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Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.
  1. A Magaudda,
  2. B Dalla Bernardina,
  3. P De Marco,
  4. Z Sfaello,
  5. M Longo,
  6. V Colamaria,
  7. O Daniele,
  8. G Tortorella,
  9. M A Tata,
  10. R Di Perri
  1. Institute of Neurological and Neurosurgical Sciences, University of Messina, Italy.

    Abstract

    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.

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