Neck
A. Context
Neck dystonia is one of the most common types. It can present in various ways, and often the patient experiences several of these pressures or strong position forces at the same time
-head tilts left or tilts right, ear lying closer to shoulder
-head turns left or turns right – at 90 degree angle
-head tilts down chin to chest or up to look at ceiling
-chin just out or chin is pulled way in toward chest
The experience of this head tilt is usually very painful. Patients report that the pressure is intense and relentless some comparing it to G forces. They often report that the pain is not just when trying to right the head to a normal position but also in the dystonic position. This means that patients are in near constant pain, and making choices every few minutes to adjust head position to find one that is less painful.
The angle of the neck can create significant practical problems. It is difficult to see straight ahead, so it is difficult to walk in a straight line, or to navigate walking along a sidewalk without risking bumping into poles or people Patients with no technical problem in their legs may find that they benefit from using a cane or walking stick just to correct a balance uncertainty. If the neck tilts down it is very hard to see above one’s head, to put objects on higher shelves, to see airplanes or to go up stairs. If the head tilts up it is hard to see what is at one’s feet and easy to trip. It is hard to put things on lower shelves or to go down stairs. If the head pulls in to the chest it is hard to swallow food.
The posture oddity is visible and creates for the patient social discomfort. Patients report low self esteem when looking in the mirror and sometimes awkwardness being seen in public. Paintings of people with odd dystonic postures have been found in history for centuries. However it is still a very poorly understood condition.
B. History
356 – 323 BC Alexander the Great -some statues show an abnormal neck position, bent slightly to the left. Sculptor Lysippus created a sculpture that Plutarch centuries later felt that was accurate about Alexander’s neck
100 – 700 AD some ceramic sculptures from the Moche civilization of Peru suggests some physical conditions that look like dystonia
175 AD Celsus wrote of rigo cervicis. he described a condition where the head was drawn down to the shoulder blades and t he chin to the chest
1100 -William of Norwich writes of an 8 year old with a twisted neck and with her left cheek touching her shoulder
1232 – El Burgo de Osma cathedral in Spain has medieval drawings with dystonic postures
1320- 1282 Nicole Oresme writes of twisted necks noted that necks of some people were thrust or twisted backwards
1494-1553- Francois Rabelais in his book “Pantagruel” wrote a satirical description of a condition of ‘wry neck’ with the head bowing of religious hypocrites . He called it ‘tortycolly” It is speculated that his term was a satirical reference to head bowing of some religious hypocrites however.
1640 – Poet Paul Scarron wrote of his own condition of head tilted on his ear and about being among the stiff -necked. He called the condition torticollis. However he said that among those with the condition, he “passed for one of the prettiest.
1737 – The twisted neck condition was studied by German doctor Georg Friedrich von Jager who called it by its Latin name, caput obstipum.
1850- Charles Dickens in David Copperfield has one character who had his head to one side “as if it was a little too heavy for him”
1888- Charcot identified a case of what seems to have been cervical dystonia in a stockbroker.
1893- Sir William Gowers describes dystonic postures in hands and feet and calls them tetanoid chorea. He draws pictures of a man with spasmodic torticollis. He describes conditions of contractions of the neck and jaw.
1896 – Thompson publishes photos of what he calls ‘wry neck”
1900- Painter Amedeo Modigliani represents some figures in a posture that looks like dystonia. His common law wife, Jeanne Hebuterne appears in some photos with her head angulated and one of Modigliani’s portraits even has a lady touching two fingers to her face, a common trick of geste antagoniste for torticollis. Many of Modigliani’s paintings show elongation and curvature and torsion of the upper body that resembles dystonia.
1910 – The twisted neck condition was called ‘wry neck’ in English or ‘Nakenmuskelkrampfe’ in German.
1942- Raymond Chandler in ‘The High Window’ describes a character whose head is drawn around to the left angle about 45 degrees and who has jaw contractions
2016 – Dr. Cynthia Comelia studies neck dystonia and quantifies its severity. Scales are developed to measure twist and motion while other scales look at nonmotor effects such as quality of life.
2017 Dr. Scott Norris studies if neck dystonia spreads to other body parts
C. What seems useful to study further
1. the precise angle of the head tilt
There are several variations of head tilt, not just left/right/ or up/ down but also pulling the chin in to the chest or jutting the chin out. Many patients report pressure from several of these at the same time. A more precise way to measure the variations would be useful.
Patients sometimes report a tilt is worse or better today but usually have no way to calibrate that. This inability to put numbers to it denies them feedback about whether a treatment, exercise or other coping strategy is working. Sometimes patients take pictures of their condition or videos to compare it from day to day or week to week. Apps may be useful to help measure the tilt.
2. the nature of the pressure
Many patients describe the intense pressure to tilt the head akin to being pinned against a wall It might be useful to measure the force or pressure – how hard a chin can push down on a sensory plate on a counter or what force the head naturally uses to push against an object held at the left. Having such an indicator would be useful to measure what pressure the body has to fight against to try to achieve a normal head position. Many patients report frustration at not being believed when they report the pressure they are under.
3. whether the condition changes
Patients report trying many strategies to improve their neck position or reduce pain. Many lean on walls or against chair backs, adjust pillows when they lie down, try neck cushions or neck braces. Often patients will use a hand to physically push the head into a more upright position. Many try gentle stretches, massages, electrical stimulation. When improvement is found it is not clear if it applies to all aspects of the dystonia. A person may have a neck that turns left, tilts down, and pulls in to the chest, all at the same time. Treatment for one tilt may not help with others.
4. the sensory tricks
The classic sensory trick is for neck dystonia.It is a touch of the cheek with one hand, that reduces the neck dystonia on the other side. Patients report surprise at the trick and several features of it.
One is that the neck stiffness disappears immediately and the effect is so fast and direct it is as if the action pushes a button. One is that the two body parts involved are not near each other or even logically connected One is that the effect does not last. It ends the second the cheek is no longer touched.
