Case 5: Joe + Maria Russo - Dementia

Dementia: Types

Alzheimer

Parkinson

Huntington

Frontotemporal

Lewy bodies

Vascular

Alzheimer Dementia: Pathophysiology

Neurofibrillary tau protein tangles + beta-amyloid plaque deposition = Neurotoxic = Neuron damage + cognitive impairment

Parkinson Dementia: Pathophysiology

Misfolded alpha-synuclein protein aggregation in brainstem, substantia nigra, cortex = Neurotoxic + Neuroinflammation = Neuron damage + cognitive impairment

Motor Symptoms: Dopaminergic neuron degeneration in substantia nigra (basal ganglia) + locus coeruleus (pons) = Dopamine deficiency = Interrupt thalamus → Motor cortex transmission

Huntington Dementia: Pathophysiology

Increased CAG repeats on huntingtin gene (HTT) = Long protein production = Neurotoxic = Damage caudate nucleus + decrease Ach and GABA = Brain atrophy + cognitive decline

Frontotemporal Dementia: Pathophysiology

Pick body (intracellular inclusion body) + ubiquitin aggregation = Neurotoxic = Frontal + temporal lobe atrophy = Cognitive impairment

• Pick Body: Tau proteins + progranulin protein (cell growth regulator) 

• Ubiquitin: Regulatory protein marker for degradation

Lewy Body Dementia: Pathophysiology

Abnormal alpha-synuclein protein + hyaline aggregation in neurons = Neurotoxic = Neuronal degeneration + cognitive impairment

Vascular Dementia: Pathophysiology

Cerebrovascular damage = Neuronal death = Cognitive impairment

• Stroke

• Cerebral small vessel disease

• Microinfarcts

Dementia: Etiologies

DEMENTIA

• Drugs:

  • Anticholinergics

  • Sedatives

  • Opioids

• Emotions: MDD

• Metabolic:

  • Hypothyroidism

  • Hyperparathyroidism

  • Electrolyte abnormalities

• Eyes + Ears: Sensory impairment

• Nutritional Deficiencies:

  • B12

  • B1

  • Folate

• Tumors + Trauma

• Infection

• Alcohol + Arteriovascular

Dementia: Progression

Subjective cognitive decline → Mild cognitive impairment (MCI) → Mild dementia → Moderate dementia → Severe dementia → Death

Loss of Function:

1. IADLs

2. ADLs

3. Bodily functions

Dementia: Subjective Cognitive Decline

Cognition: Self-reported memory concerns

Function: Fully independent

Neuropsychiatric Features: Mood + behaviour changes

Dementia: MCI

Cognition: Memory deficits (amnesia)

Function: Independent in IADLs

Neuropsychiatric Features:

• Depression

• Apathy

• Anxiety

• Irritability

Dementia: Mild Dementia

Cognition: Deficits in…

• Language

• Executive function

• Visuospatial

Function: Loss of IADLs

• Finances

• Driving

• Medications

• Appointments

Neuropsychiatric Features:

• Depression

• Apathy

• Agitation

Dementia: Moderate Dementia

Cognition: Increased deficits in working memory + language

Function: Loss of ADLs

• Personal care

• Falls

Neuropsychiatric Features:

• Psychosis

• Wandering

• Aggression

Dementia: Severe Dementia

Cognition: Profound impairment

Function: Incontinent + bedbound

• Dysphagia + aspiration risk

• Loss of speech

Neuropsychiatric Features:

• Agitation

• Apathy

• Insomnia

  • Sleep-wake reversal

Dementia: Investigations

DSM-5

Tests

• Clock-drawing test

• Mini-Cog

• Mini-Mental Status Exam (MMSE)

• Montreal Cognitive Assessment (MoCA)

Blood tests

Urinalysis

Imaging

Dementia Investigation: DSM-5

Diagnostic criteria

Decline in ≥ 1:

• Learning + memory (immediate + short-term)

• Language

  • Object naming

  • Word-finding

  • Fluency

  • Grammer

• Executive function

  • Planning

  • Decision-making

  • Working memory

• Complex attention

• Perceptual-motor

  • Visual perception

  • Coordination

  • Motor planning

• Social cognition

  • Interpretation

  • Insight into social behaviour

Additionally:

• Interference with life

• No other disorder

Dementia Investigation: Clock-Drawing Test

Domains Assessed:

• Complex attention

• Executive function

• Perceptual-motor

Interpretation: Abnormal clock = Further screening required

Dementia Investigation: Mini-Cog

Domains Assessed:

• Complex attention

• Executive function

• Learning + memory

• Perceptual-motor

Interpretation: Cognitive Impairment < 3

Dementia Investigation: MMSE

Domains Assessed:

• Complex attention

• Language

• Learning + memory

• Orientation

Interpretation: Cognitive Impairment < 24

Dementia Investigation: MoCA

Domains Assessed:

• Complex attention

• Executive function

• Language

• Learning + memory

• Perceptual-motor

• Orientation

Interpretation: Cognitive Impairment < 26

Dementia Tests: Driving Safety

Moderate - Severe: Mandatory report

Mild: Road test + Reassess every 6-12 months

Dementia Investigation: Blood Tests

Vit B12: Deficiency

TSH: Hypothyroidism

CBC: Anemia

BMP: Kidney + liver disease, hypo/hyperglycemia

Dementia Investigation: Urinalysis

Infection

Dementia Investigation: Imaging

MRI

• Structural abnormalities

• Atrophy

Dementia: Treatment/Management

Pharmacological

Nonpharmacological

Behavioural + psychological symptom (BPSD) management

Community resources

Dementia Management: Pharmacological

Cholinesterase inhibitors

NMDA-receptor antagonists

Dementia Management: Cholinesterase Inhibitors

MOA: Inhibit AChE = Increase ACh action at neural junctions = Improve neurotransmission

Ex: Donepezil, galantamine, rivastigmine

Indications:

• Alzheimer (mild-moderate)

• Parkinson

• Lewy bodies

Dementia Management: NMDA-Receptor Antagonists

MOA: Inhibit NMDA receptor = Decrease dopamine reuptake in neurons = Improve neurotransmission

Ex: Memantine

Indications:

• Alzheimer (moderate-severe)

Dementia Management: Nonpharmacological

Predictable schedule

Familiar home environment

Dementia Management: BPSD

MDD + anxiety

Agitation/aggresison + psychosis

Sleep disorders

Sundowning

BPSD Management: MDD + Anxiety

Low dose SSRI

• Ex: Escitalopram, citalopram

BPSD Management: Agitation/Aggression + Psychosis

Nonpharmacological deescalation: First-line

Low dose second-gen antipsychotic

• Avoid if possible

• Ex: Risperidone, quetiapine

Citalopram

BPSD Management: Sleep Disorders

Nonpharmacological: First-line

• Sleep hygiene

• Quiet + dark environment

Trazodone

BPSD: Sundowning

Increased neuropsychiatric symptoms in evening

• Pathophysiology: Irregular/reversed sleep-wake cycle

Sundowning: Management

Non-pharmacological: First-line

• Light therapy

• Quiet + dark environment

• Exercise

Pharmacological

• Melatonin

• Trazodone

• Citalopram

Dementia Management: Community Resources

Community-based care

Long-term care

Community-Based Care

Indications:

• Patient prefers living at home

• Supervision + functional needs

• Caregiver burnout

Limitations:

• Limited hours

• Inconsistent availability

• Cost

Ex:

• PSW

• Fall prevention programs

• Adult day programs

• Home care

PSW

Home visits for ADLs, IADLs, + supervision

Adult Day Programs

Supervised programs with activities for patient

Home Care (Ontario Health at Home)

In-home health supports to meet patient needs

• Nursing

• PT/OT

  • Medical care

Long-Term Care

Indications:

• 24h supervision needed

• Severe BPSD/aggression

• Severe caregiver burnout

Limitations:

• Low capacity

• Cost

Ex:

• Retirement home (assisted living facilities)

• Long-term care home (nursing home)

Retirement Home

Residential units for patient socialization + hospitality

• No medical care

Long-Term Care Home

Residential units for patient medical care

• Staffed with nurses + physicians

Caregiver Fatigue: Clinical Presentation

• Emotional/physical exhaustion

• Anger + frustration towards patient

• Emotional detachment from patient

• Concentration difficulties

• Sleeping difficulties 

• Social withdrawal

Caregiver Fatigue: Screening

Zarit Caregiver Burden Interview: Gold standard

• 22, 12, 4, 3-item screening

Burden Scale for Family Caregivers: Self-screening

Caregiver Self-Assessment Questionnaire

PHQ-9

• Depression + anxiety screening

Caregiver Fatigue: Treatment/Management

CBT

Psychoeducation

Respite

Practical needs support

• Financial resources

• Medical equipment/supplies

• Social support