Brain damage and IQ in unilateral Sturge–Weber syndrome: Support for a “fresh start” hypothesis

Behen, Michael E.; Juhász, Csaba; Wolfe‐Christensen, Cortney; Guy, William; Halverson, Stacey; Robert, Richard; Janisse, James; Chugani, Harry T.

doi:10.1016/j.yebeh.2011.07.010

Cited by 27 publications

(39 citation statements)

References 19 publications

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“…14 As the leptomeningeal involvement and underlying brain damage is limited to one hemisphere in 85% of the cases, SWS is an excellent clinical model for studying reorganization of the brain, including the CST, after an early (often ongoing) postnatal unilateral brain injury. 15,16 In this study, we hypothesized differential changes in the CST segments associated with hand vs. leg motor control, and also looked for patterns of structural reorganization and their relation to clinical symptoms.…”

Section: Introductionmentioning

confidence: 99%

Patterns of Structural Reorganization of the Corticospinal Tract in Children With Sturge-Weber Syndrome

Kamson

Juhász

Shin

et al. 2014

Pediatric Neurology

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Background Reorganization of the corticospinal tract (CST) after early damage can limit motor deficit. In this study, we explored patterns of structural CST reorganization in children with Sturge-Weber syndrome. Methods Five children (age 1.5-7 years) with motor deficit due to unilateral Sturge-Weber syndrome were studied prospectively and longitudinally (1-2 years follow-up). CST segments belonging to hand and leg movements were separated, and their volume was measured by diffusion tensor imaging (DTI) tractography using a recently validated method. CST segmental volumes were normalized and compared between the SWS children and age-matched healthy controls. Volume changes during follow-up were also compared to clinical motor symptoms. Results In the SWS children, hand-related (but not leg-related) CST volumes were consistently decreased in the affected cerebral hemisphere at baseline. At follow-up, two distinct patterns of hand CST volume changes emerged: (i) Two children with extensive frontal lobe damage showed a CST volume decrease in the lesional hemisphere and a concomitant increase in the non-lesional (contralateral) hemisphere. These children developed good hand grasp but no fine motor skills. (ii) The three other children, with relative sparing of the frontal lobe, showed an interval increase of the normalized hand CST volume in the affected hemisphere; these children showed no gross motor deficit at follow-up. Conclusions DTI tractography can detect differential abnormalities in the hand CST segment both ipsi- and contralateral to the lesion. Interval increase in the CST hand segment suggests structural reorganization, whose pattern may determine clinical motor outcome and could guide strategies for early motor intervention.

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Section: Introductionmentioning

confidence: 99%

Patterns of Structural Reorganization of the Corticospinal Tract in Children With Sturge-Weber Syndrome

Kamson

Juhász

Shin

et al. 2014

Pediatric Neurology

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“…Bilateral brain involvement and early‐onset, frequent seizures are major predictors of poor clinical outcome 6, 10, 11. However, studies in unilateral SWS (representing the majority of SWS cases) suggested effective compensatory mechanisms leading to relatively preserved neurocognitive functioning in a subgroup of patients with extensive damage, presumably due to early functional reorganization in the contralateral (unaffected) hemisphere 4, 5, 6. Contralateral changes have been also documented by FDG‐PET in the form of increased cortical metabolism, mostly affecting posterior regions including the visual cortex 12, 13, 14.…”

Section: Discussionmentioning

confidence: 99%

“…The clinical course and outcome of SWS is highly variable, ranging from no or minimal neurological signs to severe impairment with uncontrolled seizures, hemiparesis, visual field cut, and learning disability 3. Cross‐sectional and longitudinal neuroimaging studies showed that some children with SWS show good neurocognitive outcome despite extensive unilateral brain involvement, presumably due to early, effective functional reorganization in the contralateral (unaffected) hemisphere 4, 5, 6. In a recent study, using susceptibility‐weighted imaging (SWI), an MRI sequence uniquely sensitive to small veins in the brain,7 we have also demonstrated a modest postnatal expansion of deep medullary veins in the early clinical course of two children with SWS, and its possible protective effect against progressive cortical damage 8…”

