RBD (REM sleep behavior disorder) and other sleep issues – meeting notes

The Palo Alto (California) Parkinson’s Disease Support Group’s February 2020 meeting featured Dr. Emmanuel During, who specializes in neurology, psychiatry, and sleep medicine at Stanford.  He discussed the various types of sleep disturbances that can occur in Parkinson’s disease (PD), such as insomnia, restless legs syndrome, sleep apnea, and REM sleep behavior disorder, along with available treatments for managing these issues. There was also a question and answer session.

Though this was a Parkinson’s Disease support group meeting, these sleep issues are common in all the disorders in the Brain Support Network community, including Lewy body dementia (LBD), multiple system atrophy (MSA), progressive supranuclear palsy, and corticobasal degeneration.  Indeed, REM sleep behavior disorder (RBD) is common in LBD, MSA, and PD.

Lauren Stroshane with the Stanford Parkinson’s Community Outreach attended the meeting and shared her notes below.

Robin

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Sleep and Parkinson’s Disease – Meeting notes
Palo Alto Parkinson’s Disease Support Group Meeting
February 12, 2020
Notes by Lauren Stroshane, Stanford Parkinson’s Community Outreach

The guest speaker was Dr. Emmanuel During, who specializes in neurology, psychiatry, and sleep medicine at Stanford.

Dr. During described sleep as a “blind spot” that often goes unaddressed in medical care. Doctors rarely inquire how their patients are sleeping, so sleep issues often go unrecognized and untreated. Yet the quality and amount of sleep you get every night affects your overall health and quality of life. For many with Parkinson’s Disease (PD), sleep disturbances may have been among their earliest symptoms, before the motor symptoms like tremor or slowed movement started to manifest. Sleep also has the important function of cleaning out toxins in the brain. No matter how well we treat the motor symptoms of PD, if you are not sleeping well, you will not feel healthy.

What types of sleep issues are common in PD?

At night, sleep may be shallow or fragmented. Muscle stiffness can make it difficult to reposition during the night. Sometimes muscle cramps or dystonia occur, which may be painful or disruptive. Frequent urination and restless legs may interrupt sleep, while snoring, sleep apnea, or acting out dreams may worsen sleep quality.

During the day, many people with PD experience brain fog and have difficulty paying attention. Daytime sleepiness and general fatigue are also common.

Fatigue may consist of physical or mental exhaustion. Typically, it occurs when certain neurotransmitters (dopamine, serotonin, and norepinephrine) are low. Causes of fatigue can include poor quality sleep, depression, restless leg syndrome (RLS), and side effects of medications.

Sleepiness is different from fatigue in that it is a difficulty staying awake, like drowsiness. It may be caused by insufficient sleep or sleep apnea, which is when breathing stops repeatedly during sleep. Sleep apnea is a serious condition that decreases the amount of oxygen that the brain receives during sleep, interrupting the sleep cycle over and over. Besides making you sleepy, it also increases your risk of heart attack and dementia. Sleep apnea can be diagnosed at home or via sleep study in a lab. There are a number of different effective treatments available to manage it.

Some medications can cause sleep attacks, periods when a person can’t help falling asleep regardless of the situation. Medications which can trigger this side effect for some individuals include Mirapex (pramipexole), Requip (ropinirole), Sinemet (carbidopa-levodopa), allergy medications, anxiety medications, and some painkillers. Provigil (modafinil) and caffeine are stimulants which can be helpful for managing fatigue and sleepiness in PD, but often, if a medication is suspected of causing sleep attacks, it may need to be reduced or discontinued.

Insomnia in PD

Insomnia, or sleep fragmentation, is also common in PD. Insomnia can either consist of difficulty falling asleep at the beginning of the night, or waking up during the night and having trouble getting back to sleep again. There are a number of ways to manage insomnia, depending on what is causing it.

  • For discomfort in bed due to stiffness or dystonia, sometimes switching to long-acting carbidopa-levodopa (Sinemet ER or Rytary) can help provide on-time overnight.
  • If pain is keeping you awake, try to identify methods to address the pain, such as medication or a heating pad.
  • If you are waking up frequently during the night to urinate (nocturia), the medication can Myrbetriq (mirabegron) can be helpful. Many other drugs for this issue can cause confusion in those with PD.
  • If you address these factors and are still having insomnia, then there are other options:
    • Cognitive behavioral therapy (CBT) can be very helpful.
    • Consult a sleep doctor or neurologist to explore prescription sleep aids.
    • Try over-the-counter sleep aids such as melatonin. AVOID Benadryl and Tylenol PM, which can cause confusion in those with PD!

