Neuroimaging in the Kleine-Levin Syndrome

Abstract

Purpose of ReviewThe purpose was to review the most recent literature on neuroimaging in the Kleine-Levin syndrome (KLS). We aimed to investigate if frontotemporal and thalamic dysfunction are key KLS signatures, and if recent research indicates other brain networks of interest that elucidate KLS symptomatology and aetiology.Recent FindingsIn a comprehensive literature search, we found 12 original articles published 2013–2018. Most studies report deviations related to cerebral perfusion, glucose metabolism, or blood-oxygen-level-dependent responses in frontotemporal areas and/or the thalamus. Studies also report dysfunction in the temporoparietal junction and the oculomotor network that also were related to clinical parameters. We discuss these findings based on recent research on thalamocortical networks and brain stem white matter tracts.SummaryThe hypothesis of frontotemporal and thalamic involvement in KLS was confirmed, and additional findings in the temporoparietal junction and the oculomotor system suggest a broader network involvement, which can be investigated by future high-resolution and multimodal imaging.

Kleine Levin syndrome is associated with birth difficulties and genetic variants in the TRANK1 gene loci

“…One possible hypothesis that KLS, like bipolar disorder and other psychosis, is characterized by periodic instability of specific brain networks involving, in the case of KLS, reduced oxygen flow in cortical and subcortical areas, as reported by imaging studies(72). In this syndrome, cognitive and derealization symptoms may correlate with decreased activity in cortical areas, whereas decreased activity in thalamus and hypothalamus may be involved in the functional hypersomnia observed in disorder (72-74).…”

“…One possible hypothesis that KLS, like bipolar disorder and other psychosis, is characterized by periodic instability of specific brain networks involving, in the case of KLS, reduced oxygen flow in cortical and subcortical areas, as reported by imaging studies(72). In this syndrome, cognitive and derealization symptoms may correlate with decreased activity in cortical areas, whereas decreased activity in thalamus and hypothalamus may be involved in the functional hypersomnia observed in disorder (72-74). Involvement of circadian or behavioral rhythmicity genes may be essential for maintaining hypersomnia episodes once an episode is initiated, for example, if subjects are suddenly spending much time in bed, unexposed to light, a behavior which could result in difficulties entraining in genetically susceptible individuals (75).…”

Astrocitoma Pilocítico Cerebelar De Desenvolvimento Atípico: Relato De Caso

“…Na forma típica, os sintomas e o desenvolvimento da doença seguem padrões característicos, mas é considerada uma forma típica secundária quando sintomas neurológicos já se encontram presentes antes do primeiro episódio da síndrome e persistem entre os episódios, sendo observada em cerca de 10 % dos pacientes. A apresentação clínica da SKL é considerada como uma forma atípica se um dos principais sintomas é o oposto do classicamente descrito na síndrome (SHUKLA et al, 2008;LAVAULT et al, 2015;ARNULF, 2015;GUILLEMINAULT, 2016;LATINI;LANDTBLOM, 2018).…”

Síndrome de Kleine-Levin: atualizações de diagnóstico e tratamento/Kleine-Levin syndrome: diagnostic and treatment updates

“…Na forma típica, os sintomas e o desenvolvimento da doença seguem padrões característicos, mas é considerada uma forma típica secundária quando sintomas neurológicos já se encontram presentes antes do primeiro episódio da síndrome e persistem entre os episódios, sendo observada em cerca de 10% dos pacientes. A apresentação clínica da SKL é considerada como uma forma atípica se um dos principais sintomas é o oposto do classicamente descrito na síndrome (SHUKLA et al, 2008;LAVAULT et al, 2015;ARNULF, 2015;GUILLEMINAULT, 2016;LATINI;LANDTBLOM, 2018). SKL atípica foi definida como uma forma de distúrbio recorrente, em que hipersonia, hiperfagia e/ou hipersexualidade são substituídas, respectivamente, por insônia, anorexia e hipossexualidade.…”