Multiple System Atrophy (MSA) FAQ

Multiple system atrophy (MSA) is a rare neurodegenerative disease marked by a combination of symptoms affecting movement, blood pressure, and other body functions; hence the label "multiple system" atrophy.

According to the American Autonomic Society, Multiple System Atrophy (MSA) is a sporadic, progressive, adult-onset disorder characterized by autonomic dysfunction, parkinsonism and ataxia (a failure of muscular coordination) in any combination.

Broken down MSA stands for:

Multiple - many

System - brain structures that control different functions

Atrophy - cell shrinkage or damage

This means that cells are damaged in different areas of the brain that control different body functions. The three areas affected are the basal ganglia, cerebellum and brain stem. These areas are responsible for movement, balance and automatic body functions like bladder control.

Brain cells in the affected areas in MSA shrink (atrophy). This can sometimes be seen on MRI scans. When brain tissue is examined under a microscope, structures called glial inclusion bodies can be seen; they contain a protein called alpha-synuclein. It is the presence of these inclusion bodies in the movement, balance and automatic control centers of the brain that confirms a diagnosis of MSA.

The causes of MSA are unknown. It is still unclear as to exactly why the cells become damaged in MSA. MSA is not contagious. It does not appear to be inherited, although some research suggests there may be a predisposition within the genetic make-up of an individual for cells to become damaged.

What triggers the damage process to begin is unknown. Environmental toxins or a history of trauma have been suggested, but evidence for this as a cause is slight. This too is a focus of ongoing research.

MSA usually starts between the ages of 50-60 years, although it can affect people younger and older than this. The mean age of onset is 54. MSA usually occurs after age 50, with a slightly higher incidence in males.

Between 25,000 and 100,000 American have multiple system atrophy. The incidence (new case per 100,000 person years) for ages 50 to 99 years is 3.0 (Bower et al, 1997), or about half as frequent as progressive supranuclear palsy (PSP).

Symptoms of MSA vary in distribution, onset and severity from person to person. Because of this, three different diseases were initially described to encompass this range of symptoms: Shy-Drager syndrome, striatonigral degeneration, and olivopontocerebellar atrophy.

The reason there are 3 forms is that years ago all doctors had to go on were symptoms. If a patient had loss of balance (ataxia) and slurred speech then they knew the patient had nerve degeneration in the cerebellum, olives and pons (areas of the brain). They called this Olivopontocerebellar atrophy (OPCA).

If a patient had low blood pressure (orthostatic hypotension) they knew that the patient had nerve degeneration in the autonomic nervous system. This form is named Shy-Drager Syndrome (SDS) for Dr. Milton Shy and Dr. Glen Drager who first wrote about this disorder in 1960.

If a patient had tremors, slowness of movement and other Parkinson-like symptoms they knew that the patient had nerve degeneration in the striatonigral pathway of the brain. They called this Striatonigral Degeneration (SND).

Doctors once thought that these 3 disorders were all SEPARATE diseases. They now know that THIS IS NOT THE CASE. Autopsy evidence has shown that there appears to be a common underlying cause in all three disorders so they came up with a NEW NAME to include all 3 - MULTIPLE SYSTEM ATROPHY. Many doctors have been slow to adopt this new terminology so they continue to use the old terms SND, OPCA, and SDS when diagnosing.

The symptoms vary and depend on which particular form of MSA the patient has.

Striatonigral degeneration is practically indistinguishable from Parkinson’s.

OPCA is characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and dysarthria (difficulty in articulating words); and

Shy-Drager Syndrome is characterized by Parkinsonism plus a much more pronounced failure of the autonomic nervous system.

MSA can manifest in a variety of different ways and progresses in an unpredictable sequence and time scale for each patient. It is important to understand that having a diagnosis of MSA does not mean that you will experience all the symptoms below.

MSA can cause a wide range of symptoms, including:

· stiffness or rigidity

· freezing or slowed movements

· postural instability; loss of balance; incoordination

· Orthostatic hypotension, or a significant fall in blood pressure when standing, causing dizziness, lightheadedness, fainting, or blurred vision

· male impotence

· urinary difficulties

· constipation

· speech and swallowing difficulties

· blurred vision

The Parkinsonism of MSA is generally an akinetic rigid syndrome, similar to that of progressive supranuclear palsy (PSP). Rest tremor may occur but is not a predominant feature. Postural instability is common. Parkinsonism is generally the most common initial sign and eventually develops in about 90% of all patients.

