Sturge-Weber Syndrome without Port-Wine Facial Nevus

Ambrosetto, Paolo; Ambrosetto, G.; Michelucci, Roberto; Bacci, A.

doi:10.1159/000120140

Cited by 23 publications

(23 citation statements)

References 12 publications

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“…The clinical picture is fairly homogenous. Early developmental milestones are usually normal, with seizures appearing in childhood (Ambrosetto et al, 1983;Giroud et al, 1990). Initially, there is a good response to AED treatment, during which mental development remains normal, but thereafter the epilepsy worsens, together with progressive mental retardation in some patients.…”

Section: Epilepsy With Bilateral Occipital Calcificationsmentioning

confidence: 99%

Parietal and Occipital Lobe Epilepsy: A Review

1993

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There has been considerable recent interest in frontal lobe epileptic syndromes, and less attention paid to occipital and parietal epilepsies. The occipital and parietal lobes have arbitrary anatomical borders. The prinicpal seizure symptomatology includes somatosensory (paresthetic, painful, thermal, sexual, apraxia, disturbances of body image); visual (amaurotic, elementary and complex hallucinations, illusions) and other phenomena (anosognosia, apraxia, acalculia, alexia, aphemia, confusional states, gustatory, vertiginous, adversive, oculoclonic and eyelid flutter). The seizure symptoms are of varying localizing and lateralizing value and seizure discharges may spread rapidly and perceived symptoms may reflect secondary spread rather than the primary site of seizure onset. Recognized parietal and occipital epilepsy syndromes include benign epilepsy of childhood with centrotemporal spikes, benign epilepsy of childhood with parietal evoked spikes, benign occipital epilepsy of childhood, migraine/epilepsy syndromes, and epilepsy with bilateral occipital calcification. In addition, occipital and parietal epilepsy may be on the basis of any underlying structural lesion. There is frequently a poor correlation between clinical and EEG features. MRI and functional imaging often reveals underlying pathology. There have been no specific trials of different antiepileptic drugs for occipital and parietal seizures. Surgical treatment has its place, with attention to the risk of causing a fixed neurological deficit.

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Section: Epilepsy With Bilateral Occipital Calcificationsmentioning

confidence: 99%

Parietal and Occipital Lobe Epilepsy: A Review

1993

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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.…”

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confidence: 93%

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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“…No significant difference was present when compared with the CT findings at 12 years old (not shown here) not infrequently encountered. Although the bilateral presence of calcified lesions and absence of nevus flammeus is not uncommon in SWS [1], negative contrast enhancement in cranial MRI, lack of prominent cerebral atrophy and presence of basal ganglia calcifications in our patient, are strongly against such a diagnosis. Neurocysticercosis [11], mitochondrial disorders [12], dihydropteridine reductase deficiency [14] and hypoparathyroidism [8] which present with basal ganglia calcification were ruled out on basis of the clinical and laboratory findings.…”

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confidence: 53%