Eyes
A. Context
The particular challenges of those with blepharospasm are surveyed here. Some people have this one type of dystonia. When the involuntary muscle spasms and contractions of the muscles around the eye are accompanied by involuntary and sometimes forceful contractions of muscles of the jaw and tongue, those who have meige syndrome diagnosis may also be responding to this survey.
Patients with other forms of dystonia sometimes report occasional eye involvement. Difficulty looking straight ahead can result in seeing objects from a tilted angle or a delay in the adjustment of the eyes to a new head position.
B. Timeline
1887- Horatio Wood, neurologist in Pennsylvania describes dystoniaof the eyes and in the jaw
1900- French neurologist Henry Meige studies a condition in ten patients with jaw oddities and eyelid closure. Meige at first believed patients lacked ‘psychical equilibrium’, and suffered from melancholic temperament and emotional stress but in 1910 found evidence that there was an actual change in the activity of the midbrain in the basal ganglia.
1972 – Neurologist Dr. George Paulson studied jaw and eye dystonia in 3 patients in the US
1980 Dr. Alan Scott, a San Francisco ophthalmologist found that if a patient had eyes looking different directions, strabismus, (crossed eyes), one way to help them was to cut away the muscle that pulled the eye to one side. However this destroyed the muscle and he found that another way to fix the problem was to inject botulinum toxin to temporarily weaken that muscle. He proposed injecting botulinum toxin in the eyelid muscles of people with blepharospasm. This weakened but did not destroy the muscles around the eye.
1989- the US Food and Drug Administration approves use of botulinum toxin Botox BT-A for treatment of eye dystonia
C. What seems useful to study
Clamping shut of eyelids is not difficult to identify but slight delay in opening or closing eyes may be more subtle. Ability to focus the eyes is a separate concern. Vision is affected by fatigue so effects of dystonia may be difficult to isolate since patients with dystonia often report exhaustion and problems sleeping. Vision tends to deteriorate with age so for seniors with dystonia, it may be unclear which changes in vision are due to dystonia and which ones are due to ageing.
Some anti -anxiety medications and anti-depressants can cause blurred vision and those medications are frequently prescribed for those with dystonia.
Many people with dystonia report dizziness. Blurred vision may make a person feel dizzy, or the dizziness may be due to balance problems not related to vision. Many medications have a side effect of dizziness. The study of eye involvement and dystonia therefore is complex and the surveys look for patterns only.
Patients with cervical dystonia report that sometimes to enable a sensory trick such as lifting the head, they squint. Those in pain report that they sometimes cope by wincing, squinting nearly by reflex. It is unclear whether these two phenomena are more common in dystonia but there may be a link worth examining.
Those whose heads tilt report they are often less aware of objects on one side. Those whose heads tilt up may be able to only take occasional glimpses of what is at their feet. Those whose heads tilt down may not easily notice low hanging branches, and often report trouble seeing to go up stairs. In these ways though cervical dystonia does not affect the eyes directly, it may impair vision of the environment.
Patients with vision problems report they are sometimes perceived by the public as if they also cannot hear or process ideas. Some report that strangers immediately talk over them, talk to their companion not them, or who speak slowly and in babyish simplicity when addressing them.
D. Comments from patient experience
first signs
At first. when I had trouble focusing. I thought I just needed glasses
I felt like I had to screw up my eyes and tighten my face muscles all the time
I had eye and nose twitches
I noticed I was blinking a lot and my eyes screwed up a lot
I thought maybe I had an ingrown eyelash
My eyes began to twitch and close
My eyes opened with a lot of squinting and I could not talk well
My eyes were very heavy
One day I suddenly could not see
problem opening eyes or keeping eyes open
I could only hold my eyes open for a few seconds
With no warning my eyes close and I have to work very hard to get them to re open
My eyes just randomly close
I literally cannot open my eyes for any task that is so easy I could do it with my eyes closed.
problem closing eyes or keeping eyes closed
It was hard to close my eyelids
twitches, blinking, spasms
I have no control over the blinking. It’s like hiccups. They just happen
My eyes started twitching a lot
People might think I’m winking at them
The eye muscles felt irritated and I got exhausted from the spasms
eye floaters
My eye floaters make me think I have a bug inside my glasses
My eye floaters are like dust flying all over the place
My eye floaters are creepy, like the walls are crawling
My eye floaters freak me out
impact on daily life
I am not blind but it seems like a type of blindness
It is hard to pour hot tea and dangerous
It is hard for me to focus on the TV screen
My eyes can go into spasm when I use a computer screen
When I tried to talk, the eye problem got worse
treatment
I tried putting ice packs on my eyes to reduce the spasms
I am grateful for the doctor who spotted my problem and acted quickly.
