CurePSP Webinar summary: Dr. Hutchman 10-22-09

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CurePSP Webinar summary: Dr. Hutchman 10-22-09

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Webinar Series: Movement Disorder Specialists

October 22, 2009
(summary prepared by Wanda Dunbar)

Presented by Dr. Robert M. Hutchman, Movement Disorders Specialist,
CEO & Medical Dir., The Hutchman Clinic, The Hazel Hutchman Memorial Neuroscience Center & Neurosearch, Inc., (clinical research company), Los Angeles, CA. Completed medical residency in Neurology & fellowship in Parkinson’s Disease & Movement Disorders at Mayo Clinic in Rochester, MN. Completed second fellowship in Detroit, MI., with special training in conducting clinical drug trials and DBS surgery techniques.

Dr. Hutchman discussed: Interventions in Atypical Parkinsonian Disorders:
PSP – Progressive Supranuclear Palsy
CBD or CBGD – Cortico-Basal Ganglionic Degeneration
MSA – Multiple System Atrophy
DLBD or DLB or LBD – Diffuse Lewy Body Disease

The Motor Manifestations of Parkinsonism seen in Parkinson’s Disease (PD) and the Atypical Parkinsonian-Plus Disorders are just the tip of the iceberg. They include:

• Resting tremor – Pin-rolling character (but more common to PD)
• Bradykinesia – Slow, shuffled walking; difficulty rising from a chair, getting in and out of a car/bath, turning in bed; soft, monotonous speech; masked face, less frequent swallowing & resulting in drooling; loss of agility and fine motor skill of the hands; dry eyes due to less blinking.
• Rigidity —Subjective stiffness with aches and pains; decreased arm swing with walking, bent forward head and neck posture
• Postural instability – Due to all of the above causing imbalance and falling. PSP having trouble maintaining center of gravity.

I, typically, start treatment of Atypical Parkinsonian-Plus Disorders with Azilect, one of the new choices of Manoamine Oxidase Inhibitors (MAO-I) that should be considered for all of these patients. MAO-I’s provide mild to modest relief, are long lasting, dosed once a day. They inhibit MAO, an enzyme that metabolizes, or breaks down dopamine with little, if any side effects. Azilect has excellent evidence for slowing progression of the disease, but it is controversial. My second choice is Amantidine. It works well for patients who have tremor as main symptom and may provide mild help for slowness and stiffness. It blocks glutamate in the brain at the NMDA receptor. Side effects are typically few but may include leg swelling/mottling, nausea or confusion/hallucinosis in the cognitively impaired. It is a good choice for later disease addition to control resistant tremor, or dyskinesia resulting from aggressive treatment with dopamine drugs.

Sinemet is still the gold standard for treating PD’s motor symptoms and the Atypical Parkinsonian-Plus diseases. It is the least side effect prone, most potent medication and provides excellent relief. But it should be optimized with adjunct meds like Amantidine, Azilect or Comtan to limit its contributing to long-term motor complications (although mainly caused by the disease). In l974, Sinemet was the name given to the new combination of Carbidopa/Levodopa. Carbidopa was added to Levodopa as it chaperones Levodopa into the brain and prevents an enzyme from breaking down the levodopamine before it can enter the blood stream. Sinemet (meaning less nausea in Latin) reduced the vomiting that Levodopa causes when used alone.

Sinemet is available in immediate release: 10/100, 25/100 and 25/250 MG. The 25/100 is by far the most preferred formulation because of its high 1 to 4 Carbidopa/Levodopa ratio. It has fewer side effects than the 10/100 or 25/250MG that have a 1 to 10 ratio. There is a controlled release (CR) 25/100 and 50/200MG but these have fallen out of favor. Realistically, the immediate release is a more reliable release preparation plus Comtan or Azilect can be added, and they are as long lasting as CR Sinemet. They do not have the CR disadvantage of a slow start to begin symptom relief or be only 75% viable.

The number of Sinemet tablets used, the timing and frequency of dosing must be determined for the best effect for each patient. Typically, with Atypical Parkinson’s Disorders we start with one-half tablet 3x a day. It is very important that Sinemet be taken one hour before or one hour after meals so that the protein in the food does not interfere with absorption or compete with the drug. Also avoid citrus fruits or juices for one hour before or after meals. After one or two weeks increase the one-half tablet 3x a day by another one-half tablet at each dosage. The one tablet 3x a day at weeks 3 and 4 could be increased weekly to what is considered a good total trial dosage of about one gram or 1000 mg of Levodopa – increasing up to 3 tablets 3x a day of the 25/100MG Carbidopa/Levodopa -- 9 tablets of 100MG is just a gram. If the patient is improving, but only moderately, I’m for continuing the aggressive treatment. With minimal improvement, decrease dosage to best minimal dosage.

Sinemet is mainly intended to decrease motor symptoms and help the patient move more freely with less Parkinsonism. The Carbidopa/Levodopa can have the side effects of nausea and vomiting, hypo-tension or low blood pressure, sleepiness, feelings of heat or sweats, diskinesia or wiggly, jerky involuntary motions (like seen with Michael J. Fox on TV), psychosis, hallucinations -- visual or auditory, or delusional thoughts in the cognitively impaired. Long-term motor problems occur most often with Carbidopa/Levodopa when taken on its own or with shorter durations of each dose. Motor fluctuations occur with the wearing off of each dose, and peak dosage kinosis, although predominantly caused by the disease itself. Minimize fluctuations with earlier use of newer, longer lasting agents along with Carbidopa/Levodopa or before, such as with Azilect or Amantidine, or adjust dosage and content.

