Dementia Unizar

DEMENTIA

I Wayan Tunjung, dr.Sp.S.

Bagian NeurologiRSU Kota Mataram

DEFINISI SINDROME PENURUNAN KEMAMPUAN

INTELEKTUAL PROGRESIF YANG MENYEBABKAN DETERIORASI KOGNITIF DAN FUNGSIONAL SEHINGGA MENGAKIBATKAN GANGGUAN FUNGSI SOSIAL, PEKERJAAN DAN AKTIFITAS SEHARI-HARI.

MODALITAS FUNGSI KOGNITIF:1. ATENSI2. BAHASA3. MEMORI4. VISUOSPATIAL5. EKSEKUTIF

MODALITAS BERBAHASA:1. BERBICARA SPONTAN2. PEMAHAMAN3. PENGULANGAN4. PENAMAAN5. MEMBACA6. MENULIS

MEMORI SECARA KLINIK DIBAGI MENJADI:

1. IMMEDIATE MEMORI: RECALL DALAM HITUNGAN DETIK.

2. RECENT MEMORI: RECALL DALAM MENIT-TAHUN.

3. REMOTE MEMORI: RECALL BERTAHUN- TAHUN

Alzheimer’sDisease

•Early onset•Normal onset

Vascular (Multi-infarct)

Dementia

Lewy Body

Dementia

DEMENTIA

Other Dementias•Metabolic•Drugs/toxic•White matter disease•Mass effects•Depression•Infections•Parkinson’s

Fronto-Temporal

Lobe Dementia

s

Types of Dementia1.Cortical DementiaDisorder affecting the cerebral cortex.Exs.: Alzheimer’s and Creutzfeldt-Jakob dementia. Memory and language difficulties (Aphasia) most pronounced symptoms.Aphasia is the inability to recall words and understand common language.

2. Subcortical Dementia:Dysfunction in parts of the brain that are beneath the cortex.

Memory loss & language difficulties not present or less severe than cortical.

Exs.: Huntington’s disease and AIDS dementia complex.

Changes in their personality and attention span.

Thinking slows down.

Dementia - IncidenceSuspected that as many as 50% of people over the age of 80 develop Alzheimer’s.

5%-8% of all people over 65 have some form of dementia; number doubles every 5 years beyond that age.

Alzheimer’s causes 50%-70% of all dementia.

About 20%-30% of all dementia is believed to be caused by a vascular dysfunction (most common is multi-infarct disease).

In 2010, 57.7% of people with dementia live in low and middle income countries.

By 2050, this will rise > 70.5%.

Ferri CP et al. Lancet 2005; 366: 2112 - 2117

SO… What is Dementia? It is NOT part of normal aging! It is a disease!

It is more than just forgetfulness - which is part of normal aging

Dementia Chronic progressive disorder Deterioration in multiple aspects of cognitive function

Associated with behavioural & psychological symptoms

Severe impact on quality of life Longest duration of burden on patient, family & society

Dementia - Early StageBegins with forgetfulness - isolated incidents of memory loss do not constitute dementia.

Forgetfulness progresses to confusion and eventually disorientation.

Problem solvingJudgmentDecision makingOrienting to space and time

Personality changes - irritable, agitated, sadness (depression), manic episodes

Aging Changes in Cognition… Normal aging changes = more forgetful & slower to

learn

MCI – Mild Cognitive Impairment = 1 problem area Immediate recall, word finding & complex

problem solving problems (½ of these folks will develop dementia in 5 yrs)

Dementia = Chronic thinking problems in > 2 areas Delirium =Rapid changes in thinking & alertness

(seek medical help immediately ) Depression = chronic unless treated, poor quality , I

“don’t know”, “I just can’t” responses, no pleasurecan look like agitation & confusion

Causes of dementia Primary neurodegenerative diseases: Alzheimer’s, vascular disease, fronto-temporal dementia, Lewy body dementia

Secondary: hypothyroidism, CNS infections,vitamin B-12 deficiency, chronic subdural haematoma, tumour, etc.

