Pediatric Sturge-Weber Syndrome Medication

  • Author: Masanori Takeoka, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Jan 5, 2010
 

Medication Summary

Please refer to the various articles that describe anticonvulsant treatment of partial seizures.

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Anticonvulsants

Class Summary

These agents are used to terminate clinical and electrical seizure activity as rapidly as possible and prevent seizure recurrence.

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Carbamazepine (Tegretol)

 

Anticonvulsant effective for treatment of complex partial seizures. Appears to act by reducing polysynaptic responses and blocking posttetanic potentiation. Major mechanism of action is reduction of sustained high-frequency repetitive neural firing.

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Phenytoin (Dilantin)

 

Primary site of action of hydantoins, such as phenytoin, appears to be motor cortex, where may inhibit spread of seizure activity. May reduce maximal activity of brainstem centers responsible for tonic phase of grand mal seizures. Dosing should be individualized. If daily dosing cannot be divided equally, larger dose should be given before retiring. Phosphorylated formulation, fosphenytoin, available for parenteral use and may be given IM or IV.

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Valproic acid (Depakote, Depakene, Depacon)

 

Chemically unrelated to other drugs used to treat seizure disorders. Although mechanism of action unknown, activity may be related to increased brain levels of GABA or enhanced GABA action. Also may potentiate postsynaptic GABA responses, affect potassium channels, or have direct membrane-stabilizing effect. For conversion to monotherapy, concomitant AED dosage ordinarily can be reduced by approximately 25% every 2 wk. This reduction may be started at initiation of therapy or delayed by 1-2 wk if concern that seizures are likely to occur with reduction. Monitor patients closely during this period for increased seizure frequency. As adjunctive therapy, divalproex sodium may be added to patient's regimen at dosage of 10-15 mg/kg/d. Dosage may be increased by 5-10 mg/kg/wk to achieve optimal clinical response. Ordinarily, optimal clinical response achieved at daily doses < 60 mg/kg/d.

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Gabapentin (Neurontin)

 

Has properties in common with other anticonvulsants. However, exact mechanism of action unknown. Structurally related to GABA but does not interact with GABA receptors. Increases in daily dose are best tolerated when done slowly.

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Lamotrigine (Lamictal)

 

Triazine derivative useful in treatment of both seizures and neuralgic pain. Inhibits release of glutamate and inhibits voltage-sensitive sodium channels, which stabilizes neuronal membrane. Follow manufacturer's recommendation for dose adjustments.

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Topiramate (Topamax)

 

Sulfamate-substituted monosaccharide with broad spectrum of antiepileptic activity that may have state-dependent sodium channel– blocking action. Potentiates inhibitory activity of neurotransmitter GABA. May block glutamate activity. Not necessary to monitor plasma concentrations to optimize therapy. On occasion, addition to phenytoin may require adjustment of phenytoin dose to achieve optimal clinical outcome.

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Tiagabine (Gabitril)

 

Mechanism of action in antiseizure effect unknown. However, believed to be related to its ability to enhance activity of GABA, major inhibitory neurotransmitter in CNS. May block GABA uptake into presynaptic neurons, permitting more GABA to be available for receptor binding on surfaces of postsynaptic cells and possibly prevents propagation of neural impulses that contribute to seizures by GABA-ergic action. Dosing modification of concomitant AEDs not necessary unless clinically indicated.

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Felbamate (Felbatol)

 

Oral antiepileptic agent with weak inhibitory effects on GABA-receptor binding and benzodiazepine-receptor binding. Has little activity at MK-801 receptor-binding site of NMDA receptor-ionophore complex. However, is antagonist at strychnine-insensitive glycine-recognition site of NMDA receptor-ionophore complex. Not indicated as first-line antiepileptic treatment. Recommended for use only in patients whose epilepsy is so severe that benefits outweigh risks of aplastic anemia or liver failure. Most adverse effects during adjunctive therapy resolve as dosage of concomitant AEDs decreased.

