The patient and his wife appear for their appointment. They have a jovial quality uncharacteristic of patients coming for a second opinion on Parkinson's disease. The issue the patient has with speech is evident right away. It seems he must summon a certain energy to pronounce words, or to string words together. There is some lapse of time between questions and his response and there are none of the small inarticulations people make in standard speech; the small sounds of hm... ah...and well... are all missing. Talking and writing are the two major impairments that brought him to see a neurologist, a few years ago. The wife mentions since that time she has noticed the patient's speaking ability has deteriorated. She explains the patient sometimes tells her yes, when he means no. In addition, the patient admits his memory is not as good as it was.

The patient's general health is remarkable for hyperlipidemia- high cholesterol and triglycerides in the blood, high blood pressure, and diabetes. The patient manages all three problems with medications. The three problems contribute to give the patient small vessel disease- the smallest blood vessels that shuttle blood between arteries and veins are blocked, consequently, the patient's brain has multiple small areas which do not receive sufficient blood supply.

The patient has had the relevant tests. A PET scan revealed his brain has normal oxygen consumption, indicating no signs of Alzheimer's disease. A recent MRI suggested possible atrophy of the temporal lobes. Neuropsychological testing indicated the patient had losses in executive function, and should not be handling money; at the time he was the sole owner and director of a company with fifty employees. However, the patient scoffed at the results saying the tests were overly tedious, and he disliked the tester. Results also indicated the patient's IQ must have become compromised, as his apparent IQ did not harmonize with his position as a functioning executive. However, the movement disorder specialist fails to discuss this fact with the patient and spouse. Inquiring later about why he skipped lightly over this, the specialist noted the patient had scoffed at the results of neuropsychological tests, denying their truthfulness or value.

On physical exam, the specialist notes no rigidity, nor slowness of movement. He does find the patient has limited range of eye movements when gazing downwards, which a sign of an atypical form of PD known asprogressive supranuclear palsy (PSP). Though the patient's speaking ability is affected, he lacks many other signs typical of the disorder. Though he is a heavy man, with a substantial belly, he comes to a standing position easily from sitting in a chair. His gait is fluid, his arm swing full, and his balance normal. The specialist explains that this may be a very early case of the illness.

PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson's disease. Hence it is also called Steele-Richardson-Olszewski syndrome, for the three who focused on the disorder. The initial complaints are typically vague and an early diagnosis is always difficult. The primary complaints fall into these categories: 1) unsteady walking or abrupt and unexplained falls without loss of consciousness; 2) visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble; 3) slurred speech; and 4) various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood. http://www.ninds.nih.gov/disorders/psp/detail_psp.htm

In a review article, Dr. Marsel Mesulam described Primary Progressive Aphasia as having an insidious onset with gradual progressive impairment in finding words, naming objects, and comprehending words while engaged in conversation. Though not evident in the first years, patients exhibit prominent apathy, disinhibition, loss of recent memory, visuospatial impairments, visual- recognition problems, inability to perform simple mathematical calculations, and inability to perform pantomime movement as instructed. Dr. Mesulam notes that some patients may have signs and symptoms of illness confined to the area of language for as many as 10 to 14 years, before other problems emerge. Nowhere in the review does he mention problems with eye movements. (New Eng. J. of Med. Vol. 349:1535-1542 N.16 Oct. 16, 2003)

According to the National Institute of Neurological Disorders and Stroke, Corticobasal Degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy of multiple areas of the brain. Progressing gradually, the initial symptoms, which typically begin at or around age 60, first appear on one side of the body, but eventually affect both sides. Symptoms are similar to those found in Parkinson disease, such as poor coordination, an absence of movements, rigidity, disequilibrium or impaired balance, and limb dystonia- abnormal muscle postures. Other symptoms such as cognitive and visual-spatial impairments, apraxia- loss of the ability to make familiar, purposeful movements, hesitant and halting speech, myoclonus- muscular jerks, and dysphagia- difficulty swallowing, may also occur. The patient eventually becomes unable to walk. From the writer's perspective as an outsider, the patient seems least likely to suffer with this diagnosis. . (http://www.ninds.nih.gov/disorders/corticobasal_degeneration/corticobasal_degeneration.htm)

The movement disorder specialist recommends the patient and his wife make an appointment with another specialist whose area is cognitive neurology. This authority would be able to establish areas in which the patient has cognitive impairments. He also recommends the patient undergo another MRI within a year, to see whether changes in the brain have progressed.

Sometimes a diagnosis is unclear; sometimes a person has more than one diagnosis. The patient asks whether some pill will prevent further decline in his condition, and the specialist says there is nothing he can recommend. However, eating fruits and vegetable and performing daily exercise are general health recommendations for all. From the expression on his face, the patient stopped listening at the mention of fruits and vegetables.

