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 retina gradually thins and results in blindness.  He carries a large magnificying glass which he holds close to his nose as he peers at his list of medications.  The purpose of the visit is to seek another opinion regarding the tentative diagnosis of Parkinsonism.  He has already seen several physicians who have conflicting opinions about his tremor.
 

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 states with a serious expression that he expects 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. With a tuning fork, the doctor assesses the patient's reflexes and notes whether the patient 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 way of assessing the integrity of the long 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 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 a study in which people with Alzheimer's are getting 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.

 

 

 

 

Alot can happen in seventy-six years; twenty years of hypertension, seven years of diabetes, a quadruple bypass for the heart, three transient ischemic attacks, two strokes, thyroid imbalance, and Parkinson's disease brings the patient in to see the specialist in movement disorders. The patient was diagnosed only three months prior, and yet he is in stage three of the illness (according to the H&Y scale), evident with bilateral symptoms and impairment of balance and equilibrium. His dose of Sinemet is high, 25/250 three times per day, and yet he experienced no nausea when he began the medication.

 

The patient wears a freshly starched short sleeve linen shirt. He is balding and he wears glasses, yet he takes them off when the doctor examines his eye movements. The spouse comments the way her husband holds his lips and jaw have changed. He admits he grinds his teeth now, the doctor comments some of the jaw clenching may be an involuntary movement caused by relatively high doses of Sinemet.

 

The patient has several atypical symptoms that may be reason to consider whether he suffers from Shy Dragers Syndrome, currently called multiple system atrophy.

 

Doctors classify the disorder into 3 types:

• the Parkinsonian-type: symptoms of Parkinson's disease- slow movement, stiff muscles, and tremor

• the cerebellar-type:  causes problems with coordination and speech

• the combined-type: symptoms of both parkinsonism and cerebellar failure. Problems with urinary incontinence, constipation, and sexual impotence in men happen early in the course of the disease. Other symptoms include generalized weakness, double vision or other vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating.1

 

Patients with the disease respond less favorably to medications aimed to treat PD symptoms, though they may derive a general feeling of well- being. The wife of the patient reports he has dramatically improved with the medication. He lacked any facial expression, his arm failed to swing when he walked, and the tremor in his hands and feet was nearly constant. All of these symptoms have changed for the better with medication.

 

The loose ends hang about, unresolved. For example, when he is not speaking the patient lapses easily into a pattern of hyperventilation. Could this quirky behavior be the result of ischemia, or a lack of oxygen, to the vessels in the brainstem that govern the rate of breathing? What can account for a sudden fainting spell, besides a sudden drop in blood pressure, which commonly occurs in Shy Dragers? What can be said for the need to urinate every two hours?

 

The doctor recommends the patient stay on the current level of medication, though it may behoove him to add coenzyme Q10 to his daily intake, at levels between 300 and 900 mg per day. The specialist advocates aerobic exercise, beginning at thirty minutes, three times per week, and notes he would like to see them again in four months, to check on him.

 

Difficulty writing was the first symptom of his illness. Then his wife noted his walking was slower, and his face more fixed and rigid. Since diagnosis in 2001, he has taken a very light dose of medications: three doses of 25/100 daily and Mirapex. The doctor comments he has had a long honeymoon period; fortunate man. He sits without expression in the office chair, yet he asks questions. He wonders about the frequent hallucinations his is having.  The doctor tells him that hallucinations are very common in PD patients.  He relates a story of an older man who sees a young naked woman get into bed between him and his dozing wife. The physician asks the patient whether he reached out to see whether the woman was a hallucination, and he replied he didn't dare move, for fear he would wake his sleeping spouse. That dream the patient comments, is one he would like to have. Instead, the dog hallucination visits him nightly.

 

When the honeymoon period runs out, patients begin to experience lapses in the effectiveness of medication. These periods, commonly known as "off" periods become more pronounced as illness progresses. The doctor notes the dopamine- rich cells in the brain lose their ability to store excess dopamine, their buffering capacity wanes and patients begin to vary in their levels of function according to the level of medication that reaches the brain. Here, the physician begins speaking about the importance of avoiding proteins, especially milk proteins in the morning meal. Milk proteins compete strongly with the morning levodopa (Sinemt) for passage into the brain; their presence in the diet inhibits the ability of levodopa to get through the blood- brain barrier.  This is the reason for having a non-dairy creamer like Cremora instead of milk in coffee and cereal. Dopamine agonists, like Mirapex do not have this problem.

