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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
  1. Data on our new patients (number 1–25) is presented with previously published patients (number 26–43; shown in italics).
  2. 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.
  3. 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).