14. Movement – technical aspects – Lived Experience With Dystonia

Symptoms – Movement

A. Context

Dystonia is classified as a movement disorder . In most patients it is how they move that helps diagnose it. The problems however have been observed byresearchers to not be just about movement however. Some have speculated that the problem is also in the sensory area – how the body interacts with its environment and sends a possibly flawed message back to the brain.

Clinical studies have studied the mechanics of the movement. There is an identified delay between the desire to move and the ability of the body to respond to the command to move. There seem to be not just reaction time problems but delays at a second point called ‘gaiting’.

Patients have reported not just delay but lack of precision of their response. Some report that they automatically try to correct for the wrong motion, resulting in what to an outside observer may sees as jerking motion

Some patients report that the dystonia exists even when there is no motion and have spoken of it also a disorder of position – that they can’t stand straight or hold their head straight of their fingers naturally, even when at rest. What they report is often an ongoing pressure that forces them into a position.

Various aspects of movement seem affected – balance, stride, gait, distance between steps, tilt. An observer may not find it easy to determine what part of the motion is the dystonic part and what is the patient’s automatic or intentional effort to cope.

Those with vocal cord dystonia do not visibly have a movement disorder but report that the muscles of the speaking apparatus are challenged. Spasmodic dysphonia, or laryngeal dystonia, involves involuntary contractions of the vocal cord musclesmresulting in speech interruptions,breaks or a breathy quality to the voice.

The mechanics of what is not happening have been studied with two types identified – abbuctor and adductor. Adductor spasmodic dysphonia, the more common version, happens when vocal cords come together and stiffen, making it hard for them to vibrate. As a result the patient finds it hard to produce sounds. The muscles go into spasms, words get cut off and the voice sounds strangled. Abductor spasmoic dysphonia involves spasms that make the vocal folds open. They cannot vibrate if they are too far open, air escapes from the lungs during speech and the resulting voice often sounds breathy and weak.

B. What seems useful to study further

The senses related to motion could be studied.

Proprioception, the sensing where each body part is and what

it is doing is difficult to study. .It is not clear whether this is affected in dystonia.

Some observers assume that a person with tilted head is confused about what uprightmeans and is not aware of the head tilt. Patients report however that they areaware they are tilting, but are physically under such pressure

to tilt that it is difficult to hold the head upright.

To test this a person could look with eyes closed in a mirror

and adjust the head to upright and then open the eyes and see if

in fact the head is upright. In some cases this ability may decline

as dystonia progresses.

Equilibrioception, the sense of balance or internal GPS informs a person if

they are sitting, lying down, standing. It is not clear if this sense

is affected by dystonia.

People with Parkinson’s sometimes report they have lost a sense of their body’s position in space, their ‘sixth sense”.

Walking

Research studies have looked at balance, stride, delay of motion, timing.

What is lacking is a way for patients to calibrate

their experience and report it more precisely. They report they feel wobbly

or awkward, unbalanced. Some say they feel dangerous to those near them if they

might crash into them or dangerous to themselves if they

bump into doorjams, poles or fall on stairs.

The problems of walking seem varied for those with dystonia.

Those with cervical dystonia may not have any technical problems with legs but some report they have difficulty walking in a straight line because they can’t l ook straight ahead reliably to see where they are going. The result is they bump into things, reach out for fences, and wobble.

Dystonia seems linked to delays in responding to a desire

to move. However some treatments have been linked to

slower reaction time and it is a feature of extreme botulism. Medications

for individual patients are useful to correlate with gait study.

Voluntary or Involuntary?

The categorizing of dystonia as a disorder with involuntary muscle contractions seems to merit some nuancing. Some patients experience the condition as sudden and unintended motions, unplanned jerking motions. Howeve other patients report few such unplanned motions. What they report is ongoing resistance to planned motion.
Many report that how they move blends this resistance and their effort to overcome it, a voluntary, moment by moment adjustment. A person with cervical dystonia walking down the street may report that every few seconds they move their head to a new position to try to find one less painful. Every few seconds is frequent adjustment. When the task requires looking up to see a sign or to cross a street, they are dealing with intent to move, the drag of resistance, the adjustment they make and the huge pressure pushing the body back to its default position. The result is that dystonia as they describe it is not involuntary motion alone but a blend.