Sinemet is very affordable in the generic form. The strongest remover of symptoms available, it will remain a stable treatment for Atypical Parkinsonian Disorders, whereas we are reluctant to use the newer dopamine antagonists, Requip, Requip XL, Miraplex and Newpro. The other drugs mentioned are all good to try individually to see if well tolerated.

Now, there is Stalevo, the newest combination of carbidopa/levodopa, with Sinemet and Comtan combined in one pill like Neopolitan ice cream. Dopamine is made to last longer, be stronger to relieve more symptoms during the day. Stalevo is available in sizes 50, 75, 100, 125,150 or 200. This is the number of milligrams of Levadopa with the corresponding Carbidopa at a ratio 1 to 4 based on the most often used 25/100 MG tablet. Both Azilect and Comtan maintain a longer, stronger presence of Levodopa outside the brain but also inside the brain where it is converted to dopamine to more durably suppress the symptoms of stiffness and tremor.

Non-Motor Manifestations of Parkinsonism – These compose the main mass of the iceberg of symptoms. There are six points I cover when I see patients. They should be discussed at each appointment and trouble-shot if they are concerns: cognitive impairment and dementia, mood disorders, sleep disorders, psychosis – delusional thought, excessive daytime sleepiness and autonomic nervous system disorders. The latter are particular to MSA but are seen in PD, PSP, and less in CBG and LBD.

Cognitive Impairment & Dementia is of paramount importance and is treatable. This condition is characterized by slowness of thinking, poor short-term memory plus, at least, one area of poor thinking/mental ability, such as orientation of time/place and composing of abstract thought. It is dopamine responsive and the dopamine meds we’re using for physical symptoms may help offset cognitive slowing. Often people are made more alert, awake and improve mood but in other patients too much Sinemet might harm these factors. It is individual treatment. Most patients going from a non/under-treated state with dopamine experience improvement in cognition, mood and energy.

Roughly 40-60% of PD patients develop dementia in the later years of the disease. It can be seen as early as a year before or a year after the onset of Parkinsonism in some disorders, for example, LBD. Dementia is less likely to be seen in later years in combination with CBG or MSA. In PSP, probably 50% plus develop dementia. A prominent sign of it is the sudden standing or moving from a seated position often leading to falls as well as spontaneous speech in public. There are no specific medications for treatment of these particular disease states. Dementia can often be plagued by psychosis (hallucinations, delusions, paranoia, resulting agitation or fear) but not necessarily.

There are two main classes of medication for treatment of dementia. The first class boosts Acetylcholine. The drug, Exelon, is currently the only drug approved for Parkinson’s Disease dementia and therefore the most germain to treat Parkinsonian Disorders. Aricept and Razadyne boost Acetycholine, thereby helping with present memory and thinking. Although not guaranteed, it is hoped that they would reduce progressive memory loss.

The second class of medications reduce excessive glutamate. The drug Namenda blocks the MAND receptor for glutamate to reduce excitotoxicity or excitatory activity in the brain. Namenda is used to improve memory and thinking in the short term and, hopefully, reduce progressive, long-term memory loss. Providing anti-oxidation, typically 1000 IU of Vitamin E and 1200MG of COQ10 daily, is recommended to PD patients to help maintain memory and thinking in the long run. It is thought that, like Azilect, anti-oxidants may slow both cognitive impairment and progression of physical symptoms. Finally, regular use of non-steriodal, anti-inflammatory drugs has reduced dementia, particularly with Alzheimer’s patients. Chronic NSAID use, however, can lead to stomach ulcers or kidney disease. Products like Advil, Motrin, and Aleve are fine to take for a headache and may reduce inflammation in the brain. They would be a superior choice over Tylenol or aspirin.

Mood Disorders: Anxiety can be constant, intermittent or worsen in the “off-state” when dopamine medications, like Carbidopa/Levedopa wear off or are not working. There can be depression, such as, low energy, poor sleep quality, poor mood, feelings of worthlessness, suicidal ideation. It is important to take carbidopa/levodopa throughout the day as often as needed, such as every 6,5,4,3 hours. Frequency and dosage amount must be worked out on an individual basis, through trial and error, with the help of the physician and caregiver. One pill or maybe three pills may be required each time to keep cognitive and mood symptoms stable.

Mood disorders can also encompass social withdrawal, abulia (lack of spontaneous speech, less talkative), or apathy, and loss of interest and enjoyment. There are involuntary emotional expression disorders with inappropriate responses, like laughing at bad news and crying at good news. (pseudo-bulbar affect or palsy). These are all treatable with anti-depressants (SSRI/SNRI/NDRI class of meds, like Lexapro, Effexor, Wellbutrin, respectively), and anti-anxiety agents, even stimulants (make them more awake). Sometimes atypical anti-psychotic agents, like Seroquil, are used. Most important is the aggressive, consistent treatment with dopamine agents to maintain the “on” physical state with adequate dopamine in the brain to achieve a better stability of cognitive and mood states. SNRI and NDRI meds offer the positive effects of helping to produce stimulation -- more zest, energy and wakefulness in patients with fatigue or tiredness.

