" /> Dopa-responsive dystonia - CISMeF





Preferred Label : Dopa-responsive dystonia;

ICD-11 definition : Dopa-responsive dystonia is a dystonic disorder that typically presents in childhood with walking problems due to dystonia of the lower limbs and worsening of the dystonia towards the evening. It may also present with a ''cerebral-palsy'' like picture with onset in infancy, or more rarely as a focal dystonia with onset typically in adulthood or even as parkinsonism in adulthood.;

ICD-11 synonym : DRD - [dopa-responsive dystonia]; DYT5; Segawa syndrome;

ICD-11 acronym : DRD; DYT5;

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Dopa-responsive dystonia is a dystonic disorder that typically presents in childhood with walking problems due to dystonia of the lower limbs and worsening of the dystonia towards the evening. It may also present with a ''cerebral-palsy'' like picture with onset in infancy, or more rarely as a focal dystonia with onset typically in adulthood or even as parkinsonism in adulthood.

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25/05/2025


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