People with PD sometimes experience circadian abnormalities, in which the body’s internal clock is disrupted and confused. The circadian rhythms in our bodies regulate our cardiovascular, hepatic (liver), pancreatic, adipose (fat), and gastrointestinal systems. Our bodies use certain cues – food, sunlight, activity, and melatonin – to regulate this cycle.

For those experiencing circadian abnormalities, such as from jet lag, Dr. During recommended limiting the use of naps and trying over-the-counter melatonin tablets, 0.5 to 2 mg taken one hour before your desired bedtime.

Restless legs syndrome (RLS)

Restless legs syndrome can occur in the general population but seems to occur more frequently in PD. It is an urge to move the legs that becomes quite uncomfortable until the legs are moved or stretched. This is not the same as leg movements which may occur during sleep – RLS is a bothersome sensation while you are awake, and can prevent you from falling asleep. RLS is typically caused by inadequate levels of iron in the brain.

RLS symptoms can be triggered by rest, boredom, and being in a confined space such as a car or plane. Stretching, massage, walking, distracting, and soaking the legs in hot water can be helpful. Aggravating factors for RLS include smoking, alcohol, coffee, and lack of exercise.

If you have RLS, you may need to get your iron tested and, if it is low, take oral iron supplements at a dose recommended by your doctor. Some antidepressants (with the exception of buproprion) can be beneficial. Other medications commonly prescribed are Horizant (gabapentin enacarbil) and Lyrica (pregabalin), though these tend to be expensive. Dopamine agonist medications including Sinemet, Mirapex, Requip, and the Neupro patch can help with RLS, but must be used cautiously due to the potential to “augment” or dramatically worsen RLS symptoms over time. These medications can also cause serious side effects, such as impulse control disorder (ICD), which consists of compulsive behaviors like gambling, shopping, binge eating, and hypersexuality. Low doses of opioid medications are sometimes used for chronic RLS that does not respond to other treatments.

REM sleep behavior disorder (RBD)

The part of our sleep cycle where the deepest, most important sleep occurs is during rapid eye movement (REM) sleep, when our eyes move but the rest of our body is temporarily paralyzed. In neurologic disease, sometimes the mechanism of paralysis stops working, and our body is able to move around while we are asleep, acting out our dreams. This is called REM sleep behavior disorder (RBD), and it can be quite dangerous due to the risk for injury to oneself and others. People with RBD may flail their limbs, run, fall out of bed, or even throw punches, yet they are asleep and unaware they are doing so.

RBD occurs in 50 percent of people with PD, but can also occur long before the motor symptoms and PD diagnosis. A sleep study in the lab can confirm that RBD is present.

How to manage RBD?

Safety measures can help prevent injury:

  • Move the mattress to the floor and use bed rails to reduce the risk of falling out of bed
  • Padded furniture
  • Window protection
  • Bolster pillow between bed partners
  • Consider sleeping in separate beds or rooms for safety
  • Bed alarm to alert a caregiver if the person is out of bed

Medications can reduce the occurrence of RBD:

  • Melatonin at bedtime (usually 1-10 mg dose)
  • Clonazepam (however, this can be sedating the next day)

A new drug trial is underway for treatment of RBD in Dr. During’s lab. The study drug is sodium oxybate, which has previously been approved for narcolepsy and alcohol withdrawal. Study participants have RBD, have previously tried melatonin and clonazepam, and have been unable to tolerate these medications. To learn more about the study, contact study coordinator Adrian at [email protected]

Takeaways

Parkinson’s disease worsens sleep quality.  Poor sleep leads to poor function during the day!

He recommends the following:

  • Discuss your sleep issues with your doctor
  • Address treatable issues that are affecting your sleep, such as stiffness, need to urinate, insomnia, restless legs symptoms
  • Snoring, brain fog, and headaches when you wake up can be signs of sleep apnea
  • If you have severe restless legs, you may be low on iron
  • If you are acting out your dreams, start melatonin and get a sleep study

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Question & Answer Session

Q: What percentage of people who have REM sleep behavior disorder (RBD) will go on to develop PD?

A: All people with RBD will eventually develop a neurologic disorder. It could be PD, or possibly a type of atypical parkinsonism such as multiple system atrophy (MSA) or Lewy body dementia (LBD).

Q: What types of patients do you (Dr. During) see in your clinic?

A: Dr. During sees patients with PD or other neurologic diseases who have trouble sleeping.

