Improved definition and classification of dystonia
In 2013 Prof. Alberto Albanese et al. proposed this new and improved definition and classification of dystonia. The main aim of the new classification is to improve the clinical characteristics of dystonia and organize current knowledge regarding the ethology. Two axes were developed: Axis 1 for clinical characteristics and Axis 2 for aetiology. Additionally, the term “primary” and “secondary” dystonia is no longer used and has been replaced by isolated dystonia and combined dystonia.
Axis 1 – Clinical characteristic:
- Age of onset
Infancy (birth to 2 years)
Childhood (3–12 years)
Adolescence (13–20 years)
Early adulthood (21–40 years)
Late adulthood (>40 years)
- Body distribution
FOCAL – Only one body region is affected (e.g. blepharospasm, oromandibular dystonia, cervical dystonia, laryngeal dystonia, and writer’s cramp).
SEGMENTAL – Two or more contiguous body regions are affected( e.g. cranial dystonia)
MULTIFOCAL – Two noncontiguous or more body regions are involved.
GENERALIZED – The trunk and at least 2 other sites are involved.
HEMIDYSTONIA – More body regions restricted to one body side are involved (e.g. brain lesions)
- Temporal pattern ( e.g. Disease course and Variability)
- Associated features (isolated dystonia or combined with another movement disorder)
Axis 2 – Aetiology
- Nervous system pathology ( neurodegeneration/structural lesion/ No evidence of degeneration or structural lesion)
- Inherited or acquired
- Idiopathic (sporadic/familial)