Transient Changes on Brain Magnetic Resonance Imaging in a Patient With Sturge-Weber Syndrome Presenting With Hemiparesis

Kumar, Kishore R.; Hon, Kenneth; Schultz, David; Agzarian, Mark; Jones, David; Thyagarajan, Dominic

doi:10.1097/nrl.0b013e3181940244

Cited by 15 publications

(11 citation statements)

References 6 publications

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“…30 One single-patient case report described concurrent transient hemiparesis and abnormal magnetic resonance brain diffusion in the corresponding contralateral hemisphere. 31 These stroke-like episodes are commonly triggered by minor head injury in toddlers with SWS. 32 No data address the prevalence of these episodes, and estimates of their frequency vary widely.…”

Section: Advances and Questions In The Definition Neurological Symptmentioning

confidence: 99%

“…46 In one case, transient abnormal diffusion was identified concurrent with repeated complex partial seizures. 31 Such diffusion changes may indicate focal brain ischemia and/or seizure-induced abnormalities, typically after prolonged or frequent seizures; since seizure control may result in resolution of seizure-induced MRI abnormalities, serial MRI is important to differentiate peri-ictal diffusion from permanent brain ischemia. Positron emission tomography (PET) of brain glucose metabolism can track the extent and severity of cortical dysfunction, which often extends beyond the structural brain abnormalities detected by CT and MRI (Fig.…”

Section: Neuroimaging and Pathogenesismentioning

confidence: 99%

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Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Marchuk

Ball

et al. 2011

Develop Med Child Neuro

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AIM To review recent developments in the understanding, diagnosis, and treatment of Sturge–Weber syndrome (SWS). METHOD Members of the Brain Vascular Malformation Consortium Sturge–Weber National Workgroup contributed their expertise, to review the literature, and present promising directions for research. RESULTS The increasing number of reports dealing with SWS over the last decade reflects progress in the diagnosis and understanding of the neurological involvement. The proliferation of centers and advocacy groups to care for patients with SWS and to stimulate research has aided the development of new insights into the clinical manifestations and the pathophysiology of neurological progression, and the development of novel hypotheses to direct future research. Many key questions remain, but the tools and networks to answer them are being developed. INTERPRETATION This review summarizes important new knowledge and presents new research directions that are likely to provide further insights, earlier diagnosis, improved treatments, and possibly, prevention of this syndrome.

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Section: Advances and Questions In The Definition Neurological Symptmentioning

confidence: 99%

Section: Neuroimaging and Pathogenesismentioning

confidence: 99%

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Marchuk

Ball

et al. 2011

Develop Med Child Neuro

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“…Most patients with brain involvement will begin seizure activity during infancy, but new neurological symptoms have been shown to present in adulthood in certain cases [9, 10, 11]. Adult-onset SWS does not necessarily follow the same pattern as early onset SWS, but has not been studied extensively.…”

Section: Introductionmentioning

confidence: 99%

Sturge-Weber Syndrome

Bachur

Comi

2013

Curr Treat Options Neurol

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“…Acute hemiparesis due to chronic venous sinus occlusion is reported in SWS, albeit without DWI changes suggestive of ischaemia [5]. A recent report described increased cortical DWI signal in a patient with SWS with a subacute history of headache and hemiparesis [7], although the DWI changes only occurred after a series of prolonged seizures. Increases in cortical DWI signal have been reported in patients with SWS and chronic neurological deficit, and may reflect gliotic changes in this context [1].…”

mentioning

confidence: 99%

“…Increases in cortical DWI signal have been reported in patients with SWS and chronic neurological deficit, and may reflect gliotic changes in this context [1]. Aspirin is thought to reduce the risk of stroke-like episodes due to microcirculatory stasis [8], and its use in adults with SWS is described anecdotally [7] although there are no large clinical trials to support its use. Given the imaging changes suggestive of reversible cortical ischaemia in our patient, aspirin appears to be a rational long-term prophylactic agent against further episodes of this type.…”

mentioning

confidence: 99%

Reversible increases in cortical diffusion-weighted MR signal in a patient with Sturge–Weber syndrome and subacute hemiplegia

et al. 2011

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A 30-year-old man with Sturge-Weber syndrome (SWS) diagnosed 10 years previously was admitted with a 3 day history of headache, photophobia, drowsiness and progressive left hemiplegia. There was a history of a similar episode in 2004, complicated by a period of generalised tonic-clonic status epilepticus. He had remained seizurefree for some years on levetiracetam 500 mg bd. Examination revealed a port-wine stain in a right trigeminal V1 distribution. He was drowsy and partially oriented, with left lower facial weakness. There was complete left hemiplegia with brisk left-sided deep tendon reflexes, left extensor plantar response and left-sided sensory extinction.There was no acute haemorrhage or infarct on CT brain. CSF white cell count and protein were normal. A T1-weighted MRI brain showed dilatation of the deep venous system (Fig. 1a) and pial enhancement following gadolinium, with normal opacification of the superficial venous sinuses (Fig. 1b). There was high diffusion-weighted imaging (DWI) signal limited to the right cerebral cortex (Fig. 1c). EEG showed right-sided slowing but no epileptiform activity. He was treated with aspirin 300 mg/day and tinzaparin 17,000 u/day. Levetiracetam was increased to 1,000 mg bd. The left hemiplegia completely resolved over the next 10 days, and no seizures were observed. He complained of intermittent non-threatening formed visual hallucinations which gradually resolved. Repeat MR showed a resolution in the cortical DWI signal (Fig. 1d).Recurrent stroke-like episodes due to ischaemia or seizures occur frequently in children with SWS [3,4,9], but are less well-described in adults. Headaches, mostly with migrainous features, are common and have been associated with stroke-like episodes in one study [6]. Post-ictal Todd's paresis was also considered as a potential cause of the patient's symptoms, but the absence of definite seizures and the progressive nature of the deficit were against this, although levetiracetam was increased as a precaution against further deterioration [2].The deep venous anomalies and pial enhancement we describe are consistent with longstanding vascular changes in SWS [2,9]. The increased DWI signal over the right cerebral cortex likely represents cortical ischaemia due to microcirculatory stasis within the aberrant leptomeningeal vasculature, a mechanism which has been proposed for neurological complications of SWS [3,9]. Acute hemiparesis due to chronic venous sinus occlusion is reported in SWS, albeit without DWI changes suggestive of ischaemia [5]. A recent report described increased cortical DWI signal in a patient with SWS with a subacute history of headache and hemiparesis [7], although the DWI changes only occurred after a series of prolonged seizures. Increases in cortical DWI signal have been reported in patients with SWS and chronic neurological deficit, and may reflect gliotic changes in this context [1]. Aspirin is thought to reduce the risk of stroke-like episodes due to microcirculatory stasis [8], and its use in adults with SWS is descr...

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Transient Changes on Brain Magnetic Resonance Imaging in a Patient With Sturge-Weber Syndrome Presenting With Hemiparesis

Abstract: Although cerebral angiography could potentially demonstrate thrombosis causing large vessel obstruction, it is unlikely to have the resolution to detect stasis at a microvascular level. Microcirculatory stasis with reversible ischemia is the likely pathogenic mechanism in this case.

Cited by 15 publications

References 6 publications

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

Sturge-Weber Syndrome

Reversible increases in cortical diffusion-weighted MR signal in a patient with Sturge–Weber syndrome and subacute hemiplegia

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