Q: I have dystonia. Is that because of my sleep issues or because of my PD?

A: It is unlikely to be because of your sleep issues. Dystonia is fairly common in PD, but other things can also cause it, so it is important to speak with your neurologist to see if further workup is needed.

Q: Have you found that the quality of melatonin pills is inconsistent?

A: Yes, that is sometimes the case. He recommends a couple brands that seem to be better quality: Nature Made and Naturelle. He also suggested the website Labdoor, which ranks various supplements and minerals according to the quality of their ingredients.

Q: I often crash around 2-3pm and feel like I can’t function anymore. What should I do? 

A: This is common and expected in PD, unfortunately, as well as those without PD! If you are able to nap for 20 minutes or less, do that on a regular basis. If you can’t nap without sleeping too long, instead try going outside and doing something active, like going for a walk. Sunlight and movement will help trigger your brain that it is not time to sleep. Chewing gum also signals to your brain that it is time to eat rather than sleep. And socializing with someone is another tactic that can help wake you up.

Q: Do we only dream during REM sleep?

A: No, we dream at other points during our sleep cycle. However, REM dreams are more complex and memorable. Studies indicate the dreams we remember were typically experienced during REM sleep.

Q: Does sleep position matter?

A: If you have sleep apnea, it is best for your breathing to sleep on your side, and worst to sleep on your back. For other conditions such as RBD, it does not matter what position you sleep in.

 

2020 Brain Support Network Local Caregiver-only Support Group Meeting Dates

Since 2004 (16 years!), we have convened nine support group meetings each year for caregivers of those with Lewy body dementia (LBD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and atypical parkinsonism. We meet by disorder though the PSP and CBD groups are usually together. Contact us if you’d like to be added to the meeting reminder email list.

The discussion is led by caregivers who have lost their family members to these disorders. In most cases, the family member donated his/her brain and the diagnosis has been confirmed. (Brain Support Network can assist your family in making brain donation arrangements.) Discussion leaders include:

  • LBD: Dianne, Sharon, Alexa, Anne, and Lynn
  • PSP: Cristina and JD
  • MSA: Candy, Barbara, Karen, Jan, and Doug
  • CBD: Dick, Mindy, and Mark

These caregiver-only support group meetings are held on Sundays from 5pm to 7pm. The dates for our 2020 meetings are:

  1. January 26
  2. March 15
  3. April 26
  4. June 7
  5. July 12
  6. August 23
  7. October 4
  8. November 8
  9. December 6

In selecting these dates, we have avoided as many cultural events, sporting events, and holidays as possible. 

Please put these caregiver-only support group meeting dates on your 2020 calendars now.  An email requesting RSVPs will be sent out a week or so before each meeting. RSVPs are always due by noon on the Saturday (one day) before each meeting.

All caregivers are invited:  primary, secondary, those giving hands-on care, those managing care, and those giving emotional and informational support.  Newcomers, casual visitors, and longtime attendees are all welcome! Former caregivers–those whose loved ones have already passed away–regularly attend.  Former caregivers have been through it all and are invaluable resources to those learning to cope.

If you are an active caregiver with a loved one at home, consider asking for a “respite care grant” from your county’s agency on aging or from your local caregiver resource center (see caregiver.org/californias-caregiver-resource-centers).  Such grants pay for a caregiver to be in your home while you attend support group meetings.  The Alzheimer’s Association (alz.org/norcal) also offers respite grants for those dealing with dementia.

We occasionally have guests.  Guests have included a family consultant from Family Caregiver Alliance, a board member of the Lewy Body Dementia Association, and a board member of CurePSP.

At two separate meetings in 2019, we welcomed people with a neurological diagnosis — 3 with MSA and 4 with PSP.  Those with a diagnosis meet separately from caregivers. If you have a family member with MSA, PSP, CBD, or LBD, and they are interested in having dinner with others with the same diagnosis, please let me know AT LEAST ONE WEEK before each meeting.  These extra gatherings take a bit of coordination.  

We have over 450 members now, with attendance of 20-30 at caregiver support group meetings.  Roughly speaking, of the 450 members, 50% have an LBD connection, 30% have a PSP connection, 18% have an MSA connection, and 2% have a CBS/CBD connection or Atypical Parkinsonism connection.  (I believe we have the largest PSP, LBD, and MSA local support groups in the US.) If you have suggestions on how we can get the word out about our group, let me know!

We look forward to seeing you at some meetings in 2020!