 

The physician dips into a discussion of sleep and PD, noting the disease ruins normal sleep architecture, causing sleep to fragment. Patients may doze during the day.  Excessive daytime napping impedes sleeping ability during night hours, and works to further weaken normal sleep cycles. The body requires a certain amount of rapid eye movement sleep, when not acquired at night, the person with PD becomes susceptible to hallucinations, which are essentially waking dreams. In a study the physician conducted, he found 26% of patients with PD hallucinated; all 26% had fragmented sleep. Novel tranquilizers, such as Seroquel and Clozaril, when given in small doses in the evening counteract fragmented sleep patterns and encourage slumber. The physician prefers patients have a solid length of time given to sleep, as it is more likely they will acquire the needed amount of dreamtime. With a fixed sleep schedule, patients are less likely to hallucinate.

 

The practitioner- researcher informs the patient and family about a clinical research study he's involved in, asking whether the patient would be willing to provide a sample of blood. The aim is to find out whether an agent or biomarker exists in the blood that changes with progression of illness. By identifying such an entity, it would be possible to gauge whether medications can truly inhibit the progression of disease.

                Yenta, was the word she used to describe her brother in law. He has a lot wrong with him; the high blood pressure was discovered when he was in his thirties.  Diabetes; he uses an insulin pump, and has a neuropathy in the left leg and foot, as a consequence of the illness. He underwent cardiac ablation, had a triple by pass surgery to his heart, and wears a pacemaker. The pacemaker for his heart makes an MRI of the brain impossible. He is sometimes incontinent, has difficulty rising from a chair, and has fallen and injured his right shoulder; doctors think he has a torn labrum and will need surgery. The toes of his left foot curl up in dystonic spasms he cannot control. Some time ago, he worked as an architect, and he retains the ability to draw well, though his handwriting has succumbed to illness.

                They come for another opinion of what they can do for their family member. The patient has had speech and physical therapy. The specialist reads the notes from other physicians and the differential diagnosis; the list of possible diagnoses the patient may suffer from. Over the course of several months, the wife has seen her spouse decline in function. She contends he has lost a lot of drive.  He was a Type- A personality and now lacks the motivation for common things. His personality has become more emotional, and he admits he cries easily. The physician listening comments to the medical student sitting next to him that it sounds as though the frontal lobes are affected, as the area on both sides of the brain, dampens emotional expression.

                The specialist performs the physical examination and notes the patient's eye movements are full. The women mention the patient usually has his eyes closed, and frequently walks into objects when using his walker. This makes some sense to the specialist, as other neurologists have noted he may suffer from Supranuclear Palsy, which usually results in the patient having difficulty looking downwards,  though this is not his problem right now. Botox injections to the muscles of the foot helped relieve the uncontrollable spasms on the right. When given to the muscles around the orbit of the eyes, they have been less effective.

                The specialist is concerned the patient may suffer from normal pressure hydrocephalus, which can mimic vascular, or lower body parkinsonism. He requests a CT of the brain, which will show whether the ventricles are enlarged. If so, a neurosurgeon can place a shunt in the brain, allowing the excess cerebral spinal fluid to drain out of the brain and into the body.

                The doctor recommends the family have a consultation with a fellow neurologist trying to assemble a group of patients for a study on progressive supranuclear palsy. The colleague intends to do a drug study to discover whether a certain medication is useful in that population. At the very least, they will get another opinion from a movement disorder specialist, who will have the results of the CT of the brain to possibly to rule out normal pressure hydrocephalus.               

About five years ago she noticed the tremor in her right hand. Now as she sits in the examination room, her slightly swollen hand vacillates steadily as she rests it on the arm of the chair. She feels the muscles cramping and shortening and uses her other hand to straighten the fingers. Gradually she has lost most of the strength, dexterity and the ability to write legibly. She states she has taken Azilect for two years and sees little improvement.

 

Ah, but that's good, the doctor states. It means progression of your illness is slow; the other side of the body may be affected with disease, within that time. The comment buoys the patient's sentiments, somewhat. The specialist in movement disorders asks about her prior medical history and learns the patient underwent heart surgery to amend a leaking heart valve, seven months prior. Surgeons removed her thyroid two years ago and she suffers from high blood pressure, and high cholesterol. She states she is healthy, otherwise; comments from an optimist.

