While each disease is covered in its own chapter, discussion of items such as how it came to be named tends to extend over several chapters. It is informative, entertaining and clearly written, and the author gives the impression that what happens after a disease is discovered is more relevant than the discovery itself.

The question of what happens after a disease is discovered is crucial in determining who gets credit for the discovery.  Time and again, the author illustrates examples of this.  Broca’s area, which is the area of the brain known to be associated with aphasia, was initially discovered by Marc Dax and subsequently his son Gustave Dax.  However, the senior Dax never published his findings, and the junior Dax finally published his findings on April 28, 1865.  Broca then began to address the chronology of the discoveries.  The author writes that Broca did not question the authenticity of the report but said, “I wish to establish that it was impossible for me to guess the existence of the paper that was brought to light two years after my first publications on the subject of aphasia.”  This neatly restored the chronology despite the fact that Broca had still claimed that power of speech was represented bilaterally.  Although Marc Dax was the first to claim that the left hemisphere, when damaged, could give rise to aphasia, he failed to publish.  Meanwhile, Broca took careful notes and published all his findings; thus, it is now known as Broca’s area. 

Research is invariably subjective, no matter how objective the experimenter tries to be. Broca used several techniques to strive for objectivity in his research, such as measurements taken with special tools, and also stressed that “observations are more important than theories.” Broca felt that exacting measurements were the secret to his success but critics today see it as a “prime factor behind his failure.”  Even before the measurements were taken, the wording of the question was “clouded by the bias of the researcher.  Is the frontal lobe of women as large as that of man?”  Broca conducted no blind measurements and had no control conditions.

Disturbances of the Mind succeeds on many levels.  It discusses a range of brain diseases in a detailed and interesting manner.  It is written in a concise, clear style that both consumers and medical professional can understand. It also delves a little more deeply than just apprising the reader of a disease and its symptoms. It weaves a narrative of disease from discovery to naming to how the disease came to be known to the researcher, and notes the importance of the researcher’s actions after the disease’s discovery. The effect of researchers’ unwitting biases on the outcome of their research is also discussed.

The book covers other concepts as well, including the ways that patients afflicted with a particular disease learn to see themselves over time. For example, Tourette syndrome — which is characterized by grimaces, blinking, motor tics and in severe cases, parroting and coprolia (swearing) — can lead to social isolation.  Back in the Middle Ages, a Tourette patient would be seen as ‘possessed.’  In the early 1900s, a Tourette patient would believe that his disease was the result of a weak will and inadequate control.  Today’s Tourette patients know that they don’t have a psychological defect, that the disease is organic in nature.  This has removed the burden of shame from today’s Tourette sufferers.

The book goes into great detail in many aspects of the diseases it covers.  A notable omission in the chapter on Asperger’s Syndrome (in which patients are averse to social contact and change and have one or two obsessive interests) is the possibility of mistaken diagnoses and Asperger’s position on the autism spectrum. Nor does the author discuss the controversy over the belief that childhood vaccines cause autism, although this has been clearly, scientifically disproven.

Readers may come away with more empathy for those deemed ‘crazy.’ My limited encounters with ‘crazy’ people in the past whereupon I later discovered them to be bipolar left me with feelings of frustration and impatience as coherent communication with such persons was next to impossible.  Seen through the lens of a broadened perspective, I now realize more fully  how these symptoms are a result of organic disturbances. It allows me to develop more tolerance and patience in interactions with such persons. 

The chapter on Parkinson’s Disease also brought to mind memories of my father-in-law, who suffered greatly in his daily life from this debilitating disease.  It is interesting to note that one of today’s potential treatments involves deep brain stimulation and one of the treatments used in the late 1800s was a ‘shaking chair.’ It is also sad to note that while there have been many medical advances made, there is still no cure for Parkinson’s Disease.

To summarize, this book provides an interesting account of various neurological diseases that at times borders on the fascinating.  It provides the reader with a broadened scope of how diseases came to be discovered, and ultimately, the actions one must take in order to be credited for its discovery — for a discovery is one thing, but to bring the disease and possible treatments to other people’s attention is quite another. 

