Neurology India, Vol. 58, No. 2, March-April, 2010, pp. 336-337
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, firstname.lastname@example.org
Date of Acceptance: 08-Oct-2009
Code Number: ni10092
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).  The suggested mechanisms for accelerated myelination include cerebral venous thrombosis with restriction of venous outflow,  cerebral ischemia underlying the angioma  and transient hyperperfusion.  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  . 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, , and thus improving the quality of life by alleviating seizures and preserving the intellect of affected children.
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