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[A study of Gobbi's syndrome in Spanish population]
A Martínez-Bermejo1, I Polanco, A Royo
1Servicio de Neurología, Hospital Universitario Infantil La Paz, Universidad Autónoma de Madrid, España. med014988@nacom.es
Introduction:
The association of epilepsy, occipital cerebral calcifications and coeliac disease was recognized for the first time in 1992 by Gobbi as an independent syndrome. He emphasized that it occurred almost exclusively in persons of Italian origin.
Objective:
To define the prevalence of this syndrome in the Spanish population with a view to confirming its probably genetic etiopathogenesis.
Patients And Methods:
Neurological studies were done in 44 coeliac patients, as were cranial CT scans. All cases of Spanish origin described in the literature were noted.
Results:
In the patients with coeliac disease there was an increased incidence of crises and generalized epilepsy, but no patient was found to have occipital calcifications. Only 12 cases of Spanish origin were found, mainly in the Canary Isles and Mediterranean littoral, in spite of the high incidence of coeliac disease in Spain. No familial cases were seen.
Conclusions:
We suspect a genetic etiopathogenesis, associated with different environmental factors, to be the origin of the syndrome. This is supported by the common geographical origin of most cases and the anatomopathological findings described in those cases studied.