The cerebellar signs include finger-to-nose or heel-shin dysmetria, gait ataxia, intention tremor and nystagmus. Cerebellar signs are the first feature on only about 5% of patients. Cerebellar signs are observed in 50% of cases.1 Sporadic OPCA evolves into MSA in roughly 25% of cases within 5 years.

1 Ben-Shlomo et al, 1997

In order to diagnose multiple system atrophy, thoughtful and careful evaluation should be undertaken. Due to the variety of different ways MSA can manifest, it is often difficult to differentiate it from other neurodegenerative disorders like Parkinson's disease, progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD).

Many patients with MSA will not receive the correct diagnosis during their lifetime. This is due to the difficulty in differentiating MSA from other disorders (including relatively common degenerative disorders such as Parkinson’s disease and more rare ones such as pure autonomic failure).

MSA may be difficult to diagnose in the early stages because it can take years for key symptoms to develop. Diagnosis is made on careful history, physical examination and some tests including several types of brain imaging and autonomic function tests. However, a definite diagnosis can still only be accomplished by post-mortem examination of the brain.

There are many disorders with symptoms, which overlap with those of MSA, therefore diagnosis is usually carried out by a Neurologist or a Physician at the request of a GP. Information about the history of an individual's symptoms and an examination will be performed. There are also hospital tests that help in the diagnosis.

The initial diagnosis of MSA is usually made by carefully interviewing the patient and

performing a physical examination. A neurologist makes the diagnosis based on the history of symptoms and the findings on physical examination as well as ruling out other causes.

The physical examination may include:

· An eye examination may reveal atrophy of the iris and paralysis of eye muscles.

· Postural hypotension (drop in blood pressure associated with change in position) is evident.

· A neuromuscular examination shows abnormal reflexes and may show severe muscle wasting (atrophy). Parkinsonian movements (tremor, rigidity, and slow movements similar to Parkinson's disease) are common.

Diagnosis is largely clinical as there are no specific tests during life to confirm the disease. The testing described below can be helpful in suggesting whether a patient has multiple system atrophy, but true diagnosis can only be accomplished by examination of the brain postmortem.

Among the tests that are helpful in determining the presence of MSA are several types of brain imaging including computerized tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET). An MRI of brain can be used to rule out other pathology. There are no specific abnormalities on imaging associated with MSA.

Testing may include:

· Plasma norepinephrine levels

· Urine examination for norepinephrine breakdown products (urine catecholamines)

Disease progression in MSA is quicker than in Parkinsonism but similar or slightly slower to that of Progressive Supranuclear Palsy (PSP) (Bower, 1997). Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years. The mean survival is roughly 6 years. Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Patients usually have autonomic nervous system dysfunction first. Genitourinary dysfunction (difficulty with urination) is the most frequent initial complain in women, while impotence is the most frequent initial complaint in men.

When MSA begins with non-autonomic features, imbalance is the most common feature. This difficulty in maintaining balance may be due to either cerebellar or Parkinsonian abnormalities. Some patients complain of stiffness, clumsiness, or a change in handwriting at the onset of MSA.

MSA progresses over the course of several years to cause more widespread and severe symptoms. Orthostatic hypotension can cause fainting and falls. Loss of coordination, slowed movements, and rigidity can interfere with activities of daily living.

Some patients with MSA have mild loss of cognitive abilities, with impairments in attention and speed of thinking.

Complications include:

· Progressive loss of ability to walk or care for self

· Difficulty performing daily activities

· Injuries from falls/fainting

· Side effects of medications

The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops.

Early death is likely. The mean survival is roughly 6 years. Most people who are diagnosed with Shy-Drager syndrome die within seven to 10 years after symptoms begin. It is rare for a patient to survive 10 years.

The autonomic abnormalities are seldom the direct cause of death. A significant number of patients develop laryngeal stridor and difficulty swallowing, which can lead to pneumonia.

Pneumonia is the most common cause of death, although irregularities in heartbeat or choking may be responsible for death in some patients. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death.

In addition, many patients with MSA experience Cheyne-Stokes or periodic respiration and in some cases this may lead to a critical loss of respiratory drive, so called Ondine's curse.

Pulmonary hypertension may occur during apnea (Guilleminault et al., 1977). The most common causes of death in patients with MSA are pulmonary embolus, apnea, and intercurrent infection.

Currently there is no cure for MSA and no known means to slow progression of the disease. A number of drugs can be used to treat the various symptoms of MSA, although they become less effective as the disease progresses.

