ORIENTATIONS OF DYSTONIA
A. Background
The surveys revealed that dystonia is rarely symmetrical. This itself may be very useful to study to deter,ome what area of the brain governs that section and whether the muscle has a dysfunction or if the messaging between them is unusual.
Some people experience a left tilt, some a right tilt. This is often seen with the neck. It is also found in dystonia that affects a a limb on one side only – finger, hand, arm, leg, foot. Sidedness may also be a factor in the eyes or the lips though that seems less often reported.
It is also seen in the trunk in a tilt when sitting, standing or walking. Some patients report a sidedness in the timing of the gait of either foot, in the ability of shoulders to shrug, in the pulse on one side of the body versus the other, and in the number of audible clicks during movement on one side not the other.
It may be useful to study why a given person has a left tilt not a right tilt. Seeing if there is a relationship to their preferred hand may be useful to determine if dystona seeks a preferred muscle with preferred messaging or if it seeks a less preferred muscle, possibly weaker.
It may also be useful to study how the nondystonic side is affected. It appears that the nondystonic side, though not exhibiting the pain, muscle tightness or tremor of the dystonic side, nonetheless changes. Most sensory tricks seem to occur by touching the nondystonic side, somehow affecting with relief the dystonic side. Some patients report that touching the waist at the side has a positive pain relieving effect tough it is not surveyed whether touching the waist at the front has such an effect.
Some people experience a head pressure that has them looking down, unable to look up easily, or to look up, unable to look down easily Patients of both report problems looking at eye level. In additiok the surveys revealed that some people experience a downward pressure on the eyebrows or eyelids, so that an eye droops down.
The force of gravity may be a key factor in dystonia since people have to resist the downward push normally to hold their heads up or stand up. The surveys on daily activity and sleep found a profound relief just from lying down, or from use of cushions to support some of the constant effort to fight gravity. In the surveys here about direction, it was reported that standing up is often a problem briefly, often with dizziness or wobbliness.
Some studies have examined how dystonia progresses and to where. Patients in these surveys were asked if their dystonia moved up the body or down . It may be useful to study why there is this difference and in whom it moves. Some clinical studies suggest that with generalized and early onset dystonia the pattern is for dystonia to start in the legs and move up while in late onset the tendency is for it to start in a upper body part and move down. These phenomena may bear study.
. It may be that the body is constantly trying to fight the dystonia, mucj=h like it fights any invader virus or bacteria.Some patients report a sense of dystonia moving through the system to some patients somewhat the spread of an infection.What is different seems to be that with a virus there is progress and the virus leaves the successive parts of the body while with dystonia it tends to stay in all the ones it started in. This also however is not experience for all patients however and a few did report that the dystonia moved to another body part and left the first part.Those situations may be very useful to study as they suggest that the body is able to overcome dystonia in some ways.
Some patients experience a forward or backward body tilt. This is seen in the trunk, with the entire body leaning forward or back but also with other forms of dystonia where motion is easier going forward or going backward. In some media reports, for those rare individuals who are able to run easily but can’t walk, or those who can run backwards easily but not forward, the experience that the muscles involved themselves are in good condition. In such circumstances the problem with dystonia seems more that the brain instruction to go forward versus backward is a signficantly different instruction and that only one aspect may be affected by dystonia. Some patients report is easier to shovel snow or rake leaves if they walk backward.
Some patients experience a strong pressure to pull the chin in toward the chest. Others report a pressure to have the chin jut out though none of those were reported in the surveys. This pressure to pull in can be challenging not just for vision straight ahead but also for practical considerations like eating and even more critical ones like ability to breathe or swallow. The phenomena of tilts up or down, and or of chindpulling in or out may seem nearly the same but does in reports present as slightly different.
It is possible to have a head tilt down without the chin pulling in. The variations suggest that these may be separate pressures or body messages each. Many patients report that they are dealing with several pressures at the same time. What may be useful to study is not just when this happens and incidence but why, to whom. If the baby at birth has for instance a head tilt that corrects over time, does this suggest a muscle weakness in that are and if so, is that where dystonia hits? Baby photos may be a useful resource to study long term patterns of tilt.