coping
I don’t wear eye make up any more
I now wear dark wrap-around sunglasses outdoors because light triggers the dystonia
Lately I wear dark sunglasses indoors because mall light can give me headaches
When I use a computer I set a dark background with white lettering
E. Source of question ideas:
patient reports. clinical studies, biographies
F. Question categories
symptoms
coping
dryness
gaze
med treatment
tricks
G – Questions asked
surveys 5 and 27
H. Results
eyes
5 45 eyes 15 22 81 3
27 3 eyes 7 7 80 1
max no. respondents 45
total questions 29
likely type of dystonia – blepharospasm, cervical possibly some others
percent of all respondents doing survey 45 of 508 or 8.8%
percent of this type of dystonia in population
challenges to do this survey – eye problems would make it hard to see the survey
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. first sign
-What were the first signs of dystonia in your eyes?
20.93% My eyes felt gritty
18.60% I had a drooping eyelid
25.58% I started blinking a lot
27.91% I had trouble opening my eyes
0% I had trouble closing my eyes ( 5-10)
2. symptom progression
-eye problems progressing
4.65% My eye problem has not changed over time
9.30% The problem started with a drooping eyelid and then it got
harder to keep the eye open at all
25.58% The problem started with extra blinking and then it got harder
to keep the eye open at all
11.63% The problem started in one eye and the moved to also affect the other
2.33% The problem started in one eye and then got better but then
it happened in the other eye
13.95% unsure
46.51% not applicable (5-11)
3. symptoms
-symptoms
0% My eyes appear normal in size
0% When open, my eyes are in normal position
66.67% One of my eyelids droops
33.33% Both of my eyelids droop
33.33% One of my eyes flutters involuntarily
33.33% When I open my eyes there is a delay in one or both of them
0% If is hard for me to open my eyes at all
0% When I close my eyes there is a delay in one or both of them
0% It is hard for me to close my eyes at all
33.33%I can open and close my eyes easily
0% I sometimes have eyebrow spasms (27-1)
(small number of respondents only for this question)
4 opening or closing eyes
-What happens when you try to keep your eyes open for a few seconds?
41.46% There is no difficulty
12.20% There is slight pain
0% There is intense pain
12.20% There is shaking and tremor
21.95% I have sometimes used my fingers to try to keep my eyes open (5-14)
-opening your eyes
13.64% When I open my eyes, there is a delay for one of them to open
11.36% When I open my eyes, there is a delay for both of them to open
18.18% I can easily open my eyes within ten seconds of being asked to
13.64% If I blink, there is a delay before my eyes open again
4.55% It is difficult to open my eyes at all
11.36% It is difficult once my eyes are open to keep them open
11.36% When I open my eyes I can open them fully so they are wide open
20.45% When I open my yes they only get half open
54.55% My eyes open normally (5-2)
-closing your eyes
35.56% Since dystonia I more often close my eyes just for comfort
24.44% My eyes during the day are sometimes half closed
28.89% It is sometimes hard for me to keep my eyes open
11.11% It is always hard for me to keep my eyes open
11.11% When I close my eyes they are often squeezed shut
11.11% My eyes gently close on their own
31.11% My eyes close forcibly and involuntarily
37.78% My eyes close normally and with only normal frequency (5-1)
5. blinking, squinting
– blinking
44.19% I blink my eyes the same way and amount as normal
30.23% I blink more often since dystonia
18.60% My blinking is severe
16.28% I have involuntary winking
16.28% My eyes twitch uncontrollably
11.63% I have been told I have a tremor in my eyes (5-3)
-Some drugs can produce eye blinking. Has this been ruled out in your situation?
