Jun 20 2012

Polio Perspecvtive

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Polio Perspective – October, 2019

Millie Malone Lill, Editor        Wilma Hood, Publisher

In This Issue

               Abnormal Movements in Sleep               By Dr. Richard L. Bruno, HD, PHD www.papolionetwork.org Revised 2018 

SENIOR LOOKOUT: What you need to know about post-polio syndrome

Senior Lookout Tracy Arabian

OTHER DISABILITY RELATED NEWSLETTERS

A LITTLE BIT OF HUMOR

Senior Lookout Tracy Arabian

FLARES 101

By Millie Malone Lill

WEB CORNER

Abnormal Movements in Sleep

 Dr. Richard L. Bruno, HD, PHD www.papolionetwork.org Revised 2018

 1 Abnormal Movements in Sleep as Post-Polio Sequelae Dr. Richard L. Bruno, HD, PhD American Journal Of Physical Medicine And Rehabilitation,1998; 77:

1-6. ABSTRACT

Nearly two-thirds of polio survivors report abnormal movements in sleep (AMS), with 52% reporting that their sleep is disturbed by AMS. Sleep studies were performed in seven polio survivors to objectively document AMS. Two patients demonstrated Generalized Random Myoclonus (GRM), brief contractions and even ballistic movements of the arms and legs, slow repeated grasping movements of the hands, slow flexion of the arms and contraction of the shoulder and pectoral muscles. Two other patients demonstrated Periodic Movements in Sleep (PMS) with muscle contractions and ballistic movements of the legs, two had PMS plus Restless Leg Syndrome (RLS) and one had sleep starts involving only contraction of the arm muscles.

AMS occurred in Stage II sleep in all patients, in Stage I in some, and could significantly disturb sleep architecture even though patients were totally unaware of muscle contractions. Poliovirus-induced damage to the spinal cord and brain is presented as a possible cause of AMS. The diagnosis of post-polio fatigue, evaluation AMS and management of AMS using benzodiazepines or dopamimetic agents is described.

Despite numerous late-onset symptoms reported by polio survivors — fatigue, muscle weakness, pain, cold intolerance, swallowing and breathing difficulties — one symptom was totally unexpected: abnormal movements in sleep (AMS). As early as 1984 our post-polio patients were reporting muscle contractions as they fell asleep. The 1985 National Post Polio Survey included two questions about AMS: “Do your muscles twitch or jump as you fall asleep” and “Is you sleep disturbed by muscle twitching?”

It was surprising that 63% of the 676 respondents reported that their muscles did twitch and jump during sleep and that 52% — a third of the entire sample — said their sleep was disturbed by twitching. These percentages are markedly elevated as compared to the incidence of AMS in the general population. In one survey only 29% of those without neurological disease who were at least 50 years old reported AMS, versus 63% of surveyed polio survivors who were 52 years old on average.

In another survey only 34% of those older than 64 reported AMS, slightly more than half the incidence of AMS in the younger post-polio sample. (3) Given the apparent increased prevalence of AMS in polio survivors, and with daytime fatigue the most commonly reported PostPolio Sequelae (PPS), we were interested in objectively documenting AMS, relating them to possible disturbances in sleep architecture and identifying an effective treatment for AMS.

METHODS

Subjects. Seven polio survivors were referred for sleep studies to a sleep disorders center. This was a sample of convenience in that the subjects were patients presenting with PPS who themselves knew (three patients) or whose bedmates knew (four patients) that AMS were occurring. Patients were on average 54 years old and 44 years post acute polio, which occurred at age 10. The patients had had AMS for a mean of eight years, which was on average 35 years post acute polio. Patients reported moderate-to-severe difficulty sleeping at night and moderate-to-severe daytime fatigue that did not respond to the treatments of choice for post-polio fatigue (i.e., pacing of activities, daytime rest periods, energy conservation and use of appropriate assistive devices. (

In addition to fatigue, patients reported an average of two limbs having late-onset muscle weakness. Procedure. Patients underwent a standard polysomnographic evaluation with EEG and facial EMG recorded for sleep staging.

