Dimentia and Alziemers

Question 0

Steps in Evaluating Patient with Dementia

Answer 0

• General medical history
• General neurological history
• Neurobehavioural history
• psychiatric history
• toxic, nutritional and drug history
• Family history
• Objective exam (physical, neurological, neuropsychotic)

Question 1

Dimentia

Answer 1

Global deterioration of intellectual function in the face of unimpaired consciousness.

Question 2

Bedside Tests

Answer 2

Mini-mental statues examination (MMSE) and Montreal objective cognitive Assessment (MOCA).
Domains of cognition that are tested include:
-levels of consciousness
-orientation: time, place, person
-Memory: REmote, recent, immediate (3 object recall)
-Attention and Concetration: serial 7s and digit span
-Knowledge, insight
-Language: fluency, comprehension, repitition, object naming, tests for apraxia, reading and writing

Question 3

Parietal Cortex

Answer 3

1. Pout Reflex
2. Glabellar Reflex
3. Grasp Reflex
4. Palmo-mental reflex

Question 4

Two Ways to Think of Dementias

Answer 4

1. Part of the brain that is mostly affected.eg. Frontal (anterior) vs. Parietal (posterior) lobes/cortex
2. Rapidity of progression of dementias

Question 5

Anterior

Answer 5

Behavioural changes
loss of inhibition
antisocial bheaviour
facile and irresponsible. 
Frontotemporal dementia (Picks)
Huntingtons

Question 6

Posterior

Answer 6

Parietal and temporal lobes

Disturbances of cognitive function (memory and language) without marked changes in behaviour

Question 7

Causes of Dementia

Answer 7

1. Degenerative:alzhiemers, lewy bodies, taupalathics, Huntingtons, parkinsons, wilsons
2. CV: vascular dimentia, CNA vasculins
3. Structural: hydrocephalus, tumour, head injury, sub dural hematoma
4. Infections: Creutzfeld-jacob, neurosyphilis, HIV, Herpes simplex, Encephalitis
5. Toxic/metabollic: drugs, alcohol, toxins, vitamin deficiencies, hypothyroidism, urethramia and dialysis related, hepatic encephalopathy
6. Immune Disorders and cancer-lupus, paraneoplatic disease
7. Depression

Question 8

Lab Investigations

Answer 8

CBC, ESR, thyroid function tests, B12, folate, serum electrolytes, glucose, BUN, creatine, calcium, liver function tests, toxicity scan, RPR for syphilis, HIV serology
optional neuroimaging, CT/MRI, EEG and CSF

Question 9

Common Themes

Answer 9

Age dependent progression and worsening of dementia reflecting the loss of neurons and connections in CNS
some parts of the brain and neurons in these regions are more vulnerable to the insult (whatever it happens to be).
Proteins, that are misfolded and abnormally deposited in specific brain areas seem to play a key role in these diseases
For the most, precise causes are unknown and hence no difinitve treatment to stop progression

Question 10

Alziehmers

Answer 10

An irreversible, progressive brain disease that slowly destroys memory and thinking skills.
Although the risk of developing AD increases with age, symptoms usually appear after 60. It is not a normal part of agiing. It is caused by a fatal disease that affects the brain.
Dirupts neuron communication, metabolism and repair.

Question 11

Alziehmer symptoms

Answer 11

Impairement of memory and attention, language and communication, abstract thinking, judgement, personality changes, depression and visuo-spatial disorientation.

Question 12

Alzhiemer signs

Answer 12

motor and gait disturbances, poverty of movement and slowness
falls
problems of bladder and bowel control, seizures

Question 13

Alziehmer Etiology

Answer 13

Familial forms-less than 10% of cases
sporadic is greater than 90%
Early onset-40s and 50s. Familial and associated with susceptibility genes that include mutations of amyloid precursor protein (APP), presenilin 1 and 2 mutations
Late onset-60s or later. Mostly sporadic, apolipoprotein E4 gene, sortilin (SORL1), clusterin (CLU), complement receptors 1 (CRI), TREM2

Question 14

Neuropathology

Answer 14

Coritcal atrophy, synaptic and neuronal loss
neurofibrillarly tangles (NFTs) with paired helical filaments, abnormally hyperphophorylated forms of microtubules-associated protein, tau
Neurtic plaques with amyloid core
amyloