From: The phenomenology and natural history of idiopathic lower cranial dystonia
New patients | Combined data | |
---|---|---|
Number of the patients | 25 | 41 |
Sex | Female 18/25 (72%) | Female 24/41 (58.5%) |
Male 7/25 (28%) | Male 17/41 (41.5%) | |
Female/male (F/M ratio) | 2.6:1 | 1.4:1 |
Average age at onset (years) | 56 | 53.3 |
In female subgroup | 59.5 | 48.6 |
In male subgroup | 48.6 | 47.2 |
Areas affected | ||
Jaw only (including mixed primary movements of jaw) | 15 (60%) | 20 (48.8%) |
Lip or perioral only | 0 (0%) | 2 (4.9%) |
Tongue only | 2 (8%) | 5 (12.2%) |
Pharynx/larynx | 1 (4%) | 2 (4.9%) |
Mixed | 7 (28%) | 11 (26.8%) |
Jaw and tongue | 4 (16%) | 6 (14.6%) |
Primary movement | ||
Pure jaw deviation | 6 (24%) | 7 (17%) |
to the left | 4 (16%) | 5 (12.2%) |
Pure jaw opening | 4 (16%) | 4 (9.8%) |
Mixed | 12 (48%) | 17 (41.5%) |
Task specificity | ||
Speech | 18 (72%) | 27 (65.9%) |
Eating/chewing | 15 (60%) | 18 (43.9%) |
At rest | 9 (36%) | 11 (26.8%) |
Musical instrument | 0 (0%) | 4 (9.8%) |
Geste antagoniste | 18 (72%) | 34 (82.9%) |
Object between teeth | 17 (68%) | 31 (75.6%) |
with side specificity | 6 (24%) | 8 (19.5%) |
Holding body part lightly | 10 (40%) | 15 (36.6%) |
with side specificity | 4 (16%) | 8 (19.5%) |
Treatment | ||
THP | 21 (84%) | 29 (70.7%) |
good response | 3 (12%) | 5 (12.2%) |
CLZ | 15 (60%) | 22 (53.7%) |
good response | 2 (8%) - all mild | 4 (9.8%) |
Baclofen | 5 (20%) | 7 (17%) |
good response | 0 (0%) | 0 (0%) |
BoNT | 10 (40%) | 18 (43.9%) |
good response | 3 (12%) - all mild | 5 (12.2%) |
Dental device | 4 (16%) | 11 (26.8%) |
good response | 0 (0%) | 2 (4.9%) |