DementiaReversible:

D = Drugs, DeliriumE = Emotions (depression) and Endocrine Disorders

M = Metabolic DisturbancesE = Eye and Ear ImpairmentsN = Nutritional DisordersT = Tumors, Toxicity, Trauma to HeadI = Infectious DisordersA = Alcohol, Arteriosclerosis

Dementia Irreversible:

Alzheimer’s Lewy Body Dementia Pick’s Disease (Frontotemperal

Dementia) Parkinson’s Heady Injury Huntington’s Disease Jacob-Cruzefeldt Disease

Dementia Irreversible:

Alzheimer's most common type of irreversible dementia

Multi-Infarct dementia second most common type of irreversible dementia Death of cerebral cells Blockages of larger cerebral vessels, arteries More abrupt in onset Associated with previous strokes,

hypertension Can be traced through diagnostic procedures

DementiaLewy Body DementiaEpisodic confusion with intervals of lucidity with at least one of the following:1. Visual or auditory hallucinations2. Mild extrapyramidal symptoms

(muscle rigidity, slow movements).3. Repeated unexplained falls

Progresses to severe dementia—found at autopsy.

Dementia - DiagnosisImportant to establish the cause of the dementia - Alzheimer’s and dementia are not the same thing.

A differential diagnosis compares the symptoms of two or more diseases.

DD is important because some forms of dementia are “treatable”. Chronic drug abuse, Normal Pressure Hydrocephalus, Chronic subdural Hematoma, Benign Brain Tumors, Vitamin Deficiency, and Hypothyroidism.

Dementia - Diagnosis1. Medical History - Physician wants to determine the onset of symptoms and how they’ve changed over time.Determine risk factors for infection, family history of dementia or other neurological disease, alcohol and drug use, and a patient’s history of strokes.

Dementia - Diagnosis2. Neuropsychological Exam - Evaluates a person’s cognitive ability, e.g. orientation in time and space, memory, language skills, reasoning ability, attention, and social appropriateness.Tests involve asking a person to repeat sentences, name objects, etc.Someone with Alzheimer’s is usually cooperative, attentive, and appropriate but has poor memory.Someone with hydrocephalus is likely to be distracted and less cooperative.

Dementia - Diagnosis3. Brain Imaging/Lab Tests - CT or MRI, cerebrospinal fluid (all used to confirm a diagnosis or eliminate various possibilities)4. Blood tests - used to diagnosis neurosyphilis.5. Metabolic tests - determine treatable disorders such as a vitamin B12 deficiency6. EEG (electroencephalography) is used to diagnose Creutzfeldt-Jakob disease.

1. DEMENTIA ALZHEIMER

Where did we start?•Alzheimer’s first diagnosed in 1907 •OBS – organic brain syndrome - common term 60’s

•Psychiatric illness – mentally ill – 60’s-80’s•De-institutionalization - nursing homes – 70’s-80’s

•Little could be done once diagnosed – until the 90’s

•Families - ‘do the best you can’ – 60’s – 90’s.•Diagnosis of Alzheimer’s on autopsy only – till 90’s

Normal Brain Cells

Neurotransmitters (AChE)– being sent – message being

communicated to the next cell

Normal Brain Cells

Once the message is sent, then enzymes lock onto the messenger chemicals and take them out of circulation so a new message can be sent

What happens with Alzheimer’s Disease?Two processesCells are shrinking & dyingCells are producing less chemical to send messages

plaques tangles

Less neurotransmit

ter Further to go to get to the next cell

Enzymes (AChE inhibitors) – get to them BEFORE they

deliver their message

Brain Cells with Alzheimer’s

Alzheimer-type pathology

Silver stained plaques and tangles

• Thick arrow: senile (neuritic) plaque• Small arrow: diffuse plaque• Star: tangle

AD Pathology Amyloid plaques (Ab)

Amyloid Hypothesis

Secretase

Secretase

A

AggregatedA

APPs

N C

Cellproliferation

Calciumregulation

Membrane

KPI

Secretase Secretase

717670, 671APP

Reduced Ca++

NeuroprotectionNeuroplasticity

Increased Ca++

NeurotoxicityAbnormal outgrowth

Pathogenesis of amyloidosis in AD

AD: Risk Factors IEstablished•Age•Family history•Down’s syndrome•Apolipoprotein e4 allele•Autosomal dominant mutations: amyloid precursor protein gene (APP) chr 21, presenilin-1 gene chr 14, presenilin-2 gene chr 1. (<2% cases).