For some people, the effect only happens if one hand is used not the other, if some fingers are used not others. For some people a gloved hand can still create the effect. For some, having another person touch the chin may create the effect or having an object like a spoon touch the cheek. Others find that only their own hand creates the effect.
Some report that the effect can be produced when they just move their hand toward the cheek even before they touch it and some report that the effect can happen if they even just think of the hand touching the chin. Others however do not have that same experience.
Though the two body parts are not near each other in the body core, it is possible they are very near in the brain areas controlling them. Brain mapping has been done of some of the tricks, Do such regions overlap in some people? Do they change in size over time or with overuse or with injury? These questions merit continued study,.
It is not clear what causes neck dystonia. Researchers have noted some links to previous surgeries or traumatic injury but not all patients have had those. It may be interesting to see if as a child the patient tended to tilt their head slightly and if so, was it in that direction? A photo study might be useful to reveal if there was always a weakness in that muscle, or possibly a tendency of that area of the brain to fire slightly oddly.
Genetic studies of dystonia have made great breakthroughs but seem mainly related to generalized early onset dystonia. It may be interesting to also see if patients have had more subtle medical pasts such as weaker muscles in the neck on one side. A study of family history of other movement disorders or even auto-immune diseases in the larger family may be useful.
The nature of all of the sensory tricks holds potential for insights into brain -muscle messaging. The phenomenon of not quite touching the area of the trick and still getting the effectsuggests that the brain has an anticipation mode. It suggests that the brain plans a motion, gets ready for it, anticipates the feedback it will have to receive to confirm when the motion is complete. In general people find that when they see someone they wait at an airport for a loved one to arrive and then, see them finally, they move towards them already feeling the surge of joy they will feel when they hug. The anticipation is so strong that it starts to flood them before the event, anticipating it.
People in their memory can experience some of the same feelings they had on an earlier occasion when they hear a piece of music or see a familiar place. If the brain can conjure up emotions and use brain pathways about situations that are not currently happening this suggests the brain is not only sending messages to muscles about this moment in real time, but also about past moments and future moments. The flaw in the dystonic message may be very very specific. For times when the flaw is absent, why that brain message still works well may be nearly as useful to study as when the message is flawed.
D. Comments from patient experience
first signs
At the start it was just a stiff neck that I thought was because of bad posture at work
I got a sudden pain on the right side of my head
I was having trouble holding the weight of my head
My neck was twisted and seemed swollen
My wife noticed I was talking from the side of my mouth
tilt, lean one direction, default position
My neck locked in a downward position
My neck pulls down all the way to my chest
My neck tilted left as if it had no muscles at all there
twist – several directions
My head stooped to the right and then up
My head had 3 tilts- to the right, towards the shoulder and pulling hard and back
In my case neck muscles on both sides are affected so my head shakes but does not twist
bobbing, twitches, spasms
My head was twitching to one side
involuntary movement
My head starts to move on its own and I have to work to hold it back
When I try to look straight ahead my head goes to the right
failure to move
When I look to the right, my neck stays in position
progression
I went from my neck twisting right to it twisting left.
effect on daily life
My neck feels like it has a daily gym workout just to hold my head up
It even hurts to smile
I am not able to lift my head slowly or I go into tremors
When I snuggle up with my daughter my head knocks against hers
I look like the Leaning Tower of Pisa
I am becoming the crooked man who walked the crooked mile
People thought I must have just slept funny the night before
I can’t hold my head to focus on a small field of vision so I no longer go to live theater
sensory tricks
I was often using my hand to hold my head up
treatment
With injections of botulilnum toxin my neck is where it is supposed to be
E. How to ask
Source of question ideas
– patient reports, clinical studies, personal experience
F. Question categories
symptoms
daily activities
sens tricks
G. Questions asked -survey number, question number
surveys 9, 30
H. Results
neck
9 58 neck 22 32 75 6
30 6 neck 11 15 82 2
max no. respondents 58
total questions 47
likely type of dystonia cervical
percent of all respondents doing survey 58 or 508 or 11.4%
challenges to do this survey- ability to look at computer screen
I. 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
-dystonia body position
5.45% The position is a normal looking one, straight ahead, upright
40.00% The position is slightly tilted, bent, but not much
16.36% The position is at the extreme end of a range, far left or right, way down
way up, chin presed in or jutting way out
18.18% The various pressures I feel compromise my end up position so it is
extreme for some range and less extreme for others
16.36% unsure (9-5)
-As you move, do some muscles seem to pop out more prominently?
44.00% Yes often
12.00% Yes once in a while
18.00% No, no muscles seem more prominent
20.00% unsure ( 9-20)
-appearance
60.00% My neck muscles bulge on one side
20.00% My neck muscles bulge differently from day to day
100.00% My dystonia is visible to others because of my neck tilt
0% My dystonia is not visible to others ( 30-1)
(low number of respondents to this question)
2. range of motion
-These questions ask about your ability to look straight ahead, up and down as you wish.
