Tremor Predominant Parkinson’s Disease

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The patient who is a stocky wide- shouldered, healthy looking man, says he’s never slept through an entire night in his life. While making an extraordinary statement his face remains unexpressive, placid. His eyebrows and mouth convey no indication of emotion. The physician behind the desk shakes his head and comments the need for sleep varies considerably between people. Though few people respond to Seroquel so strangely. The first time the patient took the drug he slept for twenty-four hours straight. He describes trembling with cold for several hours on one occasion, on a separate night he blames the prescription for extreme tension, which made him pace through the house for hours. Clearly, he responds aberrantly to the medication.
The wife comments he thrashes during the night, sometimes calling out or shouting in his sleep; she reports this occurs three to four times a month. The doctor comments no one with PD has a normal sleep pattern and he describes how rapid eye movement sleep shifts to become out of phase with other sleep architecture- or wave patterns, allowing the person with PD to be able to move while dreaming. Essentially, the person is able to enact dream content, which is not possible in someone else whose sleep architecture is unaffected by illness.
The wife states they have been married forty- seven years. Her spouse leans towards her and comments they have been fighting for forty- six. An amiable banter flows between them. The wife offhandedly states her husband is an artist and describes the fine work he has been involved in, commenting he never has tremor when he’s working, it’s only later on when he’s resting. The physician responds,
“… that’s why it’s called a resting tremor…”
The patient asks whether he can increase the Mirapex he takes while experiencing stressful events, for example he is due to undergo eye surgery to for glaucoma- to release pressure on the orbits of his eyes. It requires the ophthalmologist create holes in the membrane at the back of the eye. The doctor with a head full of wavy and graying hair nods,
“Certainly, there is no contraindication to the drug.”
On physical examination, the doctor finds some rigidity on the man’s left side, and in the neck. When asked to raise his shoulders, he barely shrugs. His walk has little arm swing on either side, and when they retake their seats, the doctor asks how long ago was it when you first came in?
The wife thinks it was 2008, and the records on the computer confirm her guess. In 2008 the patient stated he had experienced symptoms of illness for five years prior to visiting a neurologist. He still gets sufficient symptomatic relief from Mirapex (pramipexole) a dopamine agonist, to avoid Sinemet. The doctor comments that most people require Sinemet after a year or two of treatment with an agonist, and he is an unusually fortunate case.

Teacher with Drug-Induced Parkinsonism

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The patient taught middle school for forty years, and she sits without leaning on the back of her chair. She comes for a consultation about whether she has Parkinson’s disease. Her husband has come with her, as a witness to the changes he has seen in her health. Her falls scared both of them. In the most recent, the patient carried groceries in each arm. She fell straight forward and broke her nose. When on the floor she was unable to rise without assistance.

The medical history of the patient has some red flags for the doctor; the patient doused her garden with spectricide and the toxin caused her thyroid to quit functioning. He mentions that there is a relationship, though not a causal one, between Parkinson’s disease and exposure to pesticides, heavy metals and other environmental toxins. She comments she has suffered from trigeminal neuralgia, inflammation of the fifth cranial nerve that produces intermittent bouts of shooting pain to the side of her face and jaw. Her husband notes he has seen tremor in her hands and a stiff walk, while the patient says she has experienced left- sided weakness, fatigue, forgetfulness, and problems with bladder control.

The patient has taken some medications that may have deleterious side effects. Pravastatin, being one of the cholesterol- lowering statins, recently made the news for its under- reported tendency to invoke muscle pain and weakness, especially in the legs. Her primary care doctor added Abilify, a novel tranquilizer to her medications when he thought Prozac was inadequate for her symptoms of depression. Abilify can block dopamine receptors and produce some signs and symptoms of parkinsonism and in addition can induce tardive dyskinesia in patients, uncontrollable movements of the face, tongue or other body parts and these may wane if discontinued, or become permanent with continued treatment.