Some patients have struggled to describe what seems like a category between voluntary and involuntary. Reflexes are in a category of movement that not usually suppressible. The startle reflex of babies, the Babinski reflex are motions, possibly related to survival. The reflex to move away from a pinprick or a burning flame also is a motion that seems suppressible only with great effort. Some patients report that when the pain of dystonia is so intense they move to a new body position to escape it, that this movement away from pain seems nearly reflexive , as if in the semi-voluntary category. They can hold still for an MRI but it is painful to do so and requires great effort. This grey area of dystonia motion then may be a fruitful area of study since it does not seem to be simply involuntary movements.

One area of interest is the phenomenon of grasp. Babies often clench their fists, and learn as they mature not to. Dystonia seems to interfere with the ability of some patients to unclench.

Some patients report that when they hear a sudden noise their startle reflex is more intense than usual as if an overreaction they can’t quite control. This oddity of reflexes working too well might be an aspect of dystonia worth study.

With voluntary motions there is habituation. Over time a motion frequently done such as swimming or biking becomes so ingrained in the brain, the synapses and neural pathways so well established that it is not necessary to consciously direct the motion much any more. The body has ‘muscle memory’ and in a way knows how to do this task

With dystonia, those who contracted it later in life report they are aware of how to do most motions and used to do them with ease, They likely developed muscle memory and brain habituation to do them. When dystonia strikes, they have sometimes express mystery about whether their body remembers how to hold the neck straight, or how to speak, Some say the new inability to do what used to be easy is shocking. Some gaze at others speaking, marvelling how easily they do it, or watch others running and marvel at how easy it is to take for granted these motions. They sometimes express sadness they have lost that ability.

However when people with dystonia experience a sensory trick, they report that suddenly their body can make that motion, just like before. This surprise, to be able to hold the head up without any pain, to be able to yell just like before however briefly merits study. The body clearly still can do the motion and has muscle memory for it.

Dystonia is often listed under hyperkinetic movement disorders along with

-tremor- rhythmic oscillations due to intermittent muscle contractions

-tics- paroxysmal muscle contractions, often temporarily suppressible

-myoclonus- arrythmic twitches, not suppressible

-chorea- unpatterned movements, dance-like, often rapid

-athetosis- often slow writhing movements, of arms and h ands

-dystonia- repetitious or sustained muscle contractions

-hemiballismus – wide, flinging movements on one side of the body only

Whether or not a motion can be suppressed, whether or not it is voluntary seems a key feature among movement disorders. It merits study here also because patients with dystonia report a nuanced experience.

First learning a motor task studies have found that movement is slow and easily disrupted. The person is incompetent and step one is to realize the task is hard. If they don’t even realize it is hard they may be unconsciously incompetent. However as they see what they need to learn they are still not competent but they can be consciously incompetent. They are figuring out what they need to learn. Once they learn the skill they become competent, but at first only consciously competent, when paying attention,’ eye on the ball’, focus, focus. Eventually over time the skilled worker gets good at the task and becomes unconsciously competent, can do it easily, ‘with his eyes closed’ as the saying goes.

People with dystonia report that they have less unconscious competence now for some motions. They are aware of challenges and trying to cope makinga more studied and conscious effort, as if they had slipped back on the learning scale. This relearning may parallel retraining the brain and muscles after a stroke. The difference seems to be that with dystonia retraining alone does not end the problem. The dystonic pressure seems to remain despite retraining. However some patients report that exercise and conscious effort at a task can provide some benefit.

At the start of learning a new motion, motor and somatosensory cortices of the brain are involved. In early stages the prefrontal and frontal cortices are active. However once the motor skill is learned, clinical studies have shown that some of those areas become less active. Researchers have disagreed about what type of plasticity mechanism is involved in the cerebellum when a motor skill is learned. The basal ganglia area seems to play a role especially in forming habitual motion.

What seems to happen with dystonia is that a former motion easy to make becomes more difficult. Some patients have compared it to learning to eat with a spoon again or learning how to hold the head up that infants had to learn. This effort seems a voluntary (not meaning willing or keen but just required) effort to relearn what had become so easy. Patients have reported that when admired for how they cope it is tempting to also respond ” I had no choice”. The emotional desire to persevere involves the will which is voluntary though survival sometimes does not give a lot of options.