7.69% People used to tell me I look at things mostly with one eye
25.00% I sometimes have to tilt my head oddly to see straight ahead
28.85 % It is a huge effort for me to look straight ahead
51.92% To look to one side is easy and to the other very difficult
44.23% It is difficult for me to look up at an airplane
13.46% It is difficult for me to look down at the floor
19.23% I can look straight ahead, left, right, up or down without any problems
1.92% unsure (9-7)
-gaze
16.67% My head tilts down so it is hard for me to look up at skyscrapers,
airplanes or clouds
16.67% My head tilts down so it is hard for me to look at upper shelves at home
or in stores
0% My head tilts up so it is hard for me to look at objects on the floor
0% My head tilts up; so it is hard for me to look at items on lower shelves
at home or in stores
33.33% I have trouble looking at objects or people on my left
66.67% I have trouble looking at objects or people on my right
16.67% My gaze and range of easy vision is not affected by dystonia ( 30-6)
-range of motion
66.67% I can easily turn my head 90 degrees to the left
16.67% I can easily turn my head 90 degrees to the right
50.00% I can easily look up at the ceiling
50.00% I can easily look down at the floor
50.00% I can easily tilt my head to lean on the left shoulder
16.67% I can easily tilt my head to lean on the right shoulder
50.00% I can easily look down at my left elbow
33.33% I can easily look down at my right elbow
33.33% When I look 90 degrees to the right, my head still tilts left
33.33% When I look 90 degrees to the left, my head still tilts right
83.33% When I try to look up, my head still tilts to one side
83.33% When I try to look down, my head still tilts to one side
16.67% My chin pulls in while I am trying to do other head motions
0% My chin juts out while I am trying to do other head motions
(30-2)
3. pain and pressure
-Pain, force and pressure on the neck
9.09% The pain is mild
32.73% The pain is intense
47.27% The pain is more of an ache
3.64% The pain is stabbing and in jabs
63.64% The pain is from extreme muscle tightness, contracting
38.18% There is pain on the other side too, from stretching
3.64% There is no pain (9-3)
-pain and other symptoms
50.00% I have severe neck pain
33.33% I have mild neck pain
16.67% I have no neck pain
33.33% The pain is constant
50.00% The pain is occasional
33.33% My head feels heavy like a bowling ball
50.00% My head feels wobbly, unstable or weak
16.67% My chin pushes in so much I worry if I can breathe
16.67% My chin pushes in so much I worry if I can swallow
0% The back of my head squeezes so tight I sometimes feel dizzy (30-3)
-These questions ask about the frequency of the force or pressure on your neck
37.04% The pressure is nonstop
16.67% The pressure is occasional, sporadic
31.48% I have attacks or storms of intense tightness and then days without
as much tightness
40.74% Every day is about the same for the pressure I feel on my neck
61.11% The pressure is less when I lie down or sleep
48.15% The pressure is less when I rest my head against a soft cushion
11.11% Nothing I do so far has changed the pressure or force I feel on my neck
(9 -4)
-These questions ask about the pressure you feel involuntarily to move our neck
8.62% My head drifts to one side
10.34% My head on its own goes back and forth as if to shake head ‘no’
6.90% My head tends to turn and gaze to the left
12.07% My head tens to turn and gaze to the right
17.24% My head tends to tilt left so the head is lying closer to the left shoulder
15.52% My head tends to tilt right so the head is lying closer to the right shoulder
0% My head tends to pull my chin in towards my chest
0% My head tends to jut my chin out straight ahead
0% My head tends to tilt forward
5.17% My head tends to tilt backward
17.24% I feel a force or pressure to move my head in at least two of the above
ways at the same time
(9-1)
-These questions ask about the intensity of the force or pressure you feel
8.93% The force I experience is gentle
32.14% The force I experience is intense, as if a vice was on me
55.36% The force I experience changes over the course of a day
39.29% The force I experience is constant, relentless (9-2)
4.. tremor, spasm, clicks
-As you try to move your head against the dystonia do you sometimes hear a tiny click?
44.00% I often hear clicks, cracks, popping sounds in my neck
34.00% I sometimes hear clicks, cracks, popping sounds in my neck
12.00% There are mores such sounds on the dystonic side than on the
nondystonic side
12.00% The clicks cause me pain
24.00% The clicks cause me no pain
10.00% I never hear any clicks when I move my neck
2.00% unsure
8.00% not applicable ( 9- 19)
-tremor, spasms and involuntary jerky movements of the head
37.74% I have occasional head tremor or gentle jiggling
28.30% I have constant head tremor or gentle jiggling
20.75% I have no head tremor
30.19% I have spasms when at rest
20.75% I have spasms when I start to move my head
24.53% I have spasms when I try to hold a new head position
5.66% I have spasms of relief when I stop trying to hold a head position
41.51% The spasms cause me pain
16.98% The spasms cause me no pain
3.77% unsure
5.66% not applicable ( 9-9)
-These questions ask about tremors and if they progressed
40.00% I have head tremors and they have not changed
0% Tremors started in my head and later also went to the hands
0% Tremors started in the head and left there and went to the hands
20.00% Tremors started elsewhere and then also happened in the head
40.00% I do not have tremors ( 30-10)
(low number of respondents to this question)
5. resistance to movement, delay
-These questions ask about your ability to move out of the dystonia position
18.87% It really hurts to be in the position but it hurts even more to try
to move out of it
16.98% It hurts at first to move out of it and then is easier again when I
move the full range to the opposite extreme – eg. hard to lift the head
to level but then easier to go from level to look way up
11.32% My head has locked in some positions and I have not been able to
easily move it out of that position at all, for several days
22.64% It is hard to hold any position except the dystonic position
28.30% When I try to hold a nondystonic position, I start to have spasms
5.66% When I finally stop trying to hold a nondystonic position, I go into
gentle spasms that seem like relief
7.55% I can move my head without pain or pressure in any position
0% I can move my head in any position without spasms
22.64% My head motions are completely not within my control
32.08% My head motions are partly in my control and partly not
50.94% Any position I am in seems a mix of what dystonia is pressing me
to do and what my body is trying to do to resist it ( 9-6)
-resistance to movement
34.62% When I try to move my head I feel a leaden cement feeling sometimes
in my jaw
19.23% When I try to move my head there is a delay before my head moves
17.31% When I try to move my head there is no resistance
15.38% unsure
19.23% not applicable ( 9- 8)
6. progression of symptoms
-progression of the dystonia
40.38% The dystonia started in my neck and has only been there
9.62% The dystonia started in my face and went later to my neck
13.46% The dystonia started in my hand or arm and went later to my neck
15.38% The dystonia started with occasional tremor that later got more frequent
11.54% The first symptom of neck dystonia was frequent headaches
0% My dystonia stopped being on one side of my neck but went to the other
15.38% My dystonia went from one side of my neck to also be on the other side
25.00% My dystonia went from my neck down to other body parts like my
throat, arms, legs
3.85% My dystonia went up from my neck to other parts of my body like my face
and eyes ( 9-10)
-progression of symptoms
83.33% My dystonia started on one side of the neck and stayed there
16.67% My dystonia started on one side and later also included the other side
0% My dystonia started on one side and then left there and moved to the
other side
(low number of respondents for this question)
7. diagnosis
-Were you checked to see (if) your neck had a bone structure problem such as
in the discs?
