The Retired Ophthalmologist

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He is subdued, well- groomed and has no objection to observers in the room. The doctor summarizes his last note, written six months earlier when the symptoms were confined to the left side of the patient’s body. Driving, hands on the steering wheel, his right hand moves along with the tremor in his left hand. That’s the only time he’s noticed tremor on the right. Bilateral symptoms indicate stage two illness. Six months ago the doctor documented, he was stage one, still the honeymoon period.

The general practitioner recently decreased the hypertensive medication he takes. Since then, he’s noticed his symptoms seem worse. He gave up his profession of forty-two years, when he felt it wise to arrive an hour early and review the charts of the previous day, to see whether he had made errors. He had trouble speaking with his patients. His voice became very soft, somewhat slurred, and people found it hard to understand him. Questioning his ability to perform as he had earlier, he sold the practice to a younger man. It’s been several months since he gave up his work and he’s been searching for ways to occupy himself during the day. He’s discovered a yoga class, attending three times a week. He exercises. The activity is not enough. A void exists where his work used to be.

The doctor notes most people with PD, the vast majority, begin taking antidepressants. Some experts even posit depression may be an integral part of the illness. The patient comments he took the antidepressant, nortryptiline several times and noticed it seemed to make him more constipated. The physician comments the medicine is an “old- fashioned” tricyclic anti-depressant with mild anti-cholinergic effects, which would benefit him. For its affects to be felt, the medication has to be taken regularly for more than a month. The patient laughs at this.

The doctor asks about sleep and the patient reveals he has occasional nightmares. In the last one, someone chases him. He turns in the dream, and in bed, and punches the guy. His fist hits the headboard. The skin of his knuckles split. His wife has chosen to sleep in another bedroom. The doctor nods, and notes that this sleep problem is called rapid eye-movement sleep behavior disorder. Most people with PD have unusual sleep architecture; most people when dreaming are incapable of moving their body. Those with the disorder are able to move and perform, acting out the scenarios of their dreams. Sleep walking and talking are other examples. To increase the patient’s sleep quality and quantity, the physician recommends Seroquel at very low doses, to be increased in dose until he can sleep at least six hours without waking up. The patient agrees he would like to sleep more. Seroquel, the Dr. explains, is considered a novel tranquilizer developed to treat psychosis, but is very effective in treating PD patients who have vivid nightmares and even visual hallucinations. Addressing his voice problem, the doctor comments a speech therapist would be able to work with him to increase volume and the clarity of his words. The man nods, he will add this to the vast span of time he has open in his afternoons.

Illness from the Islands

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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.

Unvoluntary Movements

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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.

Tourette’s Syndrome and Parkinson’s Disease

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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.

Shy Drager’s Syndrome = Multiple System Atrophy with Orthostatic Hypotension = Parkinson’s Plus Synd

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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

Naked Men

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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.

Going Green

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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.

Problems Aplenty (Liver failure, manganese and movement disorders)