Swallowing, Eating/Chewing, Taste/Smell, etc – Notes from Speech Therapist

Courtney Lewis, a speech pathologist in Australia, spoke recently to the FTD (frontotemporal degeneration) support group in Hawthorn. She addressed swallowing, eating/chewing, oral behaviors (common in the behavioral variant of frontotemporal dementia), taste/smell, and taking medication. Helpful notes from the meeting were taken by Wendy Kelso. I read these notes on the FTD Support Forum, posted by online friend Glen, whose late wife had semantic dementia. Wendy has given permission for these notes to be shared.

Note: I’ve left in all the Australian spellings and terminology (eg, “swallowing tablets” for “swallowing medication”).

Robin

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Summary of Discussion with Courtney Lewis, Speech Pathologist
FTD Support Group, Dementia Australia Hawthorn
1st November 2019
Minutes: Wendy Kelso

SWALLOWING

– Swallowing is a highly complex activity that requires planning and co-ordination of a number of brain regions
– Controlled by motor cortex – muscles/movement
– Orbitofrontal cortex – behaviour
– Precentral gyrus – voluntary initiation of the swallow reflex
– Brain stem – cranial nerves
– When you swallow, the airways are closed to prevent food going down
the wrong way
– If the windpipe does not properly close off, or if swallowing is not well coordinated, choking can occur
– Aspiration pneumonia can occur if food enters the lungs
– Malnutrition and dehydration may occur as a result of swallowing difficulties

To help the swallowing reflex:

– Verbal instructions usually don’t work – better to use tactile (touch) cues to swallow
– Tap or stroke the persons chin or cheek gently to encourage swallowing
– Take little sips of water when the person has the sensation in their mouth
– Put a cold spoon on their lips to open the mouth to allow them to be fed
– Sometimes people have forgotten what to do next after chewing and need prompting to swallow
– Try and provide food that needs less chewing – steak/tough meat requires too much chewing and is effortful to swallow
– If the mouth is overfull, this affects swallowing
– Try and encourage smaller mouthfuls of food to assist swallowing

EATING/CHEWING

– Brain changes in FTD cause changes in eating, drinking, chewing and swallowing behaviour
– Many people consume food and drink to excess and cannot control their food intake
– They may consume large quantities of sweet foods, sweet drinks and/or alcohol and cigarettes
– They may eat items that are unpleasant or non-food items, such as soap
– Sweets have a high reward centre in the brain – quickly sends pleasure information to the brain
– If the person doesn’t chew their food, it usually reflects frontal/behavioural changes rather than a problem with swallowing

Eating Tips:

– Change the environment – reduce the volume of food/drink and the number of options
– Provide healthy food options in portion controlled sizes
– Never have a buffet meal if the person overeats and cannot monitor their food intake
– Use smaller spoons, plates, cups and wine glasses
– Routine helps – regular meal times in the same place using the same cutlery and crockery. This helps the person recognise it is meal time
– Using hands to eat and finger food is fine – it can assist people to remain independent at feeding for longer
– Changing the temperature and texture can assist the food to go down
– Soup can make the food more moist
– Some people will require 1:1 supervision with meal times to assist the process and ensure safety
– Chewing requires a lot of effort – it is a form of exercise
– Sometimes people become breathless when chewing and this can lead to aspiration
– Fatigue affects chewing and swallowing

ORAL BEHAVIOURS

– Common behaviours include lip smacking, lip chewing and tongue clicking
– Strategies can include sucking or biting items such as a soft piece of fabric or using ‘chewy tubes’
– Chewy tubes are highly tactile and can be in the form of a necklace or bracelet
– If the person licks their lips, try using lip balms or chapsticks
– If the person tongue clicks or thrusts, try and reduce the sound
– If the person is a smoker, this can leave their mouth feeling very dry and they can exhibit a variety of oral behaviours
– Try and encourage sips of water

TASTE AND SMELL

– Changes to taste and smell are caused by changes in the frontal and temporal lobes in the brain
– These changes are very common in bvFTD
– The olfactory bulb (smell) is usually working but the information is not being relayed back to the other areas of the brain that control smell
– Sweet foods are highly desirable as they have a stronger more pleasant taste and have a high reward and value system in the brain
– People with FTD find it difficult to process sour/bitter and disgusting foods
– As an example, broccoli and cauliflower have a ‘negative’ taste, so is less palatable than ice cream to the person with FTD

SWALLOWING TABLETS

– Sometimes swallowing tablets with ice-cream, yoghurt or custard can help
– Many people will find it very hard to swallow tablets with water/liquid
– Swallowing tablets with water is a complex multi-step task and requires more co-ordination