59.18% Yes
36.73% No
2.04% unsure ( 9-23)
-Were you checked to see if you just have poor vision and your head tilt is to
compensate?
42.86% Yes
53.06% No
4.08% unsure ( 9- 24)
-Were you checked to see if there was any traumatic injury such as spine fracture?
44.90% Yes
44.90% No
6.12% not sure ( 9 -25)
-Were you checked to see if there was ear damage and you tilt your head to compensate?
24.49% Yes
69.39% No
4.08% not sure ( 9 – 26)
8. daily activities
-daily activities
11.76% I have trouble bathing or showering because of neck dystonia
33.33% I have trouble shampooing, drying or brushing my hair
35.29% I have some challenges eating because of neck dystonia
39.22% I have trouble carrying groceries because of neck dystonia
41.18% I have trouble seeing where I am walking because of
neck dystonia
13.73% I have trouble dressing because of neck dystonia
33.33% I am still able to do all of the above activitues
despite neck dystonia (9-13)
-reach and handling objects
66.67% It is hard to hold a phone to my ear and easier to put the phone
on speaker in front of me
16.67% It is hard to look down to see an object that fell
33.33% It is hard to reach up to objects above me
33.33% It is hard to look down to wash or manicure my hands or manipulate
objects
66.67% It is hard to find a comfortable position to hold a book and read it
16.67% It is hard to look down at food on the table in front of me
50.00% It is hard to reach up and do my hair
50.00% It is hard to look at my mirror reflection to apply make-up or shave
(30-8)
9 walk, bike, car
-walking
16.67% When walking it feels like there is a ton of bricks on my head
66.67% When walking it feels like there is a strong force pushing my
head sideways
16.67% When walking it feels like there is a string pulling my head up
33.33% I sometimes bang my head accidentally going through doorways
16.67% It is easier to walk if I focus on a stationary object
0% When I walk it feels like if I lifted my head I might fall backwards
50.00% It is hard for me to look up so it is hard to walk up stairs or up hill
50.00% It is hard for me to look down so it is hard for me to walk down stairs or
down hill
16.67% Dystonia has not affected my walking ( 30-4)
-biking and car use
66.67% It is hard for me to look straight ahead when I bike
50.00% It is hard for me to look to the left or right when I bike
50.00% Riding a bike jostles and bounces my neck
16.67% An adult tricycle is easier for me to use than a bicycle
66.67% Going over a bumpy or snowy road in a car is very hard on my neck
0% I have no problems biking with dystonia
16.67% I have no problems being in a car with dystonia ( 30-5)
-comfort in a moving vehicle
20.41% I have trouble biking because it is hard to look ahead
22.45% I can still drive but use a cushion sometimes to support my head
34.69% I can still drive but only go short distances
36.73% I am sensitive to the suspension of a car, short wheel base or tire
conditions and if those do not cushion bumps, the bumps in the road
jolt my neck painfully
6.12% I am comfortable in a vehicle with cushioned suspension and wide
wheel base like a modern bus or subway train
14.29% My neck is not jolted painfully when I am in an airplane
12.24% Riding in a car, bus, subway or airplane is not affected by my dystonia
10.20% Riding a bike or motorcycle is not affected by my dystonia
12.24% not sure
8.16% not applicable ( 9-12)
10. sleeping
-sleeping position and comfort
70.00% I am very particular about pillow position because of dystonia
22.00% I like to have a pillow beside my head
36.00% I like to have a pillow behind my head
6.00% I sometimes have a soft cushion in front of my neck
30.00% I go into spasms in some sleeping positions
38.00% To keep from pain or spasms I change sleeping position every
few minutes till I fall asleep
58.00% I have found at least one sleeping position that is eventually comfortable
10.00% Over time and with exercise I have found other sleeping positions
that are also comfortable ( 9-18)
11. coping
-These questions ask how you adjust your life to allow for the dystonia
60.38% When seated I try to find a place where I can lean my head
against a wall or cushion or room divider
52.83% When at a public lecture or film I choose a seat on purpose
so my dystonic neck tends to look that direction anyway
60.38% When possible to read I lie down and rest my head against cushions
49.06% I prefer to sit in locations where I never have to look around behind me
7.55% If I use a microphone I prefer one that is portable so I don’t have to
just face it
45.28% I put my phone on speaker rather than holding it to my neck
47.17% I have adjusted my computer arrangement so I can look at the
screen more directly ( 9- 11)
-These questions ask about ways to cope with dystonia
40.00% When driving I use my mirrors a lot to assist with vision
0% When driving I put the passenger seat way back so I can see more easily
20.00% I sometimes use a soft cushion at the back of my neck
20.00% I sometimes use a soft cushion at the front of my neck
20.00% I sometimes use a scarf for neck support
0% I sometimes use a stiff neck brace
40.00% not applicable ( 30-11)
(low number of respondents to this question)
-clothing and other adjustments
0% My dystonia is less if I wear a high collar
0% My dystonia is less if I pull a coat up high around my neck
0% A neck brace gets my head in a good position
0% A neck brace reduces pain
33.33% A neck brace makes the pain worse
16.67% A neck brace seems to get my body used to the more standard
head position
33.33% A neck brace seems to risk not training the muscles to be
strong on their own
0% I use a neck brace only for driving long distances
50.00% I have not made clothing or cushion adjustments due to dystonia ( 30-13)
12. exercise
-specific exercises you do for neck dystonia
28.00% The dystonia is less if I push the palm of my hand against the side
of my head
18.00% The dystonia is less if I push the side of my head against the palm
of my hand
10.00% The dystonia is less if I push my forehead against the palm
of my hand
16.00% The dystonia is less if I push the palm of my hand against
my forehead
40.00% The above motions do not change my dystonia
12.00% not sure
8.00% not applicable ( 9-21)
-When you stretch a dystonic muscle does one pressure get less but another stay strong or get stronger? eg. if you stretch your neck to not tilt left, it still pushes your chin in hard
35.42% Yes when one pressure is less, the other still seems strong
6.25% No, when one pressure is less, the other also seems less
52.08% not sure
6.25% not applicable (9 -22)
13. social effects
-social interaction
66.67% It is hard to look at and see someone walking towards me
66.67% I prefer to have people sit or walk on the side I naturally tilt to
33.33% With my head tilted I think I must look always puzzled
16.67% My eyes seem to take a second to catch up when I move my head
16.67% not applicable ( 30-7)
14. sensory tricks
-These questions ask about any actions you can do that suddenly reduce tremors.
