Epilepsy, occipital calcifications, and oligosymptomatic celiac disease in childhood
Hugo A Arroyo1, Susana De Rosa, Victor Ruggieri
1Department of Neurology, Hospital Nacional de Pediatría JP Garrahan, Buenos Aires, Argentina. hugoarroyo@arnet.com.ar
Insights
Early diagnosis and gluten-free diet therapy for epilepsy, occipital calcifications, and celiac disease syndrome in children can improve epilepsy outcomes and cognitive status.
Area of Science:
- Neurology
- Pediatrics
- Gastroenterology
Background:
- Epilepsy, occipital calcifications, and celiac disease form a recognized syndrome.
- This syndrome is rare, particularly outside of Italy.
- Understanding its clinical, electrophysiologic, and neuroradiologic features is crucial.
Purpose of the Study:
- To detail the clinical, electrophysiologic, and neuroradiologic characteristics of patients with this syndrome.
- To analyze the impact of early dietary intervention on epilepsy progression.
- To report on white-matter changes associated with the syndrome.
Main Methods:
- Retrospective review of 32 patients diagnosed with epilepsy, occipital calcifications, and celiac disease.
- Diagnosis of celiac disease based on European Society of Pediatric Gastroenterology and Nutrition criteria.
- Analysis of seizure types, epilepsy control, neuroradiologic findings (CT scans), and treatment outcomes.
Main Results:
- All 32 patients had occipital calcifications; 31 had partial seizures, with 21 showing occipital lobe symptoms.
- Epilepsy was generally controlled or sporadic, with 3 severe cases.
- Computed tomography revealed white-matter hypodensities around calcifications in 7 patients, which improved in 3 on a gluten-free diet.
Conclusions:
- A gluten-free diet, especially when initiated early, is associated with a favorable epilepsy outcome.
- Prompt diagnosis of celiac disease may improve epilepsy evolution and cognitive status.
- White-matter hypodensities are a notable finding in this syndrome and may be reversible with dietary therapy.
Abstract:
The association of epilepsy, occipital calcifications, and celiac disease has been recognized as a distinct syndrome. The objective of this study was to present the clinical, electrophysiologic, and neuroradiologic features in a series of patients with this syndrome. Thirty-two patients with the constellation of epilepsy, occipital calcifications, and celiac disease were identified in our epilepsy clinic. The mean age was 11 years and the mean length of follow-up was 7.4 years. The 1990 criteria of the European Society of Pediatric Gastroenterology and Nutrition were used to diagnose celiac disease. The Kruskal-Wallis statistics test was employed with a signficance of P < .05. Thirty-one patients had partial seizures, 21 of them with symptoms related to the occipital lobe. In most patients, the epilepsy was controlled or the seizures were sporadic. Three developed severe epilepsy. Occipital calcifications were present in all cases. Computed tomography in 7 patients showed hypodense areas in the white matter around calcifications, which decreased or disappeared after a period of gluten-free diet in 3 patients. A favorable outcome of epilepsy was detected in patients with the earliest dietary therapy. This study presents the largest series of children with this syndrome outside Italy. White-matter hypodensities surrounding calcifications are rarely reported. A prompt diagnosis of celiac disease might improve the evolution of the epilepsy and may improve cognitive status.
More Related Videos
09:57Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
06:04Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
Published on: August 16, 2024
Related Concept Videos
Epilepsy and Seizures: Overview
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Inborn Errors of Metabolism
Encephalitis l: Introduction
Seizures l: Introduction
Epilepsy ll: Types