Treatment aims to reduce the disabling effect of the symptoms so that as full a life as possible can be led. This includes treatment of the depression, tremor and gait disturbances, supine hypertension, orthostatic hypotension, and possible self-catheterization.

Treatment for MSA involves a combination of medication, specialized equipment and the use of therapists to manage individual symptoms. Medication regimes will vary depending on your symptoms. Your doctor will prescribe the best combination to meet your needs.

The fluctuating blood pressure makes the condition difficult to treat. Dietary changes, such as increasing salt and fluid intake, may help elevate blood pressure.

Drugs such as fludrocortisone, midodrine that control the fall in blood pressure (orthostatic hypotension) can be effective in reducing dizziness, fainting and falls. Sympathomimetics, vasoconstrictors, beta-blockers, MAO inhibitors, vasopressin, 9-fluohydrocortisone, or other medications may be used to treat low blood pressure (postural hypotension).

These drugs may cause high blood pressure when lying down. It is advisable to raise the head of your bed and have regular blood pressure checks.

A heart pacemaker programmed to stimulate the heart to beat at a rapid rate (faster than 100) may increase the blood pressure for some people.

Fludrocortisone (Florinef) - A steroid, usually taken twice a day in very small doses. May cause ankle swelling.

Ephedrine - Works quickly to raise the blood pressure, usually taken three times a day.

Midodrine (Gutron) - Works quickly to raise the blood pressure (within 30-60 minutes), usually taken three times a day. Only prescribed by specialists.

Drugs to help stiffness and slowness are the same drugs used in Parkinson's disease. Anticholinergic medications may be used to reduce early or mild tremors.

The response to medications may be disappointing. Many affected individuals either do not respond or respond poorly to treatment with anticholinergics or Levodopa.

Levodopa and dopamine agonists used to treat Parkinson's disease are sometimes effective for the slowness and rigidity of MSA. They are not as effective in MSA and can make blood pressure problems worse. It may take time to find what suits you best.

Levodopa may improve movement and balance. Doses of 1 to 1.5 gram per day must be used before unresponsiveness is declared, but generally only about 1/3 of patients respond. Carbidopa may reduce the side effects of Levodopa and make the Levodopa work better.

L-Dopa (Madopar or Sinemet)

Amantadine (Symmetrel)

Incontinence may be treated with medications or catheterization.

Drugs can reduce urgency and frequency problems.

Oxybutinin (Ditropan) - improves the tone of bladder muscles.

DDAVP (Desmopressin) - used to reduce the production of urine overnight.

Antibiotics - should be prescribed at the first signs of infection. Any infection in someone with MSA can worsen symptoms like postural hypotension dramatically.

A breathing or feeding tube may have to be surgically inserted to manage swallowing and breathing difficulties.

A gastrostomy tube, which delivers foods directly to the stomach, is occasionally needed later in the course of the disease.

Use of softer foods may improve the ability to swallow.

· Male impotence may be treated with penile implants or drugs.

· Constipation may improve with increased dietary fiber or laxatives.

· A speech-language pathologist may be able to offer strategies for improving swallowing and speaking.

S. Dickinson, E. Garland, G. Farley and T. Davis, Vanderbilt University, Nashville, TN, USA

This is a summary of an abstract that was presented at the American Autonomic Society conference. Results from this survey provide information on quality of life issues in the MSA population. To better understand the physical effects and the impact that this disorder has on lifestyle, we surveyed a national population of MSA patients.

Completed questionnaires were received from

55 males (age 64.6±1.3 yr;) and 29 females (age 64.2±1.8 yr).

Only 2% of respondents lived alone. 89% reported that the spouse was the primary caregiver. While only 36% used services requiring a fee (nurse, aide), 69% indicated that they did use medical equipment.

20% had quit work or retired in the last month, while 13% had applied for disability.

A subsample (n=12) contained responses of caregivers of deceased persons diagnosed with MSA. Death occurred within 8.9±1.1 yr of symptom onset (range of 1-18 yr).

A mean of 5.1±0.4 yr elapsed between the onset of symptoms and diagnosis.

Difficulty in performing various activities of daily living (ADLs) was assessed, with a score of "1" representing no difficulty and "5" representing an inability to perform the activity.

63% of subjects were unable to drive a car, giving this activity the highest difficulty score (3.9) 35% were unable to walk outside (difficulty score 3.6).