Useful insights may come from those with a similar disorder, Parkinson’s disease, which is more common. ( see file on Common ground with other disorders)

Tremors, shakes, spasms

Parkinson’s and dystonia sometimes have spasms in common.

However the nature of those jerks,spasms or shakes bears a study. They seem to differ in dystonia even with an individual patient at various times of the day. They seem to vary

themselves in rhythm, speed, or associated pain. Most people in the public shake periodically when cold, afraid, nervous . Most people in the general public are familiar with the way the body on lifting a heavy weight or holding up ceiling tile too long starts

to shake as if to give notice it is about to not be able to hold on much longer, Most people in the general public are familiar with the spasms of relief after an intense experience,

the body recovering after a near miss car crash.

Those’ relief’ spasms often feel good and seem part of the recovery. When a person with dystonia says they are having shakes, shudders, spasms, it is not clear if they still have normal shakes and spasms or if normal ones are augmented with dystonia.

Some patients report that they do daily exerices to strengthen their muscles but that they are pushing against a huge weight to do so, rowing against the tide. Some report experience spasms and shakes as they try this very difficult positioning. This may be

simply the spasm of muscles straining but felt more intensely due to dystonia.

Patients often report that once they stop an exercise they may then experience spasms, as the body returns to the dystonic position.Those spasmsmay be relief spasms, since some patients report they are nearly pleasant to experience. Some have even described the spasms as evidence the body is fighting. If the dystonia was the only pressure, the only message the brain was giving, then the position it forced would be a locked position. However when there is spasm, that may suggest the body is working to resist the dystonia and in minute intervals is winning.

People with severe tremor and dystonia have reported it as debilitating, especially if it interferes with ability to hold a cup, hold a pen, sit without shaking, or when it requires hanging on to a table edge to steady oneself. It may be a useful area of inquiry to not just ask about presence of tremor but about its nature and severity.

Some medications for anxiety can cause shaking. The shakiness of drug

withdrawal may be a factor for patients with dystonia, prescribed anti-anxiety or other medication as they adjust their dose. It might be a useful area of research to see if dystonic tremor is worse, not affected by or improved by medications that also can cause tremor.

C. Comments

from clinical studies and researchers

Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both.

Movement disorders are given descriptive terms that are often overlapping and ambiguous. Consistent terminology to describe these movements will clearly be helpful.

It is important to provide some type of rating scale to score the severity of the movement dysfunction

There are few validated rating scales for dystonia

1997- Two more scales were developed to rate the movement ability of people

with dystonia. The Unified Dystonia Rating Scale and the Global Dystonia

Rating Scale.

The Unified Dystonia Rating Scale rates movement in 14 different body regions

and looks at not just how severe the problem is but how long the problem lasts.

The Global Dystonia Rating Scale also looks at movement for 14 body parts

rating severity from 0 to 10. It looks at ten body areas as broad categories including in them eyes, upper face, lower face, jaw, tongue, larynx, neck, shoulders arms, elbows, hands, pelvis, legs, feet and trunk

The University of California San Diego has received funding from the Department of Defense in the US to create a way to objectively measure facial movements, angles of jaw etc in those with dystonia.

Apps have been developed to study motion, the gait of runners. There are platforms with sensors that measure gait, stride, pressure. There are apps to measure head tilt and how it changes with motion. However much of this technology seems in its infancy and not available in most clinics. Some patients have apps that measure how much they move in their sleep, that may be useful to detect whether dystonia disrupts their sleep or depth of sleep they are able to enter. However most patients have no such technology.

from patient experience

position

Now I am doing the ballerina thing

I have a new party trick – I can stand on my ankles

It feels unnatural now to sit the way the average person sits

For a second or two I am fine and then the muscles insist on going back to the weird positioning

My head is like a bent doll

My right arm and leg were held captive by their own muscles

My right foot teetered on its outside edge and arched so much I could not keep my heel in my shoe