AD: Risk Factors IIProbable:

•Depression•Hypertension•Head injury•Homocysteine

AD: Risk Factors IIIPossible:

•Gender (F>M)•Education / neuro-cognitive reserve•Diabetes•Smoking•Cholesterol•Herpes simplex virus-I?

Possible protective factors Anti-inflammatory drugs Estrogen Apolipoprotein e2 allele High neurocognitive reserve &

cognitively stimulating activities Cholesterol lowering drugs (statins) Alcohol

AD & vascular disease

Diagnosing ADDefinite AD - Histopathological evidence (requires autopsy)

- Course and examination characteristic of ADProbable AD - Deficits in > 2 areas of cognition

- Onset 40-90 (usually > 65); progressive course

- Other causes excluded Possible AD - Deficit in only 1 area of cognition

- Atypical course - Other dementia causes present

Unlikely AD - Sudden onset- Focal signs- Seizures or gait disturbance early in

course

AD - SymptomsLoss of MemoryAphasiaApraxia - (decreased ability to perform physical tasks such as dressing, eating, ADL’s

DelusionsEasily lost and confusedInability to learn new tasksLoss of judgment and reasonLoss of inhibitions and belligerenceSocial WithdrawalVisual hallucinations

AD Early StageCharacteristicsBegins with forgetfulness

Progresses to disorientation and confusion

Personality changes

Symptoms of depression/manic behaviors

InterventionsMedications - Aricept and Cognex (both are commercial names).

Both increase acetylcholine (Ach) in the brain by inhibiting the enzyme that breaks it down.

Therapy (deal with depression that often accompanies diagnosis

Counseling with family

AD - Early StageMusic TherapyUsed to relieve depressionCoupled with exercise and relaxation techniques

Increase or maintain social relationships (dancing, improvisation)

Maintain positive activities (church choir, Handbell choir, Senior social dances, etc.)

AD - Middle StageCharacteristicsNeed assistance with ADLs

Unable to remember names

Loss of short-term recall

May display anxious, agitated, delusional, or obsessive behavior

May be physically or verbally aggressive

Poor personal hygiene

Disturbed sleepInability to carry on a conversation

May use “word salad” (sentence fragments)

Posture may be altered

Disoriented to time and place

May ask questions repeatedly

AD - Middle StageInterventionsValidation Therapy

Structured Areas for Mobility

Positive, nurturing, loving environment

Music TherapyProvides avenue for social interaction (Instrumental Improvisation; TGS, Guided Music Listening)

Provides a medium for verbal/non-verbal expression (TGS)

Can help maintain cognitive and affective functioning

AD - Middle StageMT (cont’d)Music associated with positive memories will evoke a positive response.

Use client preferred music

Music from late teens through early 30’s

Lower keys (F3 to C5 for women ~ one octave lower for men

Only use sheet music when helpful ~ might be a distraction

Dancing allows for intimacy between spouses

Mallet in dominant hand, drum in non-dominant hand so one can play independently

*Careful - some may react to loud noises adversely

AD - Late StageCharacteristicsLoss of verbal articulation

Loss of ambulationBowel and bladder incontinence

Extended sleep patterns

Unresponsive to most stimuli

InterventionsCaring for physical needs

Maintain integrity of the skin

Medical interventions

Most activities are inaccessible

AD - Late StageMusic TherapyTape by bedsideGentle singing by therapist ~ one-sided, client will not participate

Can provide some connection between patient and family members through singing

Use a calm voiceUtilize touch: holding hands, hugging, rocking, hand on shoulder, etc.

Alzheimer’s drugs provide FAKE messenger

chemicals that distract the enzymes. They attach

to the Fake AChE & the message can get thru

What do Alzheimer’s drugs DO?