28.21% Yes
17.95% No
5.13% unsure
48.72% not applicable (5-19)
6. dryness, sand, grit
-These questions ask about eye comfort
38.64% My eyes often feel sandy and gritty
56.82% My eyes often feel dry
29.55% My eyes water more often since dystonia (5-6)
-eye condition
33.33% Since dystonia my eyes feel dry more often
0% Since dystonia my eyes feel watery more often
0% My eyes have not changed dryness since dystonia
33.33% Since dystonia I get more light flashes that dance around the room
(27-3)
(few respondents answered this question)
7. eyebrow control
-eyebrow control
69.77% I can raise my eyebrows at will
4.65% One of my eyebrows goes up involuntarily
16.28% I purse my eyebrows more often since dystonia
16.28% To keep my eyes open I often raise my eyebrows (5-4)
8. diagnosis
-Did the doctors rule out that you might just have conjunctivitis?
28.21% Yes
10.26% No
10.26% unsure
51.28% not applicable (5-20)
-Were your eyes examined to rule out a brown or green ring of copper deposits around the cornea?
15.79% Yes
18.42% No
39.47% unsure
26.32% not applicable ( 5-21)
9. gaze and vision
-vision
40.91% Both of my pupils are equal in size
31.82% Both of my pupils react normally to light
38.64% My vision has not changed due to dystonia
29.55% My vision has changed since dystonia
13.64% I sometimes have double vision if I look at things from certain angles
4.55% Sometimes the room looks suddenly sideways (5-8)
-These questions ask about effects if your eyes are shutting more often.
23.81% I can still see what is going on around me
11.90% I sometimes miss seeing vital parts of an event I’m watching
11.90% I am a little slower to notice when stargners appear or disappear from view (5-16)
-gaze
33.33% When my eyes are open I can see straight ahead well
33.33% My gaze is off to one side
0% Since dystonia my vision is sometimes double
0% I have problems looking to the left
66.67% I have problems looking to the right
0% I have problems looking up
0% I have problems looking down
0% If I move my eyes fast, they cross
100.00% If I move my head fast, I feel dizzy for a second
0% If I move my yes fast, my head drops down
0% When I turn my head it takes a moment for my eyes to catch up (27-2)
(few people responded to this question)
10. daily actions
-Has dystonia changed your ability to do daily tasks?
23.08% I can still read a book or newspaper
23.08% I can still use the computer
23.08% I can still watch TV and see the screen
41.03% My eyes get tired more easily when I read, watch TV or
use the computer
10.26% My vision is fine but I often can’t open my eyes well enough to see
(5-13)
11. coping / sensory tricks
-Are there things that you can do that make the dystonia less?
19.51% The dystonia is less when I look downward
7.32% The dystonia is less when I pull on an upper eyelid
2.44% If the eyelid is closed, if I manually open it, it then is
easier to keep it open
12.20% My eyes close less when I hum
24.39% My eye spasms don’t happen when I sleep
9.76% Applying slight pressure on my eyelids relieves the problem briefly
12.20% The dystonia is less when I paint
4.88% My eyes close less when I sing
0% My eyes close less when I yawn
14.63% There are other activities I do that reduce the dystonia
4.88% The dystonia is always the same and nothing I do changes it
4.88% unsure
51.22% not applicable ( 5-17)
-Things you do to cope with the eyesproblems
33.33% I sometimes use one hand to prop my eye open
66.67% The dystonia is less if I massage my cheek bone
0% The dystonia is less if I over one eye
0% The dystonia is less if I pull on my upper eyelid
0% The dystonia is less if I pull on the eyebrow
0% The dystonia is less if I just touch my eyelid
0% The dystonia is less if I look downward
0%\ The dystonis is less if I touch my forehead
0% The dystonia is less if I touch my chin
33.33% The dystonia is less if I touch my nose
33.33% The dystonia is less if I touch beside the side of the eyelid (27-4)
(only a few people responded to this question)
12. triggers, attacks
-Is sunlight hard on your eyes?