 

As the specialist dives into a description of how and who implicated dopamine as the neurotransmitter depleted in Parkinson's disease, the steady murmur of his voice and the quality of the fluorescent overhead lights lulls the listeners into a stupor. The patient has brought a cup of coffee with her, remarking it's decaffeinated, and the doctor remarks smoking and drinking coffee are two habits that are negatively correlated with illness and PD; the more one smokes and drinks coffee, the smaller the likelihood one will acquire PD.

 

On physical examination of the patient, the specialist discovers brisk reflexes; probably a byproduct of high blood pressure he comments. He inquires whether she has had an MRI of the brain, as people with long-standing high blood pressure commonly have a multitude of small white spots scattered just under the cortex of the brain. The neurologist feels the fluidity of movement from the left elbow and wrist and senses some rigidity in the muscles of the biceps. When he asks her to walk in the hallway, her gait is regular, with wide steps though she tends to hold the right arm and hand at her side, while she swings the left.

 

Sitting again in the examination room the patient asks about exercise. The doctor encourages the patient to discover when her cardiologist feels it safe to increase her heart rate, and then describes a clinical experiment in which researchers trained monkeys  to jog on a tread mill six hours a day. Those mokeys that underwent an experimental unilateral injection of a toxin that destroys dopamine neurons to one side of the brain, recuperated much faster, while those that did no exercise remained disabled. He states exercise, especially aerobic activity, enhances repair in the brain and provides a neuroprotective benefit. The patient confides she had to end her membership to the gym, as the temptation to get on the aerobic machines was overwhelming. She states she has always been an active person, and not allowing her heart rate to climb has been difficult. Yoga and Tai Chi are also helpful for those with PD, the physician states.

 

The doctor creates a chart for the patient, detailing how to increase the dosage of Sinemet. She should aim for the smallest dose that eliminates the stiffness and rigidity in her hand. He also encourages her to seek physical therapy for her hand, to regain strength and extensibility in the muscles. The doctor states he would like to see her again in three months time to see whether the transition to Sinemet has gone smoothly.

 

Before leaving, the patient reveals she lived in Alaska for sixteen years raising four children. The darkness of the winters never bothered her, or made her feel blue; an unusual blessing for a person with PD, where depression affects 70% of the patient population.

 

Entering the examination room the male patient sits in a chair with a large board for writing. Perhaps he has had a stroke and can't speak. The patient writes his wife will return soon and the physician begins speaking with him, as he sits down. This clinic, Tuesday morning clinic is a Parkinson's disease clinic, he explains to the patient, who nods in reply. In front of the doctor is a list of symptoms the patient deals with. Tremor is high on the list. The specialist continues speaking, reading the list of medications the patient relies on, and the three pages of medical history patients are asked to fill out.

 

The wife enters wearing a purple summer dress. The doctor asks when the tremor in the hands first began, and the spouse replies he has had tremor for a long time, and being a mechanic and depending on his hands, he has not worked in some time. The patient writes well, when he chooses to report something, with no indication of tremor in the handwriting. The wife clarifies the story by noting the patient required hospitalization in February after going on a drinking spree of four days. He became psychotic and lost touch with reality. In the care of a community hospital, the patient received an IV drip of saline, and nothing more. The nursing staff did not give the patient thiamine with the IV? The physician asks,and the wife, once a surgical nurse, reported never having seen anything more than saline.

 

Much earlier in his life, the patient was in a car accident that trapped his hands between his Thunderbird T- back and the asphalt road. Surgery attempted to reconstruct his fingers, but several digits remain stuck in a claw- like position. Another accident; the hammock he slept in collapsed during the night, resulting in fractures to vertebra in his neck and damage to nerves in the left arm, leaving the hand numb, and the left arm useless.

 

More recently, the wife having taken a position requiring traveling from Monday through Friday, saw little of her spouse and he began drinking heavilyHence the hospitalization for psychosis, in February. Four days after having returned home, the patient again became delirious, hallucinated and lost the ability to walk. The doctor states the scenario sounds reminiscent of Wernicke Korsakoff Syndrome, where drinkers undergo debilitating alcohol withdrawal. The wife took her spouse back to the hospital, where she claims hospital staff related they could do nothing for him. The cause of the syndrome is due to severe deficiency of thiamine, and if not treated the drinker undergoes damage to the brainstem; memory, gait and voluntary gaze are all affected.

 

On physical examination, the patient is weaker on the left, probably resulting from the old injury to the neck and damage to the nerves that innervate the arm. Muscle tone is supple, and the specialist feels no rigidity, and sees no slowness in movements. Resting tremor is slight.