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Phenobarbital (Luminal, Barbita)

 

Exhibits anticonvulsant activity in anesthetic doses and can be administered orally. If IM route chosen, inject into large muscle such as gluteus maximus, vastus lateralis, or other areas where little risk of encountering nerve trunk or major artery. Injection into or near peripheral nerves may result in permanent neurological deficit. Restrict IV use to conditions in which other routes are not feasible, either because patient unconscious, as in cerebral hemorrhage, eclampsia, or status epilepticus, or because prompt action imperative.

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Oxcarbazepine (Trileptal)

 

Pharmacological activity primarily by 10-monohydroxy metabolite. Studies indicate that this drug may block voltage-sensitive sodium channels, inhibit repetitive neuronal firing, and impair synaptic impulse propagation. This drug's anticonvulsant effect may occur by affecting potassium conductance and high-voltage activated calcium channels. Drug pharmacokinetics are similar in older children (>8 y) and adults. Young children ( < 8 y) have 30-40% increased clearance compared with older children and adults. Children < 2 years have not been studied in controlled clinical trials.

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Zonisamide (Zonegran)

 

Indicated for adjunct treatment of partial seizures with or without secondary generalization. Evidence that is effective in myoclonic and other generalized seizure types as well.

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Levetiracetam (Keppra)

 

Used as add-on therapy for partial seizures. Mechanism of action unknown. Has favorable adverse effect profile, with no life-threatening toxicity reported.

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Pregabalin (Lyrica)

 

Structural derivative of GABA. Mechanism of action unknown. Binds with high affinity to alpha2-delta site (a calcium channel subunit). In vitro, reduces calcium-dependent release of several neurotransmitters, possibly by modulating calcium channel function. FDA approved for neuropathic pain associated with diabetic peripheral neuropathy or postherpetic neuralgia and as adjunctive therapy in partial-onset seizures.

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Clonazepam (Klonopin)

 

Long-acting benzodiazepine that increases the presynaptic GABA inhibition and reduces the monosynaptic and polysynaptic reflexes. Suppresses muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters. Has multiple indications, including suppression of myoclonic, akinetic, or petit mal seizure activity and focal or generalized dystonias (eg, tardive dystonia). Reaches peak plasma concentration at 2-4 h after oral or rectal administration.

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Lorazepam (Ativan)

 

Sedative hypnotic with short onset of effects and relatively long half-life.

By increasing the action of gamma-aminobutyric acid (GABA), which is a major inhibitory neurotransmitter in the brain, may depress all levels of CNS, including limbic and reticular formation.

Important to monitor patient's blood pressure after administering dose. Adjust as necessary.

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Contributor Information and Disclosures
Author

Masanori Takeoka, MD  Assistant Professor, Department of Neurology, Harvard Medical School; Staff Physician, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Children's Hospital Boston

Masanori Takeoka, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, Child Neurology Society, and Massachusetts Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

James J Riviello Jr, MD  George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital

James J Riviello Jr, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert J Baumann, MD  Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Kenneth J Mack, MD, PhD  Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic

Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center, Washington DC

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

References

  1. Aicardi J. Diseases of the Nervous System in Childhood. 2nd ed. London: Mac Keith Press. 1998.

  2. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg. Jun 2004;23(2):87-98. [Medline].

  3. Bodensteiner JB, Roach ES. Sturge-Weber Syndrome: Introduction and Overview. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Sturge Weber Foundation. Mt Freedom, New Jersey. 1999.

  4. Boltshauser E, Wilson J, Hoare RD. Sturge-Weber syndrome with bilateral intracranial calcification. J Neurol Neurosurg Psychiatry. May 1976;39(5):429-35. [Medline].

  5. Bar-Sever Z, Connolly LP, Barnes PD. Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome. J Nucl Med. Jan 1996;37(1):81-3. [Medline].

  6. Sharan S, Swamy B, Taranath DA, et al. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS. Aug 2009;13(4):374-8. [Medline].

  7. Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am. Aug 1992;39(4):591-620. [Medline].