 Blood oxygen saturation, measured using a finger pulse oxymeter, chest and abdominal wall excursion and nasal air temperature were also recorded; video monitoring of sleep was also performed. Surface EMG was recorded from patients’ legs as well as from limbs in which AMS were reported.

RESULTS

Four types of AMS were seen: Two patients presented with what has been called Generalized Random Myoclonus (GRM) (1,6), two patients had Periodic Movements in Sleep (PMS), two had PMS plus Restless Leg Syndrome (RLS) and one had Sleep Starts (also called “hypnogogic massive myoclonic jerks”). Generalized Random Myoclonus. GRM was seen in two patients. One had had bulbar polio with little or no arm or leg involvement acutely, while the other has been diplegic since polio but had no bulbar or respiratory symptoms acutely. These patients had random contractions of muscles throughout their bodies. The former patient had such violent contractions of the trunk muscles that she was pulled into the fetal position during the night. This patient had been very aware of GRM for about 10 years. However, the other patient had been completely unaware of GRM until they were noticed by her husband.

Random, rapid muscle contractions were noted in all four limbs, jaw and pectoral muscles, in addition to slow repeated grasping movements of the hands, slow flexion of the arms and movement of the shoulders. The presence of bilateral toe flexion was notable since the patient’s right leg has always been totally paralyzed except for a minimal ability to flex her toes; the toes of her right foot contracted numerous times the during sleep.

In both patients, GRM occurred during Stage II sleep; the latter patient also had GRM in Stage I. The patient with violent trunk flexion had muscle contractions, causing a severe disturbance of sleep architecture, only during the first third of the night. She also had a few episodes of obstructive apnea that were not related to the muscle contractions but did disturb her sleep. She was prescribed clonazepam, 0.5 mg B.I.D., which eliminated her GRM. The other patient had GRM throughout the night but had no disturbance of sleep architecture and was not treated pharmacologically.

Periodic Movements in Sleep.

Two patients demonstrated PMS with contractions only of the leg muscles of which neither patient was aware. Both had limb and respiratory involvement with the acute polio and had PMS during Stage II sleep with one patient also having muscle contractions during Stage I.

The former patient had nearly continuous EMG activity in his legs throughout the night and had a severe disturbance of sleep architecture. He also had some central episodes of apnea early in the night as he was falling asleep that did not disturb his sleep. The latter patient had PMS occurring only during the first half of the night that caused no disturbance of sleep architecture. However, he had frequent hypopneas that did severely disturb his sleep. Both patients were prescribed lorazepam, 1.0 mg H.S., which eliminated the PMS.

PMS plus Restless Leg Syndrome.

Two patients had PMS plus Restless Legs Syndrome. RLS is characterized by the subjective feeling that the legs must be moved. This feeling increases during the evening, often preventing sleep onset because patients feel as if they must get up and walk. The patients with PMS plus RLS apparently had been very little affected by the acute polio, one having no polio residual and the other having one leg weakened. PMS were seen in both legs and occurred during Stage II in both patients and during Stage I in one patient.

One patient’s leg muscle contractions were so violent that she was propelled one to two inches off the surface of the bed. Although her PMS occurred only during the first half of the night, her sleep was severely disturbed and she had been very aware that she had had PMS for about 5 years. She was prescribed Ldopa/carbidopa (Sinemet) 200/50 mg, 1/2 tablet B.I.D., and clonazepam, 0.5 mg H.S. and at 3 A.M., which reduced the RLS and PMS by about 80% and allowed her to have a restful nights sleep.

The other patient did not know he had PMS, which were continuous throughout the night, and did moderately disturb his sleep architecture. He was prescribed Ldopa/carbidopa, 200/50 mg H.S., which eliminated his RLS and PMS.

Abnormal Movements in Sleep.

