What is Parkinson's Disease?
Parkinson's disease (PD) belongs to a group of conditions called
motor system disorders, which are the result of the loss of
dopamine-producing brain cells. The four primary symptoms of PD
are tremor, or trembling in hands, arms, legs, jaw, and face;
rigidity, or stiffness of the limbs and trunk; bradykinesia, or
slowness of movement; and postural instability, or impaired
balance and coordination. As these symptoms become more
pronounced, patients may have difficulty walking, talking, or
completing other simple tasks. PD usually affects people over
the age of 50. Early symptoms of PD are subtle and occur
gradually. In some people the disease progresses more quickly
than in others. As the disease progresses, the shaking, or
tremor, which affects the majority of PD patients may begin to
interfere with daily activities. Other symptoms may include
depression and other emotional changes; difficulty in
swallowing, chewing, and speaking; urinary problems or
constipation; skin problems; and sleep disruptions. There are no
blood or laboratory tests available to diagnose PD.
Is there any treatment?
At present, there is no cure for PD, but a variety of
medications provide dramatic relief from the symptoms. Usually,
patients are given levodopa combined with carbidopa. Carbidopa
delays the conversion of levodopa into dopamine until it reaches
the brain. Nerve cells can use levodopa to make dopamine and
replenish the brain's dwindling supply. Although levodopa helps
at least three-quarters of parkinsonian cases, not all symptoms
respond equally to the drug. Bradykinesia and rigidity respond
best, while tremor may be only marginally reduced. Problems with
balance and other symptoms may not be alleviated at all.
Anticholinergics may help control tremor and rigidity. Other
drugs, such as bromocriptine, pergolide, pramipexole, and
ropinirole, mimic the role of dopamine in the brain, causing the
neurons to react as they would to dopamine. An antiviral drug,
amantadine, also appears to reduce symptoms.
In some cases, surgery may be appropriate if the disease doesn't
respond to drugs. A therapy called deep brain stimulation (DBS)
has now been approved by the U.S. Food and Drug Administration.
In DBS, electrodes are implanted into the brain and connected to
a small electrical device called a pulse generator that can be
externally programmed. DBS can reduce the need for levodopa and
related drugs, which in turn decreases the involuntary movements
called dyskinesias that are a common side effect of levodopa. It
also helps to alleviate fluctuations of symptoms and to reduce
tremors, slowness of movements, and gait problems. DBS requires
careful programming of the stimulator device in order to work
correctly.
What is the prognosis?
PD is both chronic, meaning it persists over a long period of
time, and progressive, meaning its symptoms grow worse over
time. Although some people become severely disabled, others
experience only minor motor disruptions. Tremor is the major
symptom for some patients, while for others tremor is only a
minor complaint and other symptoms are more troublesome. No one
can predict which symptoms will affect an individual patient,
and the intensity of the symptoms also varies from person to
person.
What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS)
conducts PD research in laboratories at the National Institutes
of Health (NIH) and also supports additional research through
grants to major medical institutions across the country. Current
research programs funded by the NINDS are using animal models to
study how the disease progresses and to develop new drug
therapies. Scientists looking for the cause of PD continue to
search for possible environmental factors, such as toxins, that
may trigger the disorder, and study genetic factors to determine
how defective genes play a role. Other scientists are working to
develop new protective drugs that can delay, prevent, or reverse
the disease.
Hemifacial Spasm
Hemifacial spasm affects one side of the face, while
blepharospasm and Meige syndrome affect both sides. Though these
disorders are quite different and have different causes, the
patients in their search for diagnosis and treatment generally
find the same physicians. Also, botulinum toxin injections are
frequently helpful in treating both maladies. Surgical
treatment, however, is quite different. Hemifacial spasm is
frequently relieved with a surgical procedure called
microvascular decompression in which an artery at the level of
the brain stem is rerouted to relieve pressure on the facial
nerve.
