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NEUROIMAGE
Year : 2010  |  Volume : 58  |  Issue : 2  |  Page : 336-337

MR demonstration of accelerated myelination in early sturge Weber syndrome


Department of Radiodiagnosis, Christian Medical College and Hospital, Ludhiana, Punjab, India

Date of Acceptance08-Oct-2009
Date of Web Publication26-May-2010

Correspondence Address:
Uttam George
Department of Radiodiagnosis, Christian Medical College & Hospital, Brown Road, Ludhiana- 141 008, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.63769

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How to cite this article:
George U, Rathore S, Nittala P. MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India 2010;58:336-7

How to cite this URL:
George U, Rathore S, Nittala P. MR demonstration of accelerated myelination in early sturge Weber syndrome. Neurol India [serial online] 2010 [cited 2020 Jul 11];58:336-7. Available from: http://www.neurologyindia.com/text.asp?2010/58/2/336/63769


A five-month-old female infant presented with three episodes of right focal motor seizures. Clinical examination showed a capillary hemangioma over the left forehead and pre-auricular region. Seizures were controlled with intravenous antiepileptic medication following which she was started on oral maintenance antiepileptic drug (AED) therapy. Magnetic resonance imaging (MRI) of the brain with contrast study was performed in view of the port wine stain and seizures. The study showed cerebral atrophy involving the left fronto-temporal region. Abnormal white matter hypointensity on T2 weighted and T2-FLAIR was noted in the affected lobe suggestive of accelerated myelination [[Figure 1]a and b]. The left frontal diploic space appeared enlarged. Post-contrast study showed intense leptomeningeal enhancement in the left fronto-temporal region with prominent enhancing cortical/medullary veins [Figure 2]. The left choroid plexus also appeared enlarged.

Accelerated myelination in the affected hemisphere is a diagnostic feature in Sturge  Weber syndrome More Details (SWS). [1] The suggested mechanisms for accelerated myelination include cerebral venous thrombosis with restriction of venous outflow, [2] cerebral ischemia underlying the angioma [3] and transient hyperperfusion. [4] This MR pattern has been shown to be helpful in early diagnosis of SWS. The usual clinical course in SWS includes focal tonic - clonic seizures with slowly progressive hemiparesis. Recurrent seizures and long-term use of multiple AEDs lead to gradual cognitive decline and progressive cerebral atrophy. Increasing evidence suggests that early surgical intervention may alleviate seizures and prevent deterioration in mental intellect [5] . Detection of this early finding of accelerated myelination may help to initiate appropriate AED treatment or definitive surgical treatment, including hemispherectomy, localized cortical resection and corpus callosotomy, [5],[6] and thus improving the quality of life by alleviating seizures and preserving the intellect of affected children.

 
  References Top

1.Boukobza M, Enjolras O, Cambra M, Merland J. Sturge-Weber syndrome: The current neuroradiologic data. J Radiol 2000;81:765-71.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Jacoby CG, Yuh WT, Afifi AK, Bell WE, Schelper RL, Sato Y. Accelerated myelination in early Sturge-Weber syndrome demonstrated by MR imaging. J Comput Assist Tomogr 1987;11:226-31.  Back to cited text no. 2  [PUBMED]    
3.Adamsbaum C, Pinton F, Rolland Y, Chiron C, Dulac O, Kalifa G. Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations. Pediatr Radiol 1996;26:759-62.  Back to cited text no. 3  [PUBMED]    
4.Porto L, Kieslich M, Yan B, Zanella FE, Lanfermann H. Accelerated Myelination Associated with Venous Congestion. Eur Radiol 2006;16:922-6.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Arzimanoglou AA, Andermann F, Aicardi J, Sainte-Rose C, Beaulieu M-A, Villemure JG, et al. Sturge-Weber syndrome. Indications and results of surgery in 20 patients. Neurology 2000;55:1472-9.  Back to cited text no. 5      
6.Kossoff EH, Buck C, Freeman JM. Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology 2002;59:1735-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2]

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[Pubmed] | [DOI]



 

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