On physical examination, the doctor finds no stiffness or rigidity in her muscles, and her gait is normal with a full arm swing. He comments that he can detect no signs of parkinsonism. When he places a tuning fork on the bones of her foot, and she is unable to feel the metal buzzing, though she can feel the vibration in the knuckles of her hands. She is able to discern whether her toes are up or down, but her perception of temperature is also impaired. The doctor tells her that she does not have the clinical features of Parkinson’s disease now. She may have had some symptoms of parkinsonism while taking Abilify, but those have gone away after stopping the medication. Based on her examination he diagnoses a peripheral neuropathy to explain some of her symptoms. The cause of her neuropathy will require more extensive evaluation. A B-12 deficiency, low thyroid function, medications or toxic insult are possible causes of neuropathy. He conjectures a toxic bath, like the kind she experienced, might result in a neuropathy, though the lower extremity problem resembles what a diabetic patient might incur. He urges her to see another physician whose specialty is the peripheral nervous system. He hands the patient and her husband a referral form. The other specialist will thoroughly explore the function of other nerves (nerve conduction studies), and order the appropriate blood and other tests that will aid in ruling out other disorders.

Respiratory Dyskinesia

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She calls frequently seeking help for irregular, fast chest wall and diaphragm movements that make her feel she can’t breathe and that death is imminent. The doctor explains that these irregular movements are leveodopa-induced dyskinesias, known by experts in Parkinson’s Disease as respiratory dyskinesias. She wasn’t due back for another four months. Her caregiver, a woman with dark blonde bushy hair, takes care of her medications, pulling bottles from her large bag to check how many refills remain.

The problem is the pharmacist. He cancels the previous levodopa medication when she gives him a fresh prescription for a new type of dopamine replacement. The doctor throws his hands up at this, exclaiming the job of the pharmacy is to fill the prescription correctly, not to judge if a medication should be replaced.

“They should have called me, if there was some discrepancy about the medication.” His jaw set, his hands express his agitation. The patient shakes her head conceding it is an awful situation.

She is prepared though, having brought a list of concerns with her. What can be done for constipation? The problem affects most people with PD. The remedies are not consistently reliable and the doctor urges the patient to incorporate all the methods; eat prunes, drink enough water, eat enough fiber, and get regular exercise. Miralax taken daily helps pass stool by increasing its fluid content. Other patients have tried senna leaf teas, though they can induce abdominal cramping and diarrhea.

The patient worries her blood pressure gets too high during the day- sometimes rising to a systolic level of 180. The doctor mentions she takes a medication to regulate her high pressure, and should not worry needlessly. The medication, Sinemet tends to reduce blood pressure further.

The physician leans back in his chair and urges the patient must find an outlet for her anxieties, and that yoga might be helpful in controlling the dyskinesias that obstruct her ability to breathe. He mentions the mind – body connection, asking the caregiver for the usual time dyskinesia’s are worse. She replies when they have a visitor coming, when the patient gets angry or frustrated, or any other stressful time.

Second Opinion

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The dark glasses he wears lie on the physician’s desk in front of him; he had cataracts removed from both eyes, but he still suffers from macular degeneration- a condition where center field of vision blurs and eventually leads to blindness. His visit is a result of a recent diagnosis of parkinsonism. He has come for another opinion.

Losing his hearing, the patient asks the doctor to speak up, interrupting him, as he speaks. The doctor repeats himself, with abbreviated thoughts. The patient holds his palm up towards the doctor, interrupting, telling him to let him speak. His hands are big, his finger long. Several of the fingernails on his left hand are cropped off, midway through the nail. He is 88 years old and expects to live to live to 120. An American chess champion in the over 75-year division, he visits Florida once a year to compete. He claims in his youth he could play ten games blindfolded, now he can play only one game this way; his short term memory is not what it was. But it is the tremor in his hands that bothers him, especially when eating soup. Three years ago, he noticed his handwriting became larger and shakier. He comments also he has lost the bounce in his step; he no longer rises up onto his forefoot when he walks. Balancing is tricky.

