The Journal of Neuroscience, 2001, 21:RC153:1-4
RAPID COMMUNICATION
Spontaneous Remission of Paroxysmal Dystonia Coincides with
Normalization of Entopeduncular Activity in
dtsz Mutants
Mustapha
Bennay,
Manuela
Gernert, and
Angelika
Richter
Department of Pharmacology, Toxicology, and Pharmacy, School of
Veterinary Medicine Hannover, 30559 Hannover, Germany
Recent studies have shown a dramatically decreased spontaneous
discharge rate of entopeduncular neurons in a unique animal model of
idiopathic paroxysmal dystonia, the
dtsz mutant hamster. These changes
were found in animals at the age at which the most marked expression of
dystonia is usually observed. In this rodent model, the
age-dependent disappearance of stress-inducible dystonic attacks at an
age of ~10 weeks allows investigations of the relevance of
pathophysiological changes for the occurrence of dystonia by
ontogenetic studies. Therefore, we examined the entopeduncular activity
by extracellular single unit recordings in groups of
dtsz mutants and nondystonic control
hamsters at 17-22 weeks of age. In contrast to recent findings, after
the complete remission of dystonia, the mean discharge rate of
entopeduncular neurons in dtsz
mutants (28.1 ± 1.2 spikes/sec) was similar to that of
age-matched nondystonic control hamsters (30.8 ± 0.9 spikes/sec).
Thus, the disappearance of paroxysmal dystonia is accompanied by a
normalization of the entopeduncular activity in
dtsz mutants. The present data
clearly demonstrate the fundamental importance of a decreased basal
ganglia output for the expression of paroxysmal dystonia.
Key words:
animal models; basal ganglia; dyskinesia; dystonia; entopeduncular nucleus; movement disorders; single unit recordings
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