Problems Aplenty (Liver failure, manganese and movement disorders)

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A new patient arrived at the movement disorder clinic with impaired balance, bilateral tremor, cramping in the major muscle groups, depression, mood swings, tingling and numbness in the feet, and fatigue. Unaccompanied by records from previous physicians, the movement disorder specialist had no knowledge of the patient’s medical history.
Before saying a single word, the patients waxy yellowish complexion signaled poor health. An attractive person with large facial features and dark brown hair, the patient sat gingerly in the chair opposite the doctor’s desk. The specialist explained that records generally help when seeing new patients. Lacking clinical details other doctors had acquired, the specialist asked for the earliest date when the health problem became apparent. After feeling fatigued for some time the patient sought professional help and came home with a diagnosis of liver disease, hepatitis C.
Although hepatitis C damages the liver, 80% of people with the disease do not have symptoms. In those who do,symptoms may not appear for10-20 years, or even longer. Even then, the symptoms usually come and go and are mild and vague. Unfortunately, by the time symptoms appear, the damage may be very serious. The source of transmission is unknown in about 10% of people with acute hepatitis C and in about 30% of people with chronic hepatitis C. (http://www.emedicinehealth.com/hepatitis_c/page3_em.htm)
Approximately two years later the patient was diagnosed with type II diabetes. Currently using two prescriptions to manage that illness, the specialist ascribed several of the patient’s symptoms to diabetes, specifically; frequent urination, tingling and numbness in the feet. The muscle cramps and slight tremor of the hands, the physician thought were likely to be brought on by a metabolic syndrome caused by a poorly functioning liver. Shortly after, the patient commented the previous neurologist had found high blood manganese levels and was interested in having the patient undergo chelation, to reduce the manganese serum levels. Chelation involves the intake of one of several binding compounds that removes heavy metals from the body. Normally used in instances where a person has become exposed to a toxic level of lead, uranium, arsenic, copper or mercury, it has also been used to lower manganese levels in the blood. Individuals who are exposed to toxic levels of manganese (miners, welders, or ingestion of a fungicide-Maneb) develop a syndrome that resembles Parkinsons disease. In addition to slowness, rigidty and postural and action tremors, these patients also exhibit major changes in personality with irritability and anger outbursts. So it was reasonable for the patients physician to ask for a consultation with an expert in Parkinsons Disease.
The physician attributed the increased level of manganese in the blood to the liver’s inability to make a protein that binds the free metal in order to flush it from the body. Chelation is best for those who have high levels of manganese because of toxic exposure rather than for those who accumulate the metal because of liver failure. Chelation therapy is not without risk as the binding agents flush out other needed bodily minerals as well as the toxic ones. The specialist examined the patient, noting other metals, such as copper might be high in a patient with poor liver function. High copper levels bring on changes in the iris of the eye, shading the outside with a yellowish-colored ring. The patient had no such problem. He also stated the MRI would have revealed an abnormal signal in the globus pallidus, the area of the brain where the heavy metal accumulate when it is at toxic levels. Problems with globus pallidus cause a rigid-akinetic syndrome similar to Parkinsons Disease. With decreasing blood levels, this signal would subside and go away. In all, the specialist found few neurological manifestations he could claim were caused by liver disease. The patient was not confused and did not have the jerking movements of hands (asterixis) seen in patients with liver failure. He did not recommend the patient undergo chelation, feeling the process would be too stressful for a body already depleted and traumatized by poor liver function. He thought the changes in mental function, such as slowness in thinking and confusion he has experienced in the past could be due to the disruption of other neurotransmitters in brain, caused by reduced liver health. In parting, the physician referred the patient to an expert in liver disease, Dr. Eugene Schiff at the University of Miami, for a second opinion on possible treatments for hepatitis C.

Speaking and Eye Movement Problems in a Person with mild Parkinsonism

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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.

Falling Was The Earliest Sign

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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.

Reason to Get Dressed

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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.

