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Shy Drager's Syndrome = Multiple System Atrophy with Orthostatic Hypotension = Parkinson's Plus Synd
Thursday, March 25, 2010

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
Monday, March 22, 2010

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
Saturday, March 13, 2010

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)
Saturday, March 06, 2010

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.