermato la diagnosi; ottimale la risposta al trattamento cortisonico. I reperti neuroradiologici vengono in particolar modo proposti, descritti e discussi, stante le loro peculiarità.

È stata altresì condotta una revisione della casistica clinica in quei casi in cui appare rilevante la compromissione meningo-encefalitica, stante la rarità di segnalazioni in letteratura neurologica.

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Authors and Affiliations

  1. Divisione di Neurologia, Ospedale Mauriziano “Umberto I” di Torino, Largo Turati, 62-10100, Torino

    Trebini F., Appiotti A., Bacci R., Daniele D., Inglezis A. & Scarzella G.

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  1. Trebini F.
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  2. Appiotti A.
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  3. Bacci R.
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  4. Daniele D.
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  5. Inglezis A.
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  6. Scarzella G.
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Trebini, F., Appiotti, A., Bacci, R. et al. Vogt-Koyanagi-Harada syndrome: Clinical and instrumental contribution. Ital J Neuro Sci 12, 479–484 (1991). https://doi.org/10.1007/BF02335510

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  • DOI: https://doi.org/10.1007/BF02335510

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