Sleep Disorders: An estimated 95% of Parkinsonism patients have some sleep disorder whether it is nocturnal or restless mind or body causing insomnia. This is most notably
because of a lack of dopamine overnight. The Restless Leg Syndrome we treat with dopamine at bedtime, or during the night, with drugs like Requip or Miraplex, short-acting low doses of dopamine. The Sleep Behavior Disorder (Dream enactment behavior with thrashing, yelling or calling out at night, hitting or kicking a bed partner.) is seen in PD, MSA and LD. We do not see it in PSP or CBD, which are tau operatives with a different pathological cause for the disorder. The REM disorder can be an early sign, along with a loss of sense of smell with PD, SMA or LBD patients.

Restless Leg Syndrome can be seen in PD or any of the Atypical Parkinsonian-Plus Disorders along with PLMS – Periodic Limb Movements of Sleep. This is periodic, every 30, 60 or 90 seconds kicking, or triple flexion of the knee and ankle. Other things, but not as common, include night terrors: Non-REM sleep stage related screaming, moaning, and/or agitated vocalizations/awakenings.

Another disorder is Apnea – brief breathing arrest -- which is obstructive or more central to the disease itself. Nocturnal psychosis can further plague these issues. This is where the patient wakes up feeling they see a person standing in the corner of the room when, in fact, it is a shadow. Sometimes this has to be treated at bedtime with atypical anti-psychotic medications to prevent psychosis.

These sleep disorders are treatable at bedtime with sedatives, dopamine agonists, tri-cyclic agents, C-PAP – a nasal breathing device for sleep apnea, atypical antipsychotics, opiods if someone has chronic pain issues, in other words painkillers that are mildly judicial, even anti-epilepsy agents, Sinemet, and improved sleep hygiene. Don’t eat or drink in bed, or watch TV, or read in bed. Think bed is just for sleeping!

Psychosis: Most often it’s hallucinations regarding something visual, less likely to something auditory or tactile. Delusional or referential thinking -- the person on the television is talking to me -- can produce paranoia and resulting behavioral agitation or fear. Hallucinations are most prominent in the evening or at night. They are much more likely in cognitively impaired or dementia patients but are possible in any due to dopamine agonist medications, or other medications, like Sinemet, Azilect or Comtan. Also hallucinations can be caused by other illnesses/stressors, unfamiliar environments or “sundowning” -- the tendency for them to become worse when evening comes. This impairment is very treatable with atypical anti-psychotics, such as, Seroquel, which is often given at bedtime in the range of 25 to 100MG. If Seroquel is not adequate, Clozaril can be given once at bedtime and is typically very effective at 25 to 100MG. But, if needed, Seroquel or Clozaril can be given at a higher dosage at bedtime, up to 200MG. Clozaril requires weekly blood cell counts for six months to get an okay from the pharmacy each week for one week’s medication. This is because of the possible rare side effect of bone marrow suppression of white blood cell production leaving the patient susceptible to infection. After six months, blood cell checks can be every two weeks.

Excessive Daytime Sleepiness: This is the inability for an individual to maintain daytime wakefulness and/or to have the energy to interact, despite obtaining a good night’s rest. It is especially prevalent with men and LBD patients. The daytime sleepiness is made worse by poor nighttime rest, sedative effects of medications, depression and dementia. It is often mixed with a lack of motivation, apathy and abulia -- the lack of spontaneous speech.

This may lead to incompliance with the medication schedule and result in derailment of treatment because the patient is sleeping through the dosing times and meals. This may require smacks on the cheeks to arouse them to take their meals. This condition is treatable with the safe, non-amphetamine stimulants, like Nuvigil, a once-a day invigorator of the histamine in your brain, or possibly the used two-times-a day Provigil, which will become generic in the next year or two. There is also the milder pro-wake Amantidine. Ritalin can be used in rare cases when patients sleep 20 or more hours a day.

Autonomic Nervous System Disorders: Non-motor manifestations are many. This is of prime interest to MSA patients and other patients who have significant autonomic system failure -- about 25% of those with Parkinson’s Disorders.
With non-motor manifestations—
• Blood pressure and heart rate can go haywire causing lightheadedness/fainting.
• Swallowing dysfunction and acid reflux disease (Gerd)
• Gut mobility slows down, poor appetite, early satiety (early filling when eating), bloating/pain because of infrequent bowl movements and becoming packed with stool, constipation, hard BM, straining to get bowl action can all occur. A good bowl regimen needs to be implemented: plenty of fluid, stool softener maybe 3x a day, and plenty of exercise. If that isn’t enough, give a stimulant laxative, such Milk of Magnesia, a suppository, enema, or if all else fails – a prescription of Miralax or over-the-counter Glycomax.
• Urinary frequency, urgency and/or incontinence can occur. Medications are not usually used as they can lead to confusion, dry mouth, dry eyes and hypotension because of their anti-colonergic properties. Patients with these concerns need to visit their urologist for assistance.
• Erectile dysfunction in men/anorgasmia in either sex
• Thermal dysregulation (cool limbs or body)

These non-motor manifestations are treatable with good fluid management, such as drinking plenty of water during the day, adding salt to the meals (if blood pressure isn’t high), wearing compression/elastic stockings to the knee and, especially for MSA patients, aggressive use of Florinef or Fludrocortisone, up to 10 tablets a day of point 1MG. ProAmatine, up to 40MG a day in 2/3 doses split over the day, can help people maintain upright posture without hypotension. Exercise and use daily stool softener as needed. For erectile dysfunction and anorgasmia in either sex maybe use the shorter acting Viagra but do not use the other longer acting varieties.