The doctor stands and takes the man’s hand, and folds it inward towards his shoulder and out. He tells the patient his upper body is supple, without rigidity. The patient concedes he was a magician, and takes a packet of cards from a small leather case in his trouser pocket. He describes a trick he was able to do with one hand, holding the deck of cards divided into two bundles, he was able to shuffle them with one hand. Standing, he positions the cards in his left hand, and then nothing happens. The doctor follows his actions, and nods, understanding dexterity is gone from his hands. The doctor assesses the patient’s sensation with a large tuning fork asking whether he can sense the vibration of the fork, when applied to the bony prominences of his feet and legs. Noteworthy, the patient fails to feel vibration applied to the right leg. The physician explains it is a cheap and easy way of assessing the integrity of the long sensory nerves in the body, and states the lack of sensation explains some of the change in his walking style, as he appears to have a mild sensory neuropathy. The cause, the physician guesses is from compression of the nerve roots in the spine. The doctor explains we rely on three mechanisms to keep us upright in space; position sense derived from the sensory nerves in our limbs that pick up vibration, fine touch and temperature; our vestibular system of the inner ear and our vision.

When the physician summarizes his findings, he notes the patient has a mild action tremor, and a mild sensory abnormality in the right leg and foot. He would like an MRI to look at the blood vessels of the brain. However, the patient leaves on Thursday to his home state, he’d prefer to have the testing performed there. The doctor agrees to send notes to the physicians involved in his care and the conversation shifts to what sort of cutting edge therapies exist in the field of Parkinson’s and Alzheimer’s disease. Sitting behind the desk, the physician explains an experimental study in which people with Alzheimer’s are receiving GCSF (granulocyte- colony stimulating factor) to remove the amyloid plaques from the brain and improve cognition. The patient voices some interest in undergoing the same treatment, and the doctor wonders whether that would be ethical, or even practical, as the patient lacks the symptoms of those with the illness. He also notes when the amyloid is removed it can get stuck in small blood vessels, and result in micro-hemorrhages. He is unsure of the consequences of such trauma in the brain of a healthy, yet older individual. The man, wearing a woolen red sweater over a collared shirt, reaches into a file and withdraws the list of therapies he receives regularly from a physician whose specialty is aging. In the second or third line is a product called Neupogen, the same substance used in the research study for patients with Alzheimer’s.

Small and Golden-haired

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He’s small, golden haired and his voice is soft. Sitting in the chair opposite the movement disorders expert, his left hand jiggles as he rests it in his lap. After having a heart attack and triple by- pass surgery he noticed the tremor in his left hand. Sometimes now, he feels the toes of his left foot curl inward and his right hand seems shaky. An optician, he needs steady hands when he must retrieve an object from a patient’s eye. Sometimes his voice slurs, and he has to carefully enunciate his words, otherwise patients do not understand him. He and his wife have formed a plan; he plans to retire and give his practice to a younger man who has been working with him for several years.
The physician comments many people observe the first signs of illness after a trauma. The incident may be psychological, as in the death of a loved one, or a physical insult, as in a car accident. Both types of trauma exert an impact on the physical wellness of a person, allowing symptoms gone unnoticed, to suddenly become apparent. In Parkinson’s disease, physical manifestation of the disease presents when the majority of dopamine producing cells in the substantia nigra have died away.
Unlike other patients that come with a multitude of problems, typically… diabetes, high blood pressure, gastric reflux, degeneration of the disks in the back, diminished sensation in the feet, brought on by chronic high blood sugar, this patient appears healthy. He is slim, fit and well groomed. The pink button down shirt suits him. His voice is low, lacking volume, – comments the doctor, who asks whether this has always been the case. No, he admits, his voice has become much softer.
On physical evaluation the doctor finds minor rigidity in the left hand. A suggestion of rigidity is barely perceptible on the right side. In evaluating gait, the patient holds his left arm much more rigidly to his side, than the right that swings naturally with his stride. Fine motor movements decrease in amplitude and slow with repetition on the left side. Reflexes of both legs and arms are brisk, suggesting some other agent of illness at work. Parkinson’s disease patients who do not have other medical problems have normal reflexes. The physician asks whether he has had an MRI, and the patient states the most recent test reported areas of ischemia in the brain. The specialist shakes his head, agreeing the finding of brisk reflexes suggests he may have suffered a small lacunar stroke.
The doctor encourages the patient to exercise aerobically at least three times a week. He cites a research study conducted on parkinsonian monkey, in which they were trained to run on a treadmill. The running time gradually increased until they were jogging up to three hours per day. Animals who exercised were able to overcome the weakness and rigidity on one side of the body while the animals who were sedentary continued to drag one side of the body. When all the animals were sacrificed, and researchers found that those animals who had become athletes, had caused their remaining dopaminergic cells to re- sprout….Hence solid evidence, at least in monkeys, that regular aerobic exercise is good for the brain.

