Table 1 Summary of clinical features including phenomenology of 41 patients with idiopathic LCrD
From: The phenomenology and natural history of idiopathic lower cranial dystonia
Number | Gender | Age at onset | Duration | Areas affected | Primary movement | Task specificity | Precipitating event | Sensory trick | Associated symptoms | Treatment | Oral device; response |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | F | 65 | 2 | Jaw | Jaw opening | Speech, eating, chewing | Dental work | None | None | LD (no), BoNT (no, dysphagia), THP (no), CLZ (no), baclofen (unknown) | N |
2 | F | 71 | 1 | Jaw | L jaw deviation | Eating, chewing, speech | Mandibular fx after a fall | None | None | THP (yes, 2–4 mg/d) | N |
3 | F | 59 | 2 | Jaw | Jaw opening and protrusion | Speech -- > eating, chewing | Maxillary bridge replacement | Plastic between teeth | Pain/clicking in TMJ | THP (no) | N |
4 | F | 53 | 0 | Jaw | Jaw opening | Speech -- > eating | None | Plastic/tongue depressor between teeth > holding jaw | R ear clicking, lower jaw pain | THP (no), BoNT (no), Baclofen (no), TBZ (no, depression), CLZ (no, sedation), DZP (unknown) | N |
5 | F | 46 | 2 | Jaw | Jaw opening | Speech -- > eating | None | Plastic between teeth, holding jaw | None | CLZ (no), THP (unknown), BoNT (yes, mild) | Y; initial response |
6 | M | 61 | 1 | Pharynx, larynx | Pharyngeal/laryngeal dystonia | Speech (worse with ke, ge sounds) | R sinus lift and dental implant | Plastic/tongue depressor between teeth | None | THP (yes, 6–15 mg/d) | N |
7 | F | 73 | 1 | Jaw | R jaw deviation | At rest | None | Plastic between teeth on the L | None | THP (no), CLZ (1 mg/d, unknown) | N |
8 | F | 70 | 1 | Jaw | Jaw opening | Speech | Cheering a football game | Tongue depressor between lip/teeth; holding jaw | None | CLZ (no), LD (no), THP (no), Baclofen (no), BoNT (no) | N |
9 | F | 51 | 2 | Jaw | L jaw deviation | Speech, eating | R crown replacement and repair | Tongue depressor between teeth on the L | Jaw pain | CLZ (unknown) | N |
10 | F | 56 | 2 | Jaw | R jaw deviation | Speech, chewing | None | Plastic between teeth on the right and in front, holding jaw | Mild pain | THP (yes, 6 mg/d) | N |
11 | F | 58 | 1 | Jaw | L jaw deviation | Speech, eating | 3 wks after L subdural hematoma | Plastic between teeth | None | THP (unknown) | N |
12 | F | 50 | 1 | Jaw | L jaw deviation | Speech | None | Holding the L jaw, imagination about holding the jaw, tongue depressor/syringe between teeth | None | THP (unknown) | N |
13 | M | 66 | 7 | Jaw, tongue | Jaw opening and tongue mvmts | None | None | Straw in his mouth, lightly touching lips | Difficulty chewing | CLZ (no), BoNT (no), THP (unknown) | N |
14 | F | 56 | 1 | Jaw, tongue | Jaw opening, tongue protrusion, alternating tongue deviation | Speech -- > eating, chewing on the R side | 3 mo after traumatic facial abrasion | None | Clicking in jaw | LVT (no), TBZ (no), THP (unknown) | Y; no response |
15 | F | 44 | 11 | Jaw, tongue | Jaw closing, tongue retraction | Speech, chewing | Ill-fitting new denture leading to painful jaw and facial swelling | None | None | CLZ (unknown), THP (no), Baclofen (unknown), BoNT (yes, mild) | N |
16 | M | 41 | 3 | Jaw | Jaw protrusion | At rest, speech, eating | Skull fx and extensive L orbital bone damage, R lower molar extraction 1 y later | Tongue depressor or a pen cap between teeth | None | CLZ (yes, mild, 3–4 mg/d), THP (unknown) | Y; no response |
17 | M | 55 | 2 | Tongue | Tongue protrusion with 90° clockwise rotation | At rest, absent during tasks | None | None | None | LD (no), CLZ (yes, mild, 3 mg/d), BoNT (no), THP (unknown) | N |
18 | F | 78 | 4 | Lower face | Multiple mvmts with coexisting ET | Arrhythmic facial movements (talking > at rest); rhythmic facial movements (at rest > talking) | Dental procedure to crown several lower molar teeth | Holding chin with hand | Voice straining (started after the dental procedure), facial movements started 2 years after. | TBZ (no), CLZ (no), THP (unknown) | N |
