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Mov Disord. 2015 Jun;30(7):992-6. doi: 10.1002/mds.26244. Epub 2015 Apr 16.

Pallidal activity in myoclonus dystonia correlates with motor signs.

Welter ML1,2,3,4,5, Grabli D1,2,3,4,5, Karachi C1,2,3,4,6, Jodoin N4,7, Fernandez-Vidal S1,8, Brun Y1,2,3, Navarro S6, Rogers A6, Cornu P6, Pidoux B6, Yelnik J1,2,3,4, Roze E1,2,3,4,5, Bardinet E1,8, Vidailhet M1,2,3,4,5.

Author information

1
Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épiniere (CRICM), UMR-S975, Paris, France.
2
Inserm, U1127, Paris, France.
3
CNRS, UMR 7225, Paris, France.
4
Centre d'Investigation Clinique Pitié Neurosciences (Inserm CIC-1422), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
5
Département de Neurologie, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
6
Service de Neurochirurgie, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France.
7
Service de Neurologie, Centre hospitalier de l'Université de Montréal, Montréal, Québec, Canada.
8
Centre de Neuroimagerie de Recherche (CENIR), Groupe Hospitalier Pitié-Salpêtrière, Paris, France.

Abstract

BACKGROUND:

Myoclonus-dystonia related to epsilon-sarcoglycan gene mutations is characterized by myoclonic jerks and mild to moderate dystonia. The role of basal ganglia dysfunction in the pathogenesis is unknown.

METHODS:

Pallidal neuronal activity was recorded in six myoclonus-dystonia and six primary generalized dystonia patients operated on for internal globus pallidus deep brain stimulation.

RESULTS:

In myoclonus-dystonia patients compared with primary-dystonia patients, internal pallidum neurons showed higher burst frequency, lower mean burst, and pause durations. External pallidum neurons showed higher mean pause frequency. Oscillatory activity was present in 33% and 35% of internal pallidum neurons in myoclonus-dystonia and primary-dystonia patients, respectively, predominantly in the theta frequency band (3-8 Hz). In myoclonus-dystonia patients with more severe myoclonus, internal pallidum neurons exhibited a higher bursting activity with high intraburst frequency and lower oscillatory activity frequency.

CONCLUSIONS:

Myoclonus-dystonia appears to be related to specific changes in internal pallidum activity, leading to disruption in striato-pallido-thalamo-cortical circuits. © 2015 International Parkinson and Movement Disorder Society.

KEYWORDS:

deep brain stimulation; globus pallidus; myoclonus-dystonia; neuronal activity

PMID:
25880339
DOI:
10.1002/mds.26244
[Indexed for MEDLINE]

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