A Technical Description of Hemifacial Spasm
Hemifacial spasm is characterized by paroxysmal bursts of
involuntary tonic or clonic activity occurring in muscles
innervated by the facial nerve. Between spasms, the face may be
normal or slightly weak on the side of the spasms. The condition
occurs most often in middle-aged adults, with men and women
being equally affected, although I have observed it in two
teenagers. It usually begins with involvement of orbicularis
oculi muscles and simulates the typical eyelid myokymia that
affects most normal persons intermittently throughout life;
however, instead of resolving spontaneously after several days
to a few weeks, it subsequently spreads to the mid and lower
face over months to years. Hemifacial spasm generally persists
throughout life, gradually worsening over the years; however, I
have seen several patients who experienced spontaneous temporary
remissions of the condition that lasted years and others whose
remissions were permanent.
Hemifacial spasm usually is painless, but it can be associated
with ipsilateral facial pain. It may be exacerbated or activated
by cough, fatigue, or stress. The condition is present during
sleep and is often associated with both clinical and
electromyographic evidence of synkinesis (aberrant
regeneration).
The differential diagnosis of hemifacial spasm includes a wide
variety of other entities characterized by involuntary facial
movements, such as facial myokymia, which is limited to a small
area of the face; essential blepharospasm, which is always a
bilateral condition and confined to the eyelids and forehead
unless associated with Meige syndrome; Meige syndrome, which is
not only bilateral but is characterized by a different type of
spasms that are not synchronous; Tourette's syndrome, which is
only rarely unilateral and usually is associated with
purposeless vocalizations; spastic-paretic facial contracture,
in which the affected side of the face is truly paretic, rather
than slightly weak inferiorly, between spasms; and secondary
aberrant regeneration of the facial nerve following peripheral
facial nerve palsy, in which the history of a previous acute
seventh nerve palsy should suggest the correct diagnosis.
Hemifacial spasm is believed to result from irritation of the
seventh nerve at its exit from the brainstem. About 0.1-1% of
cases are associated with extra-axial lesions compressing the
seventh nerve, such as tumors (epidermoid, acoustic schwannoma,
meningioma), aneurysms, arteriovenous malformations, and
dolichoectatic basilar arteries. Even fewer cases are caused by
damage to the facial nerve by intrinsic brainstem lesions, most
often multiple sclerosis. Most cases, however, are associated
with (and possibly caused by--see below) a small artery (usually
a branch of the posterior inferior cerebellar artery) or a vein
that is compressing the seventh nerve.
Because of the small but definite probability that a patient
with hemifacial spasm has a mass lesion in the region of the
cerebellopontine angle or an infiltrating lesion in the pons,
all patients with hemifacial spasm should undergo magnetic
resonance imaging (MRI).
Cervical Dystonia
Cervical Dystonia - dystonia in the neck
What is dystonia?
Dystonia is a neurological movement disorder. It causes
involuntary muscle contractions which force the affected parts
of the body into abnormal movements or postures.
Cervical Dystonia
Cervical dystonia, sometimes known as spasmodic torticollis, is
a focal dystonia of the neck and typically occurs in people over
the age of 40. By causing neck muscles to contract
involuntarily, it produces abnormal movements and postures of
the neck and head.
The movements can lead to the head and neck twisting (torticollis)
or being pulled forwards (antecollis), backwards (retrocollis),
or sideways (laterocollis). Symptoms may vary from mild to
severe and the muscular spasms may result in pain and
discomfort. Sometimes, the condition may be partially relieved
by touching the chin, other parts of the face, or the back of
the head.
Though cervical dystonia may progress, it is not life
threatening and does not affect other brain functions such as
intellect, sensation, vision, bladder or sexual fundtion.
Cervical dystonia is unlikely to become generalised, although
occasionally another part of the body may be affected.
The condition varies from one individual to another. In some
cases it may progress for about five years and then gets no
worse. In other cases it hardly progresses at all. Occasionally,
it may remit as symptoms disappear, but may return at a later
date. Because every case of cervical dystonia is different from
every other, it is difficult to predict accurately how it may
change in the future.
What causes cervical dystonia?
Cervical dystonia is believed to be the result of abnormal
functioning of the basal ganglia, deep within the brain,
involved in the control of movement. Much research is currently
being undertaken and progress made towards a better
understanding of this abnormality.