The doctor views the MRI conducted in the community hospital and notes the poor test quality. Though judged normal, the physician notes some shrinkage of the midline cerebellum on the MRI, typical of those who drink heavily. He comments it's worth having the test repeated at the institution, as their current technology may be capable of catching something unremarkable on the poor quality image of the brain. He admits he does not know the cause of the patient's inability to speak. He asserts the problem may be a psychiatric one, though all psychiatric illness has a physical disturbance that can be explained, biochemically. The wife interjects the psychiatrist told her yesterday it is not a psychiatric problem. The doctor counsels the patient to care for himself, by eating a healthy diet, taking a multivitamin and exercising daily, and the brain will repair itself, and not to seek too many doctors as they're likely to mess things up.

 

There is no Parkinson's here, the doctor concludes. Sent by another neurologist who noted the tremor of his hands, the patient came to rule out a movement disorder. The effects of medications cause many movement disorders, the doctor concedes, and at least one of the drugs the patient uses, lists tremor as a side effect. The psychiatric medications the patient depends on have stabilized his bipolar disorder, and the neurologist feels reluctant to change any of them, though he feels the patient would benefit from physical and speech therapy, and a new MRI.

 

The patient's record describes a man of five feet, seven inches but wearing my rose- colored heels I tower above him; I stand five feet, four inches on a tall morning. He has the hunched posture seen in many Parkinson's patients and he comes to the clinic with the major complaint of falling. The nurse who accompanies him says she sees him most days, and she has not noted slowness in his motions. Perhaps this is what comes of living in a home for the elderly, the staff become accustomed to a new normal, where most of the population is slow, frail and hesitant to speak.

The doctor asks the pale patient what line of work he was in and the man reports he worked for the government in security and computer services. The physician reflects how in 1970 he used cumbersome discarded equipment from the phone company, with wires connecting networks, rather than a true computer to construct a feed automation system for a small population of monkeys. Today, a system performing the same type of tasks fits inside a tote bag.

When did you first notice the hand tremor? The doctor asks. The man replies it was approximately five years ago. The nurse states she has never seen the patient's hands shake. Problems walking developed about two years ago. He has fallen several times since then; the last accident resulted in a fractured arm. The doctor notes the patient is taking Risperdal, which is contra- indicated in patients over the age of sixty, because people in that decade of life are commonly depleted in dopamine, and the medication blocks the actioins of the little neurotransmitter that is left, resulting in a Parkinson syndrome. Hallucinations caused the nursing home doctor to order the medication for the patient. The movement disorder doctor is flustered, his tone of voice rises, as he adamantly states the drug is not recommended for those with parkinsonism or even healthy older persons. There is another medication, Seroquel that is superior for seniors with a propensity for seeing things. Later he confides physicians become too comfortable prescribing the same medications for all their patients, eschewing the newer drugs that may be more effective and have fewer complications.

The doctor rises quickly and begins the physical exam, noting the patient is quite rigid in the muscles of the right wrist. He has cogwheel rigidity in both wrists, slowness of movements, and acute stiffness and rigidity in the neck. While flexing the patient's joints he declares the left side seems worse than the right. The slowness also carries over to swallowing and blinking the eyes. The patient's record notes he takes Aricept, a medication given to improve his diminishing memory and intellect. The patient confirms he has had trouble remembering things.

After giving the patient a mini- mental examination, the physician wonders whether there may be something else behind his symptoms. The mature man has difficulty placing the twelve digits on the face of a clock, and difficulty in executing a copy of a polygon; the problem lies in spacial relations. Perhaps he has had an undiagnosed stroke, or perhaps the high blood pressure prevents oxygenation to the parietal lobe of the brain. A score of 24 correct answers from a possible 30 indicates the patient has mild to moderate cognitive deficits. The movement disorder physician urges the patient to undergo an MRI, as he has not had one, and it may give some clear reasons for his symptoms.

The doctor comments he would like them to return in four months time, to see whether the change from Risperdal to Seroquel has improved the patient's ability to move, and whether Sinemet has been effective in increasing the ability to move, swallow and blink. The patient rises without assistance from the office chair and uses the walker nearby to exit the room. He says he feels better already and heads spontaneously towards the right corridor and I correct them, to the left. The nurse takes the patient's jersey shirt in her fingers and tugs gently, "Left, baby".

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.

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.

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 Parkinsons 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.

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.

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.

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 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.

