Extent of Leptomeningeal Capillary Malformation is Associated With Severity of Epilepsy in Sturge-Weber Syndrome.

@article{Sugano2020ExtentOL,
  title={Extent of Leptomeningeal Capillary Malformation is Associated With Severity of Epilepsy in Sturge-Weber Syndrome.},
  author={Hidenori Sugano and Yasushi Iimura and Ayuko Igarashi and Mika Nakazawa and Hiroharu Suzuki and Takumi Mitsuhashi and Madoka Nakajima and Takuma Higo and Tetsuya Ueda and Hajime Nakanishi and Shin-ichi Niijima and Kostadin Karagiozov and H. Arai},
  journal={Pediatric neurology},
  year={2020},
  volume={117},
  pages={
          64-71
        }
}
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Sturge-Weber Syndrome

An early age at seizure onset and those with drug-resistant seizures had more severe degree of mental subnormality, and uncontrolled seizures, mental sub normality, visual handicap, and cosmetic disfiguration were the major impediments in life.

Sturge–Weber syndrome

Though better results in terms of seizures control and psychomotor development were reported in a limited series of children operated on early in life, the insufficient number of subjects who underwent the surgical treatment does not allow definite conclusions yet.

Outcomes of 32 hemispherectomies for Sturge–Weber syndrome worldwide

Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated, and Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom.

The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings

MRI is the imaging modality of choice in the diagnosis of Sturge-Weber syndrome patients because it is more efficient in the detection of the radiological findings related to the clinical status: seizure control, degree of psychomotor development and hemiparesis.

Sturge–Weber syndrome

The authors’ results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete, in patients with Sturge–Weber syndrome and drug-resistant epilepsy.

Sturge-Weber Syndrome. Study of 55 Patients

Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome seen over a 40-year period and were indicative of a poor prognosis.

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Medically intractable epilepsy in children can be treated effectively by surgery and the degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control.

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