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NeuroPalliative Care
Dr. J. Miyasaki, MD, MEd, FRCPC
Professor, Director Parkinson and Movement Disorders Program, Co-Director, the
Complex Neurologic Symptoms Clinic
Division of Neurology, University of Alberta
Faculty/Presenter Disclosure
• Faculty: Janis Miyasaki
• Relationships with financial sponsors:
- Grants/Research support: Allergan; Patient Centered Outcome Research Institute
-Speakers Bureau/Honoraria: N/A
- Consulting Fees: GE
- Patents: N/A
- Other: UptoDate: Royalty for book
1. Prevalence, natural history, pathophysiology, end of
life trajectory, symptoms relevant to palliative care,
treatments of the symptoms of:
2. Severe Acute Brain Injury, PD and related disorders
and dementia, MS, ALS
3. In 45 minutes with mandatory 15 minutes of
interaction
Objectives
Theoretical Trajectories of Dying
2Lunney, JR et al. Patterns of Functional Decline at End of Life. JAMA, 289(18): 2387-2392.
Opportunities for Palliative Care
Approach
Ho
spita
lizatio
n
Asp
iratio
n
Pn
eu
mo
nia
Ho
spita
l
Dia
gn
osis
Level of
Function
Time
Illnesses have Motor and Non-motor symptoms
Undetected pain is often reason behind MAID requests
Cognitive decline occurs in many illnesses thought of as
purely motor
Find a motivated neurologist in an academic setting to
partner with you
Consider attending clinics with advanced illness patients
to learn common scenarios and treatment tips
Overarching Theme of Neurologic Illness and
Palliation
Typical neuroleptics
Atypical neuroleptics – except quetiapine or clozapine
Metoclopramide, nozinan
Nearly all neurologic patients will have dementia at the
end of life – therefore, delirium may not be avoidable if
good pain control is also required
Medications to Avoid with Neurologic Patients
Ischemic stroke
Intracerebral or subarachnoid hemorrhage
Traumatic brain injury
Inflammatory brain injury
Severe Acute Brain Injury
1%/year in Canada
Third most common cause of mortality (after heart attack
and all cancers combined)
Risk factors: hypercholesterolemia
Diabetes
Smoking
Cocaine, heroine, methamphetamine
Stroke Prevalence
Behavioral Pain Scale
Common Symptoms after SABI
Paroxysmal sympathetic hyperactivitiy
Hyperthermia
Hypertension
Tachycardia, tachypnea
Increased muscle tone
Diaphoresis
Storming
Symptoms after SABI
Triggers for Conversations about Goals
Triggers for Conversations
Inability to maintain hydration and caloric intake + One
of:
Palliative Performance Scale < 40%
Weight loss >10%/6mo or >7.5%/3mo
Serum albumin low
Dysphagia severe enough to prevent receiving sufficient
food and fluid to sustain life and patient does not receive
artificial nutrition and hydration
Consider LTC and Community Pall or Inpatient
Palliative Care if:
275-500/100,000 prevalence
Average age of onset 55 years
Mean survival: 15 years
Pathophysiology: progressive neurodegenerative
disorder with unknown etiology
Parkinson disease
Parkinson’s disease – “slow” progression
Lewy body dementia – cognitive change precedes or
within 1 y of motor, cognitive fluctuations, hallucinations
Multiple system atrophy- dysautonomia + parkinsonism
or ataxia
Synucleinopathies
With permission from Lang and
Lozano, NEJM, 1998
What predicts mortality in Parkinson’s disease
Neurology 2010;75:1270
Williams,
Neurology
2006
Cause of Death Over 10 y
Williams-Gray JNNP 2013
Bothersome or disabling pain not responsive to PD
medication management
Behavioural complications requiring reduced motor
control
Caregiver distress or burnout
Recent or repeated hospitalizations
Loss of ability to drive
Falls or need for gait assistance
Cognitive impairment
Potential Triggers for Palliative Conversations
Behavioural issues: hallucinations, delusions, wandering
Significant dysphagia
Hospitalization from aspiration pneumonia
Weight loss
Existential distress
