Paediatria Croatica, Vol. 53 No. 3, 2009.
Sažetak sa skupa
CLINICAL FEATURES OF EPILEPSY IN CHILDREN WITH CENTRAL NERVOUS SYSTEM MALFORMATIONS ACCORDING TO DEVELOPMENTAL CLASSIFICATION
M. Tomasović
Sažetak
Central nervous system malformations (CNSM) and especially malformations of cortical development (MCD) are a common cause of epilepsy, although epilepsies are not always the most prominent neurologic manifestation of these disorders. Advances in developmental neuroscience and neuroimaging techniques have resulted in recognition and better understanding of CNSM, and therefore a better understanding of both normal and abnormal brain development. CNSM classification is not final and it will continue to be modified with increasing experience of geneticists, neurologists and neuroimagers. The aim of this study was to investigate the clinical features of epilepsy in 146 children with CNSM reffered to the Pediatric Clinic of the Clinical Hospital Centre Split from 1998 to 2009. Epilepsy was recognized in 68 patients (46.6 %), 37 (25.3%) of whom had the first sezures by 1 year of age, 23 had epileptic encephalopahy (EE), 39 generalized epilepsy (GE) and 6 partial epilepsy (EP). Epileptic seizures were controlled in 35 (24%), and refractory in 33 (22.6%) patients. Epilepsy is significantly more rare in isolated disorders of dorsal and ventral induction and significantly more frequent in more immature anomalous brain lesions, microcephaly and lissencephaly. Patients with more immature lesions often have multiple CNSM, seizure onset by 1 year of age, EE and refractory seizures. We established an etiological diagnosis in 62 of 146 cases (42.4%). Classification of CNSM based on radiological, clinical genetic and neurological examinations require a specialized multidisciplinary team. The input of the clinician remains necessary to link clinical, genetic and neuro-imaging data to define precisely clinico-imaging entities to improve patient treatment and disease management.
Ključne riječi
Hrčak ID:
43632
URI
Datum izdavanja:
20.11.2009.
Posjeta: 798 *