One patient was diagnosed as having a Sleep Start, her arms ballistically abducting as she began to fall asleep. She apparently was very mildly affected by the acute polio and had no AMS in the legs, even in the leg in which she reported new muscle weakness. The patient’s sleep was markedly disturbed since her arms would move as she started to fall asleep and prevent sleep onset. She was prescribed alprazolam, 0.125 mg H.S., which eliminated her AMS.

DISCUSSION

Sleep studies in this sampling of post-polio patients objectively documented three different types of AMS. Whether other types or combinations of AMS occur in polio survivors cannot be determined from this study, nor can this study or the 1985 National Post-Polio Survey state the actual incidence of AMS in polio survivors, since neither sample was random or population-based. However, the objective documentation of AMS in these post-polio patients, and the Post-Polio Survey finding that 63% of polio survivors reported muscle “twitching or jumping” as they fell asleep, suggest that AMS may in some way be related to the pathophysiology of the original poliovirus infection.

In 1964, Loeb coined the phrase “hypnic myoclonus” to describe muscle contractions during sleep onset in healthy individuals without neurological disease. Loeb described the contractions as rapid (less 0.5 seconds long), arrhythmic (occurring without a pattern) and causing a variety of movements – finger flexion, thumb adduction, forearm and foot flexion and extension, shoulder elevation and facial twitching during Stages I and II sleep – identical to those seen in the post-polio patients. However, in contrast to the post-polio patients, none of Loeb’s subjects demonstrated contractions in more than one muscle group and in none had sleep disturbed by hypnic myoclonus.

Loeb thought that hypnic myoclonus resulted from an abnormality at the level of the brain stem reticular formation causing decreased descending inhibition of anterior horn motor neurons during sleep. Martinelli  thought PMS also resulted from an increase in anterior horn cell excitability, with Walters  finding that PMS decreased with the administration of an opiate receptor agonist. Loeb and Askenasy suggested that AMS were also related to abnormal discharges from the thalamus, cerebellum and basal ganglia. 

The implication of the basal ganglia in the generation of AMS is interesting since PMS are common in patients with Parkinson’s disease, whose decreased dopamine production impairs basal ganglia functioning, as well as in patients with narcolepsy who have an increased number and sensitivity of dopamine receptors in the basal ganglia, which have been found to decrease with the administration of dopamine receptor agonists.

All of the CNS regions implicated in the pathogenesis of AMS are known to have been damaged by the poliovirus. The anterior horn motor neurons, cerebellar nuclei and reticular formation were frequently and severely damaged by the poliovirus.  The periaquiductal gray, paraventricular hypothalamus and lamina II dorsal horn neurons were all lesioned by the poliovirus ; damage to these opioid peptide-secreting neurons may be evidenced not only by AMS but also by polio survivors’ documented doubled sensitivity to pain. 

Finally, the thalamus and basal ganglia (the substantia nigra, putamen and globus pallidus) were also damaged by the poliovirus, damage that has been implicated in the pathogenesis of post-polio fatigue.  Given the distribution and extent of poliovirus lesions in all of the CNS areas implicated in the pathogenesis of AMS, we should not have been surprised in 1985 that a majority of polio survivors reported muscles that twitch and jump during sleep.

Clinical Implications.

PPS remains a diagnosis of exclusion. All possible causes for new symptoms in polio survivors, especially causes for late-onset fatigue, must be ruled out before the diagnosis of PPS is made. Therefore, it is important to rule out all sleep disorders a causes of fatigue. Clinicians need to take a thorough sleep history from their post-polio patients, asking not only about symptoms of sleep-disordered breathing, which occurs frequently in polio survivors, but also about AMS.  The patient’s bed partner must also be asked about AMS since the majority of polio survivors will not know that they have AMS. Abnormal Movements in Sleep.

The Post-Polio Institute patients are referred for a sleep study if they report daytime fatigue or if sleep-disordered breathing or AMS are suspected. The lowest dose of a short acting benzodiazepine (typically alprazolam) will be prescribed before sleep if a patient has AMS, since these medications seem to virtually eliminate GRM and PMS in our post-polio patients. Treatment of sleep-disordered breathing is deferred to the sleep disorders center as is treatment for RLS, since a dopamimetic agent in combination with a benzodiazepine may be required.