Red flags suggesting atypical parkinsonism, and treatment of DLB, MSA, PSP, and CBD

In a recent issue of the “Handbook of Clinical Neurology,” a chapter is devoted to the four atypical parkinsonism disorders — dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The authors — both movement disorder specialists at the University of Florida — indicate that “diagnosis is critical as the atypical parkinsonisms herald symptom profiles and prognoses distinct” from Parkinson’s Disease (PD). They also indicate it’s important to identify the suspected underlying pathology — whether it be alpha-synuclein (for DLB and MSA) or tau (for PSP and CBD). Identifying the underlying pathology influences clinical trial enrollment.

Here’s a link to an abstract on PubMed about the chapter:

https://www.ncbi.nlm.nih.gov/pubmed/31753139
Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.

(There is a $32 fee to download the full article.)

The authors, Drs. Melissa Armstrong and Nikolas McFarland, point out that one reason to know whether you are dealing with an atypical parkinsonism disorder rather than PD is that the survival time is less than 10 years on average and that most die from disease-related issues such as falls, aspiration pneumonia, and failure to thrive.

This statement of concern about caregivers is interesting:

“Given patients’ mounting disability over time and the need for increasing support for both instrumental activities of daily living and basic activities of daily living, caregiver support is critical. In some circumstances, clinicians will need to assist couples in identifying backup caregivers to prepare for the possibility that older caregivers may themselves experience health concerns.”

PHARMACOLOGIC TREATMENT

Pharmacologic treatment is entirely symptomatic, and includes:

MOTOR

* Parkinsonian symptoms: levodopa

* Dystonia: dopaminergic agents; botulinum toxin injections; anticholinergic agents (in younger people with MSA)

* Myoclonus: valproic acid, levetiracetam, clonazepam

The authors note that deep brain stimulation is “rarely helpful” to those with atypical parkinsonism.

NON-MOTOR

* Depression: avoid tricyclic antidepressants in those with orthostatic hypotension

* Depression and Pain: duloxetine

* Depression, Sleep, and Weight Loss: mirtazapine

NONPHARMACOLOGIC TREATMENT

Nonpharmacologic treatment is focused on dysphagia (swallowing problems) and fall prevention. Treatment also includes physical therapy, occupational therapy, and speech-language pathology.

“The role of exercise in the atypical parkinsonisms is not well-researched to date, but increasing evidence of the benefits of exercise in people with PD suggests that there may be an important role for exercise in the atypical parkinsonisms, as well. Safety is a priority and guides selection of appropriate physical interventions.”

The authors encourage neurologists to discuss advance care directives with patients and families early on so that personal preferences are known. These directives should be re-discussed along the way. Palliative care and hospice can be helpful.

The chapter includes a helpful table for physicians on “Red flags suggesting an atypical parkinsonism.” That’s copied below.

Robin

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Recognizing and treating atypical Parkinson disorders.
Armstrong MJ, McFarland N.
Handb Clin Neurol. 2019;167:301-320.

Table 16.1
Red flags suggesting an atypical parkinsonism (a)

Red flag Suggested atypical parkinsonism
Rapid disease progression Any atypical parkinsonism
Lack of a robust levodopa response Any atypical parkinsonism
Bilateral symmetric parkinsonism DLB, PSP
Early gait impairment, falls Any atypical parkinsonism (b)
REM sleep behavior disorder DLB, MSA
Early bulbar dysfunction PSP
Irregular, jerky tremor MSA, CBD
Myoclonus MSA, CBD (less common in DLB, PSP)
Supranuclear gaze palsy PSP, CBD
Dysautonomia DLB, MSA
Cerebellar signs MSA
Laryngeal stridor MSA
Perioral/facial levodopa-induced dyskinesias MSA
Early dementia DLB, PSP, CBD (b)
Apraxia of speech or progressive nonfluent aphasia PSP, CBD
Apraxia CBD, PSP
Alien limb phenomenon CBD
Higher cortical findings (e.g., agraphesthesia) CBD

(a) Most commonly associated diagnoses are listed but this does not exclude the possibility that an unlisted atypical parkinsonism could be associated with the red flag (for example, RBD is described as occurring in people with PSP, but it is more commonly associated with the synucleinopathies).

(b) Specific details of impairments may suggest a specific atypical parkinsonism.

CBD, corticobasal degeneration; DLB, dementia with Lewy bodies; MSA, multiple system atrophy; PSP, progressive supranuclear palsy.

