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Caring for Patients with
End Stage Neurological
DiseasesSamuel Frank, MD
Karen Baker, LICSW MSW
Course Materials & Disclosure
�Course materials including handout(s) and
conflict of interest disclosure statement are
available to download with this course.
�This presentation is for educational and
informational purposes only. It is not
intended to provide legal, technical or
other professional services or advice.
Objectives
� Review the differential diagnosis and clinical features of
parkinsonism
� Idiopathic Parkinson’s disease (PD)
� Other parkinsonian syndromes
� Review the clinical features of ALS
� Review the clinical features of Huntington disease
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Who Gets PD?
Epidemiology of PD
� 2:1 male:female prevalence
� Less common in Black and Asian populations
� Average onset age = 63
� 5-10% of people have symptoms < 45
� 1.6% of U.S. population over 65 has a dx of PD
� ~1 million people in North America
� Up to 130,000 people are newly diagnosed each year in
the US
Willis AW et al, 2010
Parkinsonism
� Clinical syndrome characterized by a variable
combination of:
� Tremor
� Bradykinesia
� Rigidity
� Postural instability
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Classification of Parkinson
Syndromes in a Community
Syndrome Prevalence
Idiopathic PD 85%
Medication-induced parkinsonism 7-9%
Multiple system atrophy 2-3%
Progressive supranuclear palsy 1-2%
Vascular parkinsonism 3%
MPTP, CO, Mn, recurrent head trauma rare
Postencephalitic parkinsonism None since 1960’s
Note: does not include essential tremor or dementia with motor
signs or other forms of parkinsonism
Non-Motor Symptoms of PD
� Depression or anxiety (50% of patients)
� Sleep disturbance
� Insomnia
� REM sleep beh d/o
� Poor handwriting
� Loss of smell
� Drooling
� Constipation
� Seborrheic dermatitis
� Dizziness
� Orthostatic BP
� Bladder dysfunction
� Sexual dysfunction
� Weight loss
� Dysphagia
� Dementia
Associated Problems - Late
� Dementia
� Dysphagia
� Weight loss
� Autonomic dysfunction
� Dizziness, typically from orthostasis
� Sweating
� Sexual dysfunction
� Dystonia
� Drooling
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Red Flags It May Not Be PD
� No tremor
� Symmetric findings
� Poor response to
medications
� Early falls or postural
instability
� Prominent dystonia or
ataxia
� Early dementia or
hallucinations
� Apraxia
� Early severe autonomic
dysfunction
� Gaze difficulty (especially
looking down)
� Upper motor neuron signs
Some Causes of “Parkinsonism”
� Drugs
� Anti-psychotics
� Anti-nausea
� Illicit drugs
� Vascular
� Progressive Supranuclear Palsy
� Multi-System Atrophy
� Shy-Drager
� Striato-nigral degen.
� OPCA
� CBGD
� Dementia with Lewy Bodies
� Normal Pressure Hydrocephalus
� Wilson’s disease
� Huntington’s disease
� Alzheimer’s Disease
� Dystonia
� Structural lesions
Drug Induced Parkinsonism
� Neuroleptics
� Any, except Clozapine
� Includes Zypreza, Risperdal
� Quetiepine is generally acceptable
� Keep in mind antidopaminergics for other uses
� Thorazine for hiccups
� Metoclopramide (Reglan)
� Reserpine for hypertension
� Tetrabenazine
� Illicit drugs
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Vascular Parkinsonism
� Criteria:
� Stepwise progression, risk factors for vascular disease, subacute onset, at least 2 infarcts in basal ganglia or widespread white matter abnormality.
� Typically worse in legs
� “lower body” parkinsonism
� Gait disturbance, freezing, wide base, gait apraxia
� Similar to Binswanger or NPH
� Typically no rest tremor or signs in arms
� May partially respond to levodopa
Progressive Supranuclear Palsy
� Onset after age 45
� Postural instability and falls
� Dysarthria
� Supranuclear gaze palsy
� Bilateral parkinsonism
� Predominantly akinetic-rigid
� Absence of benefit with levodopa
� Typically progresses faster that PD
� Dx to death in 9 years
� Avg time to dx is 5 years!