Dangly Earrings

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With reddish hair cut short and dangly earrings the patient sits as the doctor skims the pages of information she has filled in. Her right hand moves gently in her lap. He gazes up at her and asks what brings her to the movement disorders clinic. She looks down at her hands in her lap and raises her right hand to exhibit the fine tremble occurring between her two first fingers and thumb. As she does so the movement disappears. The physician asks when she first noticed the tremor and the woman replies it has been there awhile. At first she thought it was simply nerves, but when it kept reappearing she grew concerned and spoke with her father on the telephone. He was diagnosed with Parkinson’s disease when he was sixty five. She is forty-eight.

The physician explains to be diagnosed with PD one must have two of the three principle symptoms, and respond positively to the drug levodopa. The three classical symptoms are slowness, rigidity and tremor. Her emotions overtake her and she cries, taking a tissue from her bag, the doctor continues explaining there are far worse illnesses to be given, though there is still no cure for the progressive neurological disease. When she inquires whether her children are at risk, he comments the hereditary component is present in a minority of patients, though some studies have found a genetic component for the disease.

The doctor asks about other symptoms and the patient sighs and responds she has trouble sleeping, but has had the problem ever since she had children. Sometimes, she concedes she feels slow in the head. When she and her husband go to the movies she frequently doesn’t understand the humor. Across the desk the doctor smiles and asks whether she can recall the last show she went to. The patient dabs her eyes as her gaze drifts above the physician’s head, in thought, and she smiles ruefully unable to recall the title.

Switching seats to the examination table he asks her to raise her shoulders in a shrug. Her neck seemingly vanishes, her shoulders are so supple. Her arms and wrists however have some stiffness in the surrounding muscles. As he works to assess her rigidity, the doctor speaks about the importance of exercise. She smiles and remembers the title of the movie, Hangover Two. He commends her choice of cinema, affirming laughter is good. As he speaks her smile fades and he wonders aloud whether she is taking an antidepressant. She shakes her head no, telling him she prefers to take as little medication as possible. The physician concedes most of his PD patients eventually need an antidepressant to function at their highest quality of life. The patient looks at him without comment.

Time and a Scooter

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She is blond and well tanned. Her spouse wears green trousers a matching polo shirt. They have been married for forty nine years, which explains some of their dynamics. The husband scoffs at his wife and rolls himself to the side of his chair, away from her. She conveys he has lost some memory. The wife knows the facts and conveys them easily without stammering. She answers many of the doctor’s questions, when she sees her spouse stuck on a syllable. Like the small steps the feet take, the person with PD may have problems articulating thoughts- both repeat a motion, though little change occurs.

The doctor asks about the schedule of medications, and the wife replies that her spouse may forget a dose when occupied, so the schedule is constantly changing, though he strives to take his pills every three hours. The doctor rips a page from the pad of paper on his desk and begins constructing a table that would make up a medication diary. He states he can be of little use unless he is aware of how the patient responds to his medication; how long it takes for the pills to take effect, whether he experiences dyskinesias, and when they occur. He asks the wife to attempt the diary for a period of two weeks, so that he can see where patterns emerge. Email it to me, he says. With that information they can modify the daily course of drugs.