It also makes clear that despite the researcher’s intent to maintain complete objectivity, it is impossible to ‘observe and nothing more’ as Charcot warned others to do. Facts and findings are gathered by observations; observations are seen through the lens of the researcher and the theories the researcher holds.  Autistic children in studies performed in the 1950s were deemed to have ‘refrigerator mothers.’ This fact has now faded into the background because opposition to the independence of women has also now faded.  The researcher employs methods to maintain complete objectivity and yet the researcher is unconsciously ‘carried along by the insights of his day.’ 

Disturbances of the Mind
By Douwe Draaisma; translated by Barbara Fasting. 
Cambridge University Press UK, 2009. 
Hardcover, 360 pages
$25.99

Over time people on levodopa or dopamine agonist therapy develop involuntary movements. These are called dyskinesia. Dyskinesia in Parkinson disease is caused by medications. This can affect quality of life and may cause disability.

Neurologists from the American Academy of Neurology (AAN) are doctors who treat diseases of the brain and nervous system. They believe people with Parkinson disease should know which drugs and surgical treatments reduce their off time and
dyskinesia.

Experts in Parkinson disease reviewed all of the available studies about medical treatments and deep brain stimulation (DBS) for dyskinesia and motor fluctuations. They made suggestions that will help doctors and people with Parkinson disease make choices in their care. In some cases, there were not enough published data for or against specific therapies.

Medical Treatments to Reduce Off Time

Neurologists looked at all of the studies for medications that reduce off time. While there is stronger evidence* for some drugs, there is not enough evidence* to recommend the
value of one drug over another. There is strong evidence* that the following two drugs can decrease off time:

• Entacapone is in a group of drugs called catechol-Omethyltransferase (COMT) inhibitors. COMT inhibitors increase the length of time that each separate dose of levodopa therapy is effective and reduces per day off time.
  Entacapone acts in the bowels to increase the amount of levodopa absorbed. Side effects may include dizziness, drowsiness, hallucinations, or change in urine color.
• Rasagiline is in a group of drugs called monoamine oxidase (MAO) inhibitors. They slow the breakdown of naturally occurring dopamine and dopamine produced from levodopa. Side effects may include headache, depression, or flu-like symptoms.

There is good evidence* that these medications may reduce off time:

• Ropinirole, pramipexole, and pergolide are dopamine agonists. They act directly on dopamine receptors. They act like dopamine; they stimulate the dopamine system. Side effects may include confusion, mild nausea, or decreased appetite. Due to potential side effects such as heart and breathing difficulties, pergolide should be used with caution.
• Tolcapone is a COMT inhibitor. In rare cases, tolcapone has caused severe liver damage resulting in death. Notify your doctor immediately if you develop nausea, vomiting, abdominal pain, unusual fatigue, loss of appetite, yellow skin or eyes, itching, dark urine, or clay colored stools. These symptoms may be early signs of liver damage. Liver tests should be done often on people taking tolcapone.

There is weak evidence* that the following drugs may reduce off time:

• Apomorphine and cabergoline are dopamine agonists. They act directly on dopamine receptors. Apomorphine is injected like insulin and works rapidly. Apomorphine may cause depression, dizziness, or hallucinations. Cabergoline may cause dizziness, headache, and weakness. As of December 2005, cabergoline was not available in the United States.
• Selegiline and orally-disintegrating selegiline are MAO-B inhibitors. Side effects may include dizziness or drowsiness, abdominal pain, and anxiety.

Medical Treatments to Reduce Dyskinesia

The Parkinson disease experts also reviewed all of the available data for drugs that reduce dyskinesia.

• Amantadine reduces stiffness. There is weak evidence* that amantadine may be considered for reducing dyskinesia. Side effects may include confusion, leg swelling or rash, constipation, dizziness, lightheadedness, drowsiness, or headache.
• Clozapine is a drug used for schizophrenia. There is not enough evidence* for the use of clozapine in reducing dyskinesia. Side effects may include decrease in white blood cells, seizures, or inflammation of the heart muscle. Due to the potential harmful effects, frequent blood monitoring is required.

Surgical Treatment

A surgical procedure called deep brain stimulation (DBS) may help improve motor fluctuations and dyskinesia in people with Parkinson disease. DBS is directed at three primary targets for Parkinson. All three of these structures are deep in the brain. In DBS, an electric probe (electrode) is placed in the brain. A wire from the electrode is routed beneath the skin to a pacemaker device implanted near your collarbone. The pacemaker and electrode stimulate a specific brain structure with pulses of electricity. This regulates the structure in the brain to improve off time and involuntary movement. Only special medical centers perform this procedure.