Section: Introductionmentioning

confidence: 99%

Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome

John

Maqbool²,

Jeong

et al. 2018

Ann Clin Transl Neurol

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We present longitudinal imaging data of a child with Sturge–Weber syndrome (SWS). At age 8 months, 3 weeks after initial seizures and prolonged motor deficit, MRI showed extensive right hemispheric SWS involvement with severe glucose hypometabolism on PET. She was treated with levetiracetam and aspirin. Follow‐up imaging at age 29 months showed a robust interval expansion of enlarged deep medullary veins throughout the affected hemisphere along with a dramatic recovery of hemispheric metabolism and normalized neurocognitive functioning. These findings demonstrate a robust, multilobar hemispheric remodeling of deep venous collaterals that likely contributed to reversal of initial metabolic and neurocognitive deficits.

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“…Clinical seizure frequency was evaluated by parent interviews and medical charts, and a seizure frequency score was assigned to each patient based on clinical seizures occurring during the one year period prior to the imaging study (or since seizure onset, if seizures started less than one year before the study). The scoring system was slightly modified from a previous study on children with SWS (Behen et al, 2011), and the scores were determined as follows: 0=no seizure in the last 1 year; 1: 1–11 seizures per year; 2: 1–4 seizures per month; 3: >4 seizures per month. Similar scores were also determined at follow-up, i.e., 1 year after baseline or at the time of epilepsy surgery, if surgery was done within one year after the baseline studies (Table 1).…”

Section: Methodsmentioning

confidence: 99%

Imaging increased glutamate in children with Sturge–Weber syndrome: Association with epilepsy severity

Juhász

Xuan

et al. 2016

Epilepsy Research

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Background Sturge-Weber syndrome (SWS) is strongly associated with epilepsy. Brain tissue studies have suggested that epileptic activity in SWS is driven by glutamatergic synaptic activity. Here, we used proton magnetic resonance spectroscopic imaging (MRSI) to test if glutamate (GLU) concentrations are increased in the affected hemisphere and if such increases are associated with severity of epilepsy in children with SWS. We also studied the metabolic correlates of MRSI abnormalities, using glucose positron emission tomography (PET) imaging. Methods 3T MRI and glucose PET were performed in 10 children (age: 7–78 months) with unilateral SWS and a history of epilepsy. MRSI data were acquired from the affected (ipsilateral) and non-affected (contralateral) hemispheres. GLU, N-acetyl-aspartate (NAA) and creatine (Cr) were quantified in multiple voxels; GLU/Cr and NAA/Cr ratios were calculated and compared to seizure frequency as well as glucose PET findings. Results The highest GLU/Cr ratios were found in the affected hemisphere in all children except one with severe atrophy. The maximum ipsilateral/contralateral GLU/Cr ratios ranged between 1.0–2.5 (mean: 1.6). Mean ipsilateral/contralateral GLU/Cr ratios were highest in the youngest children and showed a strong positive correlation with clinical seizure frequency scores assessed at the time of the scan (r=0.88, p=0.001) and also at follow-up (up to 1 year, r=0.80, p=0.009). GLU increases in the affected hemisphere coincided with areas showing current or previous increases of glucose metabolism on PET in 5 children. NAA/Cr ratios showed no association with clinical seizure frequency. Conclusions Increased glutamate concentrations in the affected hemisphere, measured by MRSI, are common in young children with unilateral SWS and are associated with frequent seizures. The findings lend support to the role of excess glutamate in SWS-associated epilepsy.

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Brain damage and IQ in unilateral Sturge–Weber syndrome: Support for a “fresh start” hypothesis

Cited by 27 publications

References 19 publications

Patterns of Structural Reorganization of the Corticospinal Tract in Children With Sturge-Weber Syndrome

Patterns of Structural Reorganization of the Corticospinal Tract in Children With Sturge-Weber Syndrome

Deep cerebral vein expansion with metabolic and neurocognitive recovery in Sturge–Weber syndrome

Imaging increased glutamate in children with Sturge–Weber syndrome: Association with epilepsy severity

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