Reduced thalamic and pontine connectivity in kleine-levin syndrome.
The Kleine-Levin syndrome (KLS) is a rare sleep disorder, characterized by exceptionally long sleep episodes. The neuropathology of the syndrome is unknown and treatment is often inadequate. The aim of the study was to improve understanding of the underlying neuropathology, related to cerebral networks, in KLS during sleep episodes. One patient with KLS and congenital nystagmus was investigated by resting state functional magnetic resonance imaging during both asymptomatic and hypersomnic periods. Fourteen healthy subjects were also investigated as control samples. Functional connectivity was assessed from seed regions of interest in the thalamus and the dorsal pons. Thalamic connectivity was normal in the asymptomatic patient whereas the connectivity between the brain stem, including dorsal pons, and the thalamus was diminished during hypersomnia. These results suggest that the patient's nystagmus and hypersomnia might have their pathological origin in adjacent dorsal pontine regions. This finding provides additional knowledge of the cerebral networks involved in the neuropathology of this disabling disorder. Furthermore, these findings regarding a rare syndrome have broad implications, and results could be of interest to researchers and clinicians in the whole field of sleep medicine.
Division of Radiological Sciences, Department of Medical and Health Sciences (IMH), Linköping University , Linköping , Sweden ; Center for Medical Image Science and Visualization (CMIV), Linköping University , Linköping , Sweden.
Maria
M
Engström
Center for Medical Image Science and Visualization (CMIV), Linköping University , Linköping , Sweden ; Division of Disability Research and Linnaeus Centre HEAD, Department of Behavioral Science and Learning, Linköping University , Linköping , Sweden.
Thomas
T
Karlsson
Center for Medical Image Science and Visualization (CMIV), Linköping University , Linköping , Sweden ; Division of Neurology, Department of Clinical and Experimental Medicine (IKE), Linköping University , Linköping , Sweden.
Anne-Marie
AM
Landtblom
brain stem; cerebellum; functional magnetic resonance imaging; nystagmus; periodic idiopathic hypersomnia; pons; sleep; thalamus