Left Hand Evil

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The patient was forty-five years old when doctors told her they suspected she had Parkinson’s disease. Symptoms started in her left hand. She noticed her fingers failed to hit the keyboard correctly when she typed and it seemed clumsy when she washed the dog. Born in Italy and raised there until the age of eight, she was originally left handed. Her mother encouraged her to use her right hand, under the advice of others who told her using the left was evil. Her symptoms became aggravated when she shattered her right hip. She has undergone a total hip replacement and a subsequent surgery to further align the bones. A third surgery on the right side is scheduled to occur soon. The left hip was also been completely replaced, the damage due to the effects of arthritis.
The patient relates she takes her PD medication every 2, to 2 1/2 hours because anxiety kicks in just before the next dose. She is taking an anti-depressant as well as an anti-anxiety drug lorazepam. The movement disorder specialist notes he hopes she does not overuse the anti- anxiety agent Ativan (lorazipam), tolereance develops quickly to their benefits and tend to produce or worsen depression. He encourages her to continue on the anti-depressant but recommended she begin taking Seroquel at night. The medication allows patients to get a full six- hour night sleep, and anxiety levels tend to decrease as patients sleep better, allowing daytime hours to be more active, and less full of aggravation.
Instead of crushing her pills in her mouth, the specialist recommends she dissolve them in water, add a vitamin c capsule to prevent the medication from oxidizing, and keep the mixture in a cool dark place. This way she can sip her medication throughout the day; the more constant the levels of levodopa in the brain, the less apt she will be to suffer from motor fluctuations.
The specialist advises the patient to eat only the smallest levels of protein for breakfast and lunch, to get the most from the medication. At dinner if she is planning to go out dancing, or bowling, or engage in some activity she may chose to avoid protein at dinner as well. If she is to eat a protein heavy meal, she may also choose to up her medication slightly to compensate for the proteins in the diet that will compete with the medication for transport into the brain.
Amantadine caused small blisters to break out on the face of the patient, though she had taken the medication for its noted ability improve dyskinesias triggered by Sinemet. He inquires about past exposure to pesticides and the patient and her husband recall having their home sprayed repeatedly inside and out, and being doused with Malathion when planes were spraying to kill mosquitoes. The doctor notes a study he conducted with cotton farmers, noting the farmers and the surrounding population had high levels of the chemical Deldrin in their blood. He specifies it is not only the exposure that puts a population at risk, but the bodies’ own xenobiotic metabolism system, which determines the capacity to breakdown the chemicals once they enter. This capability is generally hereditary, akin to one’s immune system, and determines who becomes ill, and who remains unaffected.
The patient wonders whether she need consider deep brain stimulation. The specialist thinks it’s too early to be thinking of such an invasive measure, but to keep an eye on the new surgical method that targets the pedunculo- pontine nucleus, as it may prove to be helpful in correcting her problems with gait and balance in the future.

Roatan

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The patient is a small woman with thick white hair who grew up on the island of Roatan, off the coast of Honduras. She has an Island accent, but her speech is so hushed it’s hard to hear. Twice a week she spends the day at an adult care center in Hillsborough County. Otherwise, she lives with her daughter, of which she had two. In her youth, she worked for the Delmonte fruit corporation. She sits with marked stooped posture in her chair. A member of her family has brought her to the clinic for a professional consult on what likely ails her. Past documentation from an MRI noted the patient harbors a calcified meningioma. The doctor says these are quite common. Women have them more often than men, and many do not produce symptoms. The image of the patient’s brain revealed moderate diffuse atrophy and medial temporal lobe atrophy, an Alzheimer- like neuroimage.
The family member and patient note the stooped appearance began approximately three years ago when the patient’s spouse passed away. The physician inquires whether stress from the death event amplified the patient’s symptoms; the patient agrees, nodding her head.
On physical examination, the specialist finds the patient has limited ability to move her eyes upward and downward, suggestive of an atypical parkinson syndrome known as progressive supranuclear palsy PSP). When manipulating the patient’s head he notes the older woman has moderate axial rigidity. Lack of downward gaze usually causes patients to become more erect in posture, so they are looking down the length of their nose; the patient lacks the standard posture of someone with PSP. When asking the patient to perform fine hand movements he notes she has difficulty. When she concentrates, her chin has a fine tremor. Due to the difficulty she has, the doctor performs a mini- mental test. Of a possible 30 points for a perfect score, the patient had trouble with several items including the date, year and day of the week. Her total score is 4/30, denoting the patient suffers from dementia.
This is a small revelation to the family member, who concedes, the patient has been having hallucinations, especially after watching a lot of television. The doctor notes he would like to have the patient begin physical therapy and a trial of Sinemet, to see whether she receives any benefit from the medication. In benefit, the patient should become looser in her body movements, and have enhanced capacity for performing small hand motions, like buttoning a shirt. He begins a chart of increasing dosage of Sinemet, noting that it is a staircase, they can go up a step and they can also retreat a step. He says he would like to give them a prescription for the medication Seroquel, with several reservations. Sinemet can bring on hallucinations, especially in someone who is demented. They will use Seroquel in combination with Sinemet to thwart visual hallucinations and to improve sleep. Seroquel given at night will help the patient sleep better, and reset the REM cycle, he urges the patient and family member to begin both doses small, gradually working towards higher doses.
The doctor would like the patient to return in four months time, though they may call if they have questions. The member of the family notes the patient woke with a urinary tract infection one morning, and it was only discovered because she was unable to rise from bed. She ran no fever because her body did not react to the infection. This is common the physician notes, when the brain no longer receives signals from the body.

