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Distonía sensible a L-Dopa: enfermedad de Segawa

Keywords: dystonia, l-dopa sensible dystonia, segawa disease.

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Abstract:

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.

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