Figure 1
Systematic search process for patients with cervical dystonia.
1Parkinson’s disease n = 9, ataxia n = 3, progressive supranuclear palsy n = 2, corticobasal syndrome n = 1.
2Drug-induced cervical dystonia n = 8, developmental disability due to infectious brain injury in the childhood with delayed-onset cervical dystonia in the adulthood n = 1, dystonic cerebral palsy with delayed-onset dystonia in the adulthood n = 1, developmental disability due to chromosomal aberration, deformation of the spinal column and delayed-onset cervical dystonia in the adulthood n = 1, brain injury due to Arnold Chiari malformation, hydrocephalus, cerebellar tonsillectomy and posterior fossa decompression surgery prior to the onset of cervical dystonia n = 1. Of note, none of these patients had isolated cervical dystonia. None of patients met the diagnostic criteria of genetic cervical dystonia.
Table 1
Demographic and clinical characteristics.
| ALL PATIENTS n = 365 | NO OTHER NEUROLOGICAL FEATURES (I.E. ISOLATED) n = 351 | OTHER NEUROLOGICAL FEATURES n = 14 | P-VALUE | |
|---|---|---|---|---|
| Demographic features | ||||
| Age at diagnosis, median (range) | 49.0 (20–92) | 49.0 (20–92) | 56.0 (27–75) | 0.477 |
| Sex (F), n (%) | 285 (78.1%) | 273 (77.8%) | 12 (85.7%) | 0.743 |
| Family history of cervical dystonia, n (%) | 279 (7.9%) | 27 (7.7%) | 2 (14.3%) | 0.307 |
| Duration of follow-up in months, median (range) | 115 (1–313) | 115 (1–313) | 94.5 (4–313) | 0.638 |
| Clinical features of dystonia | ||||
| Effective sensory trick, n (%) | 44 (12.1%) | 43 (12.3%) | 1 (7.1%) | 1.000 |
| Pain related to cervical dystonia, n (%) | 240 (65.8%) | 233 (66.4%) | 7 (50.0%) | 0.252 |
| Head/neck tremor, n (%) | 234 (64.1%) | 224 (63.8%) | 10 (71.4%) | 0.560 |
| Upper limb tremor, n (%) | 33 (9.0%) | 31 (8.8%) | 2 (14.3%) | 0.367 |
| Brain imaging | ||||
| Brain imaging, n (%) | 282 (77.3%) | 268 (76.4%) | 14 (100%) | 0.046 |
| CT/MRI, n | 37/245 | 37/231 | 0/14 | 0.228 |
| Acquired brain lesions1, n (%) | 9/282 (3.2%) | 3/268 (1.1%) | 6/14 (42.9%) | <0.001 |
| Lesion-induced cervical dystonia2, n (%) | 6/282 (2.1%) | 0/268 (0.0%) | 6/14 (42.9%) | <0.001 |
[i] Patients with cervical dystonia with presumed idiopathic or uncertain etiology prior to brain imaging with and without other neurological features at the onset of dystonia.
1All acquired brain lesions before the onset of cervical dystonia.
2Likely (n = 4) or possible (n = 2) lesion-induced cervical dystonia (see Supplementary table 1 for more details).