Large Problems, Petite Patient

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The patient is a petite woman, fifty- four years old. Gazing at her, you notice her head shakes with a fine tremor, as does her left hand. She reports her legs have also shaken; now they appear still. Surgeons removed a malignant grade three follicular lymphoma from her abdomen and the patient underwent a course of chemotherapy. The shaking began approximately a year after she had the chemotherapy. Since then, the patient feels nauseated. She takes two prescriptions for the nausea; both are mixtures of drugs that have the potential to induce parkinson- like symptoms in the patient. The doctor notes this to the patient and she comments her doctors are trying to kill her. She explains she received the prescription through hospice. The specialist in movement disorders and the student in neuroscience consult the internet for anti- emetic drugs that do not block dopamine receptors in the brain, and find one among many that appears safer to recommend for the patient.
Skeptical why a physician would provide a prescription that could cause the patient to shake; I assume the oncologist knows what works best. The student enlightens me; commenting physicians are stuck using medications within a certain box, and rarely venture outside that zone. The patient asks about the side effects of the anti- nausea drug they recommend. The physician notes the medication may drop her blood pressure somewhat, which might be a positive side effect, as she takes a medication for high blood pressure, as well.
On physical examination, it is readily apparent the tremor is worse on the left side of her body. She notes in 2002 she suffered a, “mini- stroke”. The doctor is interested in this and questions what she means by, “mini stroke”. He gathers the patient suffered from a lacunar stroke, though it seems she fully recovered. He notes she is a complicated patient because she comes with several possible sources of tremor; the lymphoma she suffered from may bring on tremor, the medications she has taken may induce fine movements, the prior stroke may account for some of the physical symptoms evident on examination, and the final possibility is paraneoplastic syndrome. The last possibility occurs as a consequence of cancer in the body, but not due to the physical presence of cancer cells. Instead, symptoms such as ataxia- difficulty with walking and balance, dizziness, rapid uncontrolled eye movements, difficulty swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, and sensory loss in the limbs, are caused by an immune response, or a similar reaction to cancerous agents given off by tumor cells. To ascertain whether the last is occurring, the doctor asks the patient to undergo a blood test. The laboratory will check whether the patient harbors specific antibodies in her blood, anti- hu and anti-yo, which attack her own brain cells. The syndrome is quite rare. If the test is positive, the doctor feels it would be best for the patient to see a neuro- oncologist, who typically sees more cases of the disorder. Otherwise, he would like her to return in two months. He provides the patient with new prescriptions and the student notes the medications she needs to stop taking.