To date, no cure has been found. Many drugs have been tried in
the treatment of cervical dystonia, but while some of these may
provide benefit for some individuals, none is universally
effective. These drugs may also produce side effects in some
people.
Botulinum toxin injections, which weaken the muscles affected by
spasm, are the most effective treatment. Injections need to be
repeated every three months or so. In cases where little
improvement results from the injections, it may be because they
have not been accurately targeted, or the dose needs adjusting,
or a different type of botulinum toxin is required.
Sometimes electromyography (EMG) is used to identify the
appropriate muscles to inject.
How do I live with cervical dystonia?
Cervical dystonia can be a stressful condition for some to cope
with, causing them embarrassment and a loss of confidence. When
one’s head is unnaturally turned to one side, forwards or
backwards, one’s ‘body language’ is affected, and other
people may misinterpret our interaction with them.
In addition, some people find that getting around is more
difficult, as it becomes more difficult to turn to look in one
direction or another: crossing the road, or walking through a
crowd, or driving, may become problematical.
Many find that sharing their concerns with other people does
help. Others with the condition may have useful tips for coping
on a day-to-day basis and it may be helpful to contact a support
group locally, or by telephone. In addition, friends, family and
colleagues will understand your needs if you can explain how the
dystonia affects you on a day-to-day basis.
BLEPHAROSPASM
Blepharospasm is an abnormal, involuntary blinking or spasm of
the eyelids. Blepharospasm is also called “benign essential
blepharospasm, hemifacial spasm”.
Causes of Blepharospasm
Blepharospasm is associated with an abnormal function of the
basal ganglion from an unknown cause. The basal ganglion is the
part of the brain responsible for controlling the muscles. In
rare cases, heredity may play a role in the development of
blepharospasm.
Symptoms of Blepharospasm
Most people develop blepharospasm without any warning symptoms.
It may begin with a gradual increase in blinking or eye
irritation. Some people may also experience fatigue, emotional
tension, or sensitivity to bright light.
As the condition progresses, the symptoms become more frequent,
and facial spasms may develop. Blepharospasm may decrease or
cease while a person is sleeping or concentrating on a specific
task.
Blepharospasm Treatment
To date, there is no successful cure for blepharospasm, although
several treatment options can reduce its severity.
Medications taken by mouth for blepharospasm are available but
usually produce unpredictable results. Any symptom relief is
usually short term and tends to be helpful in only 15 percent of
the cases.
Myectomy, a surgical procedure to remove some of the muscles and
nerves of the eyelids, is also a possible treatment option. This
surgery has improved symptoms in 75 to 85 percent of people with
blepharospasm.
Alternative treatments may include biofeedback, acupuncture,
hypnosis, chiropractic, and nutritional therapy. The benefits of
these alternative therapies have not been proven.
BOTOX TREATMENT FOR
MOVEMENT DISORDERS
The clinic is number One in providing Botolinum Toxin Treatment
for movement disorders i.e Blephorospasm, Hemifacial Spasm,
Spasticity, Writer's Cramp and other movement disorders. Dr.
Anurag Arora is a member of International Movement Disorder
Society.
WRITER'S CRAMP
What is it?
Writer's cramp is a task-specific focal dystonia of the
hand. Symptoms usually appear when a person is trying to do a
task that requires fine motor movements. The symptoms may appear
only during a particular type of movement, such as writing or
playing the piano, but the dystonia may spread to affect many
tasks.
Two types of writer's cramp have been described: simple and
dystonic. People with simple writer's cramp have difficulty with
only one specific task. For example, if writing activates the
dystonia, as soon as the person picks up a pen or within writing
a few words, dystonic postures of the hand begin to impede the
speed and accuracy of writing. In dystonic writer's cramp,
symptoms will be present not only when the person is writing,
but also when performing other-task specific activities, such as
shaving, using eating utensils, applying make-up.
Symptoms
Common manifestations of simple writer's cramp include excessive
gripping of the pen, flexion, and sometimes deviation of the
wrist, elevation of the elbow, and occasional extension of a
finger or fingers causing the pen to fall from the hand.