34.00% The tremors are less when I touch my chin
30.00% The tremors are less when I touch my cheek
10.00% The tremors are less when I touch my temple
6.00% The tremors are less when I touch the top of my head
14.00% The tremors aer less when I touch the back of my head
12.00% The tremors are less when I touch the base of my neck
10.00% The tremors are less when I touch between my eyes
10.00% The tremors are less when I pinch my neck
4.00% The tremors are less when I touch another part of my body not
mentionned above
24.00% I have found no part of the body to touch that reduces the tremors
4.00% not sure
24.00% not applicable ( 9-15)
-These questions ask about any sensory tricks you can do to touch a part of your body and reduce the dystonia muscle tension
32.65% The muscle tightness is less when I touch my cheek
36.73% The muscle tightness is less when I touch my chin
14.29% The muscle tightness is less when I touch my temple
10.20% The muscle tightness is less when I touch the top of my head
30.61% The muscle tightness is less when I touch the back of my head
26.53% The muscle tightness if less when I touch the base of my neck
4.08% The muscle tightness is less when I touch between my eyes
14.29% The muscle tightness is less when I pinch my neck
4.08%The muscle tightness is less when I touch a body part not menionned above
38.78% There is no body part I have found to touch that reduces the muscle
tightness ( 9-14)
-sensory tricks
8.00% My dystonia is less if I bend at the waist
6.00% My dystonia is less if I use the other hand for the sensory trick
4.00% My sensory trick also works if I just move my hand towards
touching the spot and do not actually touch it
0% My sensory trick works if I just think about touching that spot
6.00% My sensory trick works if someone else touches that spot for me
30.00% My sensory trick still works regardless of if I use one finger
or two or a different finger to touch that spot
16.00% My sensory trick still works even if I use my knuckle not my finger tip
to touch that spot
28.00% unsure
28.00% not applicable ( 9- 16)
-These questions ask about ways other body parts seem to try to help you do actions despite neck dystonia
4.00% When I try to lift my head, my eyes scrunch closed
12.00% When I try to lift my head my mouth tends to open
2.00% When I try to lift my head my nostrils seem to flare
22.00% When I walk my pain is less if I scrunch my shoulders
12.00% When I walk my pain is less if I alternately shrug one shoulder
then the other
6.00% Sticking my chin out is easier if I scrunch my shoulders
32.00% I can raise my head or walk or adjust my chin without doing those
assisting movements above
18.00% unsure
12.00% not applicable (9-17)
-These questions ask about forced tricks you use to deal with the dystonia
16.67% I sometimes put my hand under my chin to forcibly lift my head
66.67% I sometimes put my hand on my cheek to forcibly turn my head
83.33% I sometimes put my hand around my head to forcibly push my head
away from a tilt
33.33% I sometimes use my hand on my forehead to push my head into position
(30-12)
-These questions ask about other tricks you have found that affect the dystonia
60.00% The dystonia is less when I yawn
80.00% The dystonia is less when I cough
20.00% The dystonia is less if I hum
40.00% The dystonia is less if I close my eyes
0% The dystonia is less if I open my eyes wide
20.00% The dystonia is less if I just imagine having a normal head position
0% The dsytonia is less if I look in the mirror
60.00% The dystonia is not affected by looking in the mirror ( 30-15)
(low number of respondents to this question)
-These questions ask about situations you have found that seem to reduce the dystonia
33.33% The dystonia is less if I touch the back of my neck
33.33% The dystonia is less if I touch the base of my jaw
0% The dystonia is less if I touch the center of my ear
16.67% The dystonia is less if I touch my cheek in front of the ear
33.33% The dystonia is less if I touch my chin
50.00% The dystonia is less if I touch my nose
33.33% none of the above ( 30-14)
15. treatment
-surgery or implants
10.42% The doctor explained to me surgery or implant options
2.08% I have had surgery for my neck dystonia
4.17% I have had deep brain stimulation implants to treat my neck dystonia
77.08% I have not had surgery or deep brain stimulation for the neck dystonia
10.42% not applicable ( 9-31)
-Have you been prescribed muscle relaxants for dystonia?
32.65% Yes and the helped
8.16% Yes and they had no effect
16.33% Yes and they did not help
38.78% Yes and there were side effects I did not like
20.41% No, I have not been prescribed them
2.04% not sure (9-29)
-Have you been given injections of botulinum toxin?
10.20% Yes I have had at least one injection
16.33% Yes I have had several injections
59.18% Yes, I have gotten regular injections for some time
14.29% No, I have never had botulinum toxin injections (9-28)
-Have you for pain been given injections of cortisone?
26.53% Yes
73.47% No ( 9- 27)
-Have you been prescribed anticholinergics or anticonvulsants for your dystonia?
10.20% Yes and they helped
6.12% Yes and they had no effect
16.33% Yes and they had a negative effect or side effect
44.90% No, I have never been prescribed them
18.37% unsure
4.08% not applicable ( 9 -30)
16. possible genetic or other links
46.81% I am of northern European ancestry
40.43% I have blue eyes
44.68% There are people in my family who have tremor of the head or hands
55.32% There are people in my family who have been treated for depression
42.55% I like to put things in order and count things and be organized
27.66% I like to be thorough and check to see if appliances and lights are
turned off
0% not sure
2.13% I prefer not to answer
6.38% not relevant ( 9 -32)
J. Analysis
Cervical dystonia is the most common type statistically according to research studies. However it has several different presentations- whether the tilt is left or right and at what angle, whether it is up towards the ceiling or down to the ground and whether the chin pulls in or juts out. It may be interesting to study why there are certain presentations for some patients. A separate examination of orientations of dystonia – left, right, up, down, open, closed is made in a later chapter.
