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: H Tanaka, J Ito, T Tasaki, K Cho
• Journal: Acta paediatrica Japonica : Overseas edition (Acta Paediatr Jpn) Vol. 37 Issue 1 Pg. 88-93 (Feb 1995) ISSN: 0374-5600 [Print] Australia
• PMID: 7754776 (Publication Type: Case Reports, Journal Article)

Chemical References

• Levodopa

Topics

• Age of Onset
• Child, Preschool
• Circadian Rhythm
• Dystonia (drug therapy, physiopathology)
• Electromyography
• Female
• Gait
• Humans
• Levodopa (therapeutic use)