Some people experience a curling of a body part, a tendency to bend and great difficulty straightening it. Others experience a body part sticking out, a toe, finger or arm, that adopts a pointed osition the person does not intend.. Some people report this phenomenon as a toe sticking straight ‘up’ and some report simply that the toes or fingers seem to separate oddly and point independently in different directions Some reportthat one toe sticks up and the others curl. These situations may be useful to study in terms of surround inhibition where it seems that a dystonic toe is most affected but the adjacent ones are responding as if trying to assist but with difficulty. In one hypothesis the toes or fingers adjacent to the dystonic one are actually too inhibited and not able to help out enough, so there is an excess of surround inhibition not a lack of it.
The curling phenomenon is seen in other types of dystonia including the neck where the body in essence has become crooked to curl one direction, or even in trunk dystonia where the entire body may seem to be curling or arching slightly. This phenomenon of curling seemsmore common than straightening as a dystonic message. That itself could be studied. Moving to a more curled position may be a default body position for pain, for avoidance, for comfort given that if extreme it approaches a fetal position. Curling also happens during a stage of cell death and it may be useful to study if there is any cell death in the dystonic muscle.
B. Sidedness and lateral orientation
-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
difficulty looking straight ahead
28.85 % It is a huge effort for me to look straight ahead (9-7)
25.00% I sometimes have to tilt my head oddly to see straight ahead (9-7)
dystonia affects one side
83.33% When I try to look down, my head still tilts to one side (30-2)
66.67% It is hard to hold a phone to my ear, better on speaker (30-8)
51.92% To look to one side is easy and to the other very difficult (9-7)
50.00% When I stop I sometimes have to sidestep to regain balance (23-3)
50.00% When I sit I slowly tilt to one side (33-1)
50.00% It is hard for me to sit upright without a support to lean on (33-1)
45.45% When I stand I tend to tilt sideways(23-10)
33.33% The dystonia started in one place and moved to same side of body (31-15)
33.33% I gradually tilt when I am sitting (23-9)
30.77% When I open my mouth it is asymmetrical, off to one side a bit. (7-5)
30.00% I can’t rotate my shoulders in small circles, equally (10-6)
29.17% When I walk it feels like I am tilting (23-3)
27.27% It usually hurts only on one side (2A-32)
25.00% I like to hold onto an armrest to keep my balance (23-9)
25.00% I cannot hunch my shoulders equally (10-5)
22.22% The trick works from the same side of the body to the dystonia (4-6)
21.74% I hear popping sounds in the muscles on my dystonic side (23-7)
17.50% My lip sometimes pulls sideways (7-1)
14.29% The parts affected are beside each other- eg. finger and wrist(11A-1)
13.64% When I open my eyes, there is a delay for one of them to open (5-2)
13.07% When I am dizzy the room or my body seem tilted (2A -17)
12.50% Lying on my nondystonic side is comfortable (25-18)
12.15% I hear more clicks from the dsytonia muscle side (2A-40)
11.64% It affected several parts of my body but all on the same side (2A-33)
8.62% My head drifts to one side (9-1)
7.69% People used to tell me I look at things mostly with one eye (9-7)
5.88% As I walk one leg often crosses behind the other ( 12A-30)
4.35% My pulse on the dystonic side seems slightly delayed (23-18)
3.57% The parts affected are not beside each other- finger,upper arm (11A-1)
1.10% hear more clicks on the dystonic side (2A-40)
left side involved and preferred
66.67% I have trouble looking at objects or people on my right (30-6)
33.33% When I look 90 degrees to the right, my head still tilts left (30-2)
17.24% My head tends to tilt left head is lying closer to left shoulder (9-1)
6.90% My head tends to turn and gaze to the left (9-1)
right side involved and preferred
33.33% I have trouble looking at objects or people on my left (30-6)
33.33% When I look 90 degrees to the left, my head still tilts right (30-2)