Multiple Systems Atrophy (MSA)
� Parkinsonism with:
� Autonomic dysfunction
� Shy-Drager Syndrome
� Cerebellar dysfunction
� Olivopontocerebellar atrophy (OPCA)
� Striatonigral degeneration (SND)
� Can be most difficult to distinguish from IPD
� Upper motor neuron dysfunction
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Cortical Basilar Ganglionic
Degeneration (CBGD)
� Onset after 45
� Asymmetric movement disorder
� Parkinsonism
� Dystonia
� Myoclonus
� Cognitive Symptoms
� Aphasia
� Ideomotor apraxia
� Alien limb
� Cortical Sensory Deficits
� No delusion or hallucination unrelated to levodopa
� Many features overlap with PSP
Alzheimer’s Disease with
Parkinsonism
Scarmeas, Neurology 9/2004
Alzheimer’s Disease with
Parkinsonism
Scarmeas, Neurology 9/2004
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Dementia with Lewy Bodies (DLB)
� Parkinsonism
� Hallucinations and dementia within one year of motor
findings
� Fluctuating cognition, attention and alertness
� Poor response to pro-dopaminergic medications
� Poor response to anti-psychotics, especially neuroleptics
Clinical Staging:
Modified Hoehn and Yahr
Stage Definition
0 No signs of disease
1 Unilateral disease (minimal functional impairment)
1.5 Unilateral plus axial involvement
2 Bilateral disease, without impairment of balance
2.5 Mild bilateral disease with recovery on pull test
3 Mild to moderate bilateral disease; some postural instability;
physically independent
4 Severe disability; still able to walk or stand unassisted
5 Wheelchair bound or bedridden unless aided
Hoehn & Yahr. Neurology. 1967;17:427-442.
Mortality in Parkinsonism
� Reduced life expectancy
� 1.5-3 times mortality rate compared to age-matched
controls
� Longer in non-demented PD cases
� Longer with L-dopa use
� PD survival > MSA, PSP
� The most common causes of death:
� pulmonary infection/aspiration, urinary tract infection,
pulmonary embolism and complications of falls, fractures
and immobility
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Treatments
� Prevention of progression
� Symptomatic - medical
� Symptomatic - surgical
� Non-pharmacologic
� Restorative
� Complementary or alternative
ALS
What is ALS ?
� A Motor Neuron Disease which affects the nerve path to
voluntary muscles resulting in progressive muscle
weakness
� Amyotrophic Lateral Sclerosis
� A myotrophic (literally without - muscle - nourishment)
� L ateral (refers to position of damage in the spinal cord)
� S clerosis (scarring or hardening)
� Identified over one hundred years ago. Called “Lou
Gehrig’s Disease” in the US
ALSA-MA Chapter © 2009
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ALS
� Progressive weakness, ultimately involving all limbs,
trunk and respiratory muscles
� Eye and sphincter control involved much later in disease
course
� Other symptoms include sialorrhea, muscle spasms,
spasticity, pain & fatigue
� Incidence 1-2 cases per 100,000
� Prevalence 6-8 per 100,000
ALSA-MA Chapter © 2009
What Causes ALS ?
No Known Cause: may be multi-factorial
� Environmental (viral or toxin)
� Genetic pre disposition
No known Cure
� One approved drug: Rilutek may slow down symptoms
Types of ALS
� Approximately 10% are “familial” or genetic.
� These families may face fear of “who will be next” or over-
burdened with care-giving.
� 90% are “sporadic” or cause unknown.
� These families are often blindsided, may not even have
heard of ALS. Unknown cause may lead to self-blame.
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Diagnosis and Progression of ALS
� No “test” for sporadic ALS, other diseases must be ruled out for 6 – 12 months.
� EMG has specific criteria, but early in disease course, some patients fit it only vaguely
� Misdiagnosis is common (Lyme disease, minor stroke, MS)
� ALS may progress very rapidly.
� 50% of people die by 3 years into illness
� Loss of breathing ability means a significant choice may need to be made about electing a tracheotomy and ventilator.
� Swallowing lost necessitating decision about PEG
Other Risks in ALS
� Cognitive loss or frank dementia in 30%.
� Higher risk of developing disease amongst veterans.
� Gulf War vets
� Disability
� May be increased in patients with TBI
Medication for ALS
� Riluzole
� May extend life up to 6 months by improving swallowing,
especially earlier in disease course
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Treatment Goals: No Cure
� Symptom Management
� Goal: Maintain Quality of Life
� Multi- disciplinary Approach
� Respiration
� Speech and Swallow
� Nutrition
� Secretions
� Augmentative Communication
� Mobility
� Assistive Technology
� Mental Health – pseudobulbar affect
� Pain Management
� Fatigue
ALSA-MA Chapter © 2009
Huntington Disease
Epidemiology
� 30,000 people in U.S. estimated to have disease
� ~300 new cases per year
� 150,000-200,000 at risk individuals
� Men and women equally affected
� Average age of onset is 39
� Range 2-90 years
� Majority have documented (or suggestive) family history
� 5-10% have juvenile HD (onset <20)
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Genetic Disease
� Each child of effected parent has 50/50 chance of inheriting disease
� Does not skip generations
� If you do not inherit the gene, you cannot pass it to the next generation
� Symptoms progress over years and begin insidiously
� The disease progresses and inevitably leads to death.
