Sleep in Gerstmann-Straüssler-Scheinker disease

Gerstmann-Sträussler-Scheinker (GSS) is a rare prion disease with heterogeneous clinical presentation. Although sleep-related abnormalities are prominent and well-known in other prion diseases such as fatal familial insomnia and Creutzfeldt-Jakob disease, information on sleep is limited in GSS. We e...

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Bibliographic Details
Published in:Sleep medicine 2023-08, Vol.108, p.11-15
Main Authors: Pérez-Carbonell, Laura, Sarto, Jordi, Gaig, Carles, Muñoz-Lopetegi, Amaia, Ruiz-García, Raquel, Naranjo, Laura, Augé, Josep María, Perissinotti, Andrés, Santamaria, Joan, Iranzo, Alex, Sánchez-Valle, Raquel
Format: Article
Language:English
Subjects:
Gerstmann-sträussler-scheinker
Prion disease
Sleep
Video-polysomnography
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Summary:Gerstmann-Sträussler-Scheinker (GSS) is a rare prion disease with heterogeneous clinical presentation. Although sleep-related abnormalities are prominent and well-known in other prion diseases such as fatal familial insomnia and Creutzfeldt-Jakob disease, information on sleep is limited in GSS. We evaluated sleep in three genetically confirmed GSS cases using clinical history, sleep scales and video-polysomnography. In addition, patients underwent neurological assessment, neurological scales, neuropsychological testing, lumbar puncture, brain MRI and brain 18F-FDG-PET. Two patients reported sleep maintenance insomnia attributed to leg stiffness and back pain while the remaining patient did not report sleep problems. Video-polysomnography showed normal sleep staging in all of them. Findings such as reduced sleep efficiency in two patients, a confusional arousal in one patient, obstructive apneas in one patient, and periodic legs movements in sleep in two patients were observed. In contrast to fatal familial insomnia, the normal sleep staging in GSS may suggest dissimilar involvement of the neuronal structures that regulate sleep. We found non-specific sleep alterations in GSS such as obstructive apneas and periodic leg movements in sleep which are of unknown origin and of uncertain clinical relevance. Studies including a larger number of patients, serial sleep evaluations and incorporating neuropathological assessment will further help to understand sleep in GSS. •Gerstmann-Sträussler-Scheinker shows normal sleep stages•Sleep alterations in Gerstmann-Sträussler-Scheinker are non-specific•Further description of sleep findings and correlation with neuropathology are warranted
ISSN:1389-9457
1878-5506
DOI:10.1016/j.sleep.2023.05.010