Acceleration in changes in functional status
Triggers
Rotigotine 3 mg (patch) = 100 mg Levodopa
Beware confusion, somnolence, psychosis
Rectal Levodopa:
Crush 10 tabs 100/25
Add to 10 ml 50% H2O, 50% glycerol + 1 g citric acid
100 ml/ml
Shake well before use
Unable to Swallow
Rotigitine patch (beware psychosis/confusion, orthostatic
hypotension)
Rectal levodopa
Give regular levodopa by PEG if in place – be aware
duration of action will be approx. 30-60 min/dose
Alternatives to Oral Levodopa
Outcomes
ESAS-PD improved significantly (56 to 40)and to similar
extent as those with endstage metastatic cancer (48 to 39)
p <0.0001 (95% CI 10,21)
Symptoms responding most to interventions: Dysphagia,
constipation, anxiety, pain, drowsiness and other
Zarit Caregiver Burden Scale (modified) improved from
mean V1 43.5 to V2 36 (p < 0.0001, 95% CI 6, 9) (max
score 96)
Cause of death in clinic
130 patients: 33 deaths
Place of death: LTC 4
Home 29 (community palliative care)
Palliative inpt unit 4
Acute care hospital 6 (no hospice bed 1)
Cause of death: aspiration pneumonia 26
died in sleep 5
other 2
NO metoclopramide, nozinan, any typical or atypical
neuroleptic
EXCEPT quetiapine and clozapine
Do NOT stop Parkinson medications unless imminently
dying
Practical Tips
Prevalence: 10-20/100,000
Life expectancy: 5 years (range 2 years-20)
Pathophysiology: synucleinopathy
Many symptoms overlap with PD, but compressed in time
course – always changing, relentless, more pain
Multiple System Atrophy
Jnnp Glasmacher, Leigh, Saha
What predicts mortality in MSA
Edmonton Symptom Assessment
System:MSA
0
2
4
6
8
10
12
Pain Tiredness Drowsiness Nausea Appetite SOB Depression Anxiety Wellbeing
MSA: Other Important Symptoms
Confusion
What predicts mortality in Progressive
Supranuclear Palsy?
Alzheimer disease: 4.4% of the population 65+
19/1000 population
Vascular dementia: 26% of all dementias
Prevalence 0.6-2% of those over 65 y
What specific dementia diagnoses do you know?
AD accounts for 60-80% of all cases
Amnestic – short then immediate, last remote
Socially appropriate
Vascular dementia 10-28%
Stepwise progression
Lewy body dementia 5-20%
Cognitive fluctuations, hallucinations, dysautonomia
Exactly like Park dis dementia except within 1 y of motor
symptoms
Dementia
5-10%
Onset 45-64 (younger than AD)
Executive dysfunction: poor decision-making, lack of
empathy, impulsiveness
Can occur with ALS
May be inherited as autosomal dominant
Frontotemporal Dementia
From Neuropalliative Care, 2018
Functional Assessment Staging
At FAST 7 25% 6 month mortality, median survival 1.3 years
Steps for Dementia Palliation
Stage/Trigger Palliative Care Interventions
Time of Dx
New behavioural symptoms
GOC
PD, POA
Rx depression, cognitive symptoms
Caregiver support
Moderate: new or inc agitation
Inc dependency
Screen and treat Psychiatric Sx
Safety screening: finances, driving,
abuse
Caregiver support
Assess care needs
Severe: incontinence
Dec ambulation, frequent falls
Dec ability to have a conversation
Choking dysphagia
Pneumonia, Weight loss,
Hospitalizations
Symptom management
GOC reassessment
De-prescribe medications of limited
benefit
Consider hospice or LTC referral
Pain Assessment in Advanced Dementia (PAINAD)
Observation 0 1 2
Breathing Normal Occ laboured
breathing, short
hyperventilation
Noisy laboured,
longer period of
hypervent or CS
Negative
Vocalization
None Occ moan/groan
Low level neg
Repeated calling
out, loud moaning,
groaning, crying
Facial Expression Smiling or
inexpressive
Sad, Frightened,
Frown
Grimacing
Body Language Relaxed Tense, distressed
pacing, fidgeting
Rigid, fisting,
pulled up, striking
Consolability No need to
console
Distressed or
reassured by
voice or touch
Unable to console,
distract
Warden, 2003
Screen for hunger, thirst, need to urinate,
defecate/constipation, inability to communicate