15.52% My head tends to tilt right, head is lying closer to right shoulder (9-1)
12.07% My head tens to turn and gaze to the right (9-1)
11.76% There is less distance between right step and next right step (12A-46)
dystonia moved to other side
33.33% Dystonia moved from one side of body to the other (31-15)
15.38% My dystonia went from one side to also be on the other side (9-10)
13.33% The dystonia started in one hand and moved to the other hand (11B-1)
11.63% Problem started in one eye, moved to also affect the other (5-11)
8.99% It affects parts of my body on the other side (2A-33)
dystonia on both sides
25.00% When I move dystonic muscle, opposite side muscle has tremor (23-19)
24.07% The trick works from a central part of body (4-6)
15.51% Often both sides hurt (2A-32)
12.00% Walking pain is less if I alternately shrug shoulders (9-17)
10.34% My head on its own goes back and forth as if to shake head ‘no'(9-1
dystonia where one side affects the other side’s problem
14.81% The trick works on either side of the body to affect the dystonia (4-6)
14.29% Touching my side at the waist reduces pain (26-2))
11.11% The trick works from the opposite side of the body to the dystonia (4-6
6.95% Touching the other side makes the dystonic side hurt less (2A-32)
C. Open or closed orientation
When muscles move they stretch or contract in the arm or leg. In the vocal cords their motion is to open or close a paasage for air to go through an opening. Dystonia affects the ability of those muscles to open or close that passage way as well as their thickening and stretching. It seems that with dystonia of the vocal cords the more common problem is for the muscles to shut together, and not be able to open well This tendency to close, seems consistent with the tendency of other forms of dystonia to curl rather than stretch as if occuping less space.
The eyelids that droop or tend to go down, so it is difficult to push them back up, in effect are clamping shut. The default position of dystonia in that case seems to close not open, to shut down not spread out.
When people are relaxed, their mouths often open in sleep but their eyes close. The relax position of open and closed seems to differ. When people die, their mouths and eyes often stay open. This suggests that the default position of the body at full relax mode, without any body control over it, is open. When dystonia makes things close up therefore, there is effort involved, and constant effort against a default mode.
When people do a sensory trick of involuntary assist, they sometimes report that they notice as they try to lift their head or manipulate their fingers, that they open their mouth. For some reason having the mouth open helps. Some even say their nostrils flare when they try to lift their head. Some patients say it is as if the brain, trying to get the head to lift, sends a message’ everybody open up’ to the mouth and nostrils.
However, oddly, when people try to move their head out of a dystonic position or try to manipulate another body part against the dystonic pressure, they often scrunch up or close their eyes. It is as if in that situation closing the eyes helps It requires effort to open the eyes and is more relaxing to close them. but the alert message of helping seems different for the two body parts and may bear study. The difference between default positions and assist positions for eyes versus mouth may be useful to study. There is a similar difference in how they handle pain since when in pain people also tend to scrunch eyes but open the mouth. In the Olympics weightlifters often open their mouths and even yell on lifting a very heavy weight. In the general populationn opening the mouth to concentrate while threading a needle also seems common. The nature of the open – closed message may be of use not just in the study of dystonia but in the general understanding of brain muscle instruction. This may reveal more of where the problem is with dystonia.
Adductor spasmodic dysphonia- the more common type of vocal cord dysdtonia and happens when spasms cause the vocal folds to clamp together and stiffen. It is difficult for the fold to vibrate to produce sounds so the voice sounds strained, athe speech may be choppy with problems starting or finishing a word.