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.

The patient is a trim and tall man who spent his youth working the family farm in South Dakota. He recalls driving the truck through the fields, spraying herbicide from two enormous containers on either side of the vehicle across multiple lines of plants, no shirt, breathing and feeling the mist settling on his bare skin. The Environmental Protection Agency states, the product 2,4-D is a herbicide used on a number of crops. "At high concentrations, it affects the central nervous system in humans, with symptoms including stiffness of arms and legs, incoordination, lethargy, anorexia, stupor, and coma." The movement disorder specialist remarks that this may be a factor that may have contributed to the development of his illness many decades later.

Asked about the earliest symptoms, the patient reported a three month interlude in his life in which he did little more than crawl in and out of bed, his fatigue was so overwhelming. Subsequently diagnosed with chronic fatigue syndrome, the patient recuperated to a large degree. The patient who is too young for retirement, describes feeling energetic for about an hour and a half every morning before feeling tiredness overtake him. He currently works less than two days per week.

Another predominant symptom is his lightheadedness. The doctor checked his blood pressure and reported it was quite low, taken in the sitting position. Blood pressure usually drops when standing. Medications for Parkinson's, levodopa and dopamine agonists both lower blood pressure in patients. The physician encouraged the patient to salt his food liberally in the weeks ahead, take his blood pressure twice a day, and see whether the lightheadedness dissipates. If adding salt is ineffective he might try wearing compression hose, sometimes called T.E.D. hose, which prevent the pooling of blood in the lower body when a person stands. The garments are tight and hot, and hard to tolerate in the heat of Florida. The last resort is a medication, Florinef, which causes the kidneys to retain sodium, which in turn causes the body to retain water, thereby increasing blood pressure.

The patient received a diagnosis of probable PD less than a year ago, though the presentation of his symptoms, namely the bothersome low blood pressure is a hint of another less favorable illness. Symptoms of trembling in the wrist and hand began on the left side, and now involve the right side. Bilateral symptoms place the patient at stage two, on the Hoehn and Yahr staging scale. The patient commented that his voice has lost volume; it used to be less whispery. His face lacked expression, as well. On physical examination the physician noted the patient's limited shoulder motion, he commented about the rigidity of axial, or trunk musculature, though his neck appeared supple. Both the physician and student were able to elicit rigidity in the arm muscles, and in gait, the patient had full arm and leg swing. Tandem gait, walking heel to toe, was more difficult and the patient spread his arms to compensate for the narrower base of support.

The less favorable diagnosis is what used to be called Shy Drager's Syndrome. Patients who can tolerate levodopa, may benefit from the medication. Toleration comes by managing the constant tendency for blood pressure to slide downwards. The physician asked the patient to return in six months. Time will tell whether the patient suffers from an atypical presentation of Parkinson's, or a more complicated illness.

The National Institute of Neurological Disorders and Stroke breaks Shy Drager's Syndrome into three possible types; Parkinsonian-type includes symptoms of Parkinson's disease such as slow movement, stiff muscles, and tremor; the cerebellar-type, which causes problems with coordination and speech; and the combined-type, which includes symptoms of both parkinsonism and cerebellar failure. Problems with urinary incontinence, constipation, and sexual impotence in men happen early in the course of the disease. Other symptoms include generalized weakness, double vision or other vision disturbances, difficulty breathing and swallowing, sleep disturbances, and decreased sweating.

http://www.epa.gov/ttn/atw/hlthef/di-oxyac.html

http://www.ninds.nih.gov/disorders/msa_orthostatic_hypotension/msa_orthostatic_hypotension.htm

Ennis O'Shay appeared early for her appointment at the movement disorder clinic. She gave full permission for her clinical history and medical case to be available for public viewing, as someone else with Parkinson's might find her problems, symptoms, and management of value. Accompanied by her husband Henry, the patient asked whether the movement disorder specialist was behind schedule, as they had not yet eaten lunch, and would do so, if the doctor would keep them waiting. Informed that the specialist had been double booked with two patients at the same hour, and was approximately thirty minutes behind schedule, the patient and her husband left for the cafe on the ground floor.

In the examination room, the blond, highly groomed woman of sixty-three stated her three current problems. As she spoke her right foot bounced at an irregular rhythm and her head twisted slightly, from side to side. She seemed unbothered by the movements, though the physician commented on the dyskinesias, asking when she had taken her last medication. She estimated it had probably been an hour, and her husband agreed. The physician commented to the medical student, the extraneous movements were peak dose dyskinesias, caused by acutely sensitive dopamine receptors. The full- cheeked student wearing a short white lab coat nodded his head in understanding, and asked the patient how long she had been taking dopamine. Mrs. O'Shay replied she was diagnosed five years ago.