Finally, a swallowing study plus good nutrition and/or a food consistency plan should be implemented for a swallowing dysfunction. Vital stimulation can be performed to build the muscles of the throat for swallowing. This is a mild electrical stimulation to the throat delivered through surface EMG electrodes and usually given by a speech therapist.

Other General Interventions of Interest for Patients and Caregivers:
Helpful activities for patients would include:Yoga, Pilates, Tai-Chi, simple daily stretching and range of motion exercises, seated cycling, physical/occupational/speech/swallowing therapy and aqua-therapy (with a partner). Botox injections can be obtained for patients with extreme rigidity of the neck as often seen in PSP and can relieve stiffness and pain. The seated cycling refers to the use of a simple Petal Exerciser having a sprocket, two petals and legs to hold it up. It can help minimize stiffness and slowness of the legs and can be procured through some multi-products catalogs and at some drugstores.

High quality patient education is very important. For this there are two excellent websites: and the The latter, authored by the Movement Disorders Society, is operated by physicians and provides information about all the Atypical Parkinson Disorders. Attend Support Groups for PD/Atypical Parkinsonism in California and other areas. You can learn about where these are and when they are meeting through Larry Shenker and other members of the CurePSP organization.

Be your own advocate!
Pursue good care from a physician in your area, preferably a Movement Disorder Specialist. You need someone who knows your condition better than a general neurologist who treats many disorders. You need a physician who specializes in Movement Disorders and who works on a regular basis with the type of disorder you have.

Question and Answer Session

Q. When will we have a cure for PSP? In the meantime what do we do?
A. I work with these patients daily and I have to say we are a long way from a cure. I’m 28 and I hope we have a cure by the end of my career. In the future we hope to use genetic screening, prevent toxic exposures we have identified and more we will identify, and learn from these disease processes as they worsen. I think the way we’re going to cure these diseases is by preventing them from ever occurring, or by greatly delaying or minimizing them, by identifying them early in patients at risk, putting patients under neuro-protective therapies, and providing generic therapies.

In the meantime, we need to all the things I have talked about here. Use aggressive intervention to maintain adequate relief of symptoms. Educate yourself by looking at website and website run by the Movement Disorder Society. It is a very educational website on all movement disorders including PSP, CBD, MSA, LBA. Go to local Parkisonian support groups; keep yourself active and supported. Trade secrets and help each other, patients and caregivers alike. Above all, get good care from a Movement Disorder Specialist who knows your disease and has seen these patients before.

Q. What exactly can Sinemet (Carbidopa/Levodopa) and other dopamine medications do to help my husband who has PSP?
A. They supplement the dopamine level in the brain to improve motor symptoms and improve quality of life.

Q. I have been diagnosed with a Parkinson’s Plus Disorder. Is Azilect really disease modifying and symptomatically relieving?
A. Yes! There is excellent evidence of its slowing of the disease. There were two large studies, Tempo and Adagio, performed on it with over six years of follow-up. Patients who took Azilect from the beginning had more improvement than those who started even six months later. Azilect is dosed once a day and is easily tolerated. It can be given alone as it works with the body’s own dopamine or as an adjunct medication, such as to Sinemet.

Q. What if any new medications are proving effective for treating PSP?
A. There have not been any new medications in the last five years specifically for treating PSP that have proven to be effective.

Q. Why do so many primary care physicians and general neurologists find it so difficult to diagnosis these movement disorders? I have had a Movement Disorder for 3 years that started with a fall. My primary care physician tested me for a stroke, the results were negative, and a neurologist could not see anything wrong.
A. The lack of a diagnosis of a Movement Disorder is a common problem. I have patients come to me with Atypical Parkinson’s Disorders that are quite obvious to me on their first visit yet over the past 2 or 3 years their condition was misconstrued by a general practitioner and/or a general neurologist. Diagnosing these disorders is something they have not been trained to do, especially the primary care physician. The neurologist in 36 months of adult neurology residency training has one month of training on Parkinson’s Disease Management leave alone Atypical Parkinson’s Disease Management. Patients need to go to academic centers to see movement disorder specialists. When I was in training I saw many of these patients. This is the more reason to go to a Movement Disorder Parkinsonian Specialist for diagnosis and a case follow-up visit, even if it is only once each year. Have this specialist follow-up on and provide guidance to your local neurologist. Be your own advocate.

Q. Do PSP patients understand what is going on around them?
A. Yes, they do and we have to act in their presence as if they were fully aware. In the early stages they are fully aware, later on they are less aware and we may have to draw their attention to things specifically. Even patients who appear quite impaired understand to a fair degree what is going on around them.

Q: Why are dopamine agonists a bad choice in treatment of Atypical Parkinsonism?
A. Agonists drugs, like Requip, Miraplex and the Newpro patch are too side effect prone causing low blood pressure induced lightheadness when upright, nausea and sleepiness. We avoid the agonists for the vast majority of these patients. They’re especially bad for MSA patients with autonomic nervous disorders.