Side effects may include thought process and speech disorders, visual and sensory disturbances, abnormal gait, lack of coordination, headaches, and seizures.

Readers should be aware that it is not easy to study surgical therapies in the same way as other medical therapies. It is difficult to design a study where neither the physician nor the patient know if the patient went through the real surgical procedure or a comparison (sham) procedure. Therefore, the evidence that DBS successfully treats Parkinson disease is weakened by the research methods involved.

There is weak evidence* that DBS using an electrode implanted in the core of the subthalamus may improve function and reduce motor fluctuations, dyskinesia, and drug usage. There is not enough information* to make suggestions about DBS in the other two areas of the brain—the thalamus and globus pallidus. There is some evidence that response to levodopa, age, and duration of Parkinson disease may predict how successful DBS of the subthalamus will be.

Your doctor should discuss potential side effects of this treatment with you. The decision to use this procedure depends on your condition and the risk for complications compared to successful outcomes.

Ten to 20 percent of people with Parkinson disease may be eligible for surgical treatments. Surgery may help long-term by reducing symptoms and improving quality of life. Talk to your neurologist early in your disease to discuss the potential for future surgical treatments.

Talk to your neurologist

Not every treatment works for every patient. A treatment decision will depend on other medical conditions you have and potential side effects. All treatments have some side
effects, the choice of which side effects can be tolerated depends on the individual. Your doctor should discuss serious side effects, if any.

This is an evidence-based educational service of the American Academy of Neurology. It is designed to provide members and patients with evidence- based guideline recommendations to assist with decision-making in patient care. It is based on an assessment of current scientific and clinical information, and is not intended to exclude any reasonable alternative methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, based on the circumstances involved.

*Note: After the experts review all of the published research studies they describe the strength of the evidence supporting each recommendation:

• Strong evidence = More than one high-quality scientific study
• Good evidence = At least one high-quality scientific study or two or more studies of a lesser quality
• Weak evidence = The studies while favorable are weak in design or strength of the evidence
• Not enough evidence = Either different studies have come to conflicting results or there are no studies of reasonable quality

Source: American Academy of Neurology.

Neurologists from the American Academy of Neurology (AAN) are doctors who treat diseases of the brain and nervous system. Experts in Parkinson disease looked at all of the studies on accurate diagnosis, disease progression, and therapies for Parkinson disease. Then they made suggestions that will help doctors and people with Parkinson disease make choices in their care. In some cases, there were not enough published data for or against specific therapies.

What is Parkinson disease?

Parkinson disease is a progressive movement disorder. This means the symptoms will gradually worsen over time. In people with Parkinson disease a vital chemical in the brain,
dopamine, slowly decreases. Dopamine makes smooth and coordinated muscle movement possible. A loss of dopamine leads to symptoms of Parkinson disease, such as:

• Shaking (tremor)
• Stiffness
• Shuffling walk
• Slowness of movements
• Balance problems
• Small or cramped handwriting
• Loss of facial expression
• Soft, muffled speech

How is Parkinson disease diagnosed?

Parkinson disease is common, but it can be difficult to diagnose. This is especially true in the early stages or in older people. A doctor will make a diagnosis after a complete medical history, review of the symptoms, and a detailed neurological exam.

Your doctor will try to find out if the symptoms are due to Parkinson disease or another condition that has similar symptoms. According to good evidence,* history of falls, no tremor, rapid progression of the symptoms, and no affect of drugs on Parkinson-like symptoms may be signs of a similar condition, not Parkinson disease.

Certain drugs are probably useful in confirming if a person has Parkinson disease versus another condition. This is called a “challenge test.” If symptoms get better while taking the drugs, the person may have Parkinson disease. The experts found there is good evidence* two drugs are probably useful in diagnosing Parkinson disease:

• Levodopa is a naturally occurring amino acid that the
  brain converts to dopamine.
• Apomorphine is a man-made form of morphine. It acts
  like dopamine and stimulates the dopamine system.