Trio of Disorders
� Movement Disorder
� Cognitive Disorder
� Psychiatric Disorder
Movement Disorder
� Involuntary movements� Chorea
� Dystonia
� Akathisia (inner feeling of restlessness)
� Inability to control voluntary movements� Balance
� Coordination
� Motor impersistance
� Grasp
� Modulation of the force of movement
� Slurred or loss of regularity of speech
� Difficulty swallowing
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Speech/Communication
� Muscles weaken
� Speech becomes difficult to understand
� Will become non-verbal but need to communicate remains
Swallowing
� Swallowing will deteriorate
� Higher risk for aspiration
� Look at diet, positioning, exercise, drinking behavior
� SLOW DOWN
� Minimize distractions
� Early discussion of G-tube preference
Cognitive & Behavioral Disorder
� Most challenging for caregivers
� Memory/Learning
� Organization
� Lack of initiation/apathy
� Delay in processing information (bradyphrenia)
� Perseveration
� Impaired judgment
� Impulse control
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Psychiatric Manifestations
� Depression & Anxiety (>50%)
� Apathy (48%)� May be secondary to depression or neuroleptic use
� Psychosis (12%)� Delusions > hallucinations
� Obsessive Compulsive Disorder
� Mania (10%)� Irritable or elevated mood
� Some meet criteria for bipolar disorder
� Sleeping Disorders
� Delirium
Advanced Disease
� Stiffness and slowness of movement
� More prominent than chorea
� Dystonia (Abnormal fixed postures)
� Weight loss
� Dementia
� Rate of progression varies, but disease range is reported to be
10-25 years
� Mean duration 19 years
Causes of Death
� Causes of death related to loss of functions:
� Pneumonia
� Aspiration
� nutritional deficiencies
� chronic skin ulcers
� Suicide risk
� ~ 25% attempt at least once
� 8-9% of all deaths due to completed suicide
� Risk factors include childlessness, depression, single, living alone, other suicides in family
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Treatments - Symptomatic
� Movement disorders
� Psychiatric treatments
� Non-pharmacologic interventions
� Always consider removing exacerbating factors
� Medications
� Environment
� Emotional triggers
Treatments – Non-pharmacologic
� Caregiver/nursing ‘routine’ care
� Social work
� Nutrition
� Social support
� Occupational therapy
� Physical therapy
� Speech therapy
Issues in Advanced Disease
� Defining “advanced”
� Tracking progression of disease
� Issues in management
� Dignity in late stage
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Rate of Progression
� Early in disease, loss of TFC is 0.5-1 point per year until
total is 5-6.
� Average TFC of a patient admitted to an extended care
facility is about 4.
� Later in HD – not well studied
� Patients may spend 5-10 years or longer under near total
to total care
Average Characteristics of
Patients in Extended Care
� Nance, 1996� 45 years old
� Disease duration = 10.0 years
� 47% women
� Previously employed
� High school graduate
� Unmarried (44% never, 33% divorced)
�Wheelock, 2003� 52 years old
� Disease duration = 8.6 years
� 63% women
Nance, 1996
Medical Issues in Advanced HD
� Behavioral disturbance
� Movements
� Including falls
� Dementia
� Delusions & other forms of psychosis
� Swallowing & Nutrition
� Weight loss/gain
� Mood & anxiety
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Everyday Issues in Advanced HD
� Medication use (and when to stop)
� How to assess pain and anxiety in the non-verbal patient
� Preventing purposeful physical violence
� Falls, falls, falls, falls, falls, . . .
� Weight in the patients who no longer walk
� How to keep patients entertained and out of trouble
Everyday Issues in Advanced HD
� Posture
� Communication with patients
� Communication devices
� Non-verbal behavior
� Screaming
� Eating/feeding with swallowing issues and time
� End of life management
� Hyperthermia and other signs of imminent death
� Pain
� Brain donation
Small Interventions Can Make Large
Differences
� Prepare for advancing disease
� CPR, feeding tubes, hospitalization, etc.
