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CC BY-NC-ND 4.0 · Sleep Sci 2021; 14(S 01): 97-100
DOI: 10.5935/1984-0063.20200113
CASE REPORTS

Sleep-related hypermotor epilepsy in a patient with mucopolysaccharidosis type III

Anna A. Abramova
1   Research Center of Neurology, Epilepsy Unit with Laboratory of Clinical Neurophysiology - Moscow - Russia.
,
Hrayr P. Attarian
2   Northwestern University Feinberg School of Medicine, Center for Sleep Disorders - Chicago - Illinois - United States.
,
Snezhana M. Dolgova
1   Research Center of Neurology, Epilepsy Unit with Laboratory of Clinical Neurophysiology - Moscow - Russia.
,
Alexandra I. Belyakova-Bodina
1   Research Center of Neurology, Epilepsy Unit with Laboratory of Clinical Neurophysiology - Moscow - Russia.
,
Elena V. Iakovenko
3   Research Center of Neurology, Department of Neurogenetics - Moscow - Russia.
,
Amayak G. Broutian
1   Research Center of Neurology, Epilepsy Unit with Laboratory of Clinical Neurophysiology - Moscow - Russia.
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ABSTRACT

Both non-epileptic sleep disturbances and epilepsy are common in patients with mucopolysaccharidoses (MPS), so diagnosis of sleep-related hypermotor epilepsy in these patients is a tackling issue. We present a case of an adult patient with MPS IIIB (Sanfilippo syndrome), who presented with numerous nocturnal events of sudden awakening and hypermotor behavior, which had been previously regarded as parasomnias. Overnight video-EEG captured numerous stereotypical seizures with ictal pattern in the frontal regions, which led the diagnosis of SHE. The patient was started with carbamazepine, which resulted in a substantial reduction in the number of seizures. Our report provides further support for use of overnight video-EEG in the differential diagnosis of sleep-related disorders in MPS, yet true incidence of SHE in MPS patients remains unknown.

Keywords:

Sleep-related Hypermotor Epilepsy - Mucopolysaccharidosis - Sanfilippo Syndrome - Hypermotor Seizures - Parasomnias

FUNDING

The study received no sponsorship.




Publikationsverlauf

Eingereicht: 06. November 2020

Angenommen: 10. Februar 2021

Artikel online veröffentlicht:
30. November 2023

© 2023. Brazilian Sleep Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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