The mission of the Shy-Drager Syndrome/Multiple System Atrophy Support Group

(SDS/MSA) is to gather information from each group of people involved (Patients,

Caregivers, Family Members, and Physicians) and disseminate that information to all.

The SDS/MSA Support Group is an information center and a "Helping Hand" for

affected people. Through various programs, including the continuation of the national patient/caregiver support group meeting, support of local groups, the toll free help line, and web site, the SDS/MSA's Support Group can reach many more people in need.

The support group also works closely with physicians who are treating the patients as well as those who are involved in the essential research to discover a cause, modes of treatment, and hopefully a cure.

The group also would like to reach out to physicians newly involved with the disease.

Information about the disease and tips on explaining it to the patient will be provide

through several modes, including the web site, links to the American Autonomic Society, access to a referral base of experienced physicians, and educational meetings.

By sharing information, resources, and support the SDS/MSA Support Group will create a "Circle of Hope" for those affected by SDS/MSA.

The SDS/MSA Support Group

2004 Howard Lane

Austin, TX. 78728

Toll Free: 1-866-SDS-4999 (Answering Machine when not available)

Phone: 1-512-856-2427 (Nights and Weekends)

Fax: 1-512-251-3315

E-mail: shydrager@yahoogroups.com

Web: http://www.shy-drager.org/

1. Advocacy/Support Organizations can help the patient and caregiver learn coping

strategies and offer support for the difficulties encountered in dealing with MSA.

2. Multiple System Atrophy Discussion Forum

3. MEDLINE plus Medical Encyclopedia

http://www.nlm.nih.gov/medlineplus/ency/article/000757.htm

4. E-medicine

http://www.emedicine.com/NEURO/topic671.htm

These are two of the popular options for MSA donations in the USA.

The Shy-Drager Syndrome/Multiple System Atrophy Support Group is a full non-profit organization approved by the Internal Revenue Service. Donations fund the entire operation of the Group. Services include:

· a toll-free number to call for information and physician referral

· the maintenance of the group website www.shy-drager.org

· access to the Shy-Drager mail list

· an annual patient/caregiver/family member meeting with prominent physicians.

Family members and friends of patients with Multiple System Atrophy or Shy-Drager

Syndrome have occasionally expressed an interest in making a financial contribution

that can be used for research on this condition. Accordingly, we have established a

fund for this purpose. All gifts are very much appreciated.

Donations for Multiple System Atrophy or Shy-Drager syndrome research can be sent to:

Vanderbilt Shy-Drager Research Fund

Vanderbilt University Medical Center

AA-3228 Medical Center North

Nashville, TN 37232-2195

Many patients express an interest in making a contribution to medical science to further research in the cause of neurodegenerative disorders. In Shy-Drager syndrome, also known as MSA, scientific study of human brain obtained after death holds the greatest hope for further understanding and ultimately development of therapies to treat the disease. The postmortem examination, or autopsy, also provides important, precise medical information to the family of the deceased.

You may wish to sign a notice of intent to make your desire for organ donation official. This notice of intent is not binding, but conveys your wish and request in writing to your family members that you wish to participate in the brain and spinal donation program.

Your affected family member may wish to donate brain tissue for special studies and research purposes. You can concur with that decision by signing a separate consent for the donation, which is often attached to his/her medical record. Upon death of your loved one, you will need to sign an autopsy permit which gives legal consent for a medical doctor to examine the body and in particular, collect brain tissue.

We know that this will be a very difficult time for you and we acknowledge the generous gift and courageous act that tissue donation is for the benefit of others. You should know that tissue removal could be done in a way that an open-casket service can be conducted.

For More Information Please contact:

Autonomic Dysfunction Center

Vanderbilt University Medical Center

AA-3228 Medical Center North

Nashville, TN 37232-2195

Phone: (615) 343-0124

Fax: (615) 343-8649

Table 2. Clinical Domains and Features in Diagnosis of MSA*

Table 3. Exclusion Criteria for Diagnosis of MSA*

Table 4. Diagnostic Categories of MSA*

Table 6. Differential Diagnosis in MSA and Parkinson Disease

Table 7. Differential Diagnosis in MSA and Pure Autonomic Failure

A drug that imitates a neurotransmitter. Dopamine agonists are drugs that imitate the

actions of dopamine.

Inability to move ("freezing") or difficulty in beginning or maintaining a body motion

A drug that blocks the action of acetylcholine, a neurotransmitter in the brain.

