Table 1.

Etiological Classification and Diagnosis of Craniocervical Dystonia

Primary craniocervical dystonia: syndromes in which dystonia is the sole phenotypic manifestation with the exception that tremor can be present as well

Secondary craniocervical dystonia: due to structural lesions and/or neural insults

vascular (e.g., ischemic stroke, hemorrhagic stroke, cerebral palsy)

infectious (e.g., human immunodeficiency virus, arboviruses)

toxins (e.g., manganese, carbon disulfide)

medications (e.g., neuroleptics, antiemetics, anticonvulsants)

autoimmune/inflammatory (e.g., multiple sclerosis, lupus erythematosus, Behcet disease)

metabolic (e.g., hypoxia, extra-pontine myelinolysis)

neoplasms (e.g., glioblastoma, metastatic tumor, meningioma)

Craniocervical dystonia-plus: disorders distinct from primary dystonia and heredodegenerative diseases with dystonia

dopa-responsive dystonia (DYT5)

myoclonus-dystonia syndrome (DYT11)

Heredodegenerative diseases with craniocervical dystonia: typically associated with pathological changes in brain tissue and may be hereditary (e.g., progressive supranuclear palsy, Parkinson disease, multiple system atrophy, corticobasal ganglionic degeneration, spinocerebellar ataxias, Huntington disease, Lubag [DYT3], rapid-onset dystonia parkinsonism [DYT12], Wilson disease, Lesch-Nyhan syndrome, pantothenate kinase-associated neurodegeneration, mitochondrial encephalopathies, Niemann-Pick type C, neuronal ceroid lipofuscinosis)

Psychogenic craniocervical dystonia: dystonia primarily due to psychological factors