title: An early onset of hereditary progressive dystonia with marked diurnal fluctuation: determining clinical status using gait analysis.
abstract: We report on a 5 year old girl who has hereditary progressive dystonia with marked diurnal fluctuation that improved remarkably after treatment with L-dopa. A gait analysis of the patient was done and we compared her gait patterns with those of a normal child. The following results were obtained. Before L-dopa treatment: (i) knee extension time was prolonged with genu recurvatum; (ii) the flexors and the extensors were simultaneously activated in the stance phase, and reciprocity between them was impaired; and (iii) recorded sole contact was a toe-only pattern. After L-dopa treatment: (i) the step cycle was shorter mainly due to a shorter knee extension time; (ii) genu recurvatum improved; (iii) reciprocal activities of the flexors and the extensors were observed; and (iv) a normal sole contact pattern was also observed. The data from the gait analysis were useful in stabilizing the subject's clinical condition and in evaluating changes to it.
authors.affiliation: Department of Pediatrics, Asahikawa Habilitation Center for Disabled Children, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Tanaka
authors.forename: J
authors.initials: J
authors.lastname: Ito
authors.forename: T
authors.initials: T
authors.lastname: Tasaki
authors.forename: K
authors.initials: K
authors.lastname: Cho
mesh_terms: D017668:Age of Onset; D002675:Child, Preschool; D002940:Circadian Rhythm; D004421:Dystonia / Q000188:drug therapy / Q000503:physiopathology*; D004576:Electromyography; D005260:Female; D005684:Gait; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use
chemical_list: D007980:Levodopa