Neuro-imaging in Kleine Levin syndrome

Kleine Levin syndrome is a rare disease affecting young people giving periodic hypersomnia combined with hyperphagia and other psychological symptoms. Our research has demonstrated hypoperfusion in temporofrontal regions on SPECT and a short term memory dysfuntion. We have a material of 15 patients and are examining them using cerebral fMRI with a cognitive paradigm for verbal memory and concentration. Preliminary results have revealed a thalamic dysfunction when compared to healthy ctrls. We also use 1H-MRS of hippocampus in search of an organic substrate for the memory dysfunction. The study aims at finding the biological substrate of this disease.

• Staff:

Anne-Marie Landtblom , Assoc. Prof.		project leader		Neurology
Maria Engström , Assoc. Prof.		fMRI		Radiology
Thomas Karlsson , Assoc. Prof.		behavioural tests		Behavioural sciences
Patrick Vigren , MD				Neurology US
Anders Tisell , PhD Student		MRS		Radiation physics
Peter Lundberg , Prof.		MRS		Radiation physics
Olof Dahlqvist Leinhard , PhD Student		MRS		Radiation physics

• Former Staff:

• Project Description:

Kleine Levin syndrome is a rare disease affecting young people giving periodic hypersomnia combined with hyperphagia and other psychological symptoms. Our research has demonstrated hypoperfusion in temporofrontal regions on SPECT and a short term memory dysfuntion (Landtblom 2002, 2003). 15 patients with Kleine Levin syndrome (KLS) and 15 healthy ctrls as well as some pts with hypersomnic syndrome without periodicity are investigated using cerebral fMRI, 1H-MRS of the hipppocampi, neuropsychological testing, SPECT of the brain and clinical status. The fMRI examination uses a cognitive paradigm for verbal memory and concentration tasks. Preliminary results has revealed a dysfunction in one nucleus of the thalami indicating a change in the neuronal circuits. We believe that the pathogenic mechanism affects both the deep cerebral nuclei and temporofrontal structures as indicated in our pervious studies with SPECT and neuropsychological testing.