Strained staff/caregiver
Past history of physical or sexual trauma may make even
good nursing care traumatic
Unmet Physical or Emotional Needs
Leading cause of institutionalization and death in older
population
Severe functional limitation occurs
Explore GOC early while patients still capable
Behavioural management is challenging
Practice Tips
290/100,000
Highest in Edmonton
Relapsing Remitting – means recovery after attacks
Primary Progressive – progressive decline after
diagnosis, may have acute relapses but less recovery
Secondary Progressive: start as relapsing remitting and
then become progressive
Related: Acute Disseminated EncephaloMyelitis
Multiple Sclerosis
Multiple disease modifying therapies mean patients receive
intensive therapy
Degree of immunosuppression correlates with risk of
Progressive Multifocal Leukoencephalopathy
Congestive Heart Failure
Pulmonary Fibrosis
Cognitive decline varies with disease burden
MS: Challenges based on Age and Previous
Treatments
Disability is assessed by the Expanded Disability Status
Scale – heavily weighted by mobility
0: asymptomatic
1-3 moderate but ambulatory independently
6 wheelchair
9 bedridden 10 dead
MS: The Challenge of Advanced Disability
Fatigue
Cognitive changes
Communication/Dysphagia
Spasticity
Tremor
Social Isolation
Marital breakdown
MS Advanced Symptoms
Early: Time of diagnosis
Acute Demyelinating Encephalomyelopathy or Neuromyelitis
Optica
First presentation requiring ICU stay
Potential Triggers for Palliative Care
Having to change or stop work
Loss of ability to drive
Chronic pain
Marital breakdown, loss of caregiver support
Wheelchair required
Urinary incontinence or catheterization required
Cognitive changes
Advanced Disease Triggers
Advancing cognitive dysfunction and dementia
Admission to long term care
De-prescribing immune modulating treatments
Dysphagia, risk of aspiration and reduced nutritional
intake
Recurrent infections/hospitalizations
Later Stage Disease Triggers
Up to 80% of patients
Spasticity
Spasm (hemifacial spasm, trigeminal neuralgia or
other)
Neuropathic pain
Skin or pressure ulcers
Pain
Physiotherapy
Baclofen 10-40 mg tid: unsteadiness, excessive
weakness, confusion
Gabapentin (?), dantrolene
Botulinum toxin
Cannabis: Delirium
Spasticity
Avoid overheating
Amantadine: anticholinergic effects 100-200 mg bid
Methylphenidate
Modafanil
Fatigue
Occupational therapy
Weights on wrist
Typically refractory to treatment
NO effective medications
DBS occasionally successful – may precipitate attack
Tremor
Awareness that cough may not be present
Careful hand feeding
PEG has not been assessed but can be useful
If drooling is disabling, consider
Atropine patch (cut in ½ or ¼)
Glycopyrolate
Botulinum toxin injections: worsen dysphagia
Dysphagia
May have any combination of problems
Overactive: spastic bladder, oxybutynin, catheterization
Detrusor sphincter dyssynergia: botulinum toxin
injection, bethanechol, catheterization
Retention: catheterization
Consider urodynamics
Bladder Dysfunction
Up to 60%
Higher risk if limbic involvement, during relapses
NOT related to duration of illness
Suicidality 4 x population controls
monitor patients treated for depression
Requests for MAID 4 x population controls in Belgium
Depression and Suicidality
Incidence 2/100,000/y
Age of Onset: 55-70 y
Mean survival: 3-5 y
Motor: fasciculations, cramps, weakness, muscle wasting
Bulbar onset: facial weakness, dysphagia, dysarthria,
respiratory failure and inability to wean from ventilator
Cognitive: Frontotemporal dementia 45-55%,
pseudobulbar affect
Amyotrophic Lateral Sclerosis
Better accuracy if patient is followed over time
Faster progression if: Age >75
Bulbar onset
BMI < 25
ALS Functional Rating Scale: 25% decline in functional
status
Prognostication
ALS Functional Rating Scale
4 3 2 1 0
Speech Normal Abn Repeat Speech combined w
