Neurocognitive and behavioral profiles of children with Landau-Kleffner syndrome

Abstract

This is a retrospective study of 14 cases of children with Landau-Kleffner syndrome (LKS), the most prominent feature of which is acquired aphasia. These children were followed at a tertiary care pediatric epilepsy center. From the research data base, all LKS cases with neuropsychological evaluation were extracted. Children ranged in age from 6 to 13 years (M = 9.12; SD = 2.19) at the time of assessment (1 to 10 years post-onset). The majority of the children were white males, and all but one continued to experience seizure activity. Global intellectual functioning ranged from 59 to 101 (M = 82.07; SD = 12.14). Across the 14 cases reviewed, the neuropsychological profiles are considered in the context of neurological and syndrome-related factors. For these cases, 86% demonstrated continued expressive, and 50% had receptive language problems with 57% exhibiting poor auditory processing. Furthermore, 50 to 57% had deficits in auditory working memory and verbal memory. Academically, the majority had poor reading fluency and comprehension; 50% exhibited difficulty with mathematics. Finally, 57% evidenced attentional or other behavioral problems. Better understanding of LKS can assist in targeted assessment and intervention planning.

Neuropsychological Assessment of Children with Landau-Kleffner Syndrome

“…In their sample of 14 children with LKS, Riccio et al reported auditory processing problems in 57%, receptive language deficits in 50%, and expressive language problems in 86%. 43 Caraballo et al reported severe aphasia in 24 of their cases (82.8%) and moderate aphasia in 5 of their cases (17.2%). 3 Twenty-four percent had a fluctuating course of aphasia, which is an often cited characteristic of LKS.…”

“…In a sample of 14 children with LKS, Riccio et al found that the majority of children had problems with reading fluency and comprehension and half had problems with mathematics. 43…”

“…38 Most recently, Caraballo et al found moderate nonlinguistic cognitive deficits in almost half of their sample (48.3%), 3 and another recent study suggested that 43 to 50% of children with LKS have deficits in visual-spatial/perceptual motor functioning. 43 In the context of neuropsychological assessment, measures of visual-spatial processing should be included that do not require a motor response. When interpreting poor graphomotor production, motor-free visual processing tasks can help determine the extent to which motor problems may interfere with performance.…”

Landau Kleffner Syndrome: An Unusual Case With Progressive Ataxia Prior To Language Regression And Autistic-Like Behaviors

“…The etiology of LKS is still unknown [22], but involvement of the frontal and/or temporal lobes is frequently stated [23]. It is unclear whether LKS represents the late manifestation of preexisting anomalies in neurodevelopment or the result of acquired pathogenetic influences such as an abnormal autoimmune response or both.…”

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

“…Среди больных СЛК описано развитие психопато-, шизофрено-и аутистикоподобного поведения, воз-никновение приступов немотивированной агрессии, тревоги и страхов [37]. Во время активного периода заболевания у всех пациентов наблюдаются различ-ной степени выраженности когнитивные нарушения: ухудшение памяти (особенно запоминания нового), внимания, скорости реакции; расстройство чтения, письма, пространственной ориентации, проявления диспраксии; тяжелые нарушения коммуникации [36,48]. Таким образом, именно речевые, поведенческие и когнитивные нарушения, а не эпилептические при-ступы определяют тяжесть заболевания и степень со-циальной дезадаптации пациентов.…”