Abstract
Introduction
The prevalence and outcome of the most frequent type of epilepsy in infancy–infantile spasms (IS)—are well characterized in the setting of most neurocutaneous disorders. By contrast, still there is no study describing the natural history of IS in the setting of Sturge–Weber syndrome (SWS).
Materials and methods
Two patients with SWS and IS were identified in our series and five in the literature. The aim of study is to evaluate the clinical, electroencephalographic (EEG) and imaging features of our cases and to compare our cases with those described in the literature. IS in the setting of SWS is an uncommon but possible event (2/19 patients seen over 13 years in our institutions).
Results
We confirmed the correlation between IS and severity of SWS cutaneous and neural (extension of leptomeningeal capillary malformation) phenotype. IS in SWS seems to be atypical both from a clinical viewpoint (they are asymmetric) and from a laboratory viewpoint (EEG is not classically hypsarrhythmic).
Similar content being viewed by others
References
Comi AM (2006) Advances in Sturge–Weber syndrome. Curr Opin Neurol 19:124–128
Cohen MM Jr (2006) Vascular update: morphogenesis, tumors, malformations and molecular dimensions. Am J Med Genet 140(19):2013–2038
Thomas-Sohl KA, Vaslow DF, Maria BL (2004) Sturge–Weber syndrome: a review. Pediatr Neurol 30:303–310
Baselga E (2004) Sturge–Weber syndrome. Semin Cut Med Surg 23:87–98
Browne TR, Holmes GL (2006) Handbook of epilepsy, 3rd edn. Lippincott Williams & Wilkins, Philadelphia
Wallace S, Farrell K (2004) Epilepsy in children. Oxford University Press, London
Guerrini R (2006) Epilepsy in children. Lancet 367:499–524
Kostagal P, Rothner AD (1993) Epilepsy in the setting of neurocutaneous syndromes. Epilepsia 34(S3):S71–S78
Cross JH (2006) Neurocutaneous syndromes and epilepsy—issues in diagnosis and management. Epilepsia 46(Suppl 10):17–23
Hartman AL, Kossof EH (2006) Epilepsy surgery for the neurocutaneous disorders. Semin Pediatr Neurol 13:63–67
Millichap JG, Bickford RG, Klass W, Backus E (1962) Infantile spasms, hypsarrhythmia, and mental retardation. A study of etiologic factors in 61 patients. Epilepsia 3:188–197
Fukuyama Y, Tsuchiya S (1979) A study on Sturge–Weber syndrome. Report of a case associated with infantile spasms and electroencephalographic evolution in five cases. Eur Neurol 18:194–204
Pietz J, Benninger C, Schäfer H, Sontheimer D, Mittermair G, Rating D (1993) Treatment of infantile spasms with high-dosage vitamin B6. Epilepsia 34:757–763
Simonati A, Colamaria V, Bricolo A, Dalla Bernardina B, Rizzuto N (1994) Microgyria associated with Sturge–Weber angiomatosis. Child’s Nerv Syst 10:392–395
Miyama S, Goto T (2004) Leptomeningeal angiomatosis with infantile spasms. Pediatr Neurol 31:353–356
Pascual-Castroviejo I, Diaz-Gonzalez C, Garcia-Melian RM (1993) Sturge–Weber syndrome: study of 40 patients. Pediatr Neurol 9:283–288
Breuner RP, Sharbrough FW (1976) Electroencephalographic evaluation in Sturge–Weber syndrome. Neurology 26:629–632
Curatolo P (2004) Tuberous sclerosis complex: from basic science to clinical phenotypes. McKeith Press, London
Ruggieri M, Pavone L (2000) Hypomelanosis of Ito: clinical syndrome of just phenotype. J Child Neurol 15:635–644
Ormrod D, McClellan K (2001) Topiramate: a review of its use in childhood epilepsy. Pediatr Drugs 3:293–319
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Barbagallo, M., Ruggieri, M., Incorpora, G. et al. Infantile spasms in the setting of Sturge–Weber syndrome. Childs Nerv Syst 25, 111–118 (2009). https://doi.org/10.1007/s00381-008-0705-6
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-008-0705-6