Bilateral occipital calcifications, epilepsy and coeliac disease

Coeliac disease - gluten enteropathy - is a chronic nutritional disorder frequently associated with neurological symptoms. It may present as neuromuscular disease with neuropathy and myositis, there can be epileptic attacks with cerebral calcifications or progressive encephalopathy with ataxia and epilepsy. The authors describe the case-history of a 18-year-old boy with coeliac disease, pharmacoresistent epilepsy and bilateral occipital calcifications. In the case-history are data on psychomotor retardation, repeated febrile attacks from the age of 9 months. Afebrile attacks started at the age of 4 years by atypical absences, at the age of 9 years dominate myoclonic-astatic attacks with falls and at the age of 10 years there are moreover generalized tonic-clonic and tonic attacks. The attacks occur daily and are pharmacoresistent. In the objective finding there was advancing mental deficiency with mild cerebellar symptomatology. On the EEG there was always a disorder of the basic activity, generalized discharges of complexes of the spike and wave with 2-2.5 c/s frequency and enhanced rhytms during sleep. The clinical form of attacks and EEG corresponded with the Lennox-Gastaut syndrome. In the CT picture from the age of 9 years cortico subcortical bilateral occipital but also parietal and frontal calcifications were present which do not change after administration of contrast substance. Results of angiographic examination were normal, in the MR examination are non-specific bilateral occipital changes. Enterobiopsy confirmed chronic enteritis with villous atrophy. The authors draw attention to the possibility that coeliac disease caused pharmacoresistent epilepsy with the finding of bilateral occipital calcifications.