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Segmental
Dystonias |
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Segmental
Dystonias affect two adjoining parts of the
body: |
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Hemidystonia
affects an arm and a leg on one side of the
body. |
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Multifocal
dystonia affects many different parts of
the body. |
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Generalized
dystonia affects most of the body,
frequently involving the legs and back.
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Treatment |
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Treatment has
been limited to minimizing the symptoms of the
disorder as there is yet no successful
treatment for its cause. |
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Reducing the
types of movements that trigger or worsen
dystonic symptoms provides some relief, as
does reducing stress, getting |
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plenty of rest,
moderate exercise, and relaxation techniques.
Various treatments focus on sedating brain
functions or blocking nerve |
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communications
with the muscles via drugs, neuro-suppression
or denervation. All current treatments have
negative side effects |
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and risks. |
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Physical
intervention |
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Physical therapy
can sometimes help with focal dystonia. A
structured set of exercises is tailored to
help the affected area. |
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Some focal
dystonias have been proven treatable through
movement retraining in the Taubman approach,
particularly in the case |
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of musicians.
However other focal dystonias may not respond
and may even be made worse by this treatment. |
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Medication |
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Different
medications on tried in an effort to find a
combination that is effective for a specific
person. Not all people will respond well |
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to the same
medications. Medications that have had
positive results in some include:
diphenhydramine, benzatropine, anti-Parkinsons |
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agents ( such as
trihexyphenidyl), and muscle relaxers (such as
diazepam). |
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Anticholinergics |
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Medications such
as anticholinergics (benzatropine), which act
as inhibitors of the neurotransmitter
acetylcholine, may provide |
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some relief. In
the case of a acute dystonic reaction,
diphenhydramine is sometimes used (though this
drug is well known as an |
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antihistamine,
in this context it is being used primarily for
its anticholinergic role.[2] In the case of
Oculogyric crisis, diphenhydramine |
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may be
administered with excellent results with
symptoms subsiding in a matter of
minutes.[citation needed] |
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Muscle
relaxants |
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Clonazepam, an
anti-seizure medicine, is also sometimes
prescribed. However, for most their effects
are limited and side effects like |
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mental
confusion, sedation, mood swings and
short-term memory loss occur. Botulinum toxin
injections into affected muscles have |
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proved quite
successful in providing some relief for around
3–6 months, depending on the kind of dystonia.
Botox injections have the |
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advantage of
ready availability (the same form is used for
cosmetic surgery) and the effects are not
permanent. There is a risk of |
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temporary
paralysis of the muscles being injected or the
leaking of the toxin into adjacent muscle
groups causing weakness or |
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paralysis in
them. The injections have to be repeated as
the effects wear off and around 15% of
recipients will develop immunity to |
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the toxin. There
is a Type A and Type B toxin approved for
treatment of dystonia; often those that
develop resistance to Type A |
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may be able to
use Type B.[10] |
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Parkinsonian
drugs |
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Dopamine
agonists: One type of dystonia,
dopamine-responsive dystonia, can be
completely treated with regular doses of
L-DOPA |
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in a form such
as Sinemet (carbidopa/levodopa). Although this
doesn't remove the condition, it does
alleviate the symptoms most of |
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the time. (In
contrast, dopamine antagonists can sometimes
cause dystonia.) |
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Baclofen |
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A baclofen pump
has been used to treat patients of all ages
exhibiting muscle spasticity along with
dystonia. The pump delivers |
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baclofen via a
catheter to the thecal space surrounding the
spinal cord. The pump itself is placed in the
abdomen. It can be refilled |
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periodically by
access through the skin. |
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Surgery |
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Surgery, such as
the denervation of selected muscles, may also
provide some relief; however, the destruction
of nerves in the limbs |
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or brain is not
reversible and should only be considered in
