Associated Facial Hemangioma and Intracranial Lesion (Weber-Dimitri Disease)

Cohen, Harry J.

doi:10.1001/archpedi.1941.02000150130016

Cited by 5 publications

(2 citation statements)

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“…Variants of the classical findings have been described in the literature such as absence of facial port-wine stain, 4 -5 extensive intracranial calcifications contralateral to the bulk of the facial naevus, 6 severe bilateral skin involvement with only unilateral cerebral involvement, 7 facial port-wine stains and glaucoma without meningeal anomalies, 8 ocular anomalies involving the scleral vessels, choroid, or retina 9 and associated hemiatrophy or hypertrophy of limbs. If seizures begin in the first months of life, there appears to be a high risk of progressive mental deterioration.…”

mentioning

confidence: 94%

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Ogunmekan

Hwang

Hoffman

1989

Can. j. neurol. sci.

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We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere. RESUME: Le role de I'hemispherectomie dans le prognostic de la maladie de Sturge-Weber-Dimitri Nous rapportons les cas de 12 patients atteints de la maladie de Sturge-Weber-Dimitri qui ont ete traites chirurgicalement entre Janvier 1975 et decembre 1987. Une hemispherectomie a ete effectuee chez 10 patients et 2 autres ont subi une lobectomie occipitale pour epilepsie refractaire au traitement. Toutes les interventions ont ete realisees entre Page de 3 et 20 mois, sauf chez deux patients ages de 8 et 9 ans respectivement. Le debut des crises convulsives se situait entre 2 et 8 mois chez tous les patients sauf chez deux qui avaient commence a 15 mois. II n'y a eu aucun deces en post-operatoire. On a du avoir recours a un shunt post-operatoire chez 3 des 12 patients (25%). On a obtenu un controle des crises pendant un an ou plus apres 1'intervention chez 11 des 12 patients. L'autre patient n'a pas eu de crise sous medication apres un suivi de moins de 12 mois. Nous n'avons pas observe de deterioration intellectuelle apres la chirurgie sauf chez 2 patients operes tardivement. Nous concluons que, chez les patients atteints du syndrome de Sturge-WeberDimitri qui presentent des crises convulsives refractaires au traitement dans les premiers six mois apres la naissance et dont Patteint hemispherique est unilateral, une resection precoce de Phemisphere atteint doit etre consideree.

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mentioning

confidence: 94%

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Ogunmekan

Hwang

Hoffman

1989

Can. j. neurol. sci.

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“…As a result, in the early literature reporting SWS, there were several publications regarding EEG findings. Most of the earliest publications were single case reports as early as 1941 (Cohen and Kay, 1941), typically describing asymmetry (Table 1). The largest by Peterman in 1958 reported 35 children with SWS, of which 26 had EEGs (Peterman et al, 1958).…”

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confidence: 99%

EEG evolution in Sturge-Weber syndrome

et al. 2014

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SUMMARY The EEG in Sturge-Weber syndrome (SWS) was theorized over 50 years ago as changing over time from normality to focal asymmetry to lastly epileptiform. We sought to validate these findings in a larger cohort today. Children with confirmed SWS and routine EEG at our center were evaluated retrospectively. An EEG score (0–3) was created and linked to patient current age, overall neurologic function, and seizure frequency. Eighty-one EEGs from 44 patients with SWS (mean age 2.0 years (range: 0.2 – 37.9 years) were evaluated and assigned an EEG score. The mean age for patients with an EEG score of 0–1 (normal or focal slowing) was 3.2 years (SEM 0.6), whereas those with an EEG score of 2–3 (focal sharp waves or frequent spike-wave bursts) was 8.7 years (SEM 1.7) (p=0.006). There was no correlation between the EEG score and either the SWS overall neuroscore or seizure subscore (measuring frequency). The EEG in patients with SWS does appear to evolve over time, becoming more abnormal with more frequent epileptiform activity, as suspected in smaller studies decades ago. This progressive change, however, did not correlate with the child’s neurologic function or seizure frequency.

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Sturge-Weber Syndrome

Bluefarb¹

1949

Arch Dermatol

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Associated Facial Hemangioma and Intracranial Lesion (Weber-Dimitri Disease)

Cited by 5 publications

References 4 publications

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

EEG evolution in Sturge-Weber syndrome

Sturge-Weber Syndrome

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