Face
A. Context
There is no official medical category of ‘face dystonia’ . However many people who have dystonia in the mouth region report oddities of the cheeks, involuntary grimacing, lip licking, tongue protruding, tics and other movements that seem vaguely captured by the general category of face. There is a separate survey for those who have dystonia in the jaw and mouth, one for those with vocal cord dystonia and one for those who have dystonia that affects eyes and eyelids.
The face survey was included to capture those who may have felt an involvement not studied by those other surveys.
B. What seems useful to study further
Facial grimacing, tics and spasms are debilitating partly because they are visible. People who experience rigidity of the cheek muscles or around the mouth and find they naturally hold their mouths wide open. In the mirror they report they look like they are eating something bitter and disgusting. Some fear they appear disapproving of anyone near them.
Patents report an involuntary tightness of the muscles that makes it painful to close the mouth but also painful to keep it open. The default position they describe, to keep the cheeks rigid and the mouth open is not a relaxed position however but also one of extremes, like a yawn or huge body stretch that is bigger than normal, but held there.
Patients report that the urge to keep the mouth in this position is very strong. The pressure to do hold a position that is itself painful is frustrating to patients but seems the same intense pressure to move to a painful position that is experienced by those with cervical dystonia where the push on the neck is irresistible though painful. This irresistible urge may be similar to that reported by those with restless leg syndrome who say they feel compelled to move their legs but when they do so do not feel full satisfaction either.
Some medications for anxiety and depression can cause muscle tightness. Typical and atypical antipychotics can sometimes create the tardif dyskinesia effect – facial grimacing, lip smacking, lip licking, puckering, chewing and sucking, tongue protrusion and tongue rolling.
A 2021 study by Fariba and Estevez says that tardive dystonia is an extrapyramidal syndrome caused by use of dopaminergic receptor antagonists.
1950s – Extrapyramidal syndromes were first noticed when a new class of neuroleptics was developed, drugs thought at first to calm psychomotor agitation. However it was discovered later that the effect of the drugs was sometimes new motor abnormalities.
1957- Schoenecker identified a condition. later called tardive dyskinesia, of bucco oral movements of such patients.
1964 – In psychiatry Faurbye used the term tardive dyskinesia
1973- Keegan and Rajputa observed dystonia tarda in a female patient with neck dystonia.
1982 – A condition was now labelled tardive dystonia.
The term ‘tardive’ indicates that the condition is late arriving, not a first effect of a medication. Patients report that if they have been taking the medication for a while, apparently without harmful effect, once the harmful effect starts to be manifest, the body may already have developed a habituation to the pill. This can mean that though it may be causing negative effects, the patient will now have to wean off the pill gradually or risk additional harmful effects of withdrawal.
Some clinical studies have found that dystonia that develops after such medication can move to other parts of the body and that the effect is not always reversible.
It is unclear how long it takes before it is evident a medication may be having negative effects, with some studies indicating a range between four days and over twenty years.
If medication increases the risk of developing dystonia, the link between type of medication and the genetic, allergy profile or other medical history of a given patient may be very important to study. The prescription of medication that has been shown by clinical studies to present a risk of development of dystonia may merit closer care among all medical practitioners including family doctors and psychiatrists. Research into the links seems very useful.
There are many types of facial involvement with dystonia and a study of possible causes of all of them would be helpful. Not all seem linked to previous medication.
C. How to ask
Source of question –
patient reports, clinical studies
D. Comments from patients
I had facial drop
I was making weird grimaces
I had cheek stretches, extended yawns and facial pulls
E. Question categories
Surveys 6, 28
F. Results
face, mouth, jaw, tongue
6 28 face 10 34 86 3
7 41 jaw,mouth 18 24 81 3
28 5 face,mouth 15 18 82 2
max no. respondents 28-41
total questions 42
likely type of dystonia face, mouth, jaw, possibly others
percent of all respondents doing survey 41 of 508 or 8%
G. Results
(The bracketed item at the end of each question set indicates the survey number and then the question number. eg. 1-3 is survey one, question 3)
1. appearance, distortion , droop
-In the mirror is your face mostly symmetrical?