Anticholinergic drugs are often effective in reducing the tremor of Parkinson's disease

A medicine used to treat Parkinson's disease.

An anticholinergic drug that is often effective at reducing parkinsonian tremor. The most common side effects include anxiety, blurry vision, dry mouth, and nausea. It may also cause confusion.

A mobility-impairment condition marked by loss of balance and decreased coordination

Slow, repetitive, involuntary movements, especially in the hands

Large clusters of neurons deep within the brain that are responsible for voluntary

movements such as walking and movement coordination. Includes the striatum, the

subthalamic nucleus, and the substantia nigra

Surgery performed on both sides of the brain

A thin layer of tightly packed cells separating the central nervous system from the

body's blood stream. This layer blocks the ability of many substances, including certain drugs, from entering the brain.

The slowing down and loss of spontaneous and voluntary movement

The generic name of a dopamine agonist drug that can alleviate Parkinson's symptoms. The most common brand name is Parlodel.

A drug often used in conjunction with levodopa—as in the drug Sinemet—to increase levodopa's efficacy by allowing more to reach the brain. Carbidopa also reduces levodopa's unpleasant side effects such as nausea.

A term referring to the brain and spinal cord

A general term for nervous disorders characterized by involuntary, random, jerking

movements of muscles in the body, face, or extremities

Computed tomography, a technique that uses a series of X-rays to create image "slices" of the body from different orientations to create a two-dimensional image of the body. The term CAT scan (computed axial tomography) refers to a specific orientation of images.

A jerky or ratchet-like sensation felt by a physician when a patient's limb is moved

around a joint

A drug that blocks an enzyme (catchol-O-methyltransferase) that breaks down

dopamine. COMT inhibitors include entacapone (Comtan) and tolcapone (Tasmar)

Application of an electrical current to a deep brain target via an implanted electrode

connected to a programmable power source inserted in the chest wall (similar to a

cardiac pacemaker)

The generic name of the drug that inhibits the enzyme monoamine oxidase type B

(MAO-B), thereby increasing the level of dopamine in the brain. The most common side effects include nausea, dizziness, insomnia, agitation, and confusion.

Identification or naming of a disease by its signs and symptoms

A neurotransmitter chemical produced in the brain that helps control movement,

balance, and walking. Lack of dopamine is the primary cause of Parkinson's symptoms.

Diagnosed (i.e. dx 5 years ago)

Slurred or otherwise impaired speech

Unsteadiness or balance problems

Involuntary, uncontrollable, and often excessive movement. These movements can be lurching, dance-like or jerky, and are distinct from the rhythmic tremor commonly

associated with Parkinson's disease. A common side effect of many drugs used to treat Parkinson's disease.

Difficulty in swallowing

Abnormal and awkward posture or sustained movements of a hand, foot, or other part of the body; may be accompanied by rigidity and twisting

The brand name for the version of deprenyl made by Somerset Pharmaceuticals

A protein that catalyzes or speeds up chemical reactions

A fast tremor (about eight cycles per second) that is most pronounced when performing an action such as writing or bringing a hand to a target

A quickening of steps and shuffling after starting to walk

Abrupt and temporary inability of Parkinson's patients to move that frequently occurs at a boundary such as a door or when exiting a car

Referring to genes, the inherited code ("DNA") for human structure and function.

Hereditary

The inherited genetic pattern that may make some individuals more prone to certain

conditions than others with a different genetic makeup

A structure (group of nerve cells) deep in the brain affecting movement, balance, and walking. It is often used as a target for pallidotomy or DBS, two surgical procedures

The variable appearance of a condition

Reduced number of movements

Immobile, expressionless face with reduced blinking

An area of cell damage or cell death

Also called L-dopa, it is the most commonly administered drug to treat Parkinson's

symptoms (its brand name is Sinemet in the United States). Levodopa helps restore

levels of dopamine, a chemical messenger in the brain responsible for smooth,

coordinated movement and other motor and cognitive functions.

Abnormal structures seen in dead or dying dopamine-producing cells of the substantia nigra in Parkinson's disease. They are frequently the most precise way to diagnose Parkinson's.

Thin metallic tubes inserted into the brain and guided by stereotactic methods. They are connected to the operating room computer and used to measure the electrical signal from brain cells during surgical procedures, such as pallidotomy.

