Paroxysmal dyskinesias in mice.
Animal models of human disease are important tools for revealing the underlying mechanisms of pathophysiology and developing therapeutic strategies. Several unique mouse calcium channel mutants have been identified with nonepileptic, episodic dyskinetic movements that are phenotypically similar to human paroxysmal dyskinesias. In this report, video demonstrations of these motor attacks are provided for two previously described mouse mutants, tottering and lethargic, as well as a new one, rocker. Semiquantitative comparisons using two different rating scales reveal differences in attack morphology, severity, and duration among the strains. These mice provide three independent models of paroxysmal dyskinesia and support for prior proposals that channelopathies may underlie the human disorders.
Shirley
Thomas L
TL
Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
Rao
Lekha M
LM
Hess
Ellen J
EJ
Jinnah
H A
HA
D000367:Age Factors; D000818:Animals; D001522:Behavior, Animal; D002819:Chorea / Q000235:genetics / Q000503:physiopathology*; D004195:Disease Models, Animal; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D012720:Severity of Illness Index