  8. Comi AM. Advances in Sturge-Weber syndrome. Curr Opin Neurol. Apr 2006;19(2):124-8. [Medline].

  9. Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol. Aug 2003;18(8):509-16. [Medline].

  10. Norman MG, Schoene WC. The ultrastructure of Sturge-Weber disease. Acta Neuropathol (Berl). Mar 31 1977;37(3):199-205. [Medline].

  11. Garcia JC, Roach ES, McLean WT. Recurrent thrombotic deterioration in the Sturge-Weber syndrome. Childs Brain. 1981;8(6):427-33. [Medline].

  12. Gomez MR, Bebin EM. Sturge-Weber syndrome. In: Butterworths B. Neurocutaneous Diseases: A Practical Approach. 1987:356-367.

  13. Aylett SE, Neville BG, Cross JH. Sturge-Weber syndrome: cerebral haemodynamics during seizure activity. Dev Med Child Neurol. Jul 1999;41(7):480-5. [Medline].

  14. Okudaira Y, Arai H, Sato K. Hemodynamic compromise as a factor in clinical progression of Sturge- Weber syndrome. Childs Nerv Syst. Apr 1997;13(4):214-9. [Medline].

  15. Maria BL, Neufeld JA, Rosainz LC. Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression. J Child Neurol. Dec 1998;13(12):606-18. [Medline].

  16. Reid DE, Maria BL, Drane WE. Central nervous system perfusion and metabolism abnormalities in Sturge- Weber syndrome. J Child Neurol. Apr 1997;12(3):218-22. [Medline].

  17. Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol. Jan 1995;10(1):49-58. [Medline].

  18. Udani V, Pujar S, Munot P, Maheshwari S, Mehta N. Natural history and magnetic resonance imaging follow-up in 9 Sturge-Weber Syndrome patients and clinical correlation. J Child Neurol. Apr 2007;22(4):479-83. [Medline].

  19. Roach ES, Bodensteiner JB. Neurologic manifestations of Sturge-Weber Syndrome. In: Sturge-Weber Syndrome. Mt Freedom, New Jersey: Sturge Weber Foundation; 1999:27-38.

  20. Huq AH, Chugani DC, Hukku B. Evidence of somatic mosaicism in Sturge-Weber syndrome. Neurology. Sep 10 2002;59(5):780-2. [Medline].

  21. Cunha e Sá M, Barroso CP, Caldas MC. Innervation pattern of malformative cortical vessels in Sturge-Weber disease: an histochemical, immunohistochemical, and ultrastructural study. Neurosurgery. Oct 1997;41(4):872-6; discussion 876-7. [Medline].

  22. Comi AM, Weisz CJ, Highet BH. Sturge-Weber syndrome: altered blood vessel fibronectin expression and morphology. J Child Neurol. Jul 2005;20(7):572-7. [Medline].

  23. Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol. May 2004;30(5):303-10. [Medline].

  24. Erba G, Cavazzuti V. Sturge-Weber Syndrome: A natural history. J Epilepsy. 1990;3(Suppl):287-291.

  25. Riviello JJ, Erba G, Lombroso CT, et al. Outcome of epilepsy surgery for Sturge-Weber Syndrome. Epilepsia. 1998;39:171.

  26. Oakes WJ. The natural history of patients with the Sturge-Weber syndrome. Pediatr Neurosurg. 1992;18(5-6):287-90. [Medline].

  27. Rochkind S, Hoffman HJ, Hendrick EB. Sturge-Weber Syndrome: Natural history and prognosis. J Epilepsy. 1990;3(Suppl):293-304.

  28. Sener RN. Sturge-Weber syndrome: a patient with a cervical port-wine nevus. Comput Med Imaging Graph. Nov-Dec 1997;21(6):359-60. [Medline].

  29. Enjolras O, Riche MC, Merland JJ. Facial port-wine stains and Sturge-Weber syndrome. Pediatrics. Jul 1985;76(1):48-51. [Medline].