It may also be interesting to look at if there was an early preference or weakness for a side, if as a child this person tended to tilt the head that direction or if they as a child tended to look at objects not with direct gaze. This survey found that 7.69% reported that people used to tell them they looked at things mostly with one eye.
Cervical dystonia is immediately visible to strangers. Patients report that has some social implications.
100% report that their dystonia is visible to others because of neck tilt.
56.00% report that some neck muscles pop out more prominently often or once in a while
The neck tilt significantly affects daily function, including seeing where you walking, looking up or down, seeing what is on either side. However it is the type of dystonia that determines what functions are difficult.
-those whose tilt up, and have trouble looking down
0%, 13.46%, 16.67%, 50.00 % reported this problem
-those who tilt down, and have trouble looking up
16.67%, 44.34% and 50.00% reported this problem
-those whose head goes left, who have trouble looking right were more numerous than those whose head goes right
17.24%-66.67% reported this problem
50.00% reported they easily look at their left elbow
17,24%-50.00% report their head is close to the left shoulder
17.24%-33.33% report that their head tilts left
-those whose head goes right, who have trouble looking left
12.07%- 33.33% reported this problem
15.52%- 33.33% report their head tilts right
15.52% -16.67% report the head is near the right shoulder
The classical picture of dystonia in paintings is cervical dystonia with a left or right neck tilt. The frequency of the tilt to go way back or way forward seems lower, in these surveys also.
The tilt of the chin to pull way in or jut out seems less common
-those whose chin goes way in, who have trouble holding it out from the chest
16.67% report this problem and their concerns about being able to breathe
or swallow because of the pressure caused
-the survey had no respondents whose chin juts out.. However those whose head tilts way back may have the experience of chin in effect jutting out also.
Terminology of the various tilts is imprecise for patients though doctors have general categories for it.
-torticollis- is horizontal chin- to shoulder turning – and involves ipsilateral splenius and contralateral sternocleidomastoid muscles
-laterocollis – is ear to shoulder tilt and involves ipsilateral sternocleidomastoid, ipsilateral splenius, ipsilateral scalene complex, ipsilater levator scapulae and ipsilateral posterior paravertebral muscles
-anterocollis – is head tilting forward- chin to chest- position It involves the bilateral sternocleidomastoid, bilateral scalene complex, bilateral submental complex muscles
-retrocollis – is head tilting back and involves the bilateral splenius, bilateral upper trapezius, bilateral deep posterior paravertebrals ‘-chin in air- position..
However many patients report that they have several of these at once.
The surveys found that
17.24% report they feel a force or pressure to move their head in at least two of these ways at the same time
18.18% report that the various pressures give them an end position that is a combination
Patients report that the neck position observed may not clearly show just dystonic pressure because they are they are constantly also trying to resist the pressure and with some partial success.
50.94% report that any position they are in seems a mix of what dystonia is pressing them to do and what their body is trying to do to resist it.
22.64% report that their head motions are not within their control but 32.08% report that their head motions are partly within their control and partly not.
Some patients report this like watching a planet rotating the sun which is simultaneously under the pressure of gravity to fall, and the physical law to continue in a straight line. The result is a curving around the sun. Patients report they are dealing with an intense pressure to be in one position and a strong internal effort to escape that pressure, simultaneously.
Any new position attained also sends a message back to the brain, and the brain responds again. Studies of where error happens, be it from brain to muscle (motor) or muscle to brain (sensory) therefore may be complicated by the fact that a 3rd feedback is also likely at work.
Terms like angle, tilt, lean, turn, may not be understood the same way by all patients and even terms like ‘flex’ are not always understood. The fact that the head can adopt so many angles in 3 dimensions makes its positioning more like a compass with possibilities of north north west etc. Patients anecdotally express a sense of slight change over the course of a day in their tilt but have no way to measure this or describe this objectively
direction
The direction of the neck tilt to left or right was asked but there was no question to link to handedness. The proportion of people who favor the left hand in the general population is 9.2% Right- handedness is much more common.
However the studies here found that tendency to tilt left is 17.24- 66.67%, as if favoring the left is more common with dystonia.
Dystonia makes the neck tilt right much less frequently at 12.07% – 33.33%
It is not clear if handedness indicates a consistent body wide strength on one side over the other.
70% of people are right eye dominant and 29% are left eye dominant.
65% of people, according to one eye clinic study, have the dominant eye the same as the dominant hand, and only 18% have the dominant eye the other side from the dominant hand. This would suggest that most people being right handed, also are right eyed, and in theory also that the right leg and the right side of the neck may be more used and stronger.
It may be useful to determine if the body of patients with left-tilt dystonia also has dominant or weaker left eye, hand, left hand or left leg. It may be interesting to study in terms of brain messaging if the pattern of tilt of dystonia is related to two other phenomena – which hand a person puts at the front when they clasp their hands, and which foot they tend to lead with when the start to walk from a standing position.
What this may reveal is a pattern of whether dystonia hits the stronger or the weaker side.
pain – type and intensity
63.64% report pain from muscle tightness and contracting
50.00% report they have severe neck pain
47.27% report pain that is more of an ache
38.18% report pain on the nondystonic side that is nonstop overstretched
32.73% report the pain is intense and only 9.09% report that it is mild
9.09% – 33.33% report they have mild neck pain
The term ‘pain’ however is a vague term. Patients may differ in what terms they feel are appropriate for what some describe it more as a strong pressure pushing down on them.
pain- duration, constancy
11.11%, 37.04% and 39.29% report the pain as constant
16.67%- 55.36% report that the pain is occasional or changing
31.48% report that the dystonia comes in attacks or storms of intense tightness
It is not clear by these surveys if the dystonia would be constant were the patient not trying to deal with it. Many patients report that lying down or using a cushion helps reduce pain, that heat helps or that even sensory tricks reduce it briefly. Medical treatment also attempts to reduce pain. The questions may have been better framed to capture if the dystonia on its own is constant.