Your doctor may also use other tests. There is good evidence* that for some patients a smell test can help doctors decide if a person has Parkinson disease versus another condition.
At this time there is not enough evidence* for or against the use of brain scans, blood tests, or other tests to diagnose Parkinson disease.

What is the prognosis for Parkinson disease?

Parkinson disease usually progresses slowly. Doctors cannot estimate exactly how quickly or slowly it will progress in a patient. This will vary from person to person. However, good evidence* shows that Parkinson disease may progress more quickly in people who are older when symptoms begin.

Parkinson disease may progress more quickly in people whose symptoms are muscle stiffness and slowness. There is weak evidence* that the disease will progress faster in men and people with a history of stroke, hearing, or vision problems.

Which initial therapies are effective for Parkinson disease?

In 2002, a group of neurologists reviewed all of the studies for the most effective drugs used to treat Parkinson disease. To treat the initial symptoms of Parkinson disease doctors may prescribe:

• Levodopa or dopamine agonists: There is strong evidence* that either levodopa or a dopamine agonist can be used to treat initial symptoms. Dopamine agonists are drugs that stimulate the dopamine system and may lessen motor complications. Levodopa is a naturally occurring amino acid that the brain converts to dopamine. Levodopa provides superior motor benefit but it is associated with a higher risk of dyskinesia.
• Selegiline: Strong evidence* shows that selegiline has very mild benefit as an initial treatment. There is not enough evidence* that it is neuroprotective.

Talk to your neurologist

People experiencing the signs of Parkinson disease should seek the care of a neurologist. Your doctor will recommend an individualized treatment plan. This may include lifestyle changes. All treatments have some side effects. The choice of which side effects can be tolerated depends on the individual.

This is an evidence-based educational service of the American Academy of Neurology. It is designed to provide members and patients with evidence- based guideline recommendations to assist with decision-making in patient care. It is based on an assessment of current scientific and clinical information, and is not intended to exclude any reasonable alternative methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, based on the circumstances involved.

*Note: After the experts review all of the published research studies they describe the strength of the evidence supporting each recommendation:

• Strong evidence = More than one high-quality scientific study
• Good evidence = At least one high-quality scientific study or two or more studies of a lesser quality
• Weak evidence = The studies while favorable are weak in design or strength of the evidence
• Not enough evidence = Either different studies have come to conflicting results or there are no studies of reasonable quality

Source: American Academy of Neurology.

Neurologists from the American Academy of Neurology (AAN) are doctors who treat diseases of the brain and nervous system. They recommend people with Parkinson disease be screened and treated if they show signs of depression or decline in their ability to think, reason, learn, or remember.

Experts in Parkinson disease, dementia, depression and psychosis reviewed all of the available studies about screening and treating depression, psychosis, and dementia in patients with Parkinson disease. They made suggestions that will help doctors, people with Parkinson disease, and their caregivers make choices in their care. In some cases, there were not enough published data for or against specific therapies.

Depression

Depression in people with Parkinson disease is common. Treating depression helps people with Parkinson disease effectively manage both conditions. Often depression is thought
of as a normal reaction to living with Parkinson disease, but it is actually a symptom of the disease.

Patients, families and friends, and physicians should be aware of the warning signs. Depressed people will have several of the following symptoms:

• Constant sad, anxious, or “empty” mood
• Feelings of hopelessness, worthlessness, helplessness
• Loss of interest in hobbies or activities
• Decreased energy
• Difficulty concentrating or making decisions
• Insomnia or early-morning awakening
• Appetite and/or weight changes
• Thoughts of death or suicide
• Restlessness, irritability

A doctor will want to know how long the person has felt this way. He or she will ask how severe the symptoms have been. A trained health care provider may use a depression
screening test to make an accurate diagnosis. During a screen for depression, the patient answers a set of questions. The questions evaluate symptoms of depression and anxiety.

The experts found good evidence* that two screening tests, the Beck Depression Inventory and the Hamilton Depression Rating Scale, are probably useful in detecting depression in people with Parkinson disease. Another screening test, the Montgomery Asberg Depression Rating Scale, had weaker evidence* and is possibly useful in detecting depression in people with Parkinson disease.