  30. Bioxeda P, de Misa RF, Arrazola JM. [Facial angioma and the Sturge-Weber syndrome: a study of 121 cases]. Med Clin (Barc). May 29 1993;101(1):1-4. [Medline].

  31. Tallman B, Tan OT, Morelli JG. Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications. Pediatrics. Mar 1991;87(3):323-7. [Medline].

  32. Williams DW 3d, Elster AD. Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome. Am J Neuroradiol. Jan-Feb 1992;13(1):291-4. [Medline].

  33. Bebin EM, Gomez MR. Prognosis in Sturge-Weber disease: comparison of unihemispheric and bihemispheric involvement. J Child Neurol. Jul 1988;3(3):181-4. [Medline].

  34. Pascual-Castroviejo I, Diaz-Gonzalez C, Garcia-Melian RM. Sturge-Weber syndrome: study of 40 patients. Pediatr Neurol. Jul-Aug 1993;9(4):283-8. [Medline].

  35. Coley SC, Britton J, Clarke A. Status epilepticus and venous infarction in Sturge-Weber syndrome. Childs Nerv Syst. Dec 1998;14(12):693-6. [Medline].

  36. Jung A, Raman A, Rowland Hill C. Acute hemiparesis in Sturge-Weber syndrome. Pract Neurol. Jun 2009;9(3):169-71. [Medline].

  37. Sujansky E, Conradi S. Outcome of Sturge-Weber syndrome in 52 adults. Am J Med Genet. May 22 1995;57(1):35-45. [Medline].

  38. Uram M, Zubillaga C. The cutaneous manifestations of Sturge-Weber syndrome. J Clin Neuroophthalmol. Dec 1982;2(4):245-8. [Medline].

  39. Shimakawa S, Miyamoto R, Tanabe T, Tamai H. Prolonged left homonymous hemianopsia associated with migraine-like attacks in a child with Sturge-Weber syndrome. Brain Dev. Oct 1 2009;[Medline].

  40. Maria BL, Neufeld JA, Rosainz LC. High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome. J Child Neurol. Dec 1998;13(12):595-605. [Medline].

  41. Klapper J. Headache in Sturge-Weber syndrome. Headache. Oct 1994;34(9):521-2. [Medline].

  42. Cheng KP. Ophthalmologic manifestations of Sturge-Weber syndrome. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Mt Freedom, New Jersey: Sturge Weber Foundation; 1999:17-26.

  43. Miles L, Eisenbaum AM, Biglan AW et al. Guidelines: Glaucoma and Sturge-Weber Syndrome, SWF Web Page.

  44. Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. Nov-Dec 1992;29(6):349-56. [Medline].

  45. Sagi E, Aram H, Peled IJ. Basal cell carcinoma developing in a nevus flammeus. Cutis. Mar 1984;33(3):311-2, 318. [Medline].

  46. Laufer L, Cohen A. Sturge-Weber syndrome associated with a large left hemispheric arteriovenous malformation. Pediatr Radiol. 1994;24(4):272-3. [Medline].

  47. Gobbi G, Bouquet F, Greco L. Coeliac disease, epilepsy, and cerebral calcifications. The Italian Working Group on Coeliac Disease and Epilepsy. Lancet. Aug 22 1992;340(8817):439-43. [Medline].

  48. Maria BL, Hoang KBN, Robertson RL et al. Imaging brain structure and function in Sturge-Weber Syndrome. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Sturge Weber Foundation. Mt Freedom, New Jersey. Sturge Weber Syndrome. 1999,43-69;43-69.

  49. Wilms G, Van Wijck E, Demaerel P. Gyriform calcifications in tuberous sclerosis simulating the appearance of Sturge-Weber disease. Am J Neuroradiol. Jan-Feb 1992;13(1):295-7. [Medline].

  50. Borns PF, Rancier LF. Cerebral calcification in childhood leukemia mimicking Sturge-Weber syndrome. Report of two cases. Am J Roentgenol Radium Ther Nucl Med. Sep 1974;122(1):52-5. [Medline].

