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Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex
- Source :
- Frontiers in Neurology, Vol 10 (2019)
- Publication Year :
- 2019
- Publisher :
- Frontiers Media S.A., 2019.
-
Abstract
- Background: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA).Methods: The study was conducted at 170 sites across 31 countries. Data from patients of any age with a documented clinical visit for TSC in the 12 months preceding enrollment or those newly diagnosed with TSC were entered.Results: SEGA were reported in 554 of 2,216 patients (25%). Median age at diagnosis of SEGA was 8 years (range, 18 years. SEGA were symptomatic in 42.1% of patients. Symptoms included increased seizure frequency (15.8%), behavioural disturbance (11.9%), and regression/loss of cognitive skills (9.9%), in addition to those typically associated with increased intracranial pressure. SEGA were significantly more frequent in patients with TSC2 compared to TSC1 variants (33.7 vs. 13.2 %, p < 0.0001). Main treatment modalities included surgery (59.6%) and mammalian target of rapamycin (mTOR) inhibitors (49%).Conclusions: Although SEGA diagnosis and growth typically occurs during childhood, SEGA can occur and grow in both infants and adults.
Details
- Language :
- English
- ISSN :
- 16642295
- Volume :
- 10
- Database :
- Directory of Open Access Journals
- Journal :
- Frontiers in Neurology
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.f608118a1e414c178daa9fb24526b834
- Document Type :
- article
- Full Text :
- https://doi.org/10.3389/fneur.2019.00705