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

Speaking and Eye Movement Problems in a Person with mild Parkinsonism

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The patient and his wife appear for their appointment. They have a jovial quality uncharacteristic of patients coming for a second opinion on Parkinson’s disease. The issue the patient has with speech is evident right away. It seems he must summon a certain energy to pronounce words, or to string words together. There is some lapse of time between questions and his response and there are none of the small inarticulations people make in standard speech; the small sounds of hm… ah…and well… are all missing. Talking and writing are the two major impairments that brought him to see a neurologist, a few years ago. The wife mentions since that time she has noticed the patient’s speaking ability has deteriorated. She explains the patient sometimes tells her yes, when he means no. In addition, the patient admits his memory is not as good as it was.
The patient’s general health is remarkable for hyperlipidemia- high cholesterol and triglycerides in the blood, high blood pressure, and diabetes. The patient manages all three problems with medications. The three problems contribute to give the patient small vessel disease- the smallest blood vessels that shuttle blood between arteries and veins are blocked, consequently, the patient’s brain has multiple small areas which do not receive sufficient blood supply.
The patient has had the relevant tests. A PET scan revealed his brain has normal oxygen consumption, indicating no signs of Alzheimer’s disease. A recent MRI suggested possible atrophy of the temporal lobes. Neuropsychological testing indicated the patient had losses in executive function, and should not be handling money; at the time he was the sole owner and director of a company with fifty employees. However, the patient scoffed at the results saying the tests were overly tedious, and he disliked the tester. Results also indicated the patient’s IQ must have become compromised, as his apparent IQ did not harmonize with his position as a functioning executive. However, the movement disorder specialist fails to discuss this fact with the patient and spouse. Inquiring later about why he skipped lightly over this, the specialist noted the patient had scoffed at the results of neuropsychological tests, denying their truthfulness or value.
On physical exam, the specialist notes no rigidity, nor slowness of movement. He does find the patient has limited range of eye movements when gazing downwards, which a sign of an atypical form of PD known asprogressive supranuclear palsy (PSP). Though the patient’s speaking ability is affected, he lacks many other signs typical of the disorder. Though he is a heavy man, with a substantial belly, he comes to a standing position easily from sitting in a chair. His gait is fluid, his arm swing full, and his balance normal. The specialist explains that this may be a very early case of the illness.
PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson’s disease. Hence it is also called Steele-Richardson-Olszewski syndrome, for the three who focused on the disorder. The initial complaints are typically vague and an early diagnosis is always difficult. The primary complaints fall into these categories: 1) unsteady walking or abrupt and unexplained falls without loss of consciousness; 2) visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble; 3) slurred speech; and 4) various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood. http://www.ninds.nih.gov/disorders/psp/detail_psp.htm
In a review article, Dr. Marsel Mesulam described Primary Progressive Aphasia as having an insidious onset with gradual progressive impairment in finding words, naming objects, and comprehending words while engaged in conversation. Though not evident in the first years, patients exhibit prominent apathy, disinhibition, loss of recent memory, visuospatial impairments, visual- recognition problems, inability to perform simple mathematical calculations, and inability to perform pantomime movement as instructed. Dr. Mesulam notes that some patients may have signs and symptoms of illness confined to the area of language for as many as 10 to 14 years, before other problems emerge. Nowhere in the review does he mention problems with eye movements. (New Eng. J. of Med. Vol. 349:1535-1542 N.16 Oct. 16, 2003)
According to the National Institute of Neurological Disorders and Stroke, Corticobasal Degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy of multiple areas of the brain. Progressing gradually, the initial symptoms, which typically begin at or around age 60, first appear on one side of the body, but eventually affect both sides. Symptoms are similar to those found in Parkinson disease, such as poor coordination, an absence of movements, rigidity, disequilibrium or impaired balance, and limb dystonia- abnormal muscle postures. Other symptoms such as cognitive and visual-spatial impairments, apraxia- loss of the ability to make familiar, purposeful movements, hesitant and halting speech, myoclonus- muscular jerks, and dysphagia- difficulty swallowing, may also occur. The patient eventually becomes unable to walk. From the writer’s perspective as an outsider, the patient seems least likely to suffer with this diagnosis. . (http://www.ninds.nih.gov/disorders/corticobasal_degeneration/corticobasal_degeneration.htm)
The movement disorder specialist recommends the patient and his wife make an appointment with another specialist whose area is cognitive neurology. This authority would be able to establish areas in which the patient has cognitive impairments. He also recommends the patient undergo another MRI within a year, to see whether changes in the brain have progressed.
Sometimes a diagnosis is unclear; sometimes a person has more than one diagnosis. The patient asks whether some pill will prevent further decline in his condition, and the specialist says there is nothing he can recommend. However, eating fruits and vegetable and performing daily exercise are general health recommendations for all. From the expression on his face, the patient stopped listening at the mention of fruits and vegetables.

Falling Was The Earliest Sign

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The patient fell several times before a neurologist ever diagnosed him with Parkinson’s disease. Falling is not a typical first symptom for someone with classical PD. The doctor asks about the first indication that hinted the patient should see a neurologist. Though the patient speaks clearly, he does not remember details well. The wife comments she thinks it was dragging of the right foot. She comments at the time, she feared her husband might have had a stroke.
There are other significant problems. The patient underwent a quadruple bypass on the vessels of his heart, and has a pacemaker. The movement disorders specialist comments though the surgeon may have cleared the blockages feeding the heart, the vessels feeding the brain have the same buildup of plaque. Patients with vascular disease may begin to show symptoms of lower body parkinsonism due to small vessel disease; shuffling gait with full arm swing. Another illness neurologists like to rule out in patients who can undergo an MRI, is normal pressure hydrocephalus. A prominent symptom in the illness along with the shuffling gait is substantial change in the person’s cognition; problem solving suffers, memory fades, the ability to plan and change plans as problems arise, becomes hard to accomplish. Because of the pace maker in his chest, the patient cannot undergo an MRI. A CAT scan, the doctor concedes, reveals little information about prior strokes the patient may have suffered.
The wife confesses the greatest problem really is the patient has become limited in what he can do. An artistic person, he does not paint or work with wood anymore, due to his frequent falls. The physician comments that maintaining an artistic outlet is very important. The stimulation artwork gives the brain as one draws or paints, is about problem solving; for example depicting a three dimensional object in a two dimensional plane. The doctor encouraged the couple to pursue an art store that sells easels that will roll up to a person’s chair. He also suggested it might be time to look into acquiring an electric cart. Access to the mall, the movies or the boardwalk should not limit the patient because his balance is poor. Along with the cart, they would need to purchase the carrier that fastens to the back of the car. Insurance will cover a large fraction of the cost, and it will help the couple maintain an active life. The last tool the doctor would like to see the patient using is a stationary bike, optimally with a chair seat, so there is no easy way to fall off.
The doctor reviews the medications the patient takes and comments the amount of levodopa is inadequate to provide a therapeutic dose, which is approximately two and a half pills of 25/100, four times per day. He draws a step- wise chart that indicates when the patient should increase the medication by a half pill. He instructs the patient to stop at a dose if he feels the medication is driving his blood pressure too low, though he cautions the patient may need to begin wearing TED hose, if low blood pressure remains an obstacle to achieving an adequate dose of medication. Proteins in the diet are very important the physician says, because they will compete with the medication to get across the blood brain barrier. A diet rich in dairy products will especially cause havoc with medication, due the quantity of free amino acids. He advised the patient to try and eat a protein- free breakfast and a low protein lunch, saving the protein filled meal for the evening, when he can anticipate being home, and feeling less effect from the medication. The doctor asked the couple to return in four months time, with a report on how things have gone.

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