  51. Terdjman P, Aicardi J, Sainte-Rose C. Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics. Aug 1991;22(3):115-20. [Medline].

  52. Marti-Bonmati L, Menor F, Mulas F. The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings. Childs Nerv Syst. Apr 1993;9(2):107-9. [Medline].

  53. Sugama S, Yoshimura H, Ashimine K. Enhanced magnetic resonance imaging of leptomeningeal angiomatosis. Pediatr Neurol. Oct 1997;17(3):262-5. [Medline].

  54. Fischbein NJ, Barkovich AJ, Wu Y. Sturge-Weber syndrome with no leptomeningeal enhancement on MRI. Neuroradiology. Mar 1998;40(3):177-80. [Medline].

  55. Adamsbaum C, Pinton F, Rolland Y. Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations. Pediatr Radiol. Nov 1996;26(11):759-62. [Medline].

  56. Griffiths PD, Blaser S, Boodram MB. Choroid plexus size in young children with Sturge-Weber syndrome. Am J Neuroradiol. Jan 1996;17(1):175-80. [Medline].

  57. Benedikt RA, Brown DC, Walker R. Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol. Mar-Apr 1993;14(2):409-15. [Medline].

  58. Juhasz C, Lai C, Behen ME, Muzik O, Helder EJ, Chugani DC, et al. White matter volume as a major predictor of cognitive function in Sturge-Weber syndrome. Arch Neurol. Aug 2007;64(8):1169-74. [Medline].

  59. Bernal B, Altman N. Visual functional magnetic resonance imaging in patients with Sturge-Weber syndrome. Pediatr Neurol. Jul 2004;31(1):9-15. [Medline].

  60. Lin DD, Barker PB, Kraut MA. Early characteristics of Sturge-Weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR Am J Neuroradiol. Oct 2003;24(9):1912-5. [Medline].

  61. Cakirer S, Yagmurlu B, Savas MR. Sturge-Weber syndrome: diffusion magnetic resonance imaging and proton magnetic resonance spectroscopy findings. Acta Radiol. Jul 2005;46(4):407-10. [Medline].

  62. Mentzel HJ, Dieckmann A, Fitzek C. Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography. Pediatr Radiol. Jan 2005;35(1):85-90. [Medline].

  63. Sivaswamy L, Rajamani K, Juhasz C, Maqbool M, Makki M, Chugani HT. The corticospinal tract in Sturge-Weber syndrome: a diffusion tensor tractography study. Brain Dev. Aug 2008;30(7):447-53. [Medline].

  64. Moritani T, Kim J, Sato Y, Bonthius D, Smoker WR. Abnormal hypermyelination in a neonate with Sturge-Weber syndrome demonstrated on diffusion-tensor imaging. J Magn Reson Imaging. Mar 2008;27(3):617-20. [Medline].

  65. Griffiths PD, Boodram MB, Blaser S. 99mTechnetium HMPAO imaging in children with the Sturge-Weber syndrome: a study of nine cases with CT and MRI correlation. Neuroradiology. Mar 1997;39(3):219-24. [Medline].

  66. Namer IJ, Battaglia F, Hirsch E. Subtraction ictal SPECT co-registered to MRI (SISCOM) in Sturge-Weber syndrome. Clin Nucl Med. Jan 2005;30(1):39-40. [Medline].

  67. Pinton F, Chiron C, Enjolras O. Early single photon emission computed tomography in Sturge-Weber syndrome. J Neurol Neurosurg Psychiatry. Nov 1997;63(5):616-21. [Medline].

  68. Chugani HT, Mazziotta JC, Phelps ME. Sturge-Weber syndrome: a study of cerebral glucose utilization with positron emission tomography. J Pediatr. Feb 1989;114(2):244-53. [Medline].

  69. Juhász C, Haacke EM, Hu J, Xuan Y, Makki M, Behen ME, et al. Multimodality imaging of cortical and white matter abnormalities in Sturge-Weber syndrome. AJNR Am J Neuroradiol. May 2007;28(5):900-6. [Medline].

