What is Paroxysmal Dystonia?
humansbeing
from the April/May Dystonia Dialogue:
What is Paroxysmal Dystonia?
Paroxysmal Dystonia refers to relativly brief attacks of dystonic movements
and postures with a return to normal posture between episodes. It is a form
of paroxysmal dyskinesias.
The paroxysmal dyskinesias are movement disorders in which the abnormal
attacks are present only during the attacks. Between the attacks the person
is generally neurologically normal, and there is no loss of consciousness
during the attacks.
The paroxysmal dyskinesias are classified as action induced (kinesigenic)
or non-action induced (non-kinesigenic).
The most common and best recognized paroxysmal dyskinesias are paroxysmal
kinesigenic choreoathetosis (PKC) and paroxysmal dystonic non-kinesigenic
choreoathetosis (PDC), aka paroxysmal dystonia.
Paroxysmal kinesigenic choreoathetosis consists of debilitating attacks of
abnormal movements, startles, hyperventilation, and stress.
The movements may be coreothetoid (a nervous disturbance marked by
involuntary and uncontrollable movements characteristic of chorea and
anthetosis), with flowing, dancelike, or slow writhing features, or
dystonia with sustained turned or twisting movements. The attacks can even
cause the person to fall. Speech can sometimes be affected by dystonia, but
there is never an alteration of conciousness.
The attacks may vary in frequency but are generally brief, lasting seconds
to five minutes. There can be as many as 100 attacks per day. A refractory
period lasting about five minutes may occur after an attack, during which
it is not possible to induce another attack.
Approximately 50 percent of people with PKC experience a change in
sensation, such as tightness, numbness, pins and needles, or tingling
before the attack.
Paroxysmal dystonic choreoathetosis consists of debilitating attacks of
primarilly dystonic movements but can also involve choreoathetosis. The
attacks range in length from minutes to several hours and may occur up to
three or four times a day. Attacks in PDC can be precipitated by a number
of factors, including alchohol, caffeine, fatigue, stress, or excitement.
The attacks are often preceded by a sencory change such as tightness,
numbness, and pins and needles, or tingling in the affected region.
The majority of cases of PKC and PDC are idiopathic (of unknown cause) or
familial (either autosomal dominant or occasionally sporadic).
The paroxysmal dyskinesias are usually dominantly inherited, but this is
not always the case. When it is an autosomal dominant disorder, a child of
one affected parent has a 50 percent chance of having the disorder. A child
whose parents both have the autosomal dominant PKC or PDC will have a 75
percent chance of inheriting the gene for the disorder.
When it is sporadic (resessive) inherited disorder, parents who both carry
the disorder in their genes have a 25 percent chance of passing it on to
their children. If only one parent is a carrier of the sporadic disorder,
his or her child will have a 50 percent chance of becoming a carrier as
well but will not actually have the disorder. Even if one does have the
genes for this disorder, paroxysmal dystonia will not always express
itself.
Secondary cause of the paroxysmal dyskinesias are abnormal blood sugars,
overactive thyroid, parathyroid disorders, focal seizures, multiple
sclerosis, encephalitis, head injury, cerebral anoxia, local basil ganglia
or thalamic lesions (such as strokes and tumors), and psychogenic causes.
The most common conditions associated with symptomatic or secondary PKC are
multiple sclerosis and head injury, whereas those with symptomatic PKC are
multiple sclerosis, perinatal encephalopathy (i.e., due to anoxia), and
psychogenic causes.
Age of onset of idiopathic (of unknown origin) cases of PKC and PDC is
usually in childhood, between 6 and 16 years, although onset can range from
a few months to 40 years old. Secondary cases can occur at any age. PKC and
PDC occur more frequently in men than women.
The work-up for the paroxysmal dyskinesias can include an
electroencephalogram (brain wave test), brain image (such as MRI or CT
scan), blood chemistries, and calcium tests.
In the idiopathic forms, these tests are generally normal whereas in the
secondary forms brain lesions may be found.
What are the Current Forms of Treatment for Paroxysmal Dyskinesia?
Unfortunatly at this time, there is no cure for dystonia, but treatments
are available. Treatment is designed to help the symptoms of spasms, pain,
and disturbed posture and function.
The goal of any treatment, including that of paroxysmal dystonia, is to
achieve the greatest benefits while incurring the fewest risks.
Our poor understanding of the pathophysiology and biochemistry of PKC and
PDC makes development of specific therapies difficult. Establishing a
satisfactory treatments scheme requires patience on the part of both the
physician and the patient, and the treatment should be tailored to the
individual person.
Medication
People with paroxysmal kinesigenic choreoathetosis respond best to
anticonvulsants such as phenytoin, primidone, carbamazepine, phenobarbital,
and diazepam. People with paroxysmal dystonic choreoathetosis, however,
generally have poor responces to anticonvulsants. Benzodiazepines, such as
clonazepam and oxazepam, the carbonic anhydrase inhibitor, acetazolamide,
and the anticholinergics may be beneficial for PDC.
The intermittent and transient nature of the paroxysmal dyskensias usually
prevent the use of therapies like botulinum toxin injections and surgeries
in most people.
Supportive Forms of Treatment
Many people have reported that in a stressful situation, symptoms may
worsen. Unfortunately, it is not possible to get rid of all stress, but a
course of stress management or relaxation techniques is beneficial.
By educating youself with information, you have taken the first step in
dealing with dystonia. Reassurance from family, friends and others who have
paroxysmal dystonia is beneficial. Support groups offer encouragement,
camaraderie, and information about new treatments and medical advances. The
DMRF maintains a network of support groups throughout North America.
Though the exact cause of dystonia is unknown, continued research offers
that answers will be found.
Gene
On Wed, 27 May 1998 17:54:51 GMT, sa...@home.not (humansbeing) wrote:
>from the April/May Dystonia Dialogue:
>
>What is Paroxysmal Dystonia?
>Paroxysmal Dystonia refers to relativly brief attacks of dystonic movements
>and postures with a return to normal posture between episodes. It is a form
>of paroxysmal dyskinesias.
>
Thank you so much for having taken the time to type
and post this information to a.s.d. for all of us to read!
Bev was looking for exactly this material a few days ago.
Regards,
Gene
http://personal.nbnet.nb.ca/ev/
http://www.geocities.com/HotSprings/Villa/2803/
humansbeing
Hi Gene,
you are welcome. I'm sorry, I didn't notice your "thankyou" post 'till just
now,... it was my pleasure. -> I hope it helps...
Sunbear
-of The Great Piney Woods of East Texas-
[what's left of them, whatcha gonna do when the well runs dry?]
-who cares anyway, nobody'll see this, so why bother?- just another
"PIPE-DREAM"
@longview.net