Q. My husband has PSP and has dyskinesia and motor fluctuations. What are these, how are they treated or prevented?
A. These are most often seen in PD but can be seen in Parkinson’s Plus Disorders especially in younger patients like in Michael J. Fox. He has impressive dyskinesia -- excessive involuntary motions, twisting and jerking – seen as the “peak effect” of medications like Sinemet, Azilect and Comtan. This is when medications taken are at their peak effect in the brain while motor fluctutations result from the wearing off of medications between doses, the “off-effect.” Sinemet, a short-acting medication, can in-train these motor fluctuations because at its peak effect it can over-shoot the desired concentration and produce dyskinesia. To prevent these conditions, from the early stages of the disorder, use not only Sinemet but also Azilect, Comtan or Tasmar to give smooth, full and adequate consistency of dopamine to the brain and thereby consistency of symptom relief. If the patient is not on Azilect or Comtan these drugs could be added or Sinemet could be taken more frequently throughout the day. Maybe 1, 2 or 3 tablets at a time of the 25/100 (no more than 300MG per dose) every three hours because the medication won’t last longer. Fill the dopamine tank as often as needed to keep physical and emotional symptoms at bay.

Q. My wife has PSP and nocturnal akathisia. What is this, how is it treated and is it similar to Restless Leg Syndrome?
A. It is seen as Restless Leg Syndrome and is usually caused by the wearing-off of daytime treatments of dopamine. Treat with later, or in the night, doses of Sinemet plus Comtan and Tasmar. Maybe give a small dose of dopamine as Requip at bedtime.

Q. I have been diagnosed with PSP, have been given dopamine medication and am having side effects of a fast heartbeat and headaches. How can these side effects best be treated and what additional ones might I expect?
A. Side effects from medications are several and are treatable. Orthostatic hypotension – low blood pressure – can produce feelings of rapid heartbeat and headaches as well as nausea, clammy or sweaty feelings, lightheadness or mental clouding until the person sits down or even faints. Although caused by the disease tsymptoms are made worse by the dopamine medications, a necessary evil of treatment. Prevention is with a good fluid management regiment. Drink more fluids, liquids, 4 to 6 glasses of water daily and, if blood pressure is not high, add 2 to 3 pinches of salt at a meal. Before standing up pump the legs at the knees/ankle to pump blood pooling in the legs back up to the head and trunk; wear elastic/compression stockings. Take Florinef , Fludrocortisone or more aggressively, Mitrodrine, to raise blood pressure throughout the day. For nausea take Tigal 300MG once a day or with each dose of Sinemet throughout the day. Treat sleepiness with Amantidine, Provigil or Nuvigil. If there is not good suppression of motor symptoms, discontinue the dopamine and exposure to side effects.

Q. My husband has PSP what role could Botox injections play in his treatment? Where is the Botox injected and would it be covered by Medicare?
A. PSP often features excessive axial rigidity in the neck and the patient cannot turn to look behind them, have trouble turning in bed, and trunkal rigidity causing imbalance and falling. For pain or neck stiffness Botox can be injected in the 50- to 100-unit spread on either side of the cervical paraspinal by a neurologist experienced in Botox injections. Some relief is expected in a week with maximum benefit in one month. Repeat every 3 months to maintain pain relief. Botox injections are typically covered by Medicare.

Q. Why do dry eyes and drooling develop? How are they best treated?
A. These conditions develop with all Parkinson’s Plus patients because they do not blink or swallow frequently enough. For the eyes use non-medicated preservative eye drops, like Artifical Tears. To stop the drooling 15 units of Botox can be injected into each side of the jaw. It starts to work in about a week. Dosage needs to be adjusted to reduce salvary production and drooling and more than 30 units may be required.

Q. My spouse has PSP. How do you scale or estimate the severity of cognitive decline or dementia?
A. We use a full MSE exam, such as the Montreal Test of Mental Status or Cokeman Short-Term Test of Mental Status. Patients are asked to interpret abstract thoughts, draw pictures, write a sentence, memorize words, etc., and we test for orientation to time and place. In 5 to 10 minutes we do a test with results scored against normal test results. A score of 26 out of 30 is normal, 21 out of 25 is mild dementia, 11 out of 20 is moderate, 0 out of 10 is severe dementia. Typically not until moderate to severe stages of PD and PD disorders do we see moderate to severe dementia. PSP frequently features mild/moderate dementia in later years with uninhibited behavior, but less in MSA and CBDG.

Q. I have PSP. Why do dysphagia (swallowing) problems develop? How is this best treated?
A. This motor problem in PSP and other disorders develop when slowness and stiffness affect the muscles of the throat. Patient may have trouble swallowing pills or may choke on dry or larger food items. Probably best to swallow with the chin down then bring the head up. Tucking the chin helps prevent food from penetrating into the airways; but if it does, pat on the back or use the Heimlich maneuver. Keep bite sizes small, moist and have patient chew food well. A swallowing test at a local hospital by a speech therapist can determine degree of impairment. Typically, they advise on food consistency and diet modifications to keep food from getting into the airways, which can lead to aspiration pneumonia. A speech therapist could perform Vital Stimulation where small sticker EMG electrodes are stuck on the throat. Very small electrical shock stimulations are applied to the throat to strengthen it, like you build arm strength with dumbbells.

Q. What labs should be done to rule out a treatable cause of cognitive decline or dementia?
A. Labs should be done, especially with PD, PSP, LBD, less with MSA or CBD, to rule out a treatable cause. Tests should include: CBC for anemia; thyroid, B-12, and foliate levels; Chem 7, electrolyte panel (for dehydration), ANA or anti-body to check for inflammatory or immune disorders, and RPL test for nervous system infection by syphilis, although rare.

