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Dementia syndrome

Dementia syndrome. Definitions. The disturbance and symptoms significantly interfere with work or usual social activities or relationsh i ps with others

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Dementia syndrome

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  1. Dementia syndrome

  2. 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

  3. 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

  4. Causes • 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

  5. 80% 90% The most frequent: • Alzheimer´s disease 50-60% • Vascular dementia • Dementia with Lewy bodies • Frontotemporal dementia

  6. Alzheimer´s disease

  7. 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

  8. 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)

  9. 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)

  10. Risk factors • Lower risk of AD ?? • antioxidants (ginkgo biloba) ? • estrogens ?? • ibuprofen ?? • smoking ????? 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

  11. Macroscopic changes • brain atrophy – temporal lobe, parietal lobe, frontal cortex, cingulate gyrus • enlargement of ventricles

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

  13. 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)

  14. 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

  15. Other factors • Presenilins 1 and 2 – transmembrane proteins • mutations in Alzheimer disease • Allele e4 of apolipoprotein E • 3 alleles – e2, e3, ee4 • e4 – higher risk of atherosclerosis and Alheimer disease

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

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