My body is twisted like a corkscrew

Standing in one place is more painful than walking

When I try to sit, I often slide down the chair and my torso twists to one side

motion

It feels like I am walking on shards of glass

I have a dromedary walk
I have a robotic walk

I walk like a wonky donkey

I walk like the Tin Man

I have trouble walking in a straight line

It’s like walking on hedgehogs

Well, moving on, which is not as easy as it sounds

It’s like having a pinball in your body bouncing back and forth between the barricades

bumping objects

Was it an accident? No, I just like to bang my head against door jams

My head bumped against doors and I would fall a lot

delay in motion

Some days I freeze up and have to rest

When I tried to move, my legs just said no

My body makes a pause for station identification

My body is on pause many times a minute

I am in perpetual ‘hold that thought’ reminder mode

I press Play on my life but the tape sticks

In the movie of my day the frame freezes momentarily

This is my life through stop motion video cam

jerks, involuntary actions

Who else gets to kick someone and say it was just a hyperactive reflex?

My husband pinned down my arm to massage my leg and my arm shot out and punched him in the face. At least he laughed

I feel like a puppet and someone else is controlling the string

My right arm seemed to have a mind of its own

My arm shot up in the air and would not go down

My arms are spawning

tingling

My tingling feels like bacon sizzling

My tingles feel like walking on ice

It feels like my body is uncooked peas sizzling in a hot pan

twitches, tremors, shakes, spasms

To relieve the spasms I should not stay in any position too long

I try to hold back the twitches

I noticed I could see the tip of my nose bobbing

A loud noise sometimes triggers a spasm

clicking sound

When my muscles click it’s like I hear gunshots

My muscles click like bubble wrap

My muscles sound like popping corn

There are constant grinding snapping sounds in my back

D/ What to ask:

Source of questions –

clinical studies, patient reports, social media postings, biographies

E. Question categories

active or still dystonia

assists – of other body parts

balance, dizziness

bend, stretch as desired

clicks, audible clicks

control of motion

curling, sticking up

delay

drag or smooth

ease or lock

fist, grip, clench, catch items

involuntary and voluntary

jerks, arm shoot out

range of motion

reaction time-reflexes changes

spasms, tremor, cramps, shakes, tics, pulsing, zaps

speed

F. – Questions asked

surveys 2A and 23

G. Results of questions – percent answers per question

symptoms

2A 217 symptoms 26 40 76 5

23 24 symptoms 18 24 81 3

max. no. respondents 217

total questions 64

likely type of dystonia – all

percent of all respondents doing survey 217 or 508 or 42.7%

H. 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. balance, dizziness

-dizziness and eyes

18.87% I feel dizzier with my eyes closed

4.72% I feel dizzier with my eyes open

17.92% Opening or closing my eyes has no effect on dizziness

22.64% I find closing my eyes relaxing

0.94% I find it hard to close my eyes

10.38% I find it hard to open my eyes

16.98% I scrunch my eyes sometimes to do some difficult body movements

38.68% My eyes do seem to affect my dystonia (2B-1)

-dizziness

23.62% I am hardly ever dizzy

36.18% I am sometimes dizzy when I stand up quickly

25.13% I am sometimes so dizzy I have to grab for a wall or furniture

11.06% When I am dizzy the room seems to be spinning

13.07% When I am dizzy the room or my body seem tilted

10.55% I am sometimes so dizzy that I fall down

9.55% I am dizzier when I take some medications (2A – 17)

-Do you have a heavy full feeling like concrete as you do some movements?

32.09% Yes, often

26.74% Yes but only sometimes

24.60% no (2A – 31)

-Do you feel that how you are trying to fight the dystonia is partly subconscious,

the body’s normal way to try to stay upright?

87.50% yes

12.50% no (20A-21)

-Are you aware of when your head or body are tilted?

82.61% yes

17.39% no

-Standing

50% I can stand comfortably for 5 minutes and not lean against anything

27.,27% I prefer to stand by leaning my head or back against a wall

13.64% I have trouble standing with my head or back against a wall

45.45% When I stand I tend to tilt sideways

13.64% When I stand I tend to tilt forwards

0% When I stand I tent to tilt backwards (23-10)

-Standing

0% I can easily stand erect without leaning on anything

0% I can stand erect with some difficulty or for brief periods only

0% When I stand sometimes my legs buckle

50% When I stand I prefer to lean against something

50% When I stand I sometimes have to step backwards to retain balance (33-2)

(this question had very few respondents)

-Sitting

37.50% I can sit straight and comfortably

33.33% I gradually tilt when I am sitting

20.83% I can easily move from one seat to the next seat beside me

37.50% I feel best when I can lean my head against a chair back

20.83% I have trouble leaning my head against a chair back

25.00% I like to hold onto an armrest to keep my balance (23-9)

-Sitting

0% I can easily sit upright

50% When I sit I slowly tilt to one side

50% It is hard for me to sit upright without a support to lean on (33-1)

(This question had very few respondents )

-Balance and motion

20.83% My balance is excellent

41.67% When I first get up from sleeping I am wobbly

29.17% When I first get up I am dizzy

29.17% When I walk it feels like I am tilting

50% When I stop and look around I sometimes have to sidestep to regain balance (23-3)

2, range of motion, stretching

-range of motion for the dystonic muscle

9.18% I have full range of motion and all positions are comfortable

18.37% I have full range of motion but some positions are very uncomfortable

40.31% I have lost a little range of motion

22.45% I have very little range of motion and some positions are impossible

(2A-19)

-range of movement

56.52% I can raise both arms easily to above my head

56.52% I can raise either foot easily well off the ground

26.09% I can turn my head easily to either side

21.74% I still have full range of motion but some motions are very painful

17.39% I do not have full range of motion of my arms

17.39% I do not have full range of motion of my feet or legs

60.87% I do not have full range of motion of my head (23-11)

3. control of motion – stop , start, free , lock, grip, curl ,voluntary or involuntary

-Do you notice all of your body movements but find you are not intending some of them?

83.33% yes

16.67% no (19-72)

-Do you feel that the dystonia is pushing your body into involuntary positions or movements?

88.89% yes

11.11% no (20A- 19)

-Intention to move

8.37% All of my movements aer voluntary, ones that I planned to do

36.95% Most of my movements are voluntary but some are involuntary

36.45% I have involuntary movements that are sudden and jerky

31.53% I have involuntary movements that are repetitive

36.45% I have involuntary movements of twisting

24.63% I have involuntary pressure to hold a position but not to always be

moving (2A – 11)

-Does dystonia seem for you a blend of voluntary and involuntary movements?

58.82% yes

41.18% no (20A- 24)

-Does it feel like you are trying to cope with the dystonia with semi-voluntary

actions, one you do but can choose not to do?

50% yes, some of it seems in my control

50% no, none of it seems in my control (20A- 22)

-Is one part of your body tending to do something but you are also working voluntarily against what it is doing? (eg trying to hold head up even if it is being pushed down)

94.12% yes

5.88% no (20A- 20)

-When awake are you always aware when parts of your body are moving?

78.95% yes

21.05% no (19-71)

-locking into a position

46.70% I feel force to go to a certain body position

24.53% I lock into a position and can’t move out of it

22.17% The position I go to is at the extreme range of body position

17.45% The position I go to is at the midway in range and looks normal

to an observer (2A – 2)

16.98% I do not go to or lock into any particular body position

-Does it seem that your fingers, toes, hands or feet are curling?

35.29% yes, sometimes

64.71% no (20A – 27)

-muscle tightness

94.12% My muscle is tight when I want it relaxed

82.35% My muscle is moving when I want it still

5.88% My muscle is relaxing when I want it tight

11.76% My muscle is still when I want it moving

5.88% My muscle is silent, not responding (20A-6)

-walking

71.43% I can easily move around my own living room

33.33% I can easily go up two stairs without handrail or support

66.67% I can walk forward 100 meters without stopping

61.90% In can walk forward, stop and then restart easily

33.33% When I walk one foot does not easily follow the other one

4.76% When I walk, my foot feels like it might fracture (23-12)

4. whether it happens when active or still

-Does the dystonia happen when you are still and not trying to move?

3.35% It happens only when I am at rest

8.13% It happens only when I start to move

5.74% It happens only once I have been moving for a while

66.99% It happens always, when I am rest or moving (2A -4)

-Did the dystonia happen first only when you moved and then also when you

were at rest?

10.20% Yes, first only at rett and then also when moving

16.33% No, first when moving and then also when at rest

1.02% No, it has alwys only been when I was at rest

10.20% No, it has always only been when I am moving

42.86% No, it has always been when I am moving or at rest both (2B – 14)

-if motion changes the dystonia

36.36% Sometimes a body part freezes on me and I can’t move it for a second

27.27% My movements are a bit jerky but I never feel I freeze up

18.18% My movements are smooth

18.18% I can move well but I have trouble raising my head to see where I am going

54.55% Even if I use a can or walker I still might bump into things if I can’t

life my head to see straight ahead

18.18% I never have trouble bumping into things when I walk (24-6)

5. speed of motion – smoothness, drag, delay, 45 seconds to discomfort

-How would you describe most of your movements?
26.00% They are mostly smooth

37.50% They are often fragmentary and jerky but I accomplish what I try to do

15.50% They are fragmentary and incomplete (2A -10)

-speed and control

57.14% Some movements seem slower since dystonia

42.86% When I try to move there is sometimes a short delay

38,10% I make repetitive movements unintentionally

23.81% If I hold a position too long, my body seems to lock

76.19% I shift position often, partly to lessen pain

38.10% I can release easily from any finger or hand position

19.05% My speed of motion has not changed with dystonia (23-14)

-Are your movements slow sustained contractions?

27.50% Yet

27.00% Not sure

31.50% no (2A -12)

(This question was probably unclearly written)

-walking

0% When I walk I swing my arms easily

0% I can walk smoothly

100% I have unsteady gait

0% When I walk, my legs sometimes buckle

50% I prefer to touch railings and walls to keep my balance

0 % I fall frequently (33-3)

(This question had very few respondents)

6. tingles, skin crawling, electric sensation, zaps

-Do you feel a pulsing in the tight muscle?

21.46% Yes, often

18.54% Yes but only rarely

39.02% There is no pulsing feeling from it. (2A -9)

– Do you have tingling of some parts of your body?

23.24% Yes very often

33.51% Yes but only occasionally

2.16% Unsure

34.05% No (2A- 35)

-electric type sensations

30.43% I sometimes get electric type zaps in some parts of my body

26.09% The vibrations feel like a low volt curent buzzing near or inside me

21.74% I have thought I was near an electric outlet or my cellphone was ringing

with these vibrations

8.70% It feels like worms are crawling under my skin

52.17% I have not noticed any electric type zaps (23-6)

7. spasm, jerk, tremor, tic, shake, pulsing, zaps

-Do you have body jerks, spasms, tremors, involuntary movements?

47.52% Yes, nearly constantly

40.59% Yes, occasionally

11.88 No (1-2)

-tics, sudden tiny movements

18.82% I have no little tics

31.18% I have occasional little tics

19.35% I have a lot of little tics

26.34%The tics seem like sudden tension of the muscles

16.13% The tics cause me pain

13.44% The tics cause me no pain

3.76% The tics give me a brief sensation of relief

39.25% The tics are sudden and unpredictable

27.96% There are more tics when I am excited or under stress

6.45% The tics are slow and sustained. They last a wile

16.13% The tics are very brief (2A-34)

-tremor and spasms

24.59% The movements are large, jerky and irregular

15.85% The movements are large, jerky but regular

12.57% The movements are slow and irregular

15.85% The movements are slow and regular

15.30% unsure

15.85% Not applicable ( 2A- 37)

(this question seems to have lacked clarity. )

-tremors, spasms

12.43% I have no tremors

22.70% I have a lot of tremors

28.11% I have occasional tremors

32.97% I have tremors in the hands

18.38% I have tremors in the legs

39.46% I have head tremors

10.81% My tremors are worse when I am sick with a fever

14.05% My tremors appear when I try to move from a dystonic position

to a more normal position

5.95% My tremors stop after a while once I get to a new body position

21.08% My tremors are intense as I try to hold a normal body position

8.11% My tremors are intense but a relief when I stop trying to

hold a normal body position

22.70% I have different types or speeds or tremors (2A -36)

-tremor

43.75% My tremors happen on their own. I do not control them at all

6.25% My tremors happen on their own but I can hold tight and stop them at will

25.00% My tremors happen nonstop or nearly nonstop

18.75% My tremors happen just briefly but stop on their own

18.75% My tremos feel like jerks of fatigue as the muscle is stretched but tired

0% My tremors feel good sometimes, like a shudder a bad experience is over

50.00% My tremors do not feel good in any way

25.00% It feels like the muscle is contracting but just out of sync

12.5% unsure

12.5% I have tremors but the questions don’t match my situation

18.75% I have no tremors (20A- 8)

-spasms and tremor

15.00% I have no tremors, shakes, tics, spasms

25.00% Tremors happen when I try to move from dystonic to normal position

35.00% Tremors happen after I tried to move to normal position and my body

settles back to dystonic position

35.00% I have involuntary jerks like sleep jerks but in the daytime

50.00% I sometimes have involuntary rhythmic head shaking

45.00% I have occasional muscle twitches in shoulder, arms, finger, calf or foot

20.00% My body makes occasional quick involuntary mvoements like hiccups of

the body

20.00% I have involuntary writhing of the body

25.00% When I try to move the dystonic muscle, the opposite side muscle

has a tremor (23-19)

-tremor

25.00% The tremor starts before I start to move, as I like up to start

6.25% The tremor happens when I start to move

18.75% The tremor happens only after I’ve been moving for a while

31.25% The tremor happens only when I try to hold a new uncomfortable position

6.25% The tremor happens only after I’ve ended trying to move a muscle, as if

relief to stop trying

12.50% I have no tremor (20A -23)

-As you try to get a muscle to move, does the muscle respond not smoothly but with excitability and jerks?

73.33% yes

26.67% no (20A- 10)

8. clicks

-audible sounds of muscles clicking

27.07% My muscles do not make click sounds different from usual

18.23% I can often hear a click when I move my dystonic muscle

12.15% I hear more clicks from the dsytonia muscle side

10.50% I can sometimes hear 3 or 3 slightly different clicks from that muscle

1.10% Even though I don’t have dystonia in my hands or toes I can often

hear more clicks when I move the hands, toe on the dystonic side

9.39% Hearing the click seems odd like I am a popcorn popping machine

7.18% I am not sure if other can hear the clicks form my body

22.10% I am unsure about if my body makes clicking sounds (2A -40)

9. reaction time

-Have you noticed a slight delay before you are able to do some motions?

43.24% yes

56.76% no (19-63)

-speed of response when you want to move

42.86% I decide to move and then move and there is no delay

29.59% There is sometimes a slight delay before my body starts to move

16.84% There is often a delay before my body starts to move (2A-18)

-delay in reacting

47.06% There is a delay between my asking the muscle to move and it moving

11.76% The delay seems to happen as I align to get ready to move

29.41% The delay seems to happen after the firing gun I as try to move

but take longer than I used to

41.18% My reaction time is faster than usual to sudden noises

35.29% My intentional motion time is slower than before dystonia (20A- 13)

-Does it feel like your reflexes are overly active?

18.42% Yes, nearly always

28.95 % Yes sometimes

52.63% No (19-60)

-Do you jump more than you used to at sudden noises?

43.24% Yes

56.76% No (19-64)

I. Analysis

There is wide variability in the experience of muscle control. However some of the ‘no’ responses may be due to people with some forms of dystonia answering questions about other forms. A better survey design would differentiate groups more efficiently.

dizziness

-Dizziness does not seem a key component of dystonia but wobbliness may be

Only 13.07% report that the room seems tilted or the body seems tilted 11.06% report that the room seems to be spinning. This suggests that the patient’s perception of position of objects and themselves is not impaired and that only their muscle control is the challenge. 82.61% report they are aware of when their head or body are tilted.

heaviness

The feeling of heaviness like concrete was reported by over half of respondents with 58.83% saying they had this heavy full feeing sometimes or often.

comfort sitting and standing

About half of respondents prefer to have some support when standing.

Only 37.5% report they can sit straight comfortably. The experience of leaning on something for support is mixed. 37.5% say they feel best when they can lean their head against a chair back and 25% say they like to hold onto an armrest for balance . 20.83% say they have trouble leaning their head against a chair back. It is not clear if this trouble is due to pain, or problem getting into a leaning position or if it is a technical one that most chair backs are not high enough to permit such leaning. Some car seats have an indent in the head rest so that head is held more upright than reclined, which for people with dystonia may cause greater pain than were it more angled to partly recline.

balance

20.83% report they have excellent balance. The wobblines reported by 41.67% on waking from sleep does seem to resolve itself indicating that the body is able to resync the motion. This suggests that the dystonia message is being adapted to by the brain.

range of motion

Loss of range of motion seems a problem with only 9.19% saying they still have full range of motion with ease. Only 18.37% report full range though some positions are uncomfortable. 62.76% report loss of some range of motion or significant loss of range of motion. There are hypotheses that the loss of range of motion is due to the now shortening and tightness of the dystonic muscle where it has lost the ability to move into the more normal position. This hypotheis of muscle atrophy through underuse or under- exercise seems common. However two factors may question this hypotheis One is in cases where treatment seems to restore normal body position so the muscle was still able to make the motion after all. The second is in the case of sensory tricks where suddenly the muscle which seemed no longer able to have a normal length or position suddenly can and with ease.

voluntary or involuntary control

The discussion of whether dystonia is involuntary motions, subconscious but somewhat voluntary, or fully voluntary is unclear from these surveys. It is commonly said in medical literature that dystonia is involuntary muscle contractions. That statement seems supported by many of the survey questions. 88.89% said that the dystonia was pushing them into involuntary positions or movements.

36.45% reported involuntary sudden jerky movements

31.53% reported involuntary repetitive movements

36.45% reported involuntary twisting movements

24.63% reported involuntary pressure to hold a position.

However when the question is asked differently, some patients feel there is a voluntary aspect of their body trying to adapt to the dystonia or respond to it.

94.12% said they feel like their body is working voluntarily against the dystonia message.

58.82% say that dystonia seems a blend of voluntary and involuntary movements?

36.95% say most of th ir movements are voluntary

8.37% say all of their movements are voluntary

Movement and position seem different aspects of muscle control. 94.12% say their muscle is tight when they want it relaxed. 82.35% say their muscle is moving when they want it still. Only 35.29% said their fingers, toes, hands or feet were curling (one assumes involuntarily) These responses suggest that dystonia is a positional disorder.

still or motion activated

On the question of whether dystonia is motion activated or also happens when at rest. answers varied. 42.86% report that it happens both when still or moving.

8.13% say it starts when they start to move. 5.74% say it happens only once they have been moving for a while. Dystonia seems linked to delayed response to the command to move but eventually the command to move overrides the delay. However where dytonia only starts once there is motion or when there has been motion for a while, this suggests the opposite- that dystonia kicks in to override the normal body motion message.

speed of motion

The speed of overall motion of those with dytsonia seems lower.

Since any continuous motion like walking is a series of many small motion commands, a delay of each of them may slow motion overall. 57.14% report that some movements seem slower since dystonia and 42.8%-43.24% -46.43 % -47.06% report there is sometimes a short delay before they move.

reaction time and reflexes

Though reaction time to move seems slowed, reflex time or startle reflex seems often enhanced. 47.37% report their reflexes are sometimes or nearly always overative and 43.24$ say they jump more than they used to at sudden noise. Patients sometimes report that their body jumps extremely to hear a dog bark even though they know rationally that it is their dog and there is no real crisis. This disconnect between what the body is considering crisis and what the mind considers crisis is a source of mystery to such patients. Some patients report that watching an exciting chase scene on TV, knowing there is no real crisis to their lives, they find their body tensing up so much with excitement that it is physically uncomfortable to watch the show. The body’s overreaction seems an exaggeration of a normal response or a combination of normal excitement tension plus dystonia. Patiens report that hough their bodies seem tense they feel emotionally much less tense at such movies than their body seems to convey.

other sensations in the muscle

Reports of experiences of tingling, electrical zap sensations, pulsing are inconsistent. These are part of the dystonia experience for some patients but not for all.

56.75% report they have tingling occasionally or often

40% report they have pulsing in the muscle rarely or often

30.43% report they have electric type zaps

The terms for tremor, jerks, spasms may themselves be imprecise and cause confusion.

88.11% report they have body jerks, spasms, tremors or involuntary movements occasionally or nearly constantly.

50.81% say they have occasional or a lot of tremors and only 12.43% say they have no tremors

18.82 % say they have ‘no’ little tics and 50.3% say they have occasional or a lot of little ticks

The phenomenon of body clicks on the dystonic side is far from negligible but does not seem a universal experience with dystonia.