Abductor spasmodic dysphonia happens when the vocal folds stay open and cannot vibrate. They are open too far. Excess air escapes from the lungs during speech and the voice sounds breathy and weak.
open is preferred position, problem closing or keeping closed
25.93% My face goes into grimace,mouth open wide sometimes or constantly(6-8)
25.00% to close my mouth there is a delay and it is hard to chew (28-3)
25.00% It is physically hard to crack a normal smile (6-2)
18.92% It is hard for me to smile a normal smile (7-16)
14.29% It is easier to lift my head if I open my mouth (26-2)
14.29% It is easier to lift my head if I open my mouth (26-2)
12.00% When I try to lift my head my mouth tends to open (9-17)
7.50% I have trouble pursing my lips (7-1)
4.72% I feel dizzier with my eyes open (2B- 1)
2.70% It is hard for me to close my mouth and I feel socially awkward (7-16)
2.00% When I try to lift my head my nostrils seem to flare (9-17)
0.94% I find it hard to close my eyes (2B-1)
0% The dystonia is less if I open my eyes wide (30-15)
0% I had trouble closing my eyes ( 5-10)
closed is preferred position, problem opening or keeping open
60.00% There is a delay when I open one or both eyes (28-4)
40.00% The dystonia is less if I close my eyes (30-15)
40.00% My eyes scrunch up more often since dystonia (28-4)
37.84% It is hard for me to hold my mouth open at the dentist’s (7-16)
35.56% Since dystonia I more often close my eyes just for comfort (5-1)
31.11% My eyes close forcibly and involuntarily (5-1)
28.89% It is sometimes hard for me to keep my eyes open (5-1)
27.91% I had trouble opening my eyes (5-10)
27.03% It is hard to open my mouth wide at the doctor’s (7-16)
25.58% problem started with extra blinking then hard to open (5-11)
25.00% it is physically hard to crack a normal smile (6-2)
24.44% My eyes during the day are sometimes half closed (5-1)
22.64% I find closing my eyes relaxing (2B-1)
20.45% When I open my yes they only get half open (5-2)
18.92% It is hard for me to smile a normal smile (7-16)
18.87% I feel dizzier with my eyes closed (2B-1)
16.98% I scrunch eyes to do difficult body movements (2B -1)
14.29% It is easier to lift my head if I close my eyes (26-2)
14.29% It is easier to lift my head if I close my eyes (26-2)
13.64% When I open my eyes, there is a delay for one of them to open (5-2)
13.64% If I blink, there is a delay before my eyes open again (5-2)
11.36% When I open my eyes, there is a delay for both of them to open (5-2)
11.36% It is difficult once my eyes are open to keep them open (5-2)
11.11% When I close my eyes they are often squeezed shut (5-1)
11.11% My eyes gently close on their own (5-1)
11.11% It is always hard for me to keep my eyes open (5-1)
10.38% I find it hard to open my eyes (2B-1)
10.00% My lips sometimes involuntarily clamp together (7-1)
9.30% The problem started with a drooping eyelid (5-11)
8.11% It is hard for me to open my mouth to brush my teeth or floss (7-16)
4.55% It is difficult to open my eyes at all (5-2)
4.00% When I try to lift my head, my eyes scrunch closed (9-17)
D. Curled or straight orientation
tends to curl or bend
66.67% My foot curls in (32-3)
58.85% One of my feet turns inward or clenches (12A- 4)
58.82% My foot arches oddly (12A-3)
53.85% I think my lower back is starting to curve (12B – 15)
50.00% When I walk a foot that is normally straight turns inward (12A-34)
38.46% My body bends so much that I have trouble standing (12B – 14)
35.29% In resting position one of my feet curls downwards (12A-5)
35.29% It seems sometimes like fingers, toes, hands or feet are curling (20A-27)
33.33% My feet twist at night (32-3)
29.41% When walking my nondystonic toes start to curl (12A-37)
29.41% When I sit, my toes curl (12A-14)
29.41% I have problems straightening my leg at the knee (12A-12)
18.75% When I sit my leg draws up towards my chest (12A-16)
15.79% -one arm bends differently from the other ( 10-7)-
14.29% It is easier to lift my head if I scrunch my nose (26-2)
6.67% Some of my toes are curling (32-4)
6.00% Sticking my chin out is easier if I scrunch my shoulders (9-17)
preferred is to straighten or point
47.06% My toes are always pointed oddly (12A-2)
35.29% -In resting position some toes stick oddly straight up (12A-6)
9.52% the pain is less if I stretch finger or toes to straightest position (23-17)
E. Up or down orientation
-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..
