Recurrent Thrombotic Deterioration in the Sturge-Weber Syndrome

García, Juan Carlos; Roach, E. Steve; McLean, William T.

doi:10.1159/000120011

Cited by 38 publications

(31 citation statements)

References 5 publications

(9 reference statements)

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“…Anecdotal reports document neurologic progression, including new neurologic deficits acquired after status epilepticus or strokelike episodes. [20][21][22] In addition, we have direct clinical experience with children examined just before and after a bout of seizures who clearly acquired new hemiparesis or visual field cut, or both, that did not fully resolve with time. In our clinical experience with roughly 75 patients with Sturge-Weber syndrome, a history of episodic neurologic deterioration is also commonly provided by the parents, particularly in infants with onset of seizures in the first year of life (unpublished findings).…”

Section: Discussionmentioning

confidence: 99%

Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

et al. 2007

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Transcranial Doppler ultrasound is a noninvasive vascular assessment technique proved useful in the management of pediatric disorders predisposed to stroke and may have similar utility for Sturge-Weber syndrome. Eight children with Sturge-Weber syndrome had velocities measured in the major cerebral arteries via the Stroke Prevention Trial in Sickle Cell Anemia methodology. Velocities and pulsatility indexes were compared between the unaffected and affected sides. All subjects had reduced velocity on the affected side; the mean middle cerebral artery percentage difference was 20% (95% CI, 15%-25%). Pulsatility index was increased on the affected side; mean middle cerebral artery pulsatility index percentage difference, 34% (95% CI, 15%-53%). Six subjects also had reduced posterior cerebral artery velocity on the affected side. Side-to-side differences in middle cerebral artery and posterior cerebral artery velocities correlated with severity of MRI asymmetry (Spearman rho = 0.88, P = .02). Decreased arterial flow velocity and increased pulsatility index in the middle cerebral artery and posterior cerebral artery suggests a high resistance pattern that may reflect venous stasis and may contribute to chronic hypoperfusion of brain tissue. Further study of Transcranial Doppler in children with Sturge-Weber syndrome is indicated.

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Section: Discussionmentioning

confidence: 99%

Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

et al. 2007

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“…Tissue hypoxia and intractable seizures have been implicated as the cause of clinical decline in SWS patients, but more recently it has been suggested that neurological deterioration results from progressive venous hypertension [3,4,6,7]. Aplasia or thrombosis of superficial cortical veins during development is thought to lead to persistence of an embryonic cortical vascular plexus that is recognised in later life as the "leptomeningeal angioma" [2].…”

Section: Discussionmentioning

confidence: 99%

Status epilepticus and venous infarction in Sturge-Weber syndrome

Coley

Britton

Clarke

1998

Child's Nervous System

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Sturge-Weber syndrome (SWS) is a neurocutaneous disorder that is typically associated with progressive neurological deterioration. We describe a 12-year-old girl with SWS who suffered a permanent cerebral insult as the result of a period of protracted status epilepticus. The case illustrates the unique susceptibility of patients with SWS to uncontrolled venous hypertension and emphasises the need for optimal seizure control and preservation of venous outflow. We discuss the relevance of our observations to haemodynamic concepts of neurological decline in SWS.

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“…In addition to anticonvulsant therapy, antithrombotic agents, such as aspirin, have been recommended to decrease thrombotic episodes and slow the progressive deterioration. 24 The nevus flammeus can be treated with cover-blend makeup or laser therapy.…”

Section: Discussionmentioning

confidence: 99%

Sturge-Weber Syndrome Presenting as an Acute Life-Threatening Event

Muñiz

2004

Pediatric Emergency Care

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Young infants may present to an emergency department after an acute life-threatening event. The differential diagnosis for an acute life-threatening event is quite extensive. Fortunately, most will have benign or treatable causes. A case of Sturge-Weber syndrome is described in a 2-day old who presented to the emergency department after an acute life-threatening event. A thorough history and detailed physical examination are generally helpful in discerning the etiology for most acute life-threatening events and may at times reveal unusual diseases such as Sturge-Weber syndrome.

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Recurrent Thrombotic Deterioration in the Sturge-Weber Syndrome

Cited by 38 publications

References 5 publications

Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

Status epilepticus and venous infarction in Sturge-Weber syndrome

Sturge-Weber Syndrome Presenting as an Acute Life-Threatening Event

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