Producción Académica UCC

Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome

Magaudda, Adriana ORCID:, Dalla Bernardina, Bernardo ORCID:, De Marco, Paolo ORCID:, Sfaello, Zenon Milciades, Longo, Michele ORCID:, Colamaria, V., Daniele, O., Tortorella, G., Tata, M.A., Di Pierri, R. and Meduri, M. (1993) Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome. Journal of Neurology. Neurosurgery and Psychiatry, 56 (8). pp. 885-889. ISSN 0022-3050

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Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified.

Tipo de documento: Artículo
Palabras clave: Adolescent. Adult. Brain Diseases. Calcinosis. Celiac Disease. Child. Epilepsies. Partial. Epilepsy Generalized. Female. Human. Laterality. Male. Occipital Lobe. Syndrome. Tomography. X-Ray Computed.
Temas: R Medicina > R Medicina (General)
Unidad académica: Universidad Católica de Córdoba > Facultad de Ciencias de la Salud
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