the most extreme cases. Recently, the
procedure of deep brain stimulation |
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(DBS) has proven
successful in a number of cases of severe
generalised dystonia.[12] DBS as treatment for
medication-refractory |
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dystonia, on the
other hand, may increase the risk of suicide
in patients. Unfortunately, reference data of
patients without DBS |
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therapy are
lacking. |
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Signs Symptoms |
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Hyperglycemia-induced involuntary movements
which, in this case, did not consist of
typical hemiballismus, but rather of
hemichorea |
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(dance-like
movements of one side of the body; initial
movements of the right arm in the video) and
bilateral dystonia (slow muscle |
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contraction in
legs, chest and right arm) in a 62-year-old
Japanese woman with type 1 diabetes.Symptoms
vary according to the |
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kind of dystonia
involved. In most cases, dystonia tends to
lead to abnormal posturing, particularly on
movement. Many sufferers |
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have continuous
pain, cramping and relentless muscle spasms
due to involuntary muscle movements. Other
motor symptoms are |
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possible
including lip smacking. |
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Early symptoms
may include loss of precision muscle
coordination (sometimes first manifested in
declining penmanship, frequent small |
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injuries to the
hands, and dropped items), cramping pain with
sustained use and trembling. Significant
muscle pain and cramping may |
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result from very
minor exertions like holding a book and
turning pages. It may become difficult to find
a comfortable position for arms |
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and legs with
even the minor exertions associated with
holding arms crossed causing significant pain
similar to restless leg syndrome. |
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Affected persons
may notice trembling in the diaphragm while
breathing, or the need to place hands in
pockets, under legs while |
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sitting or under
pillows while sleeping to keep them still and
to reduce pain. Trembling in the jaw may be
felt and heard while lying |
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down, and the
constant movement to avoid pain may result in
the grinding and wearing down of teeth, or
symptoms similar to TMD. |
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The voice may
crack frequently or become harsh, triggering
frequent throat clearing. Swallowing can
become difficult & accompanied |
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by painful
cramping. |
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Electrical
sensors (EMG) inserted into affected muscle
groups, while painful, can provide a
definitive diagnosis by showing pulsating |
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nerve signals
being transmitted to the muscles even when
they are at rest. The brain appears to signal
portions of fibers within the |
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affected muscle
groups at a firing speed of about 10 Hz
causing them to pulsate, tremble and contort.
When called upon to perform |
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an intentional
activity, the muscles fatigue very quickly and
some portions of the muscle groups do not
respond (causing weakness) |
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while other
portions over-respond or become rigid (causing
micro-tears under load). The symptoms worsen
significantly with use, |
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especially in
the case of focal dystonia, and a "mirror
effect" is often observed in other body parts:
use of the right hand may cause |
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pain and
cramping in that hand as well as in the other
hand and legs that were not being used.
Stress, anxiety, lack of sleep, |
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sustained use
and cold temperatures can worsen symptoms. |
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Direct symptoms
may be accompanied by secondary effects of the
continuous muscle and brain activity,
including disturbed sleep |
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patterns,
exhaustion, mood swings, mental stress,
difficulty concentrating, blurred vision,
digestive problems and short temper. |
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People with
dystonia may also become depressed and find
great difficulty adapting their activities and
livelihood to a progressing |
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disability. Side
effects from treatment and medications can
also present challenges in normal activities. |
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In some cases,
symptoms may progress and then plateau for
years, or stop progressing entirely. The
progression may be delayed |
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by treatment or
adaptive lifestyle changes, while forced
continued use may make symptoms progress more
rapidly. In others, the |
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symptoms may
progress to total disability, making some of
the more risky forms of treatment worth
considering. |
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An accurate
diagnosis may be difficult because of the way
the disorder manifests itself. Sufferers may
be diagnosed as having similar |
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and perhaps
related disorders including Parkinson's
disease, essential tremor, carpal tunnel
syndrome, TMD, Tourette's syndrome, or |
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other
neuromuscular movement disorders. |