She is pleased that the physician has discouraged the two doses of night- time medication. The doctor insists the patient must have six hours of sleep nightly, at least. To make this a possibility he recommends the drug Seroquel, to be taken in gradually increasing quantities until the patient finds he is sleeping through the entire night. An enlarged prostate means the man must rise to urinate several times in the night; Depends may be needed when the quality of sleep improves.

Diagnosed with the illness fifteen years ago, the gentleman underwent deep brain stimulation surgery a year and a half ago. The physician asks whether they have seen an improvement in symptoms, and the wife shakes her head, doubtful. Then she notes her spouse no longer has tremor at all. While he demonstrates his gait in the hallway outside the office, the patient’s arms swing freely. The arm swing, the wife notes is also much better, he used to carry his right arm next to his torso. He takes the same amount of dopamine replacement.

After the physical exam the wife mentions her spouse fell in a field and she was unable to help him to his feet. They had to wait for some time, until another person appeared to assist. She worries he will fall again and wonders whether the physician can help them acquire a motorized scooter. The doctor writes the couple a prescription for physical therapy, with attention to gait and balance with the request to evaluate and fit the patient with motorized scooter. The patient comments to the physician he is unlike other doctors, he has given them some time.

Wife Remains in the Waiting Room

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The patient comments he asked his wife to remain in the waiting room for several minutes while he discussed something personal with the doctor. The eyebrows of the physician rise as he asks whether it would be OK to have two medical students and an observer attend as well. The man tips his head forward slightly and says sure as he sinks slowly into the office chair. He glances at his watch and begins speaking in a low breathy voice.

Someone is trying to poison him. He’s sure of it. With some effort he takes a pill from his shirt pocket and places it on the desk in front of the doctor. The doctor frowns, gazes at the pill and asks whether he has been taking Seroquel, because the pills looks like the 50 mg tablet. Turning to the computer the doctor brings up the list of the patient’s medications as the man across from him states he found the pill in a bran muffin, that morning.

The physician nods once, and holds one finger up to the patient, who continues to expound on how he can no longer live with people who put pills in his food. He reads the note he wrote after the previous visit, aloud.

Reviewed with patient and spouse the benefits of Seroquel, or quetiapine, and how it diminishes the frequency of visual hallucinations when taken during the day and improves sleep, when taken at night. Suggested patient begin with a 25 mg tablet at night to induce sleep…

The doctor looks over at the man with the white dense eyebrows and asks whether he takes a small pink pill in the evening. The patient nods, his wife always brings him his night- time dose. He takes two and sleeps well. The doctor asks whether he takes any during the day. The patient’s eyes drift towards the ceiling, until he is looking upwards and he replies he prefers to see the things he does, than feel sleepy and dullwitted.

A light rap on the door makes all turn their heads and the nurse and spouse appear in the doorway. Motioning to the empty seat, the doctor invites the wife in and mentions they have been discussing medications. A similar age to her husband, the wife has her white hair tied back in a pony tail. She nods as the doctor continues speaking affirming the patient’s preference for hallucinations over Seroquel during the day. Her cheeks redden, and she concedes her spouse was a painter at one time and his passion for visual images might be understood better, knowing his background.

The physician approaches the patient and asks him to sit on the examination table, where he feels for rigidity in the wrists and elbows. While he examines the man, he asks who prepares his breakfast in the morning. The patient works on tapping one hand into the palm of the other, the wife replies the day nurse prepares breakfast for her spouse because she is usually out of the house by the time he wakes. As they return to their seats the physician makes eye contact with the patient, and asks whether this is an appropriate time to share the concern. The husband makes a gruff sound as he lowers himself into the seat and the doctor raises the white pill in his forefinger and thumb. The wife looks on with expectation and puzzlement as the physician explains her husband found the pill in his breakfast muffin. Her expression changes radically and she takes the arm of her husband and speaks into his expressionless face, whispering loudly she will speak with this woman.