http://en.wikipedia.org/wiki/Paraneoplastic_syndrome

The Baker

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The new patient is a woman in her late forties. She arrives with a teenage daughter in tow. Is she just nervous, or does she have tremor? Her left hand shakes visibly as she sits in the chair. Her right foot flaps under the seat. Her daughter looks at her and tells her to ‘Chill’. They have been waiting in the patient area for the last thirty minutes and the mother’s face is tight with apprehension, anxiety and anger.
The physician appears to notice the tension in the patient and apologizes for the wait, apparently the office staff has double booked patients, which never works. This seems to relieve some stress. The specialist asks why she has come and the patient concedes she has been worried about her left hand for some time, because she’s right handed, she’s put off seeing a doctor, feeling she could function with the small tremor. Socially she feels embarrassed by the jiggling in the hand. The physician asks if the tremor is worse when she uses the hand and she hesitates, but responds slowly that it is probably worse when she is not using the hand. The specialist reviews the patient’s history and notes she is otherwise healthy, with low blood pressure. He inquires into whether she grew up using well- water and she replies, yes her parents owned a dairy farm. He asks whether she was exposed to insecticides, herbicides, or heavy metals and she shakes her head yes. It was her job to place the ear tags on the lactating cows; the tags repelled flies, ticks and lice. The patient explains each cow gets a tag in each ear, similar to ear rings she clarifies. How long did she perform this work? The patient looks towards the ceiling, and admits it was for a while, maybe five years.
The specialist notes epidemiological studies have found well- water consumption and exposure to the toxins in insecticides or the like, increase the chance of acquiring the illness. He asks whether she has other family members who have been diagnosed with Parkinson’s disease or tremors and she shakes her head to the affirmative. Her father currently suffers with the illness, and her oldest brother has some issue with movements in his hand and foot, though he refuses to see a doctor. The specialist nods and notes she may have a genetic predisposition to acquiring the disease. He asks when she first recognized the tremor of her hand. The patient pauses, and the daughter answers for her mother, saying it was about a year ago, last spring. The older woman agrees, nodding her head. The daughter quips that her mom complained about the twitching to her, and she had shown her how her hand moves, involuntarily.
The doctor asks the patient to sit on the examination table so he can assess her movements. He asks about work. She has worked for Panera bread for the last eight years. She wakes early in the morning and is finished before noon; and is one in the team of bread makers. As she speaks, the doctor takes her right hand and asks her to leave the wrist loose. He moves her hand back and forth, then moves the same elbow back and forth, and reports to the young medical student who is standing, that he feels no rigidity on the right side. He performs the same actions on the left and shakes his head, yes, denoting some rigidity exists in the muscles of the left side. He asks the student to come and check, and the student appears abashed but performs the same test. He gives a brief, ‘Hm’, saying little else. The specialist asks the patient to perform various other actions, finger tip to nose eventually he asks the patient to walk in the hallway so they can observe her gait. The two men agree the way she walks indicates symptoms of Parkinson’s disease; she fails to swing the left arm, holding it rather close to her torso, though she swings her right arm fully.
They regroup in their original seats. The movement disorders specialist agrees she has some of the symptoms of the illness, though she is quite young for the disease; the average diagnosis occurs in the sixth decade. He encourages the patient to exercise daily to maintain her health. He would like to prescribe a medication that may delay some of the symptoms of the disease. Azilect should be taken once a day, at bedtime. He reassures the patient that though Parkinson’s disease has no cure, there are treatments that address the symptoms. He would like her to return to the clinic in six months, though she may call the office staff if she has questions or problems.

