Abstract

ROLON, Marcela L; YALJ, Susana; ALABART, Nora  and  MENZANO, Enrique. L-Dopa-sensible dystonia: Segawa disease. Arch. argent. pediatr. [online]. 2007, vol.105, n.6, pp.542-544. ISSN 0325-0075.

We reported the case of an eight year-old girl, hospitalized due to the appearance of acute involuntary, continuous and torsional movements in the left leg, with alteration of gait; motions worsened throughout the day and improved during sleep. As symptoms matched those of dystonia, while discarding organic causes, we performed a therapeutic test with L-Dopa, with a clear response. With this clinical picture, added to the existence of a blood relative with Parkinson disease, and having discarded the Wilson disease, we reached an L-Dopa dystonia-sensitive (Segawa disease) diagnosis, which is infrequent, chronic and drug dependent.

Keywords : Dystonia; L-Dopa sensible dystonia; Segawa disease.

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