Table 1.
Summary of clinical features including phenomenology of 41 patients with idiopathic LCrD
| 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 |
Data on our new patients (number 1–25) is presented with previously published patients (number 26–43; shown in italics).
Legend: Patient number, gender, age at onset (years), duration of the disease (years), areas that are affected (arrow shows the pattern of spreading from one region to another), primary movement, task specificity, precipitating events, sensory trick, associated symptoms, treatment employed, and trial of oral devices and response are listed in columns. Patients 1 to 25 were new and patients 26 to 43 (shown in italics) were previously published [4]. Patients 39, 40 and 44 were excluded due to co-existing blepharospasm. There is no information on precipitating event in patients 26 to 43.
M, male; F, female; L, left; R, right; mvmt, movement; invol., involuntary; >, had greater effect than; fx, fracture; wk, week; mo, month; y, year; TMJ, temporomandibular joint; sl, slight; wt, weight; b/l, bilateral; LD, levodopa; BoNT, botulinum toxin injections; THP, trihexyphenidyl; CLZ, clonazepam; mg/d, milligram per day; DZP, diazepam; LVT, levetiracetam; TBZ, tetrabenazine; N, no; Y, yes. In treatment column, response is shown in the brackets (yes, no, unknown): “yes” (in patients 1 to 25), a good response; “yes, mild”, mild response, effective dose indicated in the brackets. In the oral device column, response is shown as Y and N (yes and no).