74.07% Yes
25.93% No (6-13)
-Do the muscles of your lower face pull on their own?
23.08% Yes, often
19.23% Only once in a while
57.69% No (6-16)
-Do you feel your dystonia gives your face a distorted appearance?
18.52% Absolutely and all the time
29.63% Yes, occasionally but not happening all the time
51.85% No, I look normal most of the day ( 6-17)
-appearance
60.00% I have problems making or holding a normal smile
20.00% My face looks like I am eating something sour, frowning or disgusted
20.00% I have involuntary grimacing
40.00% My face looks like normal and my dystonia is not visible (28-1)
(small number of respondents to this question)
2. forehead
-Do you scrunch your forehead more often since dystonia?
31.71% Yes, I do it more often
56.10% No, I do it with the same frequency as usual
12.20% Unsure (5-5)
3. cheeks
-Do you have pain in your cheeks?
17.86% Often
35.71% Sometimes
46.43% No (6-4)
-Do you have an odd feeling of drooping heavy cheeks?
7.41% Yes, often
11.11% Sometimes
81.48% No (6-11)
4. tics, tremor, spasms
-Do you have facial tics?
3.70% Very often
44.44% Occasionally
51.85% No (6-14)
-For a second does it feel better after you have a facial tic?
9.52% Absolutely
90.48% No (6-29)
-Are your facial tics on only one side of the face?
27.27% Yes
72.73% No (6-15)
-Does any part of your face have a tremor?
17.86% Yes
82.14% No (6-18)
-Do you have sudden spasms of the face?
40.74% Yes
59.26% No (6-19)
5. grimaces – presence, progression
-Does your face go into an odd grimace with mouth open very wide?
7.41% Yes, constantly
18.52% Just once in a while
74.07% No (6-8)
-Does your facial grimace cause you intense pain?
23.08% Yes
76.92% No ( 6-9)
-Does your facial grimace look like you tasted bitter lemon?
27.27% Probably
72.73% Probably not (6-10)
-Did your facial grimace stay intense for months and then eventually go away?
4.17% Yes
95.83% No (6-21)
6. side of head
-Do you have pain at the side of your head?
17.86% Often
53.57% Sometimes
28.57% No ( 6-5)
-Do the muscles on one side of your face act oddly?
29.63% Yes, on one side only
18.52% On both sides but unequally
51.85% No (6-12)
7. control of movement – delay, pull, freeze, clench
-Does it take longer than it used to, to get your face muscles to move?
10.71% Yes
89.29% No (6-1)
-Is it hard to move your face muscles to ‘make a face’ for fun?
17.86% Yes
82.14% No (6-3)
-control
0% One side of my face clenches
0% My face sometimes freezes, parks, goes rigid
0% It is very difficult to close my mouth and keep it closed
25.00% When I want to close my mouth there is a delay and it is hard to chew
0% I have this strong pressure to open my mouth very wide
25.00% I get occasional sharp sudden pain in my tongue
75.00% not applicable ( 28-3)
(very few respondents to this question)
8. smiling
-Is it physically hard to crack a normal smile?
25.00% Yes
75.00% No ( 6-2)
9. daily activities
18.92% It is hard for me to smile a normal smile
8.11% It is hard for me to open my mouth to brush my teeth or floss
8.11% It is pleasant for me to keep my mouth open and have a toothbrush
or toothpick in it and seems to reduce the dystonia
37.84% It is hard for me to hold my mouth open at the dentist’s
27.03% It is hard for me to open my mouth wide at the doctor’s for a
throat exam or swab
2.70% It is hard for me to close my moth and I feel socially awkward
35.14% My ability to smile is not affected by dystonia
29.73% My ability to open or close my mouth as needed is not affected
by dystonia (7-16)
10. speaking
-Do facial spasms make it hard for you to speak?