problem looking at eye level
12.50% I have trouble reaching for an object at eye level (3A-3)
body part tilts up
15.00% My lip sometimes pulls upwards (7-1)
4.65% One of my eyebrows goes up involuntarily (5-4)
hard to look down
52.76% often or occasionally have balance problems going up and down curbs(2A-16)
33.33% It is hard to look down to wash my hands or manipulate objects (30-8)
32.29% I have trouble reaching down to pick up an object (3A-3)
25.00% It is hard for me to go downhill or downstairs because it is hard for me
16.67% It is hard to look down at food on the table in front of me(30-8)
16.67% It is hard to look down to see an object that fell (30-8)
13.46% It is difficult for me to look down at the floor (9-7)
difficulty standing up
41.67% When I first get up from sleeping I am wobbly (23-3)
36.18% I am sometimes dizzy when I stand up quickly(2A-17)
29.17% When I first get up I am dizzy (23-3)
dystonia moved upward in body
33.33% Dystonia started in fingers hand, moved up to arm,shoulders (31-15)
20.00% The dystonia moved up my arm, from fingers to wrist or arm (11B-1)
17.65% My dystonia started in the wrist and went up the arm (10-13)
13.86% It moved from my lower body to upper body (2B-9)
13.46% The dystonia started in my hand or arm and went later to my neck (9-10)
10.05% It seemed over time to move up my body (2A-33)
3.85% My dystonia went up from my neck to face and eyes ( 9-10)
body part tilts down
18.60% I had a drooping eyelid as first sign of dystonia (5-10)
16.28% I purse my eyebrows more often since dystonia (5-4)
difficulty looking up
50.00% It is hard to reach up and do my hair (30-8)
44.23% It is difficult for me to look up at an airplane (9-7)
33.33% It is hard to reach up to objects above me (30-8)
16.67% It is hard for me to look up at skyscrapers,airplanes or clouds (30-6)
16.67% It is hard for me to look at upper shelves at home or in stores (30-6)
10.42% I have trouble reaching for an object (above) eye level (3A-3)
5.00% It is hard for me to go uphill or upstairs because it is hard for me to look up
preference to sit or lie down
10.55% I am sometimes so dizzy that I fall down (2A – 17)
dystonia moved downward in body
25.00% My dystonia went from my neck down to throat, arms, legs (9-10)
24.75% It moved from my upper body down to lower body (2B-9)
13.23% It seemed over time to move down my body (2A -33)
9.62% The dystonia started in my face and went later to my neck (9-10)
6.67% The dystonia moved down my arm from arm to hand to fingers (11B-1)
5.88% My dystonia started in the arm and went down to the wrist (10-13)
F. Forward or back orientation
preference is to tilt forward, difficulty leaning back
33.33% My body feels pulled forward (12B-11)
20.83% I have trouble leaning my head against a chair back (23-9)
13.64% When I stand I tend to tilt forwards (23-10)
preference is to tilt backward, difficulty leaning forward
38.46% My body feels pulled backward (12B-10)
6.80% My head feels oddly heavy when I lie back in bathtub (2B -4)
5.17% My head tends to tilt backward (9-1)
preference to go backward
16.67% I can rake the lawn more easily walking backwards (26-12)
G. Pulling in towards body or jutting out from body orientation
preference is chin pushed into chest
16.67% My chin pushes in so much I worry if I can swallow (30-3)
6.67% My chin pushes in so much I worry if I can breathe (30-3)
H. Conclusion
The orientations of dystonia seem to be treated often as not very relevant, similar to eye color. However it is possible that the presentations actually may matter, not just to the nature of this dystonia and the brain problem, but also in why this happened with this patient, and their particular history. Such study may be very useful.
=
Lived Experience With Dystonia 