       |
HILARY STORER'S BATTLE WITH DYSTONIA
2005
Two years ago, I
awoke from an anaesthetic, having had an operation for a burst
appendix. Immediately, I found that I could not see the TV in the
ward or read. I was very light sensitive (photophobic) and had no
moisture in my eyes. My eyes blinked uncontrollably and I could
not keep them open. I was only comfortable when my eyes were kept
firmly shut or asleep.
The next three years were an expensive nightmare of visiting G
P’s, Homeopaths, Ophthalmologists, and Neurologists etc. in an
effort to find a proper diagnosis. I knew something was
drastically wrong with me, yet I was dismissed by one member of
the medical profession after another. One unsympathetic
ophthalmologist declared that I was “Old, had dry eyes and would
have to live with it!” I am in fact, middle aged, young at heart
and not prepared to give up on myself.
Weeks later, I realised that my eye condition
had deteriorated as I was blinking even more rapidly, had painful
spasms and my eyes would close tightly. By this time doctors,
family and friends thought there was something mentally wrong with
me. I knew what they were thinking and it made things worse. I
became reclusive and didn’t want to go out as I looked “funny”
with my twitches and blinks and falling over, because I couldn’t
see.
Out at a rare social occasion, I met Maureen and Steve who are on
the National Dystonia Committee. They suggested that I may have
Dystonia and that I should visit a Neurologist in Pretoria, as I
would require a proper diagnosis. It was a relief when the doctor
said that I have Dystonia, I could now put a name to my odd and
rare disease.
Dystonia
is a movement disorder and although the causes of dystonia are not
fully known it is currently thought that the condition results
from a malfunction in a part of the brain called the basal ganglia
I was treated with
Botulinum toxin injections, commonly known as Botox therapy, which
has become a very useful tool in the treatment of a number of
neurological disorders. Botulinum toxin is a protein substance
that is directly injected into muscle tissue in order to stop
abnormal muscular contraction. It has proven effective in
treating:-
-
Blepharospasm
- the involuntary forceful closure of the eyelids. Usually the
first symptom is uncontrollable blinking. Eventually the eyelids
remain completely closed all the time and even though the
patient’s vision may be normal they are functionally blind. When
accompanied by oromandibular dystonia, the condition is known as
Meiges syndrome. Injections of botulinum toxin into muscles in
the face and periocular region (around the eye) has become the
treatment of choice for patients with blepharospasm.
-
Hemifacial spasm
- the sudden, simultaneous contraction of the muscles on one
side of the face. The spasm can subside immediately or persist
for several seconds and can occur several times a day. It can be
painful and embarrassing. Botulinum toxin is an alternative
therapy to oral medication and surgery.
-
Spasmodic Torticollis
- is an asymmetric muscular spasm in the neck that results in
forceful turning of the head to one side. Additionally, the head
may be pulled forward or backward. It is the most common of the
focal dystonias (a dystonia is a state of abnormal muscle tone;
blepharospasm is another dystonia and, indeed, is the second
most common focal dystonia). Torticollis can accompany other
movement disorders and can be very painful.
-
Oromandibular dystonia
- involves continuous, bilateral (both sides) spasms of the
face, jaw, neck, tongue, larynx, and in severe cases, the
respiratory system. It’s usually treated with various
medications, although Botox therapy has proven effective for
certain cases. Because there are so many different muscles
involved, the therapy requires multiple injections.
-
Spasmodic dysphonia
– is the sudden interruption of speech due to a spasm of the
laryngeal muscles (the vocal cords). Botulinum toxin therapy has
proven effective at ameliorating the symptoms and restoring
speech fluency, although one kind of spasmodic dysphonia
(abductor spasmodic dysphonia) poses an airway obstruction risk
if certain muscles are injected.
-
Generalised Dystonia
–
The usual age of onset is between 5 and 16 years.
Parents or teachers may notice an abnormal turning in of the
foot, an awkward gait or contractions of many different muscle
groups. The involuntary dystonic movements may progress quickly
to involve all the limbs and torso, but the rate of progression
usually slows after adolescence. A genetic basis for generalised
dystonia has now been confirmed
With care and treatment from the
Ophthalmologist and the Neurologist I can see reasonably well
again, although I have "good" and "bad" days. I am however, unable
to drive, read or watch television without the aid of specially
made Blepharospasm Props (glasses with a prop to keep the eyes
from shutting).
I found invaluable support from the Dystonia
Association, South Africa and have subsequently become
their Vice-Chair and Newsletter editor. I can be contacted at
mailto:hilian@isat.co.za
|