Steady Hands for Golf

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A sixty- one year old patient comes in worried about the tremor in his hands. Told by his previous neurologist there are eighteen drugs that treat tremor, he was about to start the first trial when he opted instead to go on a long driving journey across the country. Not wanting to have to contend with the possible side effect of undue sedation, he put off treatment. The patient has no history of working with chemical toxicants. Past EMG and nerve conduction studies show he has no neuropathies, which might in some cases result in tremor. Unlike Parkinson’s disease, where tremor first presents on one side of the body, the patient’s tremor involves the hands and arms symmetrically.
The movement disorder doctor comments his symptoms bring to mind the diagnosis of essential tremor. The physician inquires whether alcohol decreases the movements, the patient notes that when dining out he, and his wife sometimes order a bottle of wine. With his first glass, he will see apparent tremor in his hands, by the time the bottle is half-empty, the tremor will have eased substantially.
On physical examination, the patient’s muscles surrounding his joints are loose and supple. The doctor notes he has a fine tremor of the head, and asks whether the patient has ever noticed it. It is very mild, the specialist assures him. Performing fine movements of the finger, in touching a pen and then his nose, the patient’s movements are obviously shaky. In writing, his hand is noticeably unsteady. He comments his characters tend to be hairy- looking, and at other times they are normal. Sometimes he says he forgets how to spell simple words, he concentrates so hard on trying to make the letters round.
The specialist peruses the file folder with details of previous tests the patient has undergone. The physician mentions the patient has some compression of nerve roots in his neck, but that is more likely, in the case of severe nerve root compression, to produce pain, weakness and muscle twitiching or fasiculations. He states the patient’s symptoms are classical examples of essential tremor, with the minor deviation that people usually notice the tremors much earlier in life. The patient is sixty- one and first noticed the tremor in his hands just over a year ago. Inderal (propranolol), a beta blocker is the medication most commonly given for essential tremor. It has an advantage over the current medication the patient uses to decrease blood pressure, as it will function for two issues- lowering blood pressure and decreasing tremor. The doctor creates a schedule for using Inderal, and mentions that essential tremor tends to increase and decrease, though with time it worsens and may come to involve the head and voice. He mentions people with debilitating tremor may choose to undergo deep brain stimulation surgery. The patient refers to his previous neurologist, who mentioned surgery for when tremor worsened to the extent he was starting to throw food around with his fork. The movement disorder physician laughs, conceding that’s a creative way of putting things and he would like him to return in six months time, so they can re-assess the situation.

Teary Eyes

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The patient has small cramped handwriting, poor sleep and difficulty turning in bed as well as rising from a chair. She has come for a second opinion on whether she has Parkinson’s disease. The movement disorder specialist asks her rhetorically how does one distinguish a Parkinson Syndrome from the true disease. He answers his own question by noting to have a syndrome a patient must have three of the four cardinal signs of the disease; resting tremor, slowness of movement, cogwheel rigidity, and/ or loss of balance. The one way to determine whether one suffers from the true illness (idiopathic Parkinsons Disease) is to see whether there is a response to dopamine. If a patient has a deficiency of the neurotransmitter, movements will increase in fluidity and speed, tremor will disappear and rigidity will ease. Certain other illnesses and medications may mimic what patients experience in the illness. Vascular disease can lead to a syndrome of lower body parkinsonism. Diabetes can bring peripheral neuropathies that may result in the patient losing her balance and falling, or have a shuffling gait. Major tranquilizers can bring on characteristics of PD because the medication blocks dopamine receptors leading to an induced state of parkinsonism.
The doctor prefers to use levodopa and carbidopa over the medication called Stalevo. He thinks Stalevo is too expensive and does not permit the patient to adjust the medication to her own needs. Sinemet, he explains to the patient means sin emesis, or no vomit. The medication is the combination of levodopa and carbidopa. It comes as a generic, is less expensive and it is easier to adjust the dose.
The patient symptoms are worse in her right arm than the left, and she is right handed. She keeps the arm tightly at the side of her body. Shrugging the shoulders result in almost no movement. Cogwheel rigidity is present in the patient’s wrist and elbow muscles. The patient confides she has fallen twice in the last year. The doctor is wary about this, he conveys patients with PD usually do not fall until the disease had progressed significantly, though it is possible she may be falling for other reasons.
At different times throughout the consultation, the patient became teary, and the doctor noted that the majority of patients with PD have significant depression. He advised her of the need for an antidepressant, stating that her disease appears quite mild. If she were on a suitable antidepressant, she might be better able to cope with the illness. The patient states she feels depressed because she does not like the limitations she has. He also indicated that the anti-depressant may eventually help her sleep better. She had confided that she has difficulty with sleep and last night only slept for two and a half hours, and got up repeatedly throughout the night.
Though the patient was not happy to receivetwo newprescriptions, the doctor asked her to return in four month’s time to re-assess her symptoms and see how she is fairing.

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