The patient fell several times before a neurologist ever diagnosed him with Parkinson's disease. Falling is not a typical first symptom for someone with classical PD. The doctor asks about the first indication that hinted the patient should see a neurologist. Though the patient speaks clearly, he does not remember details well. The wife comments she thinks it was dragging of the right foot. She comments at the time, she feared her husband might have had a stroke.

There are other significant problems. The patient underwent a quadruple bypass on the vessels of his heart, and has a pacemaker. The movement disorders specialist comments though the surgeon may have cleared the blockages feeding the heart, the vessels feeding the brain have the same buildup of plaque. Patients with vascular disease may begin to show symptoms of lower body parkinsonism due to small vessel disease; shuffling gait with full arm swing. Another illness neurologists like to rule out in patients who can undergo an MRI, is normal pressure hydrocephalus. A prominent symptom in the illness along with the shuffling gait is substantial change in the person's cognition; problem solving suffers, memory fades, the ability to plan and change plans as problems arise, becomes hard to accomplish. Because of the pace maker in his chest, the patient cannot undergo an MRI. A CAT scan, the doctor concedes, reveals little information about prior strokes the patient may have suffered.

The wife confesses the greatest problem really is the patient has become limited in what he can do. An artistic person, he does not paint or work with wood anymore, due to his frequent falls. The physician comments that maintaining an artistic outlet is very important. The stimulation artwork gives the brain as one draws or paints, is about problem solving; for example depicting a three dimensional object in a two dimensional plane. The doctor encouraged the couple to pursue an art store that sells easels that will roll up to a person's chair. He also suggested it might be time to look into acquiring an electric cart. Access to the mall, the movies or the boardwalk should not limit the patient because his balance is poor. Along with the cart, they would need to purchase the carrier that fastens to the back of the car. Insurance will cover a large fraction of the cost, and it will help the couple maintain an active life. The last tool the doctor would like to see the patient using is a stationary bike, optimally with a chair seat, so there is no easy way to fall off.

The doctor reviews the medications the patient takes and comments the amount of levodopa is inadequate to provide a therapeutic dose, which is approximately two and a half pills of 25/100, four times per day. He draws a step- wise chart that indicates when the patient should increase the medication by a half pill. He instructs the patient to stop at a dose if he feels the medication is driving his blood pressure too low, though he cautions the patient may need to begin wearing TED hose, if low blood pressure remains an obstacle to achieving an adequate dose of medication. Proteins in the diet are very important the physician says, because they will compete with the medication to get across the blood brain barrier. A diet rich in dairy products will especially cause havoc with medication, due the quantity of free amino acids. He advised the patient to try and eat a protein- free breakfast and a low protein lunch, saving the protein filled meal for the evening, when he can anticipate being home, and feeling less effect from the medication. The doctor asked the couple to return in four months time, with a report on how things have gone.

The patient suffers from very low blood pressure and parkinsonism (Shy-Drager Syndrome) and is wheelchair bound except for the days when her handsome physical therapist arrives. Then she walks with him and does all her exercises. She needs to have her hair done for therapy, and if another person is sent instead, she pretends she's sleeping in bed. The doctor gives the daughter a new prescription for therapy, which she has been paying for, though insurance only covers it for an allocated number of days.

Six months ago the doctor suggested she should not eat proteins in the morning or at the afternoon meal, to maximize delivery of levodopa from blood to brain and to determine whether levodopa is helpful in relieving the patient's Parkinson symptoms; not all patients with the disorder get relief from tremor, slowness and rigidity with levodopa. The daughter reports they attempted to stop providing milk after the last time they visited. The patient, however really enjoys a morning glass of milk. The specialist agrees that quality of life is important, and that if the patient is to continue having milk in the morning and at lunch, they need to consider increasing the morning dose of Sinemet, possibly by half a pill.

Patients with Shy-Drager syndrome lose the ability to regulate blood pressure. Sinemet, the medication containing levodopa, tends to lower blood pressure further. Within minutes of standing, the patient's blood pressure plummets depriving her brain of blood flow, making fainting likely. For this reason, she is now in a wheelchair.

To increase blood pressure, the patient takes the medication, Florinef, which causes the kidneys to increase salt retention. Her body compensates by retaining more fluid, which increases the blood volume, thereby increasing blood pressure, so she is no longer light- headed and can sit and even stand with assistance. The doctor instructs the caregivers to not allow the patient to become truly flat, or prone because she could have excessively increased intracranial blood pressure. It's preferable for patients with this disorder who are treated with Florinef to be at a slightly inclined angle when they lie in bed, as they're less likely to have excessively increased intracranial blood pressure when the head is elevated. The daughter also reports the psychiatrist has switched the patient from Lexapro to Effexor, which he claimed would also augment blood pressure. The physician asked them to return in six months, though the daughter replied she would check in with the office staff in three months time.