Celiac disease, posterior cerebral calcifications and epilepsy

doi:10.1016/S0387-7604(12)80275-0

Brain and Development

Volume 14, Issue 1, January 1992, Pages 23-29

https://doi.org/10.1016/S0387-7604(12)80275-0 Get rights and content

Ten patients (5 males) affected by epilepsy with cerebral calcifications of unknown etiology mainly located in the posterior regions were subjected to a battery of tests including an intestinal biopsy. Our aim was to establish whether or not the patients also suffered from celiac disease. Celiac disease was found in 6 patients. This result and the individual cases reported in the literature suggest that this triad of diseases (celiac disease, posterior cerebral calcifications and epilepsy) are causally related. The same HLA phenotype was found in all 10 patients, i.e., including the cases without celiac disease, suggesting an underlying disorder of the immune system. Our results emphasize that particular attention should be paid to a search for celiac disease in all patients with epilepsy and posterior cerebral calcifications.

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Citation Excerpt :
Previously, unilateral and bilateral focal T2 hyperintense white matter lesions were found [82]. Brain images play an essential role in the diagnosis of a CEC syndrome (celiac disease, epilepsy, and bilateral occipital calcification) [46,83]. The CEC syndrome is a focal or complex partial type of epilepsy usually originating from the occipital lobe.
Celiac disease (CD) is a systemic, chronic immune-mediated disorder elicited by gluten and related prolamines in genetically susceptible subjects. Main manifestations of CD involve the digestive tract; however, a growing body of evidence supports the theory that symptoms may occur in every part of the body. It is known that some patients with CD can be asymptomatic, and additionally, the incidence of “nonclassical” CD with extraintestinal presentation is apparently increasing. We aimed to perform a thorough review of existing evidence for neurological manifestations of CD, providing an up-to-date description of prevalence and examining the pathogenetic mechanisms possibly involved.
Neurological presentations are rare in children but as many as 36% of adult patients present with neurological findings. With severe malnutrition after progression of CD, different vitamin deficiencies may develop. Such problems can in turn overlap with previous neurological abnormalities including ataxia, epilepsy, neuropathy, dementia, and cognitive disorders. Here, the most prevalent clinical manifestations in adults and children have been discussed in further detail.
Further research is needed to achieve a complete understanding of the nervous system involvement in CD, but clinicians should always remember that neurological and psychiatric symptoms might be part of the CD spectrum of manifestations.
Neurological manifestations of gastrointestinal and hepatic diseases
2007, Neurobiology of Disease
Age-Specific Seizure Disorders
2006, Clinical Neurophysiology of Infancy, Childhood, and Adolescence
Neurological manifestations of gastrointestinal and hepatic diseases
2006, Neurobiology of Disease
Neurologic presentation of celiac disease
2005, Gastroenterology
Celiac disease (CD) long has been associated with neurologic and psychiatric disorders including cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. Earlier reports mainly have documented the involvement of the nervous system as a complication of prediagnosed CD. However, more recent studies have emphasized that a wider spectrum of neurologic syndromes may be the presenting extraintestinal manifestation of gluten sensitivity with or without intestinal pathology. These include migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barré-like syndrome, and neuropathy with positive antiganglioside antibodies. The association between most neurologic syndromes described and gluten sensitivity remains to be confirmed by larger epidemiologic studies. It further has been suggested that gluten sensitivity (as evidenced by high antigliadin antibodies) is a common cause of neurologic syndromes (notably cerebellar ataxia) of otherwise unknown cause. Additional studies showed high prevalence of gluten sensitivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington’s disease. It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. Studies of gluten-free diet in patients with gluten sensitivity and neurologic syndromes have shown variable results. Diet trials also have been inconclusive in autism and schizophrenia, 2 diseases in which sensitivity to dietary gluten has been implicated. Further studies clearly are needed to assess the efficacy of gluten-free diet and to address the underlying mechanisms of nervous system pathology in gluten sensitivity.

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^†: Presented at the 1st Joint Meeting of the British Society of Paediatric Gastroenterology and Nutrition and the Pediatric Gastroenterology Group of the Italian Society of Pediatrics, Parma (Italy), 21–23/9/1989; at the International Dicke Symposium, Leiden (The Netherlands), 225/1990; at the 16th Meeting of Italian Society of Neuropediatrics, Genova (Italy), 25–27/10/1990 and at the Joint Convention of the 5 th International Child Neurology Congress and the 3rd Asian and Oceanian Congress of Child Neurology, Tokyo (Japan), 4–9/11/1990.

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Celiac disease, posterior cerebral calcifications and epilepsy†

Lancet

Lancet

Brain Dev (Tokyo)

Am J Med

Neurological disorders associated with celiac disease

Brain

Neurological manifestations of malabsorption

Neurological and psychiatric complications

Neurological disorders and adult coeliac disease

Gut

Increased prevalence of epilepsy in coeliac disease

Gut

Increased prevalence of epilepsy in coeliac disease

Br Med J

A controlled study of folic acid in epilepsy

Neurology

Epilepsy and folate deficiency

Br Med J

Epilepsy with bilateral occipital calcifications: a benign onset with progressive severity

Neurology

Sturge-Weber syndrome without port-wine facial nevus: report of two cases studied by C.T

Child's Brain

Commission on classification and terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures

Epilepsia

Unexplained bilateral occipital calcifications and reduced vision

Neuropediatrics

The syndrome of epilepsy and bilateral occipital cortical calcifications

Epilepsia

Two siblings with multiple intracranial haemangiomatosis with calcifications

J Neurol