However, there is a caveat to prescribing dopamimetics for polio survivors. One of our PPS patients developed vasovagal syncope with cardiac asystole during the administration of a dopamine receptor agonist.  We consider a history of vasovagal syncope or unexplained faints a contraindication to prescribing dopamimetics for polio survivors with AMS or RLS.

SENIOR LOOKOUT: What you need to know about post-polio syndrome

Senior Lookout Tracy Arabian

Today, Oct. 24,is World Polio Day. Many people do not realize that polio still exists. There are currently two countries that are polio-endemic: Afghanistan and Pakistan.

Efforts toward the complete eradication of this disease are continuing, and, as of Oct. 16, the Global Polio Eradication Initiative reports that there have been only 88 cases of wild poliovirus in 2019.

Polio, or poliomyelitis, is an infectious viral disease that can strike at any age and affects a person’s nervous system. Between the late 1940s and early 1950s, polio crippled around 35,000 people each year in the United States. The first safe and effective polio vaccine was approved in the U.S. in 1955.

While the United States was declared polio-free in 1979, people who had the virus in their youth are now at risk of post-polio syndrome. Post-polio syndrome refers to symptoms that appear decades — an average of 30-40 years — after the initial polio illness.

Factors that can increase your risk of developing post-polio syndrome include:

  • Severity of polio infection. The more severe the initial infection, the more likely that you’ll have signs and symptoms of post-polio syndrome.
  • Age of initial illness. If you developed polio as an adolescent or adult rather than as a child, your chances of developing post-polio syndrome increase.

Common signs and symptoms of post-polio syndrome include:

  • Progressive muscle and joint weakness and pain.
  • General fatigue and exhaustion with minimal activity.
  • Muscle atrophy.
  • Breathing or swallowing problems.
  • Sleep-related breathing disorders.
  • Decreased tolerance of cold temperatures.

In most people, post-polio syndrome tends to progress slowly, with new signs and symptoms followed by periods of stability.

There are no laboratory tests specific for post-polio syndrome, and symptoms vary greatly among individuals. Physicians diagnose post-polio syndrome after completing a comprehensive medical history and physical examination, and by excluding other disorders that could explain the symptoms.

Post-polio syndrome may be difficult to diagnose in some people because other medical conditions can complicate the evaluation. Depression, for example, is associated with fatigue and can be misinterpreted as post-polio syndrome.

Post-polio syndrome is rarely life-threatening, but severe muscle weakness can lead to complications:

  • Falls due to weakness in leg muscles.
  • Malnutrition, dehydration and pneumonia due to weakness in the muscles involved in chewing and swallowing.
  • Chronic respiratory failure due to weakness in the diaphragm and chest muscles.
  • Osteoporosis due to prolonged inactivity and immobility. A person with post-polio syndrome should discuss bone-density screening with their physician.

Falling is often a concern as we age, but is a person with post-polio syndrome is at great risk of falling. Fall prevention hints include:

  • Have your hearing and vision checked regularly.
  • If prescribed a new medication, check with your doctor about side effects that might affect your balance.
  • Wear rubber-soled and low-heeled shoes that fit well.
  • Avoid wearing socks when walking inside on hardwood or linoleum flooring.
  • Be careful when walking outdoors in wet or icy conditions. Take your cellphone with you on walks.
  • Exercise regularly to maintain strong bones, strength and flexibility. Ask your doctor or health care provider about the best type of exercise program for you.
  • Keep your home safe. Remove things you can trip over from stairs and high-traffic areas.
  • Install handrails or grab bars in your bathroom or other areas where you may need extra support, such as stairs and hallways.
  • Make sure to have adequate lighting throughout your home.
  • Regular doctors visits are important as we age. If you are a polio survivor, make sure this is noted in your medical records and discuss with your doctor what you should watch for given your personal history.

