The epilepsy of Sturge-Weber syndrome: Clinical features and treatment in 23 patients

Arzimanoglou, Alexis; Aicardi, Jean

doi:10.1111/j.1600-0404.1992.tb04465.x

Cited by 83 publications

(59 citation statements)

References 12 publications

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“…[8][9][10] In the former, it is suggested that there is failure to increase cerebral blood flow to the affected hemisphere in response to the seizures. This compromises an already ischaemic cortex, leading to increased focal deficit and deteriorating cognition.…”

Section: Discussionmentioning

confidence: 99%

Transient exacerbation of hemiplegia following minor head trauma in Sturge–Weber syndrome

Zolkipli

Aylett

Rankin

et al. 2007

Develop Med Child Neuro

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Sturge‐Weber syndrome (SWS) is a sporadic disorder characterized by naevus (port wine stain), a pial angioma, and glaucoma. The angioma comprises abnormal tortuous vessels on the leptomeninges with underlying brain gliosis, calcification, and atrophy. The cerebral angioma is commonly unilateral but may be bilateral. Hemiplegia usually follows recurrent hemiconvulsions and may be related to venous stasis. The hemiplegia can be static, progressive, or fluctuating. Transient worsening of the hemiplegia can be seen with seizures and episodes resembling hemiplegic migraine. We report five patients (four females, one male) with SWS who have had transient worsening of hemiplegia following minor head injuries, occurring between the ages of 10 months and 12 years (median age 4y 6mo). An additional pilot survey suggests that this may affect up to 20% of patients.

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Section: Discussionmentioning

confidence: 99%

Transient exacerbation of hemiplegia following minor head trauma in Sturge–Weber syndrome

Zolkipli

Aylett

Rankin

et al. 2007

Develop Med Child Neuro

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“…Facial port wine stain is a congenital macular lesions which initially may be light pink in colour, thereby involving area of distribution of trigeminal nerve as documented in this case. Neurological manifestations may vary from minimal or neurological signs to uncontrolled epileptic crisis, hemiparesis, hemiatrophy, mental retardation, microcephaly and visual loss 6 . They commonly involve right side of face especially the ophthalmic distribution of trigeminal nerve and usually do not extend beyond midline 7 .…”

Section: Discussionmentioning

confidence: 99%

A case report on Sturge Weber Syndrome Seizures with Stain

Neki¹,

Shergill²,

Singh³

et al. 2017

Int. J. Curr. Res. Med. Sci.

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Sturge Weber Syndrome or encephalotrigeminal angiomatosis is non hereditary, congenital and rare disorder of unknown etiology. It is characterised by vascular malformation with capillary venous angiomas involving face, eye and leptomeninges resulting in neurological and orbital manifestations. A case of 46 years old female presented with history of tonic-clonic convulsions, evidence of Port wine stain on face since birth, characteristic CT findings diagnosed as a case of Sturge Weber Syndrome is reported here for its rarity.

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“…1 In these cases, resective procedures, typically hemispherectomy, are indicated for seizure control. The ideal timing for hemispherectomy is controversial.…”

Section: Discussionmentioning

confidence: 99%

“…Seizures are seen in more than 80% of cases, cognitive impairment in 50% and frequently glaucoma. [1][2] Seizures are the result of leptomeningeal angioma and underlying calcifications. These abnormalities are usually considered to be limited to one hemisphere.…”

mentioning

confidence: 99%

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Andrade

McAndrews

Hamani

et al. 2010

Can. j. neurol. sci.

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Sturge-Weber syndrome (SWS) is a rare congenital disease associated with venous angiomatosis of the leptomeninges and ipsilateral, cutaneous facial port-wine stain (facial angiomatosis). Seizures are seen in more than 80% of cases, cognitive impairment in 50% and frequently glaucoma. 1-2 Seizures are the result of leptomeningeal angioma and underlying calcifications. These abnormalities are usually considered to be limited to one hemisphere. Frequently, seizures in SWS are refractory to medical treatment. In those cases surgical resection of the affected areas is recommended. Some form of hemispherectomy is usually performed for SWS-associated intractable epilepsy and most patients become seizure-free after surgery. 3-5 Another possible benefit of hemispherectomy is a better cognitive profile in patients that became seizure-free after such a procedure. However, very long follow-ups addressing seizure control and cognitive deficits in hemispherectomized patients are rare and they usually show good outcomes. Here we report on a SWS patient who become seizure-free after hemispherectomy performed in the first year of life, however, seizures recurred 29 years later without any identifiable cause. She also had cognitive decline before the onset of seizures. To the best of our knowledge this is the longest seizure-free interval between hemispherectomy and seizure recurrence in SWS.

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The epilepsy of Sturge-Weber syndrome: Clinical features and treatment in 23 patients

Cited by 83 publications

References 12 publications

Transient exacerbation of hemiplegia following minor head trauma in Sturge–Weber syndrome

Transient exacerbation of hemiplegia following minor head trauma in Sturge–Weber syndrome

A case report on Sturge Weber Syndrome Seizures with Stain

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

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