Dementia syndrome

Definitions

• The disturbance and symptoms significantly interfere with work or usual social activities or relationships with others

• The syndrome associated with a progressive loss of memory and other intellectual functions that is serious enough to interfere with performing the tasks of daily life

•The decline in intellectual function, including difficulties with language, simple calculations, planning and judgment, and motor (muscular movement) skills as well as loss of memory

Signs and symptoms

• loss of cognitive ability – memory– attention– language– problem solving

• disorientation in time, in place, in persons• behavioural problems• emotional changes• depression, anxiety, paranoia

• neurodegenerative diseases– Alzheimer´s disease– Parkinson´s disease

• stroke• trauma• chronic alcohol abuse

– Korsakoff´s syndrome• genetic diseases

– Huntington disease• metabolic diseases

– Tay-Sachs disease– Gaucher disease

• infections– Creutzfedt-Jakob disease – viral encephalitis

Causes

The most frequent:• Alzheimer´s disease 50-60%• Vascular dementia• Dementia with Lewy bodies• Frontotemporal dementia

80%

90%

Alzheimer´s disease

History

1901 - German psychiatrist Alois Alzheimer identified the first case of what became known as Alzheimer's disease in a 50-year-old woman he called Auguste D (A. Deter).

Alzheimer followed her until she died in 1906, when he first reported the case publicly.

Alois Alzheimer

Auguste D

Characteristics

4 stages

• Pre-dementia– mild cognitive difficulties – memory loss - remembering recently learned facts and inability

to acquire new information– subtle problems with attentiveness, planning, flexibility, abstract

thinking • Early dementia

– the increasing impairment of learning and memory – difficulties with language - shrinking vocabulary and decreased

word fluency– executive functions, perception (agnosia), or execution of

movements (apraxia)

• Moderate dementia– inability to perform most common activities of daily living– speech difficulties become evident due to an inability to recall

vocabulary, which leads to frequent incorrect word substitutions– reading and writing skills are also progressively lost– complex motor sequences become less coordinated - risk of

falling increases– memory problems worsen – problems to recognise close

relatives– behavioural changes – irritability, lability, aggression– urinary incontinence

• Advanced dementia– completely dependent upon caregivers– language is reduced to simple phrases or even single words, or

complete loss of speech– extreme apathy and exhaustion– not be able to perform even the most simple tasks without

assistance– immobility, they lose the ability to feed themselves– death (infection, pneumonia)

Risk factors

Higher risk od AD• age

65 – 75 y 3% 75 – 85 y 19% > 85 y 50%

• positive family history

• low degree of education• depression • women > men• trauma of head

Lower risk of AD ??• antioxidants (ginkgo biloba) ?• estrogens ??• ibuprofen ??• smoking ?????

Macroscopic changes

• brain atrophy – temporal lobe, parietal lobe, frontal cortex, cingulate gyrus

• enlargement of ventricles

Microscopic changes• loss of neurons and synapses in the cerebral cortex• amyloid plaques• neurofibrillary tangles

Amyloid plaques (senile plaques)

• dense, mostly insoluble deposits of beta-amyloid peptide outside and around neurons

• beta-amyloid - small peptide (39–43 amino acids) • a fragment from the amyloid precursor protein (APP) – a

transmembrane protein of neurons• APP is critical to neuron growth, survival and post-injury

repair• in Alzheimer's disease, an unknown process (mutation of

APP gene?) causes APP to be divided into smaller fragments by enzymes (proteolysis)

• accumulation of aggregated amyloid fibrils – toxic - disrupting the cell's calcium ion homeostasis, induces apoptosis (hypothesis)

Neurofibrillary tangles

• aggregates of fibres• hyperfosforylated protein tau - accumulate inside the

cells

• normal function of tau protein - stabilizes the microtubules (important for transport in neurons) – microtubule-associated protein

• in AD - tau is hyperphosphorylated – composes pairs with other threads, creating neurofibrillary tangles and disintegrate the neuron's transport system

Other factors

• Presenilins 1 and 2 – transmembrane proteins– mutations in Alzheimer disease

• Allele 4 of apolipoprotein E– 3 alleles – 2, 3, e4– 4 – higher risk of atherosclerosis and Alheimer

disease

CategoryPossible points

Description

Orientation to time

5From broadest to most narrow. Orientation to time has been correlated with future decline.

Orientation to place

5From broadest to most narrow. This is sometimes narrowed down to streets,[and sometimes to floor.[

Registration 3 Repeating named prompts

Attention and calculation

5

Serial sevens, or spelling "world" backwards. It has been suggested that serial sevens may be more appropriate in a population where English is not the first language.

Recall 3 Registration recall

Language 2 Name a pencil and a watch

Repetition 1 Speaking back a phrase

Complex commands

6 Varies. Can involve drawing figure shown.

The mini-mental state examination (MMSE) or Folstein test - brief 30-point questionnaire test - used to screen for cognitive impairment.