Tracy Arabian is the communications officer at SeniorCare Inc., a local agency on aging that serves Gloucester, Beverly, Essex, Hamilton, Ipswich, Manchester-by-the-Sea, Rockport, Topsfield and Wenham.

Flares 101

by Millie Malone Lill

Recently, I posted the following to my Facebook page.  Normally I would not have shared this with people not in my polio group, but I realized that most able bodied people, including my family,  have no idea in this world what happens to us polio survivors.  Sometimes people just need to know what we are going through.

“I’m not looking for sympathy here, just want to explain what I mean when I say “PPS flare.” I used to call it PPS fatigue, but that doesn’t really cover it. A nap or even a day in bed does not cure it. It is more than being tired, although that is definitely a part of it. It’s being light headed with ringing in the ears, alternately shivering and sweating, shaky hands, weakness, difficulty in maintaining my balance, fuzzy thinking, much like the flu It is caused usually by my having a “good day” and thinking I can do it all, then doing it all. This is the payback. I don’t look sick, because I am not sick. I have an incurable progressive ailment. It can be controlled by my saying NO, I can’t instead of Yes, I’ll be right there, but that is very difficult for me.”

There are several flavors of Flares.  Sometimes they are not so bad and you can struggle your way through them with no one other than you being any the wiser.  Sometimes you just do a little bit more than you should, or you forget The Rule:  If you work for 10 minutes, rest for 10 minutes before you do anything more.  This type of Flare, Little Flare, is shown by a generalized fatigue and doesn’t always go into Full Blown Apocalyptic Flare.  Often a nap and a good night’s sleep will take care of it.

Sometimes you will have a What the Heck Flare.  You know, you have to go get groceries, so you also go to WalMart and do a little shopping.  This kind of Flare can be sneaky.  You might actually think you’ve gotten away with your little spree because the next day you feel fine.  However, no good shopping trip goes unpunished.  Two or three days later, you look around to see where that train is that ran over you!  What the Heck?  Yep, there you go.

Then there is the dreaded Apocalyptic Flare.  You know better, and you know a Flare is definitely in your near future, but you Do It Anyway, the biggest curse in the Post-Polio world.  You are hoping for a Little Flare or possibly a What the Heck Flare, which would at least give you a day or so between now and Ground Zero.  You do a load of laundry, go shopping, eat lunch out with friends, move some stuff you no longer want to the back of your van and then think, OK, might as well take them to the thrift shop while you are at it.  And then you remember that this is Bingo Night in your apartment complex.  It has been a very pleasant day and you are still somewhat mobile so you play Bingo for an hour or so, visit with your neighbors and then go to bed.

The next morning, you are hit by the Apocalyptic Flare From Hell.  All of the stuff in my Facebook post comes crashing down onto your defenseless body.  You lie there wondering if you have the flu, but no, it is not the flu.  There is no semi parked on your body.  And no, you are not going to die.  You may wish you could, but PPS is never that compassionate.  It does no good to berate yourself for having that one really fun, activity-filled day.  You can take pain pills if you have them, park yourself in your recliner (hopefully with your sweet little loving doggie who wants to keep you alive mainly because she has no idea how to get her dog food into her bowl without you)  and play dead for a week or two.

I know we’ve all done this, we’ve all promised ourselves we will never Do It Anyway, but we all know we are lying to ourselves and the next time we feel really good we will sign up in advance for our very own Apocalyptic Flare.  Just try not to do it too often or you might not recover.

Bingo, anyone?