11.54% Yes, often
23.08% Yes, occasionally
65.38% No ( 6-24)
-Does cheek tightness make it hard for you to form words?
11.54% Yes, often
19.23% Yes, occasionally
69.23% No (6-26)
-Does the facial grimace make it hard for you to speak?
7.69% Yes, often
26.92% Yes, occasionally
65.38% No (6-25)
11. sensory tricks, -eg. yawn
-Does any sensory trick reduce muscle tightness in your face?
33.33% Yes
66.67% So far I have not found one ( 6-28)
-Does any sensory trick reduce your facial tics?
27.27% Yes
72.73% No ( 6-27)
-Does it reduce muscle tightness if you practise yawning?
30.43% Yes
69.57% No ( 6-30)
H. Analysis
Problems reported for this type of dystonia seem to be both functional and social.
Functionally the issue does seem to be involuntary movement of muscles though patients anecdotally may use different terms for this. Some patients describe facial pulling, extended yawning, or cheek stretches, which suggests that the involuntary part is not so much a movement as an urge to hold tight an exaggerated position.
48.15% report that the muscles on one or both sides of their face act oddly
42.31% report that the muscles of their lower face pull on their own. often or once in a while
31.71% report they scrunch their forehead more often since diagnosis.
18.52% report that they have drooping of cheeks and an odd feeling of heaviness
The sensation of heaviness or leaden weight is reported anecdotally by patients with several forms of dystonia and may be useful to study. Some with cervical dystonia report changes in a sense of buoyancy. It is not clear if this sensation of droop, heaviness, dead weight is damage in the message back to the brain or if the body part is unable to respond because there is cell damage.
Patients report both an involuntary aspect of these positions and a delay in trying to get out of them.
25.00% report a delay in trying to close the mouth, a delay that impacts ability to chew
17.86% report that it is hard to ‘make a face’ for fun
10.71% report a delay in trying to get their face muscles to move
Problem opening the mouth is more commonly reported than is the problem closing the mouth. It is not clear if in effect these are the same problem of lack of control to move out of a position either to open wider or to close. Those who have dystonia in the mouth and jaw were invited to participate in the survey on mouth and jaw (G) and their results may be useful to add on this topic.
37.84% report it is hard to hold the mouth open at the dentist’s
27.03% report it is hard to open the mouth wide at the doctor’s
2.70% report it is hard to close the mouth
(A separate study of the orienations of dystonia – open, closed, left, right, bent, stretched is made in a later chapter)
problems socially or concern with physical appearance
48.15% report that their face has a distorted appearance occasionally or all the time
25.93% report their face is not mostly symmetrical
There are problems reported with social interactions
18.92% – 25.00% – 60.00% report problems making or holding a smile
20.00% – 27.27% report that the grimace probably looks like they tasted sour or bitter, are frowning or disgusted
25.93% report they had odd grimaces constantly or once in a while
spasms, tremor,tics
48.14% report they have facial tics occasionally or very often
40.74% report they have sudden spasms
17.86% report they have a tremor
In anecdotal reports the pressure to make a grimace is intense and feels nearly primal even though it causes pain.
pain
90.48% report that it does not feel better briefly after they have a facial tic
71.43% report they have pain at the side of their head often or sometimes
53.57% report they have pain in their cheeks often or sometimes
23.08% report that their facial grimace causes them intense pain
9.52% report that it feels better briefly after they have a facial tic
In these surveys 4.17% reported that they had facial grimaces for months but that they eventually went away. The question however does not capture if there was also a change in medication over that interval and that would be useful to ask.
The phenomenon of facial grimacing is one of the stereotypes in the general public, along with jerky movements, mouth gaping open, jerky facial positions, that goes with being ‘handicapped’ and in some cases mocked. The cruel stereotypes are assigned to many people with a wide range of actual physical or mental challenges but in the general public are often globbed together as one negative stereotype. The survey on moods attempts to capture some of the psychological effects of dystonia.
Lived Experience With Dystonia 