Donepezil, Rivastigmin, Galantamine (Aricept, Exelon, Reminyl (Razadyne)

2. DEMENTIA VASCULAR

Vascular DementiaNerve cells are OKBlood supply is damagedNo oxygen gets to the cellNo nutrients get to the cell

Then … the nerve cells die

Vascular DamageHealthy cell with

oxygen and nourishment

Dead nerve cell - no blood supply

No message

Training to care for people with dementia

What is Vascular Dementia?Second most common form of dementia after Alzheimer’s

disease.Occurs when the blood supply to the brain is interrupted by

a blocked or diseased vascular system.

Training to care for

people with dementia

A Little Bit of HistoryArteriosclerosis and senile dementia described as different syndromes as early as 1899.

Mayer-Gross et al in 1969 reported hypertension to be the cause in 50% of patients.

Hachinski in 1974 used the term multi-infarct dementia.

In 1985 the term vascular dementia was used by Loeb.

Training to care for

people with dementiaDifferent Types 0f Vascular Dementia

1. Mild vascular cognitive impairment2. Multi-infarct dementia3. Vascular dementia due to a strategic single infarct.4. Vascular dementia due to lacunar lesions5. Vascular dementia due to haemorrhagic lesions6. Binswanger disease7. Subcortical vascular dementia8. Mixed dementia (combination of AD and

Vascular)

Training to care for

people with dementia

Multi-infarct dementiaMost common formCaused by a series of small strokes or TIA.Damage caused to the cortex of the brainArea associated with learning, memory and language.

Can be temporary but over time with repeated incidents become permanent

Symptoms include severe depression, mood swings and epilepsy

Training to care for

people with dementiaBinswanger’s Disease

(or Subcortical vascular dementia)

Associated with stroke-related changes.Damage to tiny blood vessels of the white matter, deep within the brain.

Symptoms develop more gradually and include

Slowness, lethargy, difficulty walking, emotional ups and downs, lack of bladder control.

Signs and Symptoms of Vascular Dementia

Physical signs/symptomsMemory problems, forgetfulnessDizzinessLeg or arm weaknessLack of concentrationMoving with rapid, shuffling stepsLoss of bladder or bowel control

Training to care for

people with dementia

Behavioural signs/symptomsDepressionSlurred speechLanguage problemsAbnormal behaviourWandering/getting lostLaughing/crying inappropriatelyDifficulty following instructionsProblems handling money

Training to care for

people with dementia

Risk Factors that increase risk of developing Vascular Dementia

Training to care for

people with dementiaRisk Factors

High blood pressureSmokingDiabetesHigh CholesterolHistory of mild warning strokesEvidence of arterial disease elsewhereHeart rhythm abnormalitiesLack of physical activityFatty dietGender – Men are at a slightly higher risk.Age – Usually between 60 and 75, incidence increases with age.

Training to care for

people with dementia

GOOD NEWS

Unlike Alzheimer’s Disease, there are ways to prevent and reduce the severity of vascular dementia.

Training to care for

people with dementiaMedical management of:

High blood pressureHigh CholesterolHeart diseaseDiabetesAdministering medication to prevent clots forming eg AspirinDrugs to relieve restlessness or depressionRecent research indicates that cholinesterase inhibitor

medications which are helpful with Alzheimer’s disease may be of benefit in Vascular Dementia eg Aricept, Reminyl.

In some cases surgery such as carotid endarterectomy may be indicated.

Unlike Alzheimer’s Disease, there are ways to prevent and reduce the severity of vascular dementia.

Receiving Rehabilitative Support:

PhysiotherapyOccupational TherapySpeech Therapy To help the person regain lost functions.

A Healthier Lifestyle:

•A healthy diet•Regular Exercise•Stop smoking•Moderate intake of alcohol

Training to care for

people with dementiaProgression of Vascular Dementia

Onset gradual or dramatic

StabiliseTIA/Stroke

StabiliseTIA/Stroke

Typically Vascular Dementia progresses gradually in a stepwise fashion in which

a person’s abilities deteriorate after a stroke, then stabilise until the next stroke.

TERIMA KASIH…