Good Practice Guide


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The classification of dystonia was revised in 2013 (Albanese 2013).
The new classification is based on 2 axes: clinical and aetiological.

Clinical axis

Time of onset   

Early onset dystonia appearing up to the age of 20 (often generalises to affect multiple body areas). Broken into sub-categories as follows:

  • Infancy (0–2 years)
  • Childhood (3–12 years)
  • Adolescence (13–20 years)

Adult onset dystonia appearing after the age of 20 (usually remains localised with restricted progression to adjacent muscles). Broken into sub-categories as follows:

  • Early adulthood (21–40 years)
  • Late adulthood (>40 years)

Body distribution   

  • Focal: single body region.
  • Segmental: contiguous body regions (Example: cranial and cervical, cervical and upper limb.)
  • Multifocal: non-contiguous body regions (Example: upper and lower limb, cranial and upper limb.)
  • Generalised: the trunk and at least two other sites (often includes arms and/or legs).
  • Hemidystonia: half of the body (usually secondary to structural lesion in contra-lateral basal ganglia).

Temporal pattern   

  • Persistent: symptoms persist to approximately same extent throughout the day.
  • Action-specific: symptoms only present when performing a particular task (such as writing or playing music).
  • Diurnal: symptoms fluctuate through the day in a regular circadian pattern.
  • Paroxysmal: symptoms occur in brief episodes with normality in between.

Isolated / Combined  

  • Isolated: torsion dystonia does not appear in combination with any other movement disorder (with the exception of tremor).
  • Combined: torsion dystonia combined with other movement disorders(such as myoclonus or parkinsonism). There is no evidence of neurodegeneration.

Aetiological axis

Inheritance   

  • Inherited: linked to a known gene locus or identified genetic defect.
  • Acquired: dystonia has an identified environmental cause such as a brain lesion, another neurological disorder or exposure to drugs or chemicals. Examples: dystonia due to brain tumour, off-period dystonia in Parkinson’s disease, tardive dystonia which is drug-induced, some forms of cerebral palsy including premature delivery and birth injuries, neurodegenerative and metabolic disorders.
  • Idiopathic: dystonia has no identified cause.

 Last reviewed June 2014

Disclaimer
The Dystonia Society provides the information on this page as general information only. It is not intended to provide instruction and you should not rely on this information to determine diagnosis, prognosis or a course of treatment. It should not be used in place of a professional consultation with a doctor.
The Dystonia Society is not responsible for the consequences of your decisions resulting from the use of this information, including, but not limited to, your choosing to seek or not to seek professional medical care, or from choosing or not choosing specific treatment based on the information. You should not disregard the advice of your physician or other qualified health care provider because of any information you receive from us. If you have any health care questions, please consult the relevant medical practitioner.

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