Q. My spouse has PSP. Should Levadopa be taken 1 hour before or 2 hours after a meal rather than with meals? If the sufferer is not responding to the Levadopa should dosage
be increased to 2000MG to see if a positive result is possible?
A. Yes!! It should be taken 1 hour before a protein meal and 2 hours after a meal because protein can disturb absorption of Sinemet. Split the medication into 3 doses or 3 tablets 3x a day to about 900 MG. This is the benchmark of about one gram of Levadopa. If you don’t see much improvement, continue to go up in dosage to 4 or 5 doses a day, and increase it up to 4 tablets or 400MG at each dose, or as much as 2000MG a day until you see noticeable benefit and improved quality of life. If needed, go up to 20 tablets or more a day of 25/100MG of Sinemet. Watch for side effects. Be a true advocate for the patient.

Q. My relative has PSP. Does the COQ10 supplement help and what is it used for?
A. About 7 years ago a 6-month study was done in the Northwest with 80 patients taking 1200, 600 and 300MG per day. Those taking 1200MG had a slower progression of their physical symptoms. The results were not as impressive as the Azilect trials, Tempo and Adiago. The COQ10 study was done on PD patients and may be applicable to PSP and other Parkinsonian disorders. If I were such a patient I would take 1MG of Azilect and 1200MG of COQ10 per day. NIH is studying Creatine at 5000MG per day as a neuroprotectant for PD, the Net-PD Study. The Parkinson’s Study Group is currently looking at doses of COQ10 of up to 2400MG per day.

Q. My husband has PSP. He does not sleep at night, but sleeps a great deal of the day. How much sleeping is too much during each day? Can too much daytime sleep disrupt sleep at night?
A. Sleeping more than three hours during the day is too much and the patient will not
sleep at night. Medications are used both for wakefulness and for sleeping.

Q. My husband has PSP. What causes the excessive daytime sleepiness common to patients with Parkinson’s Disease and the other movement disorders? How is it treated?
A. The wakefulness is caused by a disturbance in the part of the brain controlling wakefulness, the brain stem system (paraponteen reticular activating system) and; likewise, cortical involvement with pathological changes like the neurofibulary tangles seen in PSP. Sleepiness is increased by dopamine medication, muscle relaxants and sedatives for anxiety or antipsychotics, like Seroquel if used during the day. Minimize the use of these medications during the day except for dopamine (providing motor functions are responsive to it). Make nighttime sleep go well. Offer sleep aids. Make sure the patient is not suffering from a lack of Sinemet overnight and has been screened for excessive sleep apnea. If necessary, use a c-pap device. Amantidine is used for patients with mild sleepiness with reports of less trunkel imbalance; sometimes more pep, energy or wakefulness. Also there is the more robust Provigil or Nuvigil.

Q. My husband has PSP and the doctor is talking about giving him Provigil or Nuvigil. What do these prescription medicines do?
A. They are robust modern stimulants that specifically treat excessive daytime sleepiness, fatigue, apathy or the lack of zest. Provigil at 100 to 200MG is taken 2x a day; the newer Nuvigil at 150 to 250MG once a day. These drugs are off-label, not FDA approved for Parkinsonian disorders, but they work. FDA has not given a full indication for these drugs because a large clinical study costing $20 million has not been done. This is the only reason for the technicality. Our experience has been if the patient suffers from depression, is being treated with anti-depressants and still has excessive daytime sleepiness, this can help get the drugs approved by the insurance company. Also, sleep apnea with treatment and excessive daytime sleepiness are indications for Provigil and Nuvigil, and may aid in receiving the insurance company’s approval.

Q. My relative has PSP. Why do bowl problems, weight loss and appetite loss develop in Parkinsonism? How is this best treated?
A. These are autonomic system disorders common to all Parkinsonian Disease Disorders. The bowl problem occurs because the bowl is slowing down due to the body’s autonomic system slowing down. The patient encounters loss of the sense of smell that may affect the patient’s taste and thereby have less appetite. With the bowl slowing down the patient may feel full because of the irregular bowl movements and constipation. This can lead to low blood sugar and the patient struggling for energy during the day or to impacted bowl leading to the need for manual impaction removal or even surgery. The patient may lose 10 to 40 lbs. Treatment is with a good bowl regimen. (See Autonomic Nervous System Disorders/gut mobility in main speech).

Q. My husband is in the early stages of PSP with his full mental capabilities. Our children and me have been discussing a feeding tube with him. He does not want one but we would like him to get one. Should the family try to convince him to accept a feeding tube?
A. No! The family should heed the desires of the patient. He should (while still early in the disease) put his wishes in a Living Will. It should be authored by an attorney and legally sealed by a notary. This is usually only an issue in advanced stages of the disease when keeping a person alive with a feeding tube does not make sense.

Q. I have CBD and have been told that I have cortical sensory loss. What is cortical sensory loss and how is it treated?
A. It is not treatable. Cortical degeneration or the CBD/CBGD refers to the basal ganglia degeneration that causes physical motor loss of function in Parkinsonism. The C or D in CBD refers to the loss of cortical cells that help us perceive sensation and produce higher thinking. This loss of these cells causes a loss of normal personality function and may lead to dementia in some CBD patients; but it is not as common as it is with PSP. CBD patients may develop stereo agnosia – loss of ability to determine 3-dimensional shape—where they cannot tell (when blindfolded) that they are holding a ball in the effected hand, but they can with the good hand.