Palliative care discussions in MSA – when to have, what to discuss, etc.

This is an interesting study published in May 2017 about palliative care discussions (or the lack there of) among 22 Canadian patients with multiple system atrophy (MSA) seen at a movement disorder center.  “Palliative care discussions” include diagnosis and symptom management, prognosis, goals of care, and comfort measures.  Interestingly only 16 of the 22 patients discussed these issues with their MDs, according to medical records.  (So maybe not all “discussions” are recorded in patient charts.)

You can find the full article here:  (available at no charge)

https://www.cambridge.org/core/journals/canadian-journal-of-neurological-sciences/article/palliative-care-discussions-in-multiple-system-atrophy-a-retrospective-review/4F1398B9A85ECA7588455DCAD237C6B3/core-reader

(Thanks to long-time friend Vera James for sharing this link with me.)

The authors argue that “Early and effective advance care and end-of-life planning discussions are…essential for effective palliative care in MSA. … [These] discussions … will allow people with MSA to make treatment decisions that better align with their goals of care prior to an emergent situation.”

And the authors argue that advance care planning must taken into account respiratory dysfunction.  “Respiratory dysfunction, including but not limited to stridor, is the leading cause of death for people living with MSA, and it may present acutely with or without prior evidence of stridor.”

Perhaps these discussions are not occurring because stridor and sleep apnea are NOT the most common symptoms seen in MSA?  Both symptoms occur among a minority of patients.  Among the 22 Canadian patients, the most common symptoms were parkinsonism, orthostatic hypotension, GI/GU dysfunction, gait impairment, and ataxia.

But even patients with “modified diets did not have a documented discussion about possible future requirements for artificial feeding.”

However, if you have stridor, your medical team is more likely to discuss advance care planning with you (and document it in the medical record).  “Five of the six patients with stridor had a documented discussion about tracheostomy before one was required for life-sustaining purposes. Of the three patients who required emergent tracheostomy, one had a documented discussion declining tracheostomy in the management of stridor. Nevertheless, this patient was later intubated in intensive care secondary to respiratory failure.”

Of the 22 patients, 6 were deceased.  Though one of those six had a DNR (do-not-resuscitate) order in place, resuscitation was attempted by the care facility where the patient was living.  And only 2 of those six had a healthcare agent listed in the medical record.  Four of the six died in ICU.  Of those four, “discussions surrounding resuscitation and goals of care took place” days or hours before death.

The authors say:

“Our chart review shows clear gaps in the current approach to advance care planning, goals of care and palliative care discussions in patients living with MSA, at least at our centre. Important advance care planning and end-of-life discussions took place very late in the disease trajectory and often at a time when the affected person was no longer able to participate. While our study is limited to a retrospective review of charts at a single centre, it nonetheless shows significant gaps in the provision of care.”

To remedy this situation, the authors propose a framework for palliative care discussions in MSA:

“In the early stages of MSA, it is important to determine who the person with MSA would like to have present for important healthcare discussions in general, and then establish each patient’s readiness to discuss end-of-life decisions. Since diagnosis of MSA is difficult, there may be a period of ambiguity regarding the diagnosis, and the patient might not yet be receptive to hearing about the disease trajectory and his/her prognosis. Nevertheless, even without a determinate diagnosis, identifying personal supports and discussing personal values will help to inform and simplify future discussions regarding end-of-life care, when appropriate. Information regarding diagnosis, expected disease trajectory, symptom management and prognosis should be disclosed to the person with MSA and his/her family or an appointed decision maker, where possible. Physicians should initiate and document the results of important discussions, after assessing the patient’s readiness for such discussions. These include the identification of a substitute decision maker or power of attorney for healthcare, the presence of advance care plans, preferences regarding artificial nutrition and hydration, including their expected benefits or the lack thereof, end-of-life care wishes and preferred place of death. While tracheostomy may extend the length of life, it does not, in our view, extend or improve the quality of life. Therefore, it is important to discuss tracheostomy and to document the patient’s wishes surrounding tracheostomy before an unexpected emergent event.”

The authors expect the neurologist to be active participants in the palliative care process.  Neurologists can also make referrals to more specialized palliative care services.

“For some people, a referral [to palliative care] at the time of diagnosis might be appropriate, whereas for other people it may be more appropriate to initiate a referral once further severe symptoms develop, including dysphagia, stridor, sleep-disordered breathing, severe orthostatic blood pressure changes, increasing falls or other symptoms of distress.”

Robin