  70. Riela AR, Stump DA, Roach ES. Regional cerebral blood flow characteristics of the Sturge-Weber syndrome. Pediatr Neurol. Mar-Apr 1985;1(2):85-90. [Medline].

  71. Moore GJ, Slovis TL, Chugani HT. Proton magnetic resonance spectroscopy in children with Sturge-Weber syndrome. J Child Neurol. Jul 1998;13(7):332-5. [Medline].

  72. Brenner RP, Sharbrough FW. Electroencephalographic evaluation in Sturge-Weber syndrome. Neurology. Jul 1976;26(7):629-32. [Medline].

  73. Sassower K, Duchowny M, Jayakar P. EEG evaluation of children with Sturge-Weber Syndrome and Epilepsy. J Epilepsy. 1994;7:285-289.

  74. Jansen FE, van Huffelen AC, Witkamp T. Diazepam-enhanced beta activity in Sturge Weber syndrome: its diagnostic significance in comparison with MRI. Clin Neurophysiol. Jul 2002;113(7):1025-9. [Medline].

  75. Jordan LC, Wityk RJ, Dowling MM, DeJong MR, Comi AM. Transcranial Doppler ultrasound in children with Sturge-Weber syndrome. J Child Neurol. Feb 2008;23(2):137-43. [Medline].

  76. Di Trapani G, Di Rocco C, Abbamondi AL. Light microscopy and ultrastructural studies of Sturge-Weber disease. Childs Brain. 1982;9(1):23-36. [Medline].

  77. Hoffman HJ. Benefits of early surgery in Sturge-Weber syndrome. In: Tuxhorn I, Holthausen H, Boenigk H, eds. Paediatric Epilepsy syndromes and their surgical treatment. London: John Libbey and Company; 1997:364-370.

  78. Simonati A, Colamaria V, Bricolo A. Microgyria associated with Sturge-Weber angiomatosis. Childs Nerv Syst. Aug 1994;10(6):392-5. [Medline].

  79. Kossoff EH, Hatfield LA, Ball KL. Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. J Child Neurol. Aug 2005;20(8):678-82. [Medline].

  80. Kossoff EH, Balasta M, Hatfield LM, Lehmann CU, Comi AM. Self-reported treatment patterns in patients with Sturge-Weber syndrome and migraines. J Child Neurol. 2007. Jun 2007;22(6):720-6. [Medline].

  81. Roach ES, Riela AR, McLean WT, et al. Aspirin therapy for Sturge-Weber Syndrome. Ann Neurol. 1985;18:387.

  82. Morelli JG. Port-wine stains and the Sturge-Weber syndrome. In: Bodensteiner JB, Roach ES, eds. Sturge Weber Syndrome. Mt Freedom, New Jersey: Sturge Weber Foundation; 1999:11-16.

  83. Morelli JG, Enjolras O, Goldberg G et al. Treatment of Port wine stains. SWF Web Page.

  84. Troilius A, Wrangsjo B, Ljunggren B. Potential psychological benefits from early treatment of port-wine stains in children. Br J Dermatol. Jul 1998;139(1):59-65. [Medline].

  85. Bruce DA. Neurosurgical aspects of Sturge-Weber syndrome. In: Bodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. Mt Freedom, NJ: Sturge Weber Foundation; 1999:39-42.

  86. Roach ES, Riela AR, Chugani HT. Sturge-Weber syndrome: recommendations for surgery. J Child Neurol. Apr 1994;9(2):190-2. [Medline].

  87. Rappaport ZH. Corpus callosum section in the treatment of intractable seizures in the Sturge-Weber syndrome. Childs Nerv Syst. Aug 1988;4(4):231-2. [Medline].

  88. Holmes GL. Surgery for intractable seizures in infancy and early childhood. Neurology. Nov 1993;43(11 Suppl 5):S28-37. [Medline].