.
Anecdotally pain reported by some is so intense that despair sets in. People may differ however in pain threshold, or in personality and desire to appear strong or to admit to the pain. These factors are not captured by the survey but may be factors in what doctors are told about their pain.
spasms, tremor
Terms may not be understood the same way by all patients. – tremor, spasms, jiggling, involuntary jerks, shaking, quivering or quaking.
66.04% report they have constant or occasional head tremor
30.19% report spasms at rest
24.53% report spasms when they try to hold a new head position
20.75% report spasms when they start to move the head
5.66% report spasms of relief when they stop trying to hold a head position
The spasms may differ technically. Spasms at rest suggest the body struggles to hold any position. Spasms to start a motion suggest there is a resistance. 19.23 % of those asked dreport a delay before their head moves. This delay of response is also reported by those with gait dystonia,by those who have chewing problems and by those who have problems opening or closing their eyes. The delay suggests a brain to muscle problem in sending the message or a muscle to brain problem in responding. The phenomenon of gating not just reaction time seems a very useful area of study.
Spasms while trying to hold a head position however seem more likely due fatigue after great effort, much like weight lifters start to shake just before they no longer can lift the weight. The body tires in the fight.
Spasms that start after the effort is complete, as the body settles back to the dystonia position, are reported by some patients as relief. This type of spasm seems more like the shaking a person has after a near miss traffic accident, where the body has been tense and now can finallt admit that stress, shake the tension out, get back to the more familiar mode. Patients anecdotally report that relief spasms, of all types, are more likely to feel pleasant while the others do not. However only a very small number of respondents in this study reported such relief spasms.
clicks
78.00% report they often or sometimes hear clicks, cracks, popping sounds in the neck.
This phenomenon has also been noted in those with jaw dystonia as they move the jaw, and in surveys of those with shoulder dystonia. It may be useful to study whether in any joint there is a bubble of excess gas when a muscle makes a cracking sound, and that this is created more during dystonia.
24.00% report that the clicks cause no pain while 12.00 % report they do cause pain.
41.51% report the spasms cause them pain while 16.98% report that spasms cause them no pain
It may be useful to study if the clicks and spasms are the body trying to cope with the dystonia and if so, if these are in some ways providing benefit.
progression of symptoms
83.33% of respondents said their dystonia started on one side of the neck and stayed there.
across
15.38%- 16.67% reported that the dystonia went from one side of the neck to also be on the other side
This particular phenomenon was not clearly surveyed. It is not clear if the respondent was saying that the tilt actually changed from tilt left to tilt right, or if they meant that the pain on one side also became a pain on the other side. With dystonia, a left tilt makes the muscles on the left very short and tight but also overstretches the muscles on the right to permit the head tilt. Both sets seem to endure significant discomfort because both are in extreme positions for a long time. It is not clear if this was also reported as the dystonia having moved and a clearer question would have been useful.
Some clinical studies have examined whether, when dystonia spreads, it tends to move up or down the body.
up
13.46% reported that dystonia started in hand or arm and moved up to neck
3.8% reported that dsytonia in the neck moved up to face and eyes\
down
25.00% reported that dystonia went down from neck to throat, arms, legs
9.62% reported that dystonia went down from face to neck
daily activities
Though having a sore neck is a common public experience briefly, the experience of dystonia has a greater effect than many may realize.
66.67% have trouble holding a phone to the ear or holding a book
66.67% report it is hard to look straight ahead when biking
66.67% report that it is hard to look at and see someone walking towards them
66.67% report that going over a bumpy or snowy road in a car is hard on the neck
50.00% have trouble reaching up to do their hair or looking in the mirror to apply
make-up or shave
41.18% have trouble seeing where they are walking
39.22% have trouble carrying groceries
36.73% report painful neck jolts in cars of some suspension or wheel base
35.29% have challenges eating
34.69% can still drive but only go short distances
33.33% sometimes bang their head accidentally going through doorways
33.33% have trouble shampooing, drying or brushing their hair, reaching up for
objects above them or down to wash their hands
13.73% have trouble dressing
11.76% have trouble bathing or showering
sleeping
Clinical studies that report that dystonia is absent during sleep are not consistent with the results of these surveys. More research would be useful, since need to sleep eventually overcomes all pain for a time and sleep itself does not prove absence of pain.
It is also possible that levels of sleep are factors in the different experience of pain and that deep sleep is not affected.
The surveys revealed some of the concerns patients experience as they try to sleep
The position of lying down
61.11% report that the pressure on their neck is less when they lie down or sleep
A cushion or pillow
The benefit of the cushion may be to help support the weight of the head or may be a help to resist the need to constantly fight against gravity
60.38% when seated try to find a place to lean the head against a wall, cushion or
room divider
60.38% report that when possible, to read they lie down and rest the head against cushions
48.15% report the pressure is less when they rest their head against a soft cushion
20.00% report they sometimes use a soft cushion at the back of the neck
20.00% use a soft cushion at the front of the neck
20.00% report they sometimes use a scarf for neck support
Getting into a comfortable, nonpainful position
58.00% report they have found at least one sleeping position that is eventually
comfortable. Though statistically one cannot make a lot of conclusions
based on who did not answer, this statistic may suggest that a large
number do not find comfortable positions much at all.
10.00% report that over time and with exercise they have found other sleeping
positions that are also comfortable
Spasms
30.00% report they go into spasms in some sleeping positions
changing position to find one of less pain
38.00% report that to keep from pain or spasms they change sleeping position every
few minutes till they fall asleep
This question series did not ask about warmth and heat. In the ‘coping ‘section of surveys many reported that heat was helpful for getting to sleep.
The use of medication for sleep may also be related. The survey on medications may be of interest. Use of medications or heat does not seem to eliminate dystonia for all patients, though it may reduce it.
Many patients express strong interest in pillows. It is not clear if these pillows provide a tactile warmth and comfort effect, if they provide a physical support to prevent spasms, or if they provide a sensory trick. Further study may be useful to find out what aspects of pillows matter and what technically the benefit is.
