[Familial idiopathic paroxysmal kinesigenic dyskinesia: its natural history and a descriptive study in three Spanish families].
BACKGROUND
Paroxysmal kinesigenic dyskinesia (PKD) is a disorder that is characterised by brief episodes of involuntary movements triggered by other sudden movements.


METHODS
Here we describe the cases of nine patients from three unrelated families who had PKD in its familial idiopathic form.


RESULTS
The majority of the patients (77.7%) were males. The mean age at onset was 10.3 years. All the subjects presented sudden movements as factors that precipitated the crises, which lasted < 10 s in 88.8% of cases. Two thirds (66.6%) of the patients were treated with carbamazepine, phenytoin and valproic acid and all of them responded well. Spontaneous remission occurred in 62.5% of the patients over 20 years of age and in a further 25% there was a significant decrease in the number of crises.


CONCLUSIONS
In our sample the proportion of patients who presented spontaneous remission was greater than in that reported in previous studies. As in other series, we found positive responses to antiepileptic drugs other than carbamazepine.
Palau-Bargues
Juan
J
Servicio de Neurología, Hospital Universitario La Fe, Avenida Campanar 21, Valencia, Spain. palaubargues@gmail.com
Rubio-Agusti
Ignacio
I
Burguera
Juan Andrés
JA
Vilchez-Padilla
Juan Jesús
JJ
Villanueva
Vicente E
VE
D000293:Adolescent; D002648:Child; D002819:Chorea / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D012189:Retrospective Studies; D013030:Spain