Q.I have CBD and have been told I have an Alien Limb Syndrome (ALS). According to Google this rare condition is where the person is unable to recognize their affected limb as their own, and regard it as being alien to them. Is there any treatment and will it get worse?
A. Alien Limb Syndrome is specific to CBD and is not seen in PSP or MSA and rarely in the other Parkinsonian disorders. There is no good treatment. Dopamine is used, but the limb does not respond well and gets progressively worse. The bad arm can move or grab out of the patient’s control as if it were alien to them and acts antagonistically to the good arm.

Q. My sister has CBD and has been told her motor symptoms are similar to those found in Parkinson’s Disease: Akinesia/bradykinesia (absence of movement), rigidity, tremor and limb dystonia (abnormal muscle postures). Do these motor systems that appear on the one side also develop on the other side?
A. If this patient has CBD, these symptoms will not develop on the other side. They are exclusively unilateral, occurring only on one side.

Q. Please explain what Orthostatic Hypotension (OH) is and why does it develop in MSA? What is the recommended treatment regimen?
A. Orthostatic Hypotension is low blood pressure when standing and is common in MSA and less so in the other Parkinsonian disorders. It is affecting the autonomic nervous system. Treatment is with plenty of fluids, additional salt (if no high blood pressure), pump legs/knees before standing, and wear elastic/compression hose to the knees. For more advanced cases take 1/10 tablets of Fludrocortisone each morning to supplement adrenal gland and kidney function to maintain normal fluid volume or pressure. Or, use Midodrine (ProAmatine) to constrict blood vessels to push more blood up into the brain, thereby preventing lightheadness, etc.

Q.My husand has MSA. Why does sexual dysfunction develop in Parkinsonism and how is this best treated?
A. Any of the Parkinsonian Disorders, but especially MSA, can have problems with autonomic functions of the body. Sexual Dysfunction can affect either sex but is more obvious in men with their erection. Women can have dysfunction of the tissue in their nipples and clitoris. This is challenging to treat. Viagra can be used. It dilates the sexual tissue allowing more blood to flow in to produce more erect tissue and to help achieve orgasm. The vasodilation it produces can drop blood pressure and lead to lightheadness, etc. People with MSA have to be cautious of using Viagra (maybe less so in the other PD disorders). I would never endorse use of Levitra or Cilias. Much longer lasting they provide up to 24 to 36 hours of potential symptoms from vasodilation. Viagra, the shortest acting, has maybe 2 to 6 hours of function. A couple could take it and chance vasodilation or try local treatments, like the muse pellet. A pellet of vasodilation medicine is inserted directly into the penis through a small, gentle plastic mechanism. It is effective 30 to 60 minutes and protects the rest of the body from low blood pressure.

Q. I have MSA-P. What is the maximum dose of Fludrocortisone or Midodrine (ProAmatine) that can be taken in a day?
A. Aggressive treatment is advised. Take 10 tablets of Fludrocortisone in the morning, each at 0.1MG for Multiple System Atrophy with Parkinsonism (MSA-P) as well as disautonomia (trouble with the autonomic nervous system). If needed for lightheadness take 20MG one to 3x a day. Midodrine is used at doses up to 40MG a day. Split these up into 5, 10 or 20MG per day depending on need for aggressive pressurization of the blood vessels. Give 1 to 3x a day with each dose lasting four hours enabling the patient to get through the day without lightheadness.

Q. What medicine(s) are best to treat the muscle stiffness that occurs especially in the later stages of CD? What is the benefit of using Comtan or Tasmar for CBD?
A. The same dopamine medications as I discussed earlier are used for this muscle stiffness; Sinemet, possibly in larger amounts or more frequently. Azilect is taken along with each dose of Sinemet, and 200MG of both Comtan and Tasmar with each dose of Sinemet thoughout the day for muscle stiffness. Tasmar has caused liver problems so every six months a liver check is needed. I am not a proponent of muscle relaxants although some patients have benefited when prescribed by others. Have Botox injections (described earlier) for stiffness in the neck and the low back. Use non-medical treatment: range of motion exercise, message and physical and occupational therapy to keep patient active and limber.

Q. The cost of nursing staff to provide daily care is very expensive. Is there anything that can be done to assist with the cost obligation?
A. There is no good answer unless the patient has Long-Term Care Insurance. There is little that can be done to assist with the cost of nursing staff for custodial or respite care unless it is a volunteer. Look into local organizations for some type of assistance. Contact your local Alzheimer’s Association as they are very well organized and might can assist in some way.

Q.I have PSP. Should I remain at home during the course of this disease (my preference) versus moving to an Assisted Living Facility? Cost is not an issue in this decision.
A. It is a very personal decision. Most people prefer to stay at home which would be my preference. Patients are more comfortable being in their own home with their spouse/family.

Procure caregivers fulltime or part-time to assist the family. Utilize the Assisted Living Facility should your family caregiver become overwhelmed or to obtain better trained care.

Q. My Mom has one of these disorders and has a problem accepting it. Her sister who passed away from a different disease wasn’t ashamed and took advantage of everything that was presented to her. What can I do to make my mother accept her condition?
A. Have her be educated about the disease by looking at websites and viewing CurePSP Webinars. Have her talk to a qualified physician who in a compassionate way can educate her about the disease, maybe treat her and perhaps she can get better. We need to support people with these disorders, treat them, and if they can get better, it will help!