Sometimes the disorder progresses to include the elevation of
shoulders or the retraction of arm while writing. Tremor is
usually not a symptom of writer's cramp. The symptoms usually
begin between the ages of 30 and 50 years old and affect both
men and women.
Cramping or aching of the hand is not common. Mild discomfort
may occur in the fingers, wrist, or forearm.
A similar cramp may be seen in musicians as the violin is bowed,
in certain athletes such as golfers, or in typists.
If writer's cramp causes any type of impairment, it is because
muscle contractions interfere with normal function. Features
such as cognition, strength, and the senses, including vision
and hearing are normal. While dystonia is not fatal, it is a
chronic disorder and prognosis is difficult to predict.
Cause
Writer's Cramp is believed to be due to abnormal functioning of
the basal ganglia, which are deep brain structures involved with
the control of movement. The basal ganglia assists in initiating
and regulating movement. What goes wrong in the basal ganglia is
still unknown. An imbalance of dopamine, a neurotransmitter in
the basal ganglia, may underlie several different forms of
dystonia, but much more research needs to be done for a better
understanding of the brain mechanisms involved with dystonia.
Cases of inherited writer's cramp have been reported, usually in
conjunction with early-onset generalized dystonia, which is
associated with the DYT1 gene.
Treatment
Treatment for dystonia is designed to help lessen the symptoms
of spasms, pain, and disturbed postures and functions. Most
therapies are symptomatic, attempting to cover up or release the
dystonic spasms. No single strategy will be appropriate for
every case.
The goal of any treatment is to achieve the greatest benefits
while incurring the fewest risks. It is to allow you to lead a
fuller, more productive life by reducing the effects of dystonia.
Establishing a satisfactory regimen requires patience on the
part of both the affected individual and the physician.
The approach for treatment of dystonia is usually three-tiered:
oral medications, botulinum toxin injections, and surgery. These
therapies may be used in alone or in combination.
Complementary care, such as physical therapy and speech therapy,
may also have a role in the treatment management depending on
the form of dystonia. For many people, supportive therapy
provides an important adjunct to medical treatment.
Although there is currently no known cure for dystonia, we are
gaining a better understanding of dystonia through research and
are developing new approaches to treatments.
SPASTICITY
Spasticity is common in varying degrees after spinal cord
injury. Spasticity is a non-specific symptom, which may occur in
many problems associated with spinal cord injury.
Spasticity is the involuntary movement (jerking) of muscles,
which occurs because message can travel from parts of your body
to the spinal cord and cause reflex activity (muscle movement).
This is possible because the spinal cord has certain normal
automatic functions, which are under the influence of the brain.
These functions include muscle tone and reflexes. Most spinal
cord injured persons have a healthy, intact spinal cord below
the immediate area of their injury, and thus these automatic
activities can continue to exist. However, they are no longer
under the regulating influence of the brain and are thus
exaggerated. This is called spasticity.
Any sensory stimulus below the level of injury can cause
spasticity, for example, a change in body position (i.e.,
movement of an extremity), bladder irritation, pressure sores,
fractured bones or a bowel program.
Sometimes, minor degrees of spasticity may be helpful to you.
Due to the muscle movement, your circulation is improved and the
movement also helps to maintain the shape and bulk of your
muscles. However, if it is severe, spasticity can interfere with
functional activities such as transfers, weight shifts, gait
training, etc.
Some stimuli can cause a change in your spasticity. Anything
that would ordinarily be uncomfortable or painful can cause an
increase in your spasticity. If you experience a major increase
in spasticity, possible causes are:
* Skin problems - a skin sore or ingrown toenail
* Bladder problems - high residuals, infection or bladder stones
* Bowel problems - constipation, impactions or hemorrhoids
* Medical problems - viral syndrome (infection, influenza,
intestinal flu), heterotopic ossification or a spinal
cyst.
Although a change in the pattern of your spasticity does not
always indicate another problem, it is wise to check all
possibilities.
If your spasticity is severe and interferes with function, there
are several medications that may be of help -- Valium, Dantrium,
and Lioresal. A surgical procedure called a radiofrequency
rhizotomy is sometimes indicated in the treatment of severe
spasticity. If you have increased spasticity, which persists,
consult your physician.
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