A health care provider will prescribe a treatment based on the test results. The experts found weak evidence* that amitriptyline may be considered to treat depression in people
with Parkinson disease. Amitriptyline is in a class of drugs called tricyclic antidepressants. These drugs have an effect on chemicals in the brain that affect mood and behavior. The side effects of some of these drugs can be harmful to people with Parkinson disease. Talk to your neurologist, mental health provider, or pharmacist about possible side effects. Some of the side effects include dry mouth, daytime drowsiness, and difficulty urinating—especially in men. There is not enough evidence* regarding the effectiveness of other treatments. Your doctor will use his or her judgement to determine use of these drugs.

Treatment for depression in people with Parkinson disease can be managed by your neurologist or a mental health professional who is in close communication with your neurologist.

Hallucinations and Delusions

Hallucinations consist of seeing or hearing things that are not really there. Examples are seeing animals, insects, children, or a shadow in the room. Over time, the hallucinations may
become frightening or threatening. Delusions are fixed thoughts that are not based in the real world. Examples would be believing that nursing staff want to harm you, that your spouse is having an affair, or that people are stealing from you.

Hallucinations and delusions are dangerous because people may act on them and this can result in injury to themselves or those around them. It is also distressing to have delusions or threatening hallucinations for both the patient and the family.

Hallucinations and delusions are the result of the combination of Parkinson medications acting on previous personality traits or, more commonly, some degree of memory and thinking problems (dementia) associated with Parkinson disease.

At this point, there is no accurate screening test for hallucinations. If these symptoms are present, you or your care partner should tell your neurologist. Medications can be adjusted or new medications such as clozapine or quetiapine can control hallucinations and delusions.

Dementia

Older people with Parkinson disease may develop dementia. It is more common in those over 70 years old. Dementia is a medical term referring to difficulties with recent memory
(e.g., the person can’t remember what happened yesterday, but can remember events from years ago). Two terms used are Parkinson disease dementia and dementia with Lewy
bodies. Most scientists believe they are the same thing. Signs of Parkinson disease dementia include changes in alertness, withdrawal, loss of problem-solving skills, and lack of flexibility in thinking (getting stuck on one topic). Trained doctors diagnose dementia using screening tests.

During a test for dementia, the patient answers a series of questions. These questions evaluate memory, problemsolving ability, attention span, and language skills. The
experts found good evidence* that two tests are probably useful in detecting dementia with Parkinson disease, the Mini-Mental Status Examination (MMSE) and CAMCog.

The experts found good evidence* that two drugs may be considered to manage dementia in people with Parkinson disease. These drugs are rivastigmine and donepezil. Rivastigmine may be considered for the treatment of people with Parkinson disease and dementia with Lewy bodies Disease. The benefit with rivastigmine is small and tremor may worsen. Donepezil is possibly effective in improving thought processes in people with Parkinson disease and dementia, but the benefit is also small.

A person with Parkinson disease and dementia requires regular checkups with his or her doctor to ensure the therapies are working.

For Care Partners

Caring for a person with Parkinson disease and dementia is stressful. Care partners should talk to others about any frustrations they are experiencing. Talk to friends or family
members, or join a support group for care partners. This can be very helpful. Care partners need to take care of themselves. If the care partner can’t take a break, he or she can burn out, develop mental and physical health problems, and become unable to care for the person
with Parkinson disease.

Talk to your neurologist

Any change in mood or behavior; problem solving ability; ability to think, reason, or concentrate in a person with Parkinson disease is worth a visit to a neurologist or mental health professional. A doctor will recognize the symptoms of depression, dementia, or other mental health conditions.

This is an evidence-based educational service of the American Academy of Neurology. It is designed to provide members and patients with evidence- based guideline recommendations to assist with decision-making in patient care. It is based on an assessment of current scientific and clinical information, and is not intended to exclude any reasonable alternative methodologies. The AAN recognizes that specific patient care decisions are the prerogative of the patient and the physician caring for the patient, based on the circumstances involved.

*Note: After the experts review all of the published research studies they describe the strength of the evidence supporting each recommendation:

• Strong evidence = More than one high-quality scientific study
• Good evidence = At least one high-quality scientific study or two or more studies of a lesser quality
• Weak evidence = The studies while favorable are weak in design or strength of the evidence
• Not enough evidence = Either different studies have come to conflicting results or there are no studies of reasonable quality

Source: American Academy of Neurology.