70.00% are very particular about pillow position
36.00% like to have a pillow behind the head
22.00% like to have a pillow beside the head
6.00% sometimes have a soft cushion in front of the neck
heaviness, feeling of weight
Patients sometimes report a sensation of the head being heavy, cement like, like a bowling ball. This may indicate resistance to being moved. However it may also be related to the experience some report of difficulty swimming, of a change in the sense of buoyancy. This may merit study, particularly since some clinical studies have found that
after deep brain stimulation patients often can no longer swim.
66.67% report that when walking it feels like there is a strong force pushing the head
sideways
34.62% report that when they try to move their head they feel a leaden cement feeling
sometimes in the jaw
16.67% report that when they are walking it feels like there is a ton of bricks on the head
sensory tricks
Sensory tricks were frequently reported. The surveys found a range of types of tricks that reduce the dystonia. It may be interesting to study what types of tricks work with what types of neck tilt to see if there is a pattern.
forced tricks
Physically forcing the head to more normal position is reported by many patients.
83.33% sometimes put their hand around their head to forcibly push the head
away from a tilt
66.67%sometimes put the hand on the cheek to forcibly turn the head
33.33% sometimes use a hand on the forehead to push the head into position
16.67% sometimes put a hand under the chin to forcibly lift the head
sensory tricks – classical type
36.73% touch the chin to reduce muscle tension, 34.00% to reduce tremor
32.65% touch the cheek to reduce muscle tension, 30.00% to reduce tremor
30.61% touch the back of the head to reduce muscle tension, 14.00% to reduce tremor
26.53% touch the base of the neck to reduce muscle tension, 12.00% to reduce tremor
14.29% touch the temple to reduce muscle tension, 10.00% to reduce tremor
14.29% pinch the neck to reduce muscle tension, 10.00% to reduce tremor
10.20% touch the top of the head to reduce muscle tension, 6.00% to reduce tremor
4.08% touch between the eyes to reduce muscle tension, 10.00% to reduce tremor
4.08% touch another body part to reduce muscle tension, 4.00% to reduce tremor
These patterns confirm the historic pattern of the sensory trick in paintings, where touching the chin or cheek is most common.
The greater sensitivity of some body parts to producing the trick seems a parallel declining pattern for muscle tension or tremor in most situations. It may be useful to study if those areas of the body tend to have more nerves or fewer.
However the declining pattern is not fully consistent. The area between the eyes seems more sensitive to tremor than to muscle tension according to these surveys.
The area between the eyes is of particular interest because it is often an area touched in thought, to enable concentration among the general population and to have a somewhat calming effect.
The relationship of these points to acupuncture points may also be useful to study. In this series questions were not asked about some other reputed acupuncture points that affect the neck, such as the web between the thumb and first finger, or the area on the palm just below the third and fourth fingers. Anecdotally some patients report immediate end of tremor at touching some of these points and this could be studied.
Why some points affect pressure and some tremor is not clear. Anecdotally patients report that one trick works for muscle tension while another works for spasms.
sensory tricks using assistance from other body motions
22.00% report that pain walking is less if they scrunch their shoulders
12.00% report that walking pain is less if they alternately shrug shoulders
12.00% report that when they try tlift the head their mouth tends to open
However incidence of other involvement was small
4.00% report that eyes scrunch closed when they try to lift the head
2.00% report that nostrils flare when they try to lift the head
sensory tricks that do not require direct touch of a given finger
30.00% report that they can use one or two or different fingers for the effect
16.00% report that using the knuckle not the finger tip works
6.00% report that the trick works if someone else touches the spot for them
6.00% report that the trick works even when done by the other hand
4.00% report that the trick works if they just move the hand towards the
spot but do not touch it
0% report that the trick works if they just think about touching that spot
These are small numbers and it is possible that respondents were not clear on the question since 28.00% reported they were unsure and 28.00% that the question was inapplicable.
other actions where dystonia seems reduced or gone
The idea of a completely unrelated activity suddenly reducing dystonia was reported by some respondents.
80.00% said it is less when they cough
60.00% said it is less when they yawn
40.00% said it is less if they close their eyes
20.00% said it is less if they hum
Those activities are often instinctive, done involuntarily as well as voluntarily. That may mean that the body has an override that prioritizes the messages as survival level urgency that can override dystonia.
It is also possible that the mechanics involved in those actions are so vastly different from the neck dystonia that the mind is able to do them because they involve parts of the body not affected by the dystonia damage. The phenomenon of vocal cord dystonia where people can barely speak but can yell seems similar – the movements required for the task are not impaired though they are similar to the muscle motions that are impaired.
A similar thing happens apparently with those with leg dystonia who can barely walk but who can run, or those who cannot run forwards but can run easily backwards. These oddities may be simply asking for function of different muscles or of the same muscles of dystonia but in a way so different by command that the dystonia is not happening.
treatment
The survey series on medical treatments and on effects and side effects may be useful to also consult. Patients with dystonia of the neck reported higher rates of treatment by botulinum toxin than by cortisone injections.
59.18% report they have received regular injections of botulinum toxin for some time
26.53% report they had had one or several injections of botulinum toxin
However
26.53% reported they had been given injections of cortisone
The survey did not reveal whether this lower use of cortisone was due to patient or doctor preference or cost or availability of the treatment. However the variation may be useful to study if the definition of the problem is the same, for instance pain.
genetics and possible susceptibility
Anecdotally patients have sometimes observed coincidences in their backgrounds.This survey series attempted to see if there were patterns. The questions were optional as were all questions.
There seem to be some patterns with cervical dystonia and this may be of potential interest for future study.
55.32% reported they had family members who have been treated for depression
46.81% reported they are of northern European ancestry
44.68% reported there are people in their family with tremor or head or hands
42.55% reported the like to put things in order, count things and be organized
40.43% reported they have blue eyes
The survey on identifiers may also be of interest though the response rate was low. It seems that the questions are useful to ask to trace possible causal links or risk factors. However patient concerns about privacy or shyness may be hurdles to answering them.
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Lived Experience With Dystonia 