Wanting to address her issues, the patient stated Henry would prefer she not drive. She gazed at her husband, and let him speak. The husband had a head of white wavy hair, and appeared several years older, though trim. He had worn his bright green trousers on the golf course earlier in the morning, and lacked the opportunity to change clothing. Not embarrassed by the loud color, he made his case to the physician, that he feared his wife might kill someone accidentally, and the victim's family sue them. The physician looked up from the notes he was taking and commented whether they had thought of increasing the liability coverage on their car insurance, and the patient replied of course; it was already at the maximum. The physician stated the AARP offers Driver's Safety programs and a physical therapist would be able to ascertain whether the patient is a hazard on the road. He offered to give the couple a referral to a therapist who routinely performs that sort of work. Henry agreed, asking whether Ennis would be willing to give up driving if the therapist found her perilous to other drivers. She nodded her head and agreed, cautioning he would need to hire a driver for her. This he consented to do.

The second problem arose at night. She was seeing naked men in the house. There was silence in the examination room and the physician asked how she knew they were hallucinations. Mrs. O'Shay responded that she called to them and told them to come to her, but they rarely paid attention and preferred to speak with each other. At first, she thought they must be Henry's friends, but why were they naked? The doctor smiled and asked what the men do when she sees them. She waved her hand, and answered, "Oh everything." Sometimes they cook in the kitchen and it smells like frying chicken. They dig and plant flowers in the ground outside the front windows. They work on the house carrying tools around and hammering.

Henry agreed she had been seeing naked men, and it only occurs at night. The physician wondered whether she might be over- medicated, and asked for Ennis' daily dose of medications. Before assessing the medication schedule, the doctor asked about the third problem. Mrs. Ennis conceded this was a bit embarrassing, but so be it. The older woman confessed she had become quite amorous of late, and had been using an implement to satisfy herself. The desire was something she had been reluctant to discuss with him at previous visits, given the personal nature of the issue, but it had been a year perhaps, and the need seemed to be increasing. The specialist asked whether she was taking dopamine agonists, and she nodded. The doctor replied dopamine agonists are known to induce hypersexuality, and patients regain their former levels of desire when abstaining from the medication.

The couple had a written copy of the dosing schedule the patient followed, and shared this with the specialist. They agreed to discontinue the dopamine agonist and lowered the evening dose of Sinemet, and the physician noted they could call the secretary if needed, but he would see them back in the office in six months.

The fluorescent lights in the examination room turned the patient's skin a strange yellowish- green color, comparable to someone with chronic hepatitis C. Diagnosed with PD about eight years ago the patient's main problem was gait. Navigating corners had become tricky, and resulted in the patient executing a series of fast little steps; in people with PD the shuffling gait is also called festinating gait. The specialist noted the same sort of issue applies to speech, with PD patients stuttering, unable to progress beyond a certain repeated syllable. Speech also softens in PD, becoming whispery and losing volume due to increasing rigidity of the diaphragm and muscles that span the ribs. While speech therapy helps the patient make sentences with sequential words, physical therapy can focus on gait, and balance to avoid falls.

The movement disorder physician questioned the patient about whether problems in gait coincided with times in which medication had not been working, for example, upon waking in the morning. The patient commented there was no obvious change in symptoms, in instances where medication was taken later than usual. Stalevo and other dopamine yielding drugs may not be very helpful when sudden immobility or freezing complicates the picture. The specialist informed the patient and partner that surgeons at the University of Florida and at the University of Toronto are placing stimulators in the brain, at the site called the pedunculo- pontine nucleus, PPN for short. The procedure is still in experimental trials to uncover its level of effectiveness in improving gait for patients with Parkinson's disease.

The physician also mentioned a group of Japanese investigators who experimented many years ago with L-threo-dihydroxyphenylserine or droxydopa, claiming the compound had symptomatic beneficial effect for patients with freezing syndromes. Currently being used in Asia for various conditions, and has completed stage two clinical trials for orthostatic hypotension in the USA, the medication is a precursor of the neurotransmitters norepinephrine and epinephrine, and is used generally to increase the concentrations of the neurotransmitters in the brain and body. Recent studies have found it effective in raising the blood pressure of patients with Multiple System Atrophy, who suffer with orthostatic hypotension; large drops in blood pressure due to abrupt changes in physical position. From wikipedia, ...' works by increasing levels of norepinephrine and epinephrine in the peripheral nervous system inducing tachycardia or increased heart rate and hypertension or increased blood pressure, thus enabling the body to maintain blood flow upon and while standing.'

