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 significance 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. (J Child Neurol 2002;17:800—806).
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References
1.
Chapman Rwg, Laidlow JM, Colin-Jones D., et al: Increased prevalence of epilepsy in coeliac disease. BMJ1978;2:250—251.
2.
Cooke WT, Smith WT: Neurological disorders associated with adult celiac disease. Brain1966;89:683—722.
3.
Garwicz S., Mortensson W.: Intracranial calcification mimicking the Sturge-Weber syndrome. A consequence of cerebral folic acid deficiency?Pediatr Radiol1976;5:5—9.
4.
Ambrosetto P. , Ambrosetto G., Michelucci R., Bacci A.: Sturge-Weber syndrome without port wine facial nevus. Childs Brain1983;10:387—392.
5.
Sammaritano M., Andermann F., Helanson D., et al: The syndrome of epilepsy and bilateral occipital cortical calcifications. Epilepsia1985;26:532.
6.
Piattella L. , Zamponi N., Cenci L., et al: Calcificazioni bioccipitali ed epilepssia: Malattia di Sturge-Weber atipica o nuova sindrome? Boll Lega Ital Epi1986;54/55:139—142.
7.
Battistella PA, Mattesi P., Casara Gl , et al: Bilateral cerebral occipital calcifications and migraine-like headache. Cephalalgia1987 ;7:125—128.
8.
Giroud M., Nivelon-Chevalier A., Vicard C., et al: Epilepsie et calcifications corticales occipitales bilatèrales. Un entititè nouvelle?Presse Med1987;6:308—309.
9.
Taly AB, Nagaraja D., Das S., et al: Sturge-Weber-Dimitri disease without facial nevus. Neurology1987;37:1063—1064.
10.
Del Giudice E., Romano A., Pelosi L., et al: Bilateral cerebral posterior calcifications and epilepsy: Atypical Sturge-Weber or a new nosological entity? in Faienza C, Prati GL (eds): Diagnostic and Therapeutic Problems in Pediatric Epileptology. Amsterdam, Elsevier Science Publishers, 1988, 183—184.
11.
Gobbi G., Sorrenti G., Santucci M., et al: Epilepsy with bilateral occipital calcifications: A benign onset with progressive severity. Neurology1988;38:913—920.
Giroud M., Borsotti JP, Michiels R., et al: Épilepsie et calcifications occipitales bilatérales: 3 cas. Rev Neurol (Paris)1990;146:288—292.
14.
De Marco P. , Lorenzin G.Growing bilateral occipital calcifications and epilepsy. Brain Dev1990;12:342—344.
15.
Del Giudice E., Romano A., Pelosi L., et al: Unexplained bilateral occipital calcifications and reduced vision. Neuropediatrics1984;15:218—219.
16.
Sammaritano M., Andermann F., Melanson D., et al: The syndrome of intractable epilepsy, bilateral occipital calcifications and folic acid deficiency. Neurology1988;38(Suppl 1):239.
17.
Gobbi G., Bouquet F., Greco L., et al: Coeliac disease, epilepsy, and cerebral calcifications. Lancet1992;340:439—443.
18.
Magaudda A. , Dalla Bernardina B, De Marco P, et al: Bilateral occipital calcifications epilepsy and coeliac disease: Clinical and neuroimaging features of a new syndrome. J Neurol Neurosurg Psychiatry1993;56:885—889.
19.
Bye Ame, Andermann F., Robitaille Y., et al: Cortical vascular abnormalities in the syndrome of celiac disease, epilepsy, bilateral occipital calcifications, and folate deficiency. Ann Neurol1993;34:399—403.
20.
Lea ME, Harbord M., Sage MR: Bilateral occipital calcification associated with celiac disease, folate deficiency, and epilepsy. AJNR Am J Neuroradiol1995;16:1498—1500.
21.
Pinilla Moraza J, Gil Pujades A, Labarga Echeverria P, Astiazarán Echevarría F: Epilepsia, calcificaciones cerebrales y enfermedad celíaca. Un nuevo caso . Rev Neurol1995;10:214—215.
22.
Cuveillier JC, Vallee L., Nuyts JP: Le syndrome maladie coeliaque, calcifications cerebrales, et epilepsie. Arch Pediatr1996 ;3:1013—1019.
23.
Hernandez MA , Ortigosa L., de la Colina G., Togores JM: Epilepsy, cerebral calcifications and latent coeliac disease: Clinical and evolution. Epilepsia1997;38(Suppl 7):121.
24.
Martinez-Bermejo A., Polanco I., Royo A., et al: A study of Gobbi's syndrome in a Spanish population. Rev Neurol1999;29:105—110.
25.
Arroyo HA, De Rosa S., Ruggieri V., et al: Epilepsia, calcificaciones cerebrales y enfermedad celíaca. Arch Arg Pediatr1995;93:310—316.
26.
Arroyo HA, De Rosa S., Fejerman N., and Members of the Working Group.Epilepsy, cerebral calcification, and celiac disease: Argentine multicentre experience, in Gobbi G, Andermann F, Naccarato S , Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease. London, John Libbey & Company, 1997, 93—101.
27.
Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia1981;22:489—501.
28.
Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia1989;30:389—399.
29.
Jaspers H.: Report of committee on methods of clinical exam in EEG. Electroencephalogr Clin Neurophysiol1958;10:370—375.
