Focal cortical dysplasia: molecular disturbances and clinicopathological classification (review).
PBN-AR
Instytucja
Instytut "Pomnik - Centrum Zdrowia Dziecka"
Informacje podstawowe
Główny język publikacji
en
Czasopismo
International Journal of Molecular Medicine
ISSN
1107-3756
EISSN
1791-244X
Wydawca
SPANDIDOS PUBL LTD
DOI
URL
Rok publikacji
2016
Numer zeszytu
5
Strony od-do
1327-1337
Numer tomu
38
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Autorzy
Pozostali autorzy
+ 3
Słowa kluczowe
en
focal cortical dysplasia
malformations of cortical development
intractable epilepsy
seizures
tuberous sclerosis
Streszczenia
Język
angielski
Treść
Focal cortical dysplasia (FCD) is one of the most important causes of drug-resistant epilepsy in paediatric patients, particularly in those below the age of 3. Even though over 40 years have passed since the first description of the entity by Taylor, the exact mechanisms causing these cortical abnormalities remain unelucidated. In this review, we summarise the current knowledge on clinical and histopathological aspects, taking into account the new classification system proposed by the International League Against Epilepsy. We focus on the clinicopathological associations and differences in post-surgical outcome among FCD subtypes, in particular isolated FCD vs. FCD associated with principal lesions, which have not been summarised to date. We also recapitulate genetic studies, pointing to the possible mechanisms of the cortical dysregulation and drug resistance, and summarise novel factors which may contribute to epileptogenesis in FCD. Furthermore, we compare FCD type IIB (FCDIIB) with brain tumours found in a neurocutaneous disorder, tuberous sclerosis, as we evaluate the hypothesis that FCD IIB may be a local form of this disease.
Cechy publikacji
review-article
Inne
System-identifier
0000016934
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