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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 1998-4022
Vol. 58, Num. 2, 2010, pp. 336-337

Neurology India, Vol. 58, No. 2, March-April, 2010, pp. 336-337

Neuroimage

MR demonstration of accelerated myelination in early sturge Weber syndrome

Uttam George, Shubhra Rathore, Pramod Nittala

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

Correspondence Address: Uttam George, Department of Radiodiagnosis, Christian Medical College & Hospital, Brown Road, Ludhiana- 141 008, Punjab, India, ubgeorge@gmail.com

Date of Acceptance: 08-Oct-2009

Code Number: ni10092

PMID: 20508372

DOI: 10.4103/0028-3886.63769

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 (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

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]

Copyright 2010 - Neurology India

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[ni10092f2.jpg] [ni10092f1.jpg]
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