WEB CORNER

Finding Love as a disabled woman:

https://themighty.com/2019/10/find-love-disability/?utm_source=newsletter_disability&utm_medium=email&utm_campaign=newsletter_disability_2019-10-24&$deep_link=true

Why entrepreneurship is popular with peope with disabilities

https://themighty.com/2019/10/entrepreneurship-popular-disabilities/?utm_source=newsletter_disability&utm_medium=email&utm_campaign=newsletter_disability_2019-10-24&$deep_link=true

When kids ask about disabilities

https://themighty.com/2019/10/kids-ask-comment-disabilities/?utm_source=newsletter_disability&utm_medium=email&utm_campaign=newsletter_disability_2019-10-24&$deep_link=true

 

Ghost viruses and the Taliban stand in the way of polio vaccination

https://www.mtpr.org/post/ghost-viruses-and-taliban-stand-way-wiping-out-polio

16 side effects that tell you to call your doctor

https://themighty.com/2019/10/medication-side-effects-chronic-illness/?utm_source=newsletter_chronic_illness&utm_medium=email&utm_campaign=newsletter_chronic_illness_2019-10-23&$deep_link=true

What they don’t prepare you for when you are chronically ill

https://themighty.com/2019/10/what-they-dont-prepare-you-for-when-you-are-chronically-ill/?utm_source=newsletter_chronic_illness&utm_medium=email&utm_campaign=newsletter_chronic_illness_2019-10-23&$deep_link=true

How to care for your mental health when you have a chronic illness

https://advice.shinetext.com/articles/how-to-care-for-your-mental-health-when-you-have-a-chronic-illness/?utm_source=newsletter_chronic_illness&utm_medium=Newsletter&utm_medium=email&utm_campaign=newsletter_chronic_illness_2019-10-23&$deep_link=true

Polio expert advocates increased medical attention to counter effects of disease on survivors

https://www.sunnewsonline.com/polio-expert-advocates-increased-medical-attention-to-counter-effects-of-disease-on-survivors/

My wheelchair accessible adventure on Amtrak

https://spintheglobe.net/dir/2019/10/18/accessible-amtrak-autotrain/?lm=757fc47dae9c49305ed7909a743f7933&ls1=98ff620d828644636bf152f00ec8df4e03cb9bf1&ls2=905621682ee2eb35a61019f83a0dda2124926fd64f36b424c14166cb20a7a4ee

The pressures of having an invisible disability

https://themighty.com/2019/10/invisible-disability-pressures/?utm_source=newsletter_disability&utm_medium=email&utm_campaign=newsletter_disability_2019-10-17&$deep_link=true

OTHER DISABILITY RELATED NEWSLETTERS

https://paper.li/e-1452749534#/

https://paper.li/postpolionews/1291300268#/

htps://www.papolionetwork.org/newsletter.html

https://paper.li/e-1452749534#/

http://www.post-polio.org/

https://www.polioaustralia.org.au/http://www.postpoliosupport.com/summaries.htmlhttps://polioepic.org/http://snapsnv.org/

A LITTLE BIT OF HUMOR

Everyone can use a little “grammar” update now and then so here’s yours for today… enjoy!

Is it “complete”, “finished” or “completely finished”?  No English dictionary has been able to adequately explain the difference between these two words – “Complete” or “Finished”.

In a recent linguistic competition held in London and attended by, supposedly, the best in the world, Samdar Balgobin, a Guyanese man, was the clear winner with a standing ovation which lasted over 5 minutes.

The final question was: ‘How do you explain the difference between COMPLETE and FINISHED in a way that is easy to understand?  Some people say there is no difference between COMPLETE and FINISHED.’

Here is his astute answer:

“When you marry the right woman, you are COMPLETE. When you marry the wrong woman, you are FINISHED. And when the right one catches you with the wrong one, you are COMPLETELY FINISHED!”

He won a trip around the world and a case of 25 year old Scotch!

 

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6 Responses to “Polio Perspecvtive”

  1. Thomas Christian says:

    Ole better git a hearing ade?

  2. Millie Lill says:

    Ruth, read all you can online and I also suggest that you join one of the Facebook polio sites. You can learn a lot from those of us who have been there and done that.

  3. Hilary Boone says:

    Why do we still have so much hassle trying to get decent medical care. Sharing experiences really does help us realise ‘It’s not just me’ which slightly lessens the frustration and stress. Millie as usual you have done a great job.

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