Small, cramped handwriting that is a symptom for many Parkinson's patients

The brand name of a dopamine agonist, pramipexole, made by Pharmacia, which is

often used to treat Parkinson's disease

Drugs that enhance the effect of dopamine by preventing enzymes from breaking them down

Refers to several conditions, many of them neurodegenerative, that prevent normal

movement. Some are characterized by either lack of movement (bradykinesia,

hypokinesia, etc) or excessive movement (chorea, athetosis, dystonia, tremor). Besides Parkinson's, other conditions often defined as movement disorders include essential tremor, multiple system atrophy, progressive supranuclear palsy, Huntington's disease, Tourette's syndrome and cerebral palsy.

Three-dimensional images of the brain obtained in a scanner using a powerful magnet

A degenerative condition characterized by low blood pressure when standing,

parkinsonism, rigidity, ataxia, fainting, and incontinence.

Refers to conditions such as Parkinson's that are characterized by the loss of cells in

the central nervous system

A physician specializing in diseases and disorders of the brain, spinal cord, nerves, and muscles, including stroke, Parkinson's disease, epilepsy, Alzheimer's disease, and muscular dystrophy

A nerve cell used to transmit information within the central nervous system

A doctor who operates on the brain and central nervous system

A chemical that carries impulses from one neuron to another

Sudden loss of activity of levodopa lasting minutes to hours after a brief period of

effectiveness. The term also sometimes refers to a cyclical response to medication

where the patient can function adequately at times but is too stiff and immobile to

function at other times.

Sudden drop in blood pressure upon standing.

A surgical procedure in which lesions are produced in the globus pallidus region of the brain in an effort to lessen Parkinson's symptoms such as tremors, rigidity, and

Bradykinesia

Antiquated term referring to paralysis or an uncontrollable shaking of the body.

Parkinson's disease was originally called the "shaking palsy"

Antiquated name for Parkinson's disease

Generic term referring to slowness and mobility problems that look like Parkinson's

disease. Several conditions, such as multiple system atrophy and progressive

supranuclear palsy, and a number of medications produce this appearance.

The brand name for the dopamine agonist bromocriptine that is made by Novartis

Abbreviation for Parkinson's disease

The generic name of a dopamine agonist used to treat Parkinson's disease. The brand name is Permax

The brand name for the dopamine agonist pergolide that is made by Eli Lilly

An acronym for "positron emission tomography," an imaging technique used to monitor and produce pictures of metabolic or biochemical activity in the brain

One of the characteristic slower tremors in the fingers of Parkinson's patients; the

alternating movements of the thumb and forefinger give the appearance of rolling a

small object between the fingers

The expected future course of an illness

A degenerative disease of unknown cause characterized by problems looking up and down, frequent falls and parkinsonism that is not helped consistently by levodopa

Abnormal stiffness in a limb or other body part. It is most apparent when an examiner moves a patient's limb -- as in cogwheeling.

A degenerative condition characterized by low blood pressure when standing. It may

lead to parkinsonism, rigidity, ataxia, fainting, or incontinence.

The brand name of the most commonly prescribed version of the drug levodopa, made by Du Pont Pharmaceuticals

Brain surgery, guided by brain images from CAT or MRI scans, usually involving a

metallic frame bolted to a patient's head to prevent any movement

Also known as the corpus striatum, it is the largest component of the basal ganglia in

the brain and controls movement, balance, and walking

Literally means "black substance." A part of the basal ganglia, located in the midbrain, that is rich in dopamine-producing nerve cells and the black pigment neuromelanin (hence its name). In Parkinson's the loss of nerve cells from this region leads to a dopamine deficit and subsequently to Parkinson's symptoms.

A nerve center near the substantia nigra. The STN may be targeted for deep brain

stimulation (DBS) to reduce Parkinson's symptoms

The brand name of the COMT inhibitor tolcapone, that is made by Roche Laboratories.

A surgical procedure in which cells in the thalamus are destroyed in an effort to

eradicate debilitating tremors.

A mass of gray matter (nerve cells) located deep in the brain that is responsible for

motor control and serves as a relay center for sensory signals.

A drug in the COMT inhibitor class that is sometimes prescribed in tandem with

levodopa. The drug has been known to cause serious liver problems and has been

withdrawn from the Canadian and European markets.

Unwanted rhythmic movements (may be fast or slow) that may affect the hands, head, voice or other body parts.

An external or environmental factor such as head trauma, exposure to a toxin, or stress that contributes to the development of a condition or disease.

Loss of effectiveness of Parkinson's medications between doses. If the effectiveness of a medication does not last until the next dose is due, it "wears off".

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