Outcome at Adulthood of the Continuous Spike–waves During Slow Sleep and Landau–Kleffner Syndromes

Abstract

Summary:Purpose: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike-waves during slow sleep (CSWS) and Landau-Kleffner syndromes, which are two rare epileptic syndromes occurring in children.Methods: We enrolled seven young adults, five who had a CSWS syndrome, and two, a Landau-Kleffner syndrome in childhood. We evaluated their intellectual level as well as their oral and written language and executive functions.Results: This study confirmed that the epilepsy associated with these syndromes has a good prognosis. Only one patient still had active epilepsy. However, the neuropsychological disorders particular to each syndrome persisted. Only two patients had followed a normal pathway in school. Three of the five patients with a CSWS syndrome during childhood remained globally and nonselectively mentally deficient. We found no evidence of the persistence of a dysexecutive syndrome in this study group. The intellectual functions of the two patients with Landau-Kleffner syndrome were normal; however, their everyday lives were disrupted by severe, disabling language disturbances. We discuss the role of some prognostic factors such as the location of the interictal electric focus and the age at onset of CSWS.Conclusions: These two epileptic syndromes of childhood are very similar in many respects, but their clinical outcomes in adulthood are different.

Sequential Prefrontal Lobe Volume Changes in Epileptic Patients with Continuous Spikes and Waves During Slow Sleep

“…The relation between the density of paroxysmal anomalies and neuropsychological regression is based on clinical findings, notably, the parallel between paroxysmal anomalies duration and ultimate neuropsychological outcome, as well as between the neuropsychological disturbances and the location of the interictal epileptic focus (Tassinari et al, 2005;De Negri, 1994;Morikawa et al, 1992). In addition, the most convincing theory maintains that focal www.intechopen.com epileptic activity produces a disturbance in the maturation of cortical zones, mainly in the associative areas (Praline et al, 2003). Nonconvulsive status epilepticus (SE) in adult animals leads to widespread neuronal necrosis in vulnerable regions (Wasterlain et al, 1993).…”

Landau–kleffner Syndrome (Acquired Epileptic Aphasia) With a Focus on Electroencephalographic Criteria

“…В этих случаях речь идет о региональных острых волнах и комплексах острая-медленная волна невысокого индекса, возникающих главным образом во сне и не оказывающих негативно-го воздействия на когнитивные функции пациентов. Разумеется, при длительном персистировании реги-ональной эпилептиформной активности необходимо проводить высокоразрешающую МРТ для исключения симптоматического характера заболевания [34]. …”

Rehabilitation issues in Landau–Kleffner Syndrome

“…Reports vary on long-term prognosis, ranging from age-appropriate vocational and social functioning [4] to extremely limited independence [10,15]. An estimated 50% of children experience partial remission and 10-20% of children experience almost total remission [16]. However, persistent language deficits are seen in most cases and specific deficits in phonological short-term memory (but not necessarily lexico-semantic short-term memory [17]) are seen in all cases [8,18].…”