The patient and caregiver listen as the researcher describes what he feels will be the next breakthrough for Parkinson's disease. In his opinion, the next innovation will not come in the form of surgery, but as scientists discover ways to harness the brain's capacity to replenish its own neurons.

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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.

Two illnesses so disparate, one characterized by sudden repetitive tics, the other by stooped posture and slowness, have some aspects in common.   People with Tourette's control their tics to some degree; their movements were coined 'unvoluntary', by the well- know movement disorder specialist Tony Lang because they lie in the grey zone between willed actions and uncontrollable performance. Patients with levodopa-induced chorea are at the mercy of their movements; their actions are involuntary, uncontrollable by the patient.

People with Tourette's appear to have exquisitely sensitive dopamine receptors. Patients who have had PD for many years and been treated with medications, develop very responsive dopamine receptors in reaction to the depletion of dopamine- rich cells. As the population of the dopamine containing cells within the substantia nigra dwindles, the receptors in the striatum become more capable of responding to the slightest increase in the neurotransmitter. For PD patients, the ultra- sensitive receptors cause levodopa-induced chorea, the uncontrollable dance-like movements of the limbs, head and torso. For the person with Tourette's, they bring on grunting, coprolalia (swearing) and repetitive movements.

A young patient sought help in managing a tic disorder. Diagnosed in early years with attention deficit, hyperactivity disorder, the young student took Ritalin. Ritalin, or the generic methyphenidate, is a mild central nervous system stimulant, prescribed for adults with narcolepsy- a disorder of random sleep episodes and excessive daytime fatigue. In children, the drug has an inverse affect, rather than perking them to wakefulness, it enhances the ability to focus, control one's actions, and remain still or quiet. Physicians typically avoid prescribing the drug to adults with ADHD because it speeds them up, increasing the hyperactivity they already possess. The student received a prescription from a prior neurologist for the drug Adderall, an amphetamine, commenting the medication turned the patient into a jack-in-a -box. The specialist nodded; surprised the medication would be favored for someone with underlying tics, as it would magnify them.

The physician recommended pimozide, having the brand name Orap.  Purported to improve the tics of 70% of patients who use it, the medication belongs to the class of drugs known as major transquilizeers which block the activity of dopamine in the brain.  However, in small doses the specialist thought it appropriate for tics, in larger doses and with long-term use, the drug can cause tardive dyskinesia, a syndrome of involuntary movements brought on by chronic blockade of dopamine receptors.  They discussed alternative treatments including a medication known as Xenazine (tetrabenazine).   Tetrabenazine is a medication that depletes dopamine storage and was recently approved for the treatment of chorea associated with Huntingtons disease.  However,  it has long been used in Europe and Canada to treat the tics of Tourettes syndrome.  In the USA, doctors can use tetrabenazene for treatment of tics, but insurance may not cover the cost because it is not officially approved for this use.

A psychiatrist in the Virgin Islands treated the patient for fourteen years. When the movement disorder specialist looked at the list of medications, he was impressed and satisfied with the regimen. Only after the patient had fallen, spent a week in the hospital then been transferred to a rehabilitation facility where he spent most of his time in bed, did his health appear to be threatened. A family member commented he couldn't lift himself up from a lying position, when he first arrived in the States. With family care and physical therapy, he appeared at the clinic using a walker.

Seeking to know more of the patient's history, the doctor asked about the first symptoms of illness. Tremor began in the right hand and after two years or so, spread to the left hand. The doctor commented two types of PD are generally recognized, the tremor dominant type and the rigid, akinetic kind. He commented the patient is lucky to have the tremor dominant type, though the illness is more apparent, it also progresses more slowly. On physical examination, the physician noted the patient was quite stiff in the neck and torso, but an increased level of Sinemet would relieve some of this. Happy the patient was having physical therapy, he recommended therapy continue until Medicare refused to cover the cost.

