The spectrum from BCECTS to LKS: The Rolandic EEG trait-impact on cognition.
The laboratory hallmark of BCECTS is the rolandic discharge (RD) in the EEG of patients, occurring in a characteristic topographical, vigilance-related, event-related, and age-related pattern, disappearing during puberty. RDs are present in 2% of healthy children. About 8% of children with RDs have epilepsy. An increased prevalence rate of RDs is found in children with cognitive and behavioral disorders, with headaches and some genetic syndromes. In some patients, the cognitive disorders are transient but in others they are progressive, resulting in stable mental retardation after puberty. A recent study of 36 BCECTS patients addressed the following questions. (1) the possible relationship between the severity of RDs and the neuropsychological deficits; (2) the profile of neuropsychological deficits; (3) changes of cognition related to EEG changes; and (4) effects of therapy. No correlation was found between global IQ and the severity of the RDs. All the children had at least one specific learning disorder (sometimes long-lasting). When the children were treated, a correlation between cognitive and EEG improvement could not be demonstrated. Recently, 21 patients without epilepsy but with attention deficit and hyperactivity and/or learning disorders were studied: an open treatment trial with sulthiame resulted in improved sustained and selective attention. The neurobiology of RDs and their relationship to cognitive dysfunction and epilepsy requires further study.
Stephani
Ulrich
U
University Children's Hospital, Department of Neuropediatrics, Kiel, Germany. stephani@pedneuro.uni-kiel.de
Carlsson
Goran
G
D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D002540:Cerebral Cortex / Q000503:physiopathology; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D004569:Electroencephalography / Q000706:statistics & numerical data*; D019305:Epilepsy, Rolandic / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D005858:Germany / Q000453:epidemiology; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D007859:Learning Disabilities / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data*; D012720:Severity of Illness Index; D013843:Thiazines / Q000627:therapeutic use; D016896:Treatment Outcome; D000096926:Benzenesulfonamides
D013843:Thiazines; C084593:sulthiame; D000096926:Benzenesulfonamides