19 | F | 67 | 7 | Tongue | Tongue protrusion | At rest, chewing/eating | None | None | Mild drooling and sl softening of voice, jaw tremor. Mouth ulcer | TBZ (unknown) | N |
20 | F | 63 | 1 | Jaw | Jaw opening, jaw protrusion | Speech (words with an "r" or "l", at rest | Holding a plastic syringe in the left corner of mouth | Difficulty swallowing liquids | LD (no), LZP (no), Baclofen (unknown) | N | |
21 | M | 45 | 1 | Jaw | Jaw closing, jaw protrusion and L jaw deviation | At rest | None | Tongue depressor between teeth on the right side, touching L lower cheek | Clicking noise at the L jaw, jaw pain (L > R) | CLZ (no), Cyclobenzaprine (yes, mild), Jaw surgery (no), THP (unknown), BoNT (unknown) | Y; no response |
22 | M | 45 | 2 | Jaw, tongue | Jaw opening, tongue retraction | At rest, chewing | ?cocaine abuse | None | None | Clonidine (yes, mild), CBZ (mild), CLZ (unknown), THP (unknown) | N |
23 | M | 51 | 1 | Jaw | L jaw deviation, mild jaw protrusion | At rest | None | Firm pressure on R cheek, holding object between teeth on the L side | None | LD (no), BoNT (unknown) | N |
24 | F | 51 | 15 | Lip, tongue, perioral muscles | Invol. dystonic mvmt of face, lips and tongue; invol. sucking mvmt of mouth | Speech, eating | None | Tongue depressor between teeth, putting hand on face | Dysphagia, 8-lb wt loss, biting cheek-- > mouth ulcers | THP (no), CLZ (no, confusion, memory problem), BoNT (yes, mild) | N |
25 | M | 25 | 25 | Jaw, perioral muscles | Mouth closure with sl jaw opening and protrusion | Speech, praying, chewing | None | Tongue depressor between teeth (L > R), touching jaw/chin lightly | Pain at b/l lower jaw, burning sensation in buccal mucosa, biting buccal mucosa-- > bleeding | CLZ (no, 1.5 mg/d), THP (no, 2 mg/d, dose inadequate) | N |
26 | M | 50 | Tongue | Tongue rolling | Speech | Toothpick in mouth; touch lips with finger | TBZ (no); THP (unknown) | Y; good response | |||
27 | M | 38 | Jaw | L jaw deviation | Speech, chewing | Pipette between left molars; touch left jaw | CLZ, THP (no) | Y; no response | |||
28 | F | 60 | Jaw | Jaw closure | Non-specific, worse with speech | Touch L cheek; plastic tube between teeth | BoNT (unknown) | N | |||
29 | F | 43 | Jaw | Jaw closure and L jaw deviation | Bassoon -- > speech, chewing | Device over molar; plastic appliance between teeth | BoNT (yes) | Prior device; good response | |||
30 | M | 41 | Jaw | Jaw closure | French horn -- > speech, drinking | Bite plate; straw between teeth on L | BoNT, CLZ, baclofen, THP (no to all) | Y; brief response | |||
31 | F | 41 | Jaw | Jaw protrusion and left jaw deviation | Speech | Straw between teeth, L > R | BoNT (unknown) | Y (bite blocks); help when bite | |||
32 | M | 33 | Lip | Lip pulling to the corners of the mouth | Speech | Straw between upper lip-teeth; hold upper lip with 2 fingers | THP (yes, 6–18 mg/d) | N | |||
33 | F | 51 | Platysma, face | Neck pull forward, grimacing | Non-specific | Straw between teeth on either side | CLZ (yes, 0.5 mg/d), BoNT (no) | N | |||
34 | M | 64 | Pharynx, larynx | Pulling | Paradoxical; worse at rest, speech better | Plastic between teeth | CLZ (unknown) | N | |||
35 | F | 68 | Jaw, tongue | Lateral movements of jaw, tongue rolling | Non-specific | Straw/spoon between teeth | THP (no); CLZ (yes, 1 mg/d) | Y; good response | |||
36 | F | 66 | Tongue | Tongue pushing against teeth (protrusion?) | Non-specific | Paper towel over lower front teeth; toothpick | Unknown | Unknown | |||
37 | M | 48 | Perioral muscles | Rotation of perioral muscles | Speech | Straw in his mouth | BoNT (yes) | Y; unknown | |||
38 | M | 44 | Tongue | Tongue rolling | Flute -- > speech | Plastic pipette in cheek | THP (yes, 4–6 mg/d) | N | |||
41 | M | 30 | Embouchure | Lowering, protrusion | Trombone | Touch chin or L side of face | None | N | |||
42 | M | 66 | Jaw, tongue | Jaw opening, tongue movements | Paradoxical; worse at rest, speech better | Straw between teeth | THP, CLZ, BoNT (no to all) | Not yet referred to M.G. | |||
43 | F | 42 | Jaw, lips | Jaw and lip opening | Speech, chewing | Touch R temple | CLZ, THP, BoNT, baclofen (no to all) | Y; brief response |