In addition to gradually increasing the level of Sinemet, for which he drew out a chart for the family, the doctor suggested the patient begin using the medication, Ditropan at night. The drug increases the bladder's holding capacity, so one can sleep an entire night without the urge to urinate. On the cautious side, when given with other drugs, specifically Artane, it may increase a patient's risk of acquiring a bladder infection. The physician warned the patient and family to be aware of this, and cut the patient's dose of Artane to avoid possible problems. An adequate dose of Seroquel at night would ensure the patient falls asleep and remains so until the following morning. He stressed the patient avoid daytime napping and make an effort to exercise daily.

The doctor, not feeling rushed, as he allocates an hour for new patients, digressed a bit on medications. Artane, he stated, is one of the oldest drugs given to those with PD and is quite effective for tremor. To its detriment, the medication can interfere with short-term memory in patients over the age of 60, worsens constipation and interferes with bladder function. Sinemet, so named for sin emesis- Latin for without vomit, has three potential side effects- nausea; that's why it's best to take the drug with food, it can cause drops in blood pressure, and it may bring on hallucinations, especially in patients not sleeping well at night.

The physician urged the patient continue taking Mirapex and amantadine as he had previous been instructed. After the change in dosage of Sinemet, which would take approximately a month to fully accomplish, they could tweek the doses of other medications. In addition, he advised the patient to avoid protein- rich foods throughout the day, eating instead a diet rich in fruit, vegetables, and carbohydrates. Eating foods that lack amino acids enables Sinemet to act effectively and reduce symptoms of illness. At dinner, with the idea the evening will be quiet, spent reading or watching television and not line dancing, the patient should take the daily requirement of protein. The neutral amino acids in protein will interfere with the transport of levodopa from blood to brain, so the patient is apt to feel slower and not have the same response to medications, as when taken without protein during the day. To manage the illness effectively, it seems best to plan ahead of time what foods to eat at a particular meal. The doctor stressed he would like the patient to return in three months time, but that if problems arose to call his assistant, and he provided them a card with the appropriate office number.

Somewhere between the extremes of voluntary willed and involuntary, uncontrollable movement lies the gray area of unvoluntary movement. People with tics are thought to suffer from unvoluntary movements because they are able to consciously suppress urges to perform the motion.  While restraining themselves, the desire to perform the action builds until they are unable to contain themselves and they release a flurry of tics.

A patient appeared in the movement disorder clinic, referred by his neurologist because the patient's case was beyond his level of expertise. The patient reported an overwhelming need to move his legs or change position- something one would commonly hear from a person suffering from restless legs syndrome.  Indeed, the patient had taken Mirapex, a drug prescribed for that disorder. Unfortunately, the medication did little his need to move, so that the odd movements he felt compelled to perform embarrassed him, and causing him to seek a doctor's opinion. Movement disorders come in patterns. The patterns are based on physiological derangement of cells, in the case of Parkinson's disease movements eventually arise when the population of dopamine producing neurons has diminished remarkably within the substantia nigra.

The patient's movements were similar to those in Monty Python's Ministry of Silly Walks, there were random and odd. The patient reported he didn't know why he performed them, other than to calm a restless need he felt within his body. The loved one of the patient, who accompanied him to the appointment, stated he also moves oddly while sleeping.

The patient's medical history included a clipped aneurysm; the bulging blood vessel had bled irregularly and given the patient tremendously painful headaches. He had been advised to have surgery, which he had done. He also suffers from diabetes, had undergone rotator cuff surgery and spinal surgery, in which a surgeon fused several levels of vertebra in his spine.

Diabetes invariably brings vascular disease, as the endothelial cells lining the smallest vessels in the brain and body degenerate because they are unable to utilize sugar. Neuropathies occur as a consequence of inadequate function of the blood vessels serving nerves and may present with decreased sensation in the feet and fingers. Diabetes may have contributed to the sensory changes in his limbs and could contribute in part to restless leg syndrome. But the extent of the unusual movements appeared much beyond the typical movements of the limbs seen in restless leg syndrome.

On physical examination, the clinician found no rigidity in the muscles of the arms. Reflexes were symmetrical and normal. The clinician documented the patient's responses to fine motor testing as the medical student led the examination. Discussing his thoughts with the patient and significant other, the physician suggested the patient increase his level of antidepressant and add a small dose of the generic medication primozide, or the brand name equivalent Orap,  given commonly to those suffering with tics.  He also requested the patient obtain records of the neurosurgery performed nearly ten years ago, to ascertain if an MRI of the brain can be done. The practitioner requested the patient return in three months time, when a further discussion including past records can occur, and after gauging whether the current medications prove helpful.