� Revisit use of medications
� Positioning
� Routine/predictability
� Adjunctive therapies such as speech & language,
music, recreational, and art therapies may improve
quality of life
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Medications Can Be Used in
Advanced HD for:
� Chorea
� Neuroleptics, tetrabenazine, amantadine, clonazepam
� Mood
� Anti-depressants, mood stabilizing agents
� Weight loss
� Megace, cannabinoids, olanzapine, low dose
mirtazipine
� Behavioral dyscontrol
� Anything that can get into the patient and sedates
Long on Information, Short on Time
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Family and caregiver
support Karen Baker, LICSW MSW
Stressors for the family with Huntington’s
Disease
� HD is a genetic disease
� Several family members can be disabled or dependent
within one family, leaving the caregivers outnumbered
by the “patients”
� Family may still be actively grieving deaths of other
relatives
� Family may be angry at previous generation for not
disclosing genetic risk
Stressors for the family with HD
� HD’s cognitive deterioration makes it very challenging
for one to succeed in school, finish college or training
programs, receive promotions. Many people with HD
are out of work, applying for disability benefits.
Caregivers face a loss of income while taking time from
work to provide care. Imagine the financial toll to a
family over several generations.
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Stressors for the family with HD
� A person with HD has passed on a 50/50 chance that
biological offspring will inherit the gene. Families live
in great fear of children inheriting the disease.
� Genetic testing has been available since 1994 but raises
several ethical issues. Much consideration should be
taken prior to testing, ideally with the support of a
genetic counselor. Families may strongly disagree with
pre-symptomatic testing.
Stressors for the family with HD
� Risk of suicide needs to be addressed for those testing
positive. Counseling and evaluation for anti-depressant
medication is strongly advised.
� Financial planning should be considered. Once a person
tests positive, there is a risk of not being able to
purchase disability, long term care, or life insurance.
� Relatives may argue over whether to test or not,
causing additional family conflict. An individual may
test in secret, not feel comfortable sharing results with
family, especially if the parent does not wish to test or
admit the diagnosis.
� People at risk feel “under a microscope” as relatives are
looking for symptomatic behavior
Stressors for the family with HD
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Stressors for the family with HD
� Opportunity to finalize advanced directives can be lost
by cognitive impairment
� Fear of discrimination leaves at-risk individuals private,
secretive, afraid to even attend an HD support group
� Families live in great fear of who may show signs of HD
next
Stressors for the family with ALS
� Two types of ALS:
� Approximately 10% are “familial” or genetic. These
families may face fear of “who will be next” or over-
burdened with care-giving.
� 90% are “sporadic” or cause unknown. These families
are often blindsided, may not even have heard of ALS.
Unknown cause may lead to self-blame.
Stressors to the family with ALS
� No “test” for sporadic ALS, other diseases must be ruled
out for 6 – 12 months.
� Misdiagnosis is common (Lyme disease, minor stroke),
can lead to anger or mistrust towards medical
community.
� ALS may progress very rapidly.
� Loss of breathing ability means a significant choice may
need to be made about electing a tracheotomy and
ventilator.
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Stressors to the family with ALS
� Cognitive loss is a risk (10 – 25%).
� Studies have shown a higher risk amongst Veterans.
Stressors to the family with PD
� Long course of PD can lead to care-giver exhaustion.
Common stressors to all families
� “There is no cure” may not be heard by the individual
or family due to lack of diagnosis, denial, or cognitive
impairment.
� Participation in clinical research trials to find a cure is
encouraged, but can lead to ethical issues. Participants
may believe they are receiving a “cure”, but can be
disappointed when study ends. Participants may use
research to avoid making decisions.
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Stressors to all families
� “Envy” towards other diseases with a cure or more
support or media coverage
� Cognitive and speech loss limits the window of
opportunity to address advanced directives.
Suggestions to utilize disease-specific
non-profit agencies
� Several non-profit agencies can offer support to families
and professional caregivers:
� Educational materials (printed books, conferences,
webinars)
� Training to professional caregivers
� Support Groups
� Assistance with referrals to specialists
� Fund-raising and advocacy events
Suggested non-profits
� Huntington’s Disease Society of America
� www.hdsa.org
� ALS Association
� www.als.org
� American Parkinson Disease Association
� www.apdaparkinson.org
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Questions and further information
� Karen Baker LICSW MSW
kbaker@hdsa.org
� Samuel Frank, MD
samfrank@bu.edu
Course Evaluation & Post-Test
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Education Network.
To conclude this course and to obtain a certificate of
completion, you must finish the evaluation and post-
test.
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