Yellow Polo Shirt

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His belly fits snuggly beneath a yellow polo shirt. His arms move naturally and freely with every step while he walks in the hallway. He turns without hesitation, wearing black athletic shoes and shorts, and returns to his seat in the small office. In the chair he comments when his medication is not working he can barely move his feet. The spouse beside him is tall with thick graying hair tied away from her face. Before resuming his seat the doctor confides that in the old days before levodopa, people with PD would gradually bend at the waist, until becoming quite stooped in posture. He demonstrates the posture rounding his shoulders and allowing his head to sag forward. The spinal bones would acclimate and take on the imposed curve in the back, so the permanent posture became bowed forward, though It’s hard to imagine this large man impeded by illness.

He asks about the blue pill he takes at night, relating that when he takes it, it’s hard for him to get up in the morning. He won’t take it for fear he’ll lose his job. The doctor comments, it’s not an all or nothing proposition. They can cut the dose by half. Doxepin has several benefits; it has anti- depressant effects, inhibits the bladder and increases sleep.

The physician notes the patient’s blood pressure, 94/ 57 taken while sitting. He comments it would be even lower if the nurse had taken it while standing. It’s too low for someone who works outside and is liable to become dehydrated and have his pressure fall even lower; he’s apt to faint. Medications for Parkinson’s disease cause blood pressure to drop, while the illness affects the ability of smooth muscle in blood cells to contract, allowing blood to pool in the lower body when standing and working for prolonged periods of time. He checks the other medications and notes the patient takes three other drugs to lower his blood pressure, and wonders aloud who manages his general health. His spouse, in the chair by his side, replies he will see the general practitioner the following morning, and should discuss which of the three medications would be best to forgo.

Dying FishTremor

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The doctor says the worse you sleep, the more likely you are to hallucinate under the influence of the anti-parkinson meds. The patient took a sleeping pill a friend gave him, a valium, and slept like a baby. The physician explains those drugs are fine for the short-term. Taken repeatedly they lose their efficacy, and over time people become dependent on them; without the medication they feel anxious and irritable. Being depressants, people with Parkinson’s disease should avoid drugs that depress the nervous system; they tend to worsen the depression that accompanies PD.

He’s forgetting a lot of things. He had a girlfriend who cooked for him, but she left him.

“And you’ve forgotten her.” The doctor comments. I can’t tell if he smiles.

He doesn’t want to take Seroquel, it gives him a headache. The doctor urges him to give the medication a chance, he has not yet found the right dose that will help him sleep. The patient comments his mother takes it also, and she hallucinates all the time. Explaining the medication is given to decrease hallucinations and promote sleep, the physician says doses can range from 25 mg up to 600 mg. Everyone has to discover their own requirement, between getting a solid night’s sleep, and being excessively sleepy the following morning, or not sleeping at all. He asks whether the drug comes in a generic, and the doctor nods, though the generic quetiapine fails to be listed among his choices to prescribe.

He isn’t able to work. His mind is failing and he has noticeable tremor in his left hand. The hand flails about the physician’s desk like a dying fish. He has to wait two years until he can apply for complete disability though he was diagnosed with PD a year ago. Concerned about paying his bills, he doesn’t want to declare bankruptcy. The Seroquel is expensive.

The doctor types while he asks the patient what sorts of things he does during the day. Wearing a baseball cap and white athletic shoes, the patient says he rides his bike in the neighborhood. The doctor nods commending the activity telling him he needs to exercise daily.

He drives two and a half hours from Sebring for his appointments, but he’s content to do so, it’s only every six months. As he leaves the doctor assesses the muscles in his upper body, noting they feel fluid. In the hallway he walks with a bilateral arm swing, if it wasn’t for the tremor of his left hand he might appear normal.

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