  89. Alexander GL, Norman RM. Sturge-Weber syndrome. In: Vinken PJ, Bruyn GW, eds. Handbook of Clinical Neurology. 14. 1972:223-240.

  90. Hoffman HJ, Hendrick EB, Dennis M. Hemispherectomy for Sturge-Weber syndrome. Childs Brain. 1979;5(3):233-48. [Medline].

  91. Ogunmekan AO, Hwang PA, Hoffman HJ. Sturge-Weber-Dimitri disease: role of hemispherectomy in prognosis. Can J Neurol Sci. Feb 1989;16(1):78-80. [Medline].

  92. Arzimanoglou A. The surgical treatment of Sturge-Weber Syndrome with respect to its clinical spectrum. In: Tuxhorn I, Holthausen H, Boenigk H, eds. Paediatric Epilepsy Syndromes and Their Surgical Treatment. 1997:353-363.

  93. Arzimanoglou A, Aicardi J. The epilepsy of Sturge-Weber syndrome: clinical features and treatment in 23 patients. Acta Neurol Scand Suppl. 1992;140:18-22. [Medline].

  94. Gilly R, Lapras C, Tommasi M. [Sturge-Weber-Krabbe disease. Notes on 21 cases]. Pediatrie. Jan-Feb 1977;32(1):45-64. [Medline].

  95. Kossoff EH, Buck C, Freeman JM. Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology. Dec 10 2002;59(11):1735-8. [Medline].

 
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A child with Sturge-Weber syndrome with bilateral facial port-wine stain.
Cranial CT scan showing calcifications.
MRI image in Sturge-Weber syndrome.
Single-photon emission computed tomographic scan in Sturge-Weber syndrome.
Single-photon emission computed tomographic scan in Sturge-Weber syndrome.
Table 1. Clinical Manifestations of Sturge-Weber Syndrome
Risk of SWS with facial PWS8%
SWS without facial nevus13%
Bilateral cerebral involvement15%
Seizures72-93%
Hemiparesis25-56%
Hemianopia44%
Headaches44-62%
Developmental delay and mental retardation50-75%
Glaucoma30-71%
Choroidal hemangioma40%
Table 2. Developmental Morbidity Associated with Seizures in Adults with SWS
With Seizures (%) Without Seizures (%)
Developmental delay450
Emotional/behavioral problems8558
Need for special education710
Employability4678
Table 3. Summary of Work-up Findings in Sturge-Weber Syndrome
CSF analysisElevated protein
Skull x-rayTram-track calcifications
AngiographyLack of superficial cortical veins



Nonfilling dural sinuses



Abnormal, tortuous vessels



CT scanCalcifications, tram-track calcifications



Cortical atrophy



Abnormal draining veins



Enlarged choroid plexus



Blood-brain barrier breakdown (during seizures)



Contrast enhancement



MRIGadolinium enhancement of LA



Enlarged choroid plexus



Sinovenous occlusion



Cortical atrophy



Accelerated myelination



SPECTHyperperfusion, early



Hypoperfusion, late



PETHypometabolism
EEGReduced background activity



Polymorphic delta activity



Epileptiform features



Table 4. Seizure Control in Sturge-Weber Syndrome
Study Complete Partial Refractory/No Control
Gilly et al[94] NA*NA37%
Sujanski and Conradi[37]



(adults)



27%49%24%
Sujanski and Conradi[37, 17] (all ages) 50%39%11%
Pascual-Castroviejo et al[34] 47%12%28%
Oakes[26] 10%NA83%
Sassower et al[73] NANA43%
Arzimanoglou and Aicardi[92] NANA39%
Erba and Cavazzuti[24] 50%NANA
Toronto[77, 90] NANA32%
*NA = not available
Table 5. Surgical Results of Hemispherectomy and Limited Resection from 3 Centers
Center Hemispherectomy Seizure Free Limited resection Seizure Free Improved
Toronto12111182
Paris551578
Boston98630
Total2624321810
24 of 26 patients with hemispherectomy - Seizure free
28 of 32 patients with limited resection - Seizure free or improved
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