nonvocal
communication
none
Salivation None Slight Moderate Marked excess Drooling
Swallow Normal Early
problem
Dietary
consistency
Tube feeding Nothing by
mouth
Handwrit
ing
Normal Slow Not all
legible
Able to hold pen but
not write
Unable to
hold pen
Cutting
food and
feeding
Normal Slow Needs some
help
Food must be cut Need to be
fed
ALS Functional Rating Scale
4 3 2 1 0
Turn in Bed Normal Slow With difficulty Initiate but
not alone
Helpless
Walking Normal Early prob Walks with
assistance
Nonambulat
ory
No purposeful
leg movement
Dressing
and
Hygiene
Normal Effortful Some help Needs
assistance
Total Care
Climbing
Stairs
Normal Slow Mild
unsteadiness
Needs
assistance
Unable to do
Breathing Normal SOBOE SOB at rest Intermittent
ventilator
Ventilator
dependent
Riluzole: inhibits glutamate release, extends life by 3
months
Edaravone: Slows progression as measured by ALSFRS
Unclear if any survival benefits
Treatments
Most ALS Programs will have established pathways for
non-invasive ventilation using BiPAP
Initially used at night
Use may extend into day
Choice to stop BiPAP at this point or any time after
<10% of US patients pursue tracheostomy and
mechanical ventilation
Non-Invasive Ventilation
Physiotherapy
Little high level evidence
Baclofen 10 mg tid up to 80 mg/d
Tizanidine
Benzodiazepines: clonazepam 0.5-2 mg tid
Botulinum toxin
Levetiracetam
Spasticity
Sudden, involuntary, painful contractions
Baclofen, vitamin E, gabapentin (open label evidence)
Quinine sulfate: associated with higher risk of cardiac
arrhythmia and removed from US market
Mexiletine
ECG for prolonged QT
Cramps
Frontotemporal dementia patients early on can be
socially appropriate and not able to reason
Early loss of executive function: use Montreal Cognitive
Assessment Scale
Cognitive impairment present in 50% of patients
Potential Pitfalls
At time of diagnosis, a palliative approach is appropriate
Change in ALS FRS
Progressive weight loss
FVC < 50% or MIP < -60 cmH20
Worsening dysarthria or dysphagia
Serious Illness Triggers
Difficulty communicating
Dyspnea
Choking episodes
Insomnia
Pain
Depression mood (40%), Anxiety (30%) and confusion
(10%)
Common Problems in the Last Month of Life
Early morning HA
Vivid dreaming
Dyspnea on exertion
Inability to lie flat
Nocturnal hypercapnea
Respiratory Impairment
If FVC <50% or MIP < -60 cm H2O
BiPAP is typically organized by the ALS clinic
Improves QOL but does not extend survival for those with
bulbar symptoms
Non-Invasive Ventilation
<10% pursue this option
Patients report sustained QOL
Family report significant decrease in QOL
Tracheostomy and ventilator support
Lung recruitment strategies: partner with respirologist
early in course of illness
Bronchodilators
Glycopyrrolate for sialorrhea: 1-2 mg qid (delirium)
Morphine in low dose for dyspnea
Dyspnea Treatment
Early requests often due to fear or unaddressed
symptoms
Oregon review: ALS second most common condition
Loss of autonomy
Inability to engage in activities
MAID
Medication Oral Rectal IV IM SC
Phenytoin + No + No No
Valproic Acid + + + No No
Levetiracetam + + + + +
Phenobarbital + + + + +
Carbamazepine + + No No No
Midazolam + + + + +
Lorazepam + + + + +
Typical neuroleptics
Atypical neuroleptics – except quetiapine or clozapine
Metoclopramide, nozinan
Nearly all neurologic patients will have dementia at the
end of life – therefore, delirium may not be avoidable if
good pain control is also required
Medications to Avoid with Neurologic Patients
1. People DO die of neurologic illness
2. Symptom burden is high and often over y – decades
3. Symptoms typically include motor and non-motor
4. Treatments may not be compatible with optimal motor
function
5. Treatment may not be typical of the palliative care
toolkit
6. Medications can worsen mental status
Conclusions
References
Thank you