30.
Marsh NN: Studies of intestinal lymphoid tissue. The predictive value of raised mitotic indices among jejunal epithelial lymphocytes in the diagnosis of gluten sensitive enteropathy. J Clin Pathol1982;35:517—525.
31.
Walker Smith JA, Guandalini S., Schmitz J., et al: Revised criteria for the diagnosis of coeliac disease. Report of a working group of ESPGAN. Arch Dis Child1990;65:909—911.
32.
Spencer J., Isaacson PG, Mac Donald TT, et al: Gamma-delta T cells and the diagnosis of celiac disease. Exp Immunol1991;85:109—113.
33.
De Rosa S., Litwin N., Dávila Mtg, et al: Correlación de anticuerpos antigliadina y antiendomisiales clase IgA (AGA-IgA, EMA-IgA) con histología intestinal en la enfermedad celíaca . Acta Gastroenterol Latinoam1993;23:19—25.
34.
Palavecino EA , Motta AH, Awad J., et al: HLA and celiac disease in Argentina: Involvement of the DQ subregion. Dis Markers1990;8:5—10.
35.
Gobbi G., Bertani G., and the ItalianWorking Group on Celiac Disease and Epilepsy: in Gobbi G, Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease . London, John Libbey & Company , 1997, 65—79.
36.
Vascotto M., Fois A.: Frequency of epilepsy in celiac disease and vice versa: A collaborative study , in Gobbi G, Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease. London, John Libbey & Company, 1997, 105—110.
37.
Del Giudice E : Gluten free diet and evolution of the seizures in celiac disease and epilepsy, in Gobbi G , Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease. London, John Libbey & Company, 1997, 89—92.
38.
Bardella MT , Molteni N., Prampolini L., et al: Need for follow up in celiac disease . Arch Dis Child1994;70:211—213.
39.
Ambrosetto G., Antonini L., Tassinari A.: Occipital lobe seizures related to clinically asymptomatic celiac disease in adulthood. Epilepsia1992;33:476—481.
40.
Tiacci C., D'Alessandro P., Cantisani TA, et al: Epilepsy with bilateral occipital calcifications: Sturge Weber variant or a different encephalopathy?Epilepsia1993;34:528—539.
41.
Fois A., Vascotto M., Di Bartolo RM, Di Marco V: Celiac disease and epilepsy in pediatric patients . Childs Nerv Syst1994;10:450—454.
42.
Fois A., Balestri P., Vascotto M., et al: Progressive cerebral calcifications, epilepsy, and celiac disease . Brain Dev1993;15:79—82.
43.
Piattella L. , Zamponi N., Cardinali C., et al: Endocranial calcifications, infantile celiac disease, and epilepsy. Childs Nerv Syst1993;9:172—175.
44.
Cajade AG, Cohen JA, Heiner LA: Vascular causes of white matter diseases. Neuroimaging Clin North Am1993;3-2:361—377.
45.
Hinchey J., Chaves C., Appignani B., et al: A reversible posterior leukoencephalopathy syndrome. N Engl J Med1996;334:494—500.
46.
Aicardi J.: Diseases of the nervous system in childhood, in Clinics in Development Medicine. London, Mac Keith Press,1992, 518—588.
47.
Aicardi J., Goutieres F.: A progressive familial encephalopathy in infancy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis. Ann Neurol1984;15:49—54.
48.
Razavi-Encha F., Larroche JC, Gaillard D.: Infantile familial encephalopathy with cerebral calcifications and leukodystrophy. Neuropediatrics1988;19:72—79.
49.
Cohen BH, Packer RJ: Adverse neurologic effects of chemotherapy and radiation therapy, in Berg BO (ed): Neurologic Aspects of Pediatrics. Butterworth Heinemann, Stonehan, 1992, 567—594.
50.
Holmes Gkt: Neurological and psychiatric complications in celiac disease, in Gobbi G, Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease . London, John Libbey & Company , 1997, 261—264.
51.
Longo M., Magaudda B., Dalla Bernardina B., et al: Sindrome delle calcificazioni occipitali bilaterali, epilessia, e malattia celiaca. Diagnosi differenziale con la Sturge-Weber. Studi multicentrico in 20 casi. Neuroradiologia1992;109—112.
52.
Arroyo HA, Avalos M., Fejerman N.: Epilepsia, calcificaciones cerebrales, enfermedad celíaca y síndrome de Sturge Weber: Sus diferencias. Rev Neurol1995;23(Suppl):381.
53.
Howdle PD: Immunological aspects of celiac disease: Some systemic perturbations, in Gobbi G, Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease. London, John Libbey & Company, 1997, 47—54.
54.
Rush JP, Inman R., Bernstein M., et al: Isolated vasculitis of the central nervous system in a patient with celiac disease. Am J Med1986;81:1092—1094.
55.
Mazzilli MC, Bonamico M.: Role of the HLA class molecules in the pathogenesis of celiac disease, in Gobbi G, Andermann F, Naccarato S, Banchini G (eds): Epilepsy and Other Neurological Disorders in Celiac Disease. London, John Libbey & Company, 1997, 55—58.
56.
Herrera M., Theiler G., Augustovski F., et al: Molecular characterization of HLA class II genes in celiac disease patients of Latin American Caucasian origin. Tissue Antigens1994;39:1—5.
