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LETTER TO EDITOR
Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 797-799

Stroke-like episodes in Sturge-Weber syndrome


1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India
2 Department of Neuroimaging and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Acceptance22-Jul-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
Syam Krishnan
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.72185

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How to cite this article:
Baheti NN, Krishnan S, Thomas B, Kesavadas C, Radhakrishnan A. Stroke-like episodes in Sturge-Weber syndrome. Neurol India 2010;58:797-9

How to cite this URL:
Baheti NN, Krishnan S, Thomas B, Kesavadas C, Radhakrishnan A. Stroke-like episodes in Sturge-Weber syndrome. Neurol India [serial online] 2010 [cited 2020 Apr 4];58:797-9. Available from: http://www.neurologyindia.com/text.asp?2010/58/5/797/72185


Sir,

Sturge- Weber syndrome More Details (SWS) is a rare sporadic neurocutaneous syndrome, characterized by leptomeningeal and cutaneous angiomas of the face, typically in the ophthalmic and maxillary distributions of the trigeminal nerve. [1] Transient stroke-like episodes are a rare presentation in SWS. The occurrence of such episodes, if unaware of, poses a diagnostic challenge to the clinician.

A 6-year-old girl was noted to have a port wine nevus in V1 and V2 division of bilateral trigeminal nerves at birth. Psychomotor development was normal. At the age of three years, the child had brief seizures, characterized by deviation of head and eyes to the left with clonic jerking of left upper and lower limbs. She was initiated on carbamazepine with no further seizures. She presented to us with sudden-onset holocranial persistent headache of four days' duration and acute onset left sided weakness of six hours duration prior to presentation. Examination showed left hemiparesis. Computerized tomography scan head (done at the age of 3 years) showed calcification in right subcortical parietal white matter. Brain magnetic resonance (MR) imaging done during this admission showed mild gyral edema over the right temporo-occipital region and bilateral (right>left) posterior cerebral hemispheres pial angiomatosis[Figure 1]. Contrast-enhanced MR angiogram and digital subtraction angiogram showed no evidence of arterial or venous thrombosis. Electroencephalogram (EEG) showed excess of slow wave-activity (1-1.5 Hz) over the right hemisphere without any epileptiform activity [Figure 2]. The child was given IV lorazepam without any improvement in limb power or change in EEG. She was initiated on aspirin (3 mg/kg); and over the next one week, her neurologic deficit improved completely and she was discharged home. A follow-up susceptibility-weighted imaging at the time of discharge showed reappearance of susceptibility artifacts over the region of angiomatosis [Figure 2].
Figure 1 :MR brain axial images. T2-weighted (a) and FLAIR (b) images showing increased signals in gyriform pattern over the right temporo-occipital region suggesting mild gyral edema. Post-gadolinium contrast FLAIR image (c) showing diffuse enhancement of the subarachnoid space over both posterior cerebral hemispheres (right>left), consistent with pial angiomatosis. Diffusion-weighted image (d) and apparent diffusion coefficient image (e) show no diffusion restriction. Susceptibility-weighted image (f) showing decreased venous prominence over the right temporo-occipital region

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Figure 2 :Susceptibility-weighted imaging (SWI) 1 week later (after complete recovery). Axial SWI shows reappearance of the veins in the right temporo-occipital region

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Transient focal deficits (stroke-like episodes) are a unique feature in SWS. These symptoms can be caused by temporary ischemia of the cortex underlying the vascular malformation or postictal depression of the brain activity. [2] Adequate differentiation between epileptic and ischemic origins of these events is of utmost importance, as this will have consequences on management. Advanced MR imaging techniques can potentially help to differentiate between the two. However, these have never been tried in this setting. Classically these episodes have been attributed to venous stasis within the leptomeningeal malformation causing repeated thrombosis, resulting in recurrent infarction. [2] Angiographic studies, however, have documented patent venous channels, suggesting stasis at a microvascular level. [3] Abnormalities of the microcirculation in angioma cause stasis and anoxia of the underlying brain, leading to disruption of the blood-brain barrier, increased capillary permeability, focal alterations in pH, calcium deposition and neuronal dysfunction. [3] This corresponds to the gray matter edema, as seen on MR imaging in our patient

About 75% to 90% of children with SWS develop seizures by three years of age. [4] Ictal epileptiform activity or postictal depression of activity can be another cause of transient neurological dysfunction. Rarely focal inhibitory motor status manifests as hemiplegia without any change in sensorium, and EEG shows focal slowing without epileptiform abnormalities. [5] Clinical and electrographic improvement after intravenous lorazepam can resolve this issue. Perfusion imaging in our patient [Figure 3] showed decreased relative cerebral blood flow and volume with prolonged mean transit time, thus ruling out inhibitory motor seizures. [6]
Figure 3 :Perfusion-weighted imaging showing decreased cerebral blood flow in the relative cerebral blood flow map (a); decreased blood volume in the relative cerebral blood volume map (b) and increased transit time in the mean transit time map (c) over the right temporo-occipital region

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Despite weakness persisting and progressing over days, repeat diffusion MR imaging showed no restriction, suggesting a reversible pathology. Our patient consistently showed decreased artifacts, suggesting deficient oxygen utilization with increased ratio of oxyhemoglobin to deoxyhemoglobin. [7] The above changes reverted once deficit improved, suggesting some reversible cause. Although the exact reason is unknown, it may represent preferential dilatation of abnormal venous channels with increase blood flow.

The value of aspirin in the treatment of such transient weakness in SWS has been suggested previously. In a study of 14 patients, a 65% decrease of stroke-like events was found after administration of low-dose aspirin (2-3 mg/kg/d). [8] Our patient also stabilized after being initiated on aspirin, and weakness improved completely over a week. Therefore, we suggest that low-dose aspirin should be considered for individuals with manifestations of a paroxysmal hemiparesis without ictal epileptiform activity on EEG.

 
 » References Top

1.Roach ES. Diagnosis and management of neurocutaneous syndromes. Semin Neurol 1988;8:83-96.  Back to cited text no. 1
    
2.Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatr Neurol 2004;30:303-10.  Back to cited text no. 2
    
3.Garcia JC, Roach ES, McLean WT. Recurrent thrombotic deterioration in the Sturge-Weber syndrome. Childs Brain 1981;8:427-33.  Back to cited text no. 3
    
4.Maria BL, Hoang K, Robertson RL, Barnes PD, Drane WE, Chugani HT. Imaging brain structure and functions in Sturge-Weber syndrome. In: Bodensteiner JB, Roach ES, editors. Sturge-Weber syndrome. Mount Freedom, NJ: The Sturge-Weber Foundation; 1999. p. 43-69.  Back to cited text no. 4
    
5.Smith RF, Devinsky O, Luciano D. Inhibitory motor status: Two new cases and a review of inhibitory motor seizures. J Epilepsy 1997;10:15-21.  Back to cited text no. 5
    
6.Oguz KK, Senturk S, Ozturk A, Anlar B, Topcu M, Cila A. Impact of Recent Seizures on Cerebral Blood Flow in Patients With Sturge-Weber Syndrome: Study of 2 Cases. J Child Neurol 2007;22:617-20  Back to cited text no. 6
    
7.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. 2007;28:900-6.  Back to cited text no. 7
    
8.Maria BL, Neufeld JA, Rosainz LC, Drane WE, Quisling RG, Ben- David K, et al. Central nervous system structure and function in Sturge-Weber syndrome: Evidence of neurologic and radiologic progression. J Child Neurol 1998;3:606-18.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]

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