Ictal SPECT in Sturge-Weber syndrome

Bilgin, Özgür; Vollmar, Christian; Peraud, Aurelia; Fougère, Christian la; Beleza, Pedro; Noachtar, Soheyl

doi:10.1016/j.eplepsyres.2007.12.006

Cited by 10 publications

(6 citation statements)

References 14 publications

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“…When hemispherectomy is not successful, seizures are usually seen shortly after surgery 12 . The longest seizure-free interval reported so far after hemispherectomy has been two years only in a patient that had functional hemispherectomy 13 . The usually poor cognitive profile in those cases is attributed to the effects of ongoing seizures, natural progression of the disease or a combination of these factors.…”

Section: Discussionmentioning

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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Section: Discussionmentioning

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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“…In these patients, abnormalities of CBF in the affected areas can help physicians locate the seizure focus. Lennox-Gastaut syndrome [49] and Sturge-Weber syndrome [50][51][52] have been evaluated with PET and SPECT imaging which have helped to identify affected areas for possible surgical treatment. Specific mass lesions observed in disorders such as tuberous sclerosis [12,53], dysembryoplastic neuroepithelial tumors [54], and long-term epilepsy associated tumors [55] can be evaluated with MRI to identify the lesions as well as with PET or SPECT to identify those specific lesions which develop into seizure foci.…”

Section: Comparison Studies Between Spect Pet and Mri In Seizure DImentioning

confidence: 99%

Role of SPECT and PET in pediatric epilepsy

Mashayekh

Newberg

2015

J Pediatr Epilepsy

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Single photon emission computed tomography (SPECT) and positron emission tomography (PET) offer valuable information for the diagnosis, management, and follow up of patients with seizure disorders. These imaging studies can also contribute to the understanding of the pathophysiology of epilepsy. Intractable epilepsy in children is a serious disorder that has an overwhelming effect on physical, psychological, and social development. Functional imaging with PET and SPECT has been useful in a variety of seizure types and is typically more sensitive compared to anatomic imaging methods such as magnetic resonance imaging for localizing non-lesional seizure foci. SPECT and PET may be useful in guiding the strategy of implantation in patients submitted to undergo invasive recordings. However, the ability of PET and SPECT to localize a seizure focus may be crucial for tailoring the surgical resection area in patients with intractable seizures. Since most seizure cases begin in childhood, PET and SPECT imaging for seizure disorders is frequently used in this population. In this article, we review the literature on the current uses and indications of PET and SPECT in the study and management of epilepsy in children.

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“…Eine Interpretation der Perfusions-Daten ist daher nur im Kontext der klinischen Information aus EEG und Anfallssemiologie zum Zeitpunkt der Injektion möglich. Die iktale SPECT-Untersuchung hat einen besonders hohen diagnostischen Stellenwert bei MRT-negativen Patienten, bei Patienten mit nicht lokalisiertem oder artefaktverdecktem EEG, sowie bei voroperierten Patienten, bei denen EEG-Befunde wegen veränderter elektrischer Leitfähigkeit durch die Resektion und Knochenlücken schwer zu lokalisieren ist (Bilgin et al, 2008). Nach erfolgreicher iktaler SPECT Untersuchung während eines Anfalles kann ergänzend eine interiktale Untersuchung durchgeführt werden, in der sich die Anfalls-spezifische Perfusionssteigerung nicht zeigt.…”

Section: Single-photon-emissions-computer-tomographie (Spect)unclassified