Electrophysiological characteristics of limbic and motor globus pallidus internus (GPI) neurons in two cases of Lesch-Nyhan syndrome

Détails

ID Serval
serval:BIB_B9AAC11E814F
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Electrophysiological characteristics of limbic and motor globus pallidus internus (GPI) neurons in two cases of Lesch-Nyhan syndrome
Périodique
Neurophysiologie Clinique
Auteur(s)
Pralong  E., Pollo  C., Coubes  P., Bloch  J., Roulet  E., Tetreault  M. H., Debatisse  D., Villemure  J. G.
ISSN
0987-7053
Statut éditorial
Publié
Date de publication
12/2005
Peer-reviewed
Oui
Volume
35
Numéro
5-6
Pages
168-73
Notes
Case Reports
Journal Article --- Old month value: Nov-Dec
Résumé
OBJECTIVE: Lesch-Nyhan syndrome is a rare and debilitating condition characterized by dystonia and self-mutilating behavior. In order to shed light on the pathophysiology of dystonia, we report the pallidal electrophysiological activity recorded in two patients during deep brain stimulation surgery (DBS). METHODS: Microrecordings were performed on 162 neurons along four tracks aimed at the right and left anterior (limbic) and posterior (motor) globus pallidus internus (GPI). RESULTS: Regardless of the anesthetic agent used (propofol or sevoflurane), both patients showed similar neurons firing rates in the four regions studied, namely the limbic and motor portions of the globus pallidus externus (GPE) or GPI. In both patients, firing rates were similar in the GPE (12.2+/-1.8 Hz, N=38) and GPI (13.2+/-1.0 Hz, N=83) portions of the limbic track, while the motor GPE fired at a higher frequency (23.8+/-2.7 Hz, N=18) than the motor GPI (12.5+/-1.4 Hz, N=23). CONCLUSIONS: These results demonstrate that light propofol or sevoflurane anesthesia influences pallidal activity in a similar way. Electrophysiological recordings suggest that Lesch-Nyhan syndrome might be characterized by analogous firing frequencies in the limbic GPE and GPI while motor GPE would tend to fire at higher rate than the motor GPI. It is therefore tempting to suggest that the symptoms that are observed in Lesch-Nyhan syndrome might result from motor GPI inhibition. SIGNIFICANCE: This observation may confirm the Albin and Delong's model of the basal nuclei in hypokinetic and hyperkinetic disorders.
Mots-clé
Child Electric Stimulation Therapy Electrophysiology/methods Globus Pallidus/*physiopathology Humans Hypoxanthine Phosphoribosyltransferase/deficiency Lesch-Nyhan Syndrome/*physiopathology Limbic System/*physiopathology Motor Neurons/*physiology Sleep/physiology
Pubmed
Web of science
Création de la notice
06/02/2008 10:03
Dernière modification de la notice
20/08/2019 15:27
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