Q. Is there any current medical literature to validate spine cell treatments for MSA? I have spoken with Dr. Frank Morales who is doing treatments in Nogales, Mexico. He reported he has formed a team of researchers in a collaborative effort to establish one of the first Stem Cell – Genetic research facilities in Mexico City at the University Instituto Politecnico Nacional, one of the largest universities in Latin America.
A. I have had two patients go to see Dr. Morales and I understood that he was doing transplants of bone marrow cells. This included some blood cells for bloodline cells since blood cells come from the cells in the bone marrow. I have not heard of spine cell treatments. A lot more needs to done before we can start to use brain or spine cell transportations into the brains of patients for any PD or Parkinson’s Plus treatments. I have to hear more about what Dr. Morales and the X-Cel Center in Germany are doing and their results. I had one patient go to the center in Germany and two to Mexico, and they felt mildly invigorating results but nothing very significant changed in their basic symptoms or quality of life. Such treatment is very controversial and its effective-ness yet to be determined. I am very confident, optimistic and excited about therapies to be developed in the coming years that will really make a difference in treatment of Parkinsonian disorders. Therapies could be stem cell treatments from the bone marrow, or better, embryonic brain tissue or possibly spinal cell treatments, whether taken from an embryonic source, or more likely from an adult autopsy, or by organ transportation sources, and genetic therapies.

Q. How do I reach the Parkinsonian Support Groups?
A. There are five CurePSP sponsored live online discussion support groups: PSP Spouses (caregivers), PSP Adult Children, PSP Patients, MSA Spouses and MSA Patients. To join anyone of them, you can contact me, Larry Schenker, the CurePSP Webinar Coordinator at
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Postby eplowman » Thu Dec 03, 2009 12:36 pm

Thank you for making this summary available.

I wish Dr. Hutchman would look more closely at the issue of using Sinemet with PSP patients. While it appears to have relevance for PSP-P patients, experience and observations posted here in the forum suggest the opposite is true for PSP-RS patients. MOST PSP patients have the RS (Richardson's Syndrome) form of the disease.

PSP-P patients, like PD patients, often exhibit low levels of dopamine in the brain. Sinemet apparently can help remedy this deficiency, at least temporarily.

However, Sinemet rarely helps PSP-RS patients. Any improvement, rarely seen, is extremely short-lived, often lasting for only a day or so, according to experiences reported in this forum. Worse, the side effects can be awful, including hallucinations. Worse than the PSP symptoms.

Many of the neurologists who are most knowledgeable about, and experienced with, PSP do =not= prescribe any drugs at all for PSP-RS patients. It would be helpful, I think, for Dr. Hutchman to have more interaction with such other neurologists, and evaluate their reasoning.

I am not a doctor. Just a reporter (with high respect for medical professionals) who tries to observe closely actual experiences and reports of people "who are there." As a caregiver, I had 24/7 contact with my loved one with PSP month after month and year after year. Our movement disorder specialist, whom we both liked very much, had 30 minutes exposure to my loved one every six months -- one hour per year, The knowledge and insights we caregivers gain from concentrated, continuous contact with our loved one as the disease progresses should not be dismissed lightly.

With all due respect,

ed p.
|My wife of 56 years was Rose b. 1930, dx 1999, symptoms from 1997; d. 06/21/08; PSP-rs autopsy confirmed.
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Postby Robin » Thu Dec 03, 2009 3:05 pm

More comments (not as good as Ed's, however) were posted on this not-very-good webinar here:
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Postby eplowman » Thu Dec 03, 2009 7:38 pm

Robin or anyone else who might know:

Near the top of his presentation, Dr. Hutchman addressed this topic: "The Motor Manifestations of Parkinsonism seen in Parkinson’s Disease (PD) and the Atypical Parkinsonian-Plus Disorders."

The first manifestation he lists is "resting tremor." This is NOT a symptom associated with PSP, according to what has been reported in the forum and in key scientific literature about PSP. In fact, it appears to be a "differential" in diagnostics, pointing away from PSP. Is it known to be a characteristic symptom in any of the other atypical Parkinson's Plus diseases (MSA, CBD, LBD)?

Hallucinations and psychotic symptoms are NOT normally associated with PSP patients who are NOT taking =any= of the drugs that were recommended in this presentation. Are such symptoms common in patients with MSA, CBD, and LBD who also are not taking such drugs?

Frankly, I find it troubling that drugs known to CAUSE such symptoms in PSP patients are prescribed to "treat" the symptoms they cause! And as indicated in my earlier post, I also find it troubling that so-o-o much emphasis was placed on Sinemet and other agents aimed at altering dopamine when that is not a relevant issue at all in the majority of PSP cases (except for the adverse side effects it can cause for PSP patients). If all of the paragraphs about treatment with Sinemet and related agents were removed, it would appear that very little content applicable to PSP would remain. Just my cursory observation.

This was by most appearances a presentation primarily about Parkinson's Disease, not atypical Parkinsonism, despite the description of the webinar. And that, too, is troubling because of the confusion it could cause among atypical Parkinsonism sufferers and their caregivers. There must have been a breakdown in planning. But we live and learn....

ed p.
|My wife of 56 years was Rose b. 1930, dx 1999, symptoms from 1997; d. 06/21/08; PSP-rs autopsy confirmed.
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Postby Robin » Thu Dec 03, 2009 10:05 pm

The whole darn webinar was frustrating and I prefer not to re-live it. I agree with was basically a PD presentation made to look like an atypical parkinsonism presentation.
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