The Natural History of Patients with the Sturge-Weber Syndrome

Oakes, W. Jerry

doi:10.1159/000120677

Cited by 64 publications

(21 citation statements)

References 8 publications

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“…6 Seizures occur in roughly 75% of SWS patients with unilateral brain involvement and in approximately 95% of patients with bilateral brain involvement. 7,8 Rarely, instead of presenting with acute seizures, infants with SWS brain involvement present with early handedness, evidence of hemiparesis, or a visual gaze preference.…”

Section: Neurologic Signs and Symptoms In Sturge-weber Syndromementioning

confidence: 99%

Presentation, Diagnosis, Pathophysiology, and Treatment of the Neurological Features of Sturge-Weber Syndrome

2011

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Background Sturge-Weber syndrome (SWS) is a neurovascular disorder with a capillary malformation of the face (port-wine birthmark), a capillary-venous malformation in the eye, and a capillary-venous malformation in the brain (leptomeningeal angioma). Although SWS is a congenital disorder usually presenting in infancy, occasionally neurologic symptoms first present in adulthood and most affected individuals do survive into adulthood with varying degrees of neurologic impairment including epilepsy, hemiparesis, visual field deficits and cognitive impairments ranging from mild learning disabilities to severe deficits. SWS is a multi-system disorder that requires the neurologist to be aware of the possible endocrine, psychiatric, ophthalmologic, and other medical issues that can arise and impact the neurologic status of these patients. Some of these clinical features have only recently been described. Review Summary This review summarizes the neurologic manifestations of SWS, discusses issues related to the diagnosis of brain involvement, relates major neuroimaging findings, briefly describes the current understanding of pathogenesis, and provides an overview of neurologic treatment strategies. Conclusion Recent clinical research has highlighted several novel and lesser known aspects of this clinical syndrome including endocrine disorders. Functional imaging studies and clinical experience suggests that neurologic progression results primarily from impaired blood flow and that prolonged seizures may contribute to this process. Treatment is largely symptomatic although aggressive efforts to prevent seizures and strokes, in young children especially, may impact outcome.

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Section: Neurologic Signs and Symptoms In Sturge-weber Syndromementioning

confidence: 99%

Presentation, Diagnosis, Pathophysiology, and Treatment of the Neurological Features of Sturge-Weber Syndrome

2011

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“…Many of these neurologic deficits fully or partially resolve but in young children are more likely to result in accrual of permanent disability. Seizures occur in roughly 75% of patients with unilateral brain involvement, and 95% of patients with bilateral brain involvement [13, 14]. It is essential to educate parents on recognizing seizures, as SWS seizures often do not present in the manner that parents expect.…”

Section: Introductionmentioning

confidence: 99%

Sturge-Weber Syndrome

Bachur

Comi

2013

Curr Treat Options Neurol

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“…Seizure activity, especially if within the first year of life, is associated with worse prognosis. 12 Occasionally, a subarachnoid hemorrhage may occur. Skull radiographs may show calcifications of doubled contoured lines (''railroad track pattern'') that follow the brain convolutions in the damaged areas.…”

Section: Discussionmentioning

confidence: 99%

“…8,18,19 The symptom causing the initial presentation to the hospital is usually seizure which occurs in 55% to 97% of patients. 12,13 The facial nevus flammeus was a primary complaint in 10% and glaucoma in 7% of patients with SturgeWeber syndrome. The majority of seizures occurs within the first year of life and begins as focal motor and progress to become generalized.…”

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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The Natural History of Patients with the Sturge-Weber Syndrome

Cited by 64 publications

References 8 publications

Presentation, Diagnosis, Pathophysiology, and Treatment of the Neurological Features of Sturge-Weber Syndrome

Presentation, Diagnosis, Pathophysiology, and Treatment of the Neurological Features of Sturge-Weber Syndrome

Sturge-Weber Syndrome

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

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