20.45% No, sunlight does not bother my eyes any more than it did
before dystonia
20.45% Yes, sunlight is a little harder on my eyes now
38.64% Yes, sunlight is very hard on my eyes now
31.82% Any bright light seems to trigger my dystonia (5-7)
-light and its effect on the eyes
45.24% It is very hard on my eyes to see headlights of an approaching car
16.67% Variable light in a room, with areas of darkness and areas of light, sets
off my dystonia
30.95% My dystonia is less intense when the room is dimly lit
9.52% Light and darkness do not seem to affect my dystonia ( 5-15)
-attacks of dystonia in the eyes
36.59% I sometimes have attacks of when the eye dystonia is worse
7.32% The eye dystonia is constant and there are not periods when it is worse
4.88% The eye distonia is occasional and mild only
7.32% Before an attack I see an aura
2.44% Before an attack I have a visual disturbance
4.88% unsure
51.22% not applicable (5-12)
13. medical treatment
surgery
45.95% I have never had surgery for my eyes, eyebrows or eyelids
2.70% I have had eye surgery
2.70% I have had surgery to remove part of the muscle in my eyebrow
8.11% I have had surgery to remove part of the muscle in my eyelid
2.70% After surgery my eyes opened and closed better
2.70% unsure
43.24% not applicable (5-22)
-treatments
33.33%I had botulinum toxin injections and they helped
33.33% I had botulinum toxin injections and they did not help
0% I had eyelid surgery and it helped
0% I had eyelid surgery and it did not help (27-7)
(low number of respondents to this question)
14. devices
-eyewear for dystonia
60.53% I wear eyeglasses or contact lenses and did so before dystonia
13.16% My eyeglass prescription has not changed since the dystonia
44.74% My eyeglass prescription has changed since the dystonia
31.58% I always have worn sunglasses in bright sunlight
44.74% I wear sunglasses more often since dystonia
2.63% I have always used goggles when swimming
2.63% I use goggles more when swimming, since dystonia
2.63% I have tried eye crutches on my glasses (5-18)
J. Analysis
These questions are intended for those with diagnosis of blepharospasm or any other dystonia related eye problem With the high rate of answers marking ‘inapplicable’ it appears that some respondents with dystonia in other body parts may have answered these questions based on some eye involvement but not necessarily official diagnosis of dystonia there. This means the results are not dependable for blepharospasm alone. There may be trends there for blepharospasm but a more differentiated question system would have been useful.
Patients with other areas of dystonia sometimes have some eye involvement. Meige syndrome is dystonia affecting eyes and jaw, tongue and sometimes neck, so some answering may have fit that category.
Answering an online written survey when a person has vision problems would be daunting. It is likely that those who did respond went to considerable trouble to do so, possibly getting help with reading. A survey of people with vision problems using spoken question and spoken response would likely be useful.
eye condition
33.33% – 56.82% reported dry eyes
25.58% -33.33% reported eye flutters, spasms, involuntary blinking or winking
20.93% -38.64% reported gritty eyes
0 – 29.55% reported watery eyes
9.30% – 18.60% reported drooping in one or both eyelids
seeing lights
33.33% reported getting light flashes that dance around the room
7.32% reported seeing an aura before a dystonia attack
2.44% reported having a visual disturbance before an attack
effect of light
59.09% reported that sunlight is a little harder or very hard on their eyes now
45.24% reported that it is very hard on their eyes to see headlights of
an approaching car
44.74% reported they wear sunglasses more often since dystonia
31.82% said any bright light seems to trigger their dystonia
The difficulties some report in watching TV or using a computer could have been studied more thoroughly to see if the problem was in opening the eye or from the screen being a light source difficult to look at.
41.03% report that their eyes get tired more easily when they read, watch TV
or use the computer
difficulty opening eyes – they tend to stay closed
31.11% report that their eyes close forcibly and involuntarily
28.89% report that it is sometimes hard to keep the eyes open
27.91% reported a first symptom as trouble opening the eyes
24.44% report that during the day their eyes are sometimes half closed.
11.11 % report that their eyes gently close on their own
difficulty closing eyes – they tend to stay open
0% reported as first symptom trouble closing the eyes.
(A separate study of orientations and dystonia, open or closed, left or right, bent or stretched is in a later chapter.)
sensory tricks
66.67% report the dystonia is less if they massage a cheek bone
33.33% report that it is less if they touch beside the side of the eyelid
33.33% report that it is less if they touch their nose
21.95%-33.33% report they sometimes use a finger or hand to try to prop the eye open
19.51% say the dystonia is less when they look downward
12.30% report it is less if they hum
12.20% that it is less when they paint
7.32% report it is less when they pull on an upper eyelid
4.88% report it is less when they hum
4.88% that it is less when they sing
vision itself
100.00% report that if they move their head fast, they feel dizzy for a second
66.67% report problems looking to one side
38.64% say their vision has not changed since dystonia
33.33% say their gaze is off to one side
29.55% say their vision has changed
13.64% say they sometimes have double vision
(this set of questions seems to have been answered by those with several types of dystonia not solely by those with eyelid involvement. A more clearly differentiated type of question linked to diagnosis would have been useful)
Lived Experience With Dystonia 