Top

Published in:

01-01-2009 | Original Paper

Infantile spasms in the setting of Sturge–Weber syndrome

Authors: Massimo Barbagallo, Martino Ruggieri, Gemma Incorpora, Piero Pavone, Caterina Nucifora, Alberto Spalice, Andrea Domenico Praticò, Agata Polizzi, Lorenzo Pavone, Paola Iannetti

Published in: Child's Nervous System | Issue 1/2009

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).

Comi AM (2006) Advances in Sturge–Weber syndrome. Curr Opin Neurol 19:124–128PubMedCrossRef

Cohen MM Jr (2006) Vascular update: morphogenesis, tumors, malformations and molecular dimensions. Am J Med Genet 140(19):2013–2038CrossRef

Thomas-Sohl KA, Vaslow DF, Maria BL (2004) Sturge–Weber syndrome: a review. Pediatr Neurol 30:303–310PubMedCrossRef

Baselga E (2004) Sturge–Weber syndrome. Semin Cut Med Surg 23:87–98CrossRef

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 367:499–524PubMedCrossRef

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

10.

Hartman AL, Kossof EH (2006) Epilepsy surgery for the neurocutaneous disorders. Semin Pediatr Neurol 13:63–67PubMedCrossRef

11.

Millichap JG, Bickford RG, Klass W, Backus E (1962) Infantile spasms, hypsarrhythmia, and mental retardation. A study of etiologic factors in 61 patients. Epilepsia 3:188–197PubMed

12.

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 18:194–204PubMedCrossRef

13.

Pietz J, Benninger C, Schäfer H, Sontheimer D, Mittermair G, Rating D (1993) Treatment of infantile spasms with high-dosage vitamin B6. Epilepsia 34:757–763PubMedCrossRef

14.

Simonati A, Colamaria V, Bricolo A, Dalla Bernardina B, Rizzuto N (1994) Microgyria associated with Sturge–Weber angiomatosis. Child’s Nerv Syst 10:392–395CrossRef

15.

Miyama S, Goto T (2004) Leptomeningeal angiomatosis with infantile spasms. Pediatr Neurol 31:353–356PubMedCrossRef

16.

Pascual-Castroviejo I, Diaz-Gonzalez C, Garcia-Melian RM (1993) Sturge–Weber syndrome: study of 40 patients. Pediatr Neurol 9:283–288PubMedCrossRef

17.

Breuner RP, Sharbrough FW (1976) Electroencephalographic evaluation in Sturge–Weber syndrome. Neurology 26:629–632

18.

Curatolo P (2004) Tuberous sclerosis complex: from basic science to clinical phenotypes. McKeith Press, London

19.

Ruggieri M, Pavone L (2000) Hypomelanosis of Ito: clinical syndrome of just phenotype. J Child Neurol 15:635–644PubMedCrossRef

20.

Ormrod D, McClellan K (2001) Topiramate: a review of its use in childhood epilepsy. Pediatr Drugs 3:293–319CrossRef

Title: Infantile spasms in the setting of Sturge–Weber syndrome
Authors: Massimo Barbagallo
Martino Ruggieri
Gemma Incorpora
Piero Pavone
Caterina Nucifora
Alberto Spalice
Andrea Domenico Praticò
Agata Polizzi
Lorenzo Pavone
Paola Iannetti
Publication date: 01-01-2009
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 1/2009
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-008-0705-6

2024 ASCO Annual Meeting

Springer Medicine

Infantile spasms in the setting of Sturge–Weber syndrome

Abstract

Introduction

Materials and methods

Results

2024 ASCO Annual Meeting

Springer Medicine

Abstract

Introduction

Materials and methods

Results

Please log in to get access to this content

Other articles of this Issue 1/2009

Comparison of shunt infection incidence in high-risk subgroups receiving antibiotic-impregnated versus standard shunts

Shortening of ventricular shunt catheter associated with cranial growth: effect of the frontal and parieto-occipital access route on long-term shunt patency

Local chemotherapy in the rat brainstem with multiple catheters: a feasibility study

Sagittal craniosynostosis combined with ossified cephalhematoma—a tricky and demanding puzzle

Cerebellar pilocytic astrocytoma presenting with intratumor bleeding, subarachnoid hemorrhage, and subdural hematoma

Pediatric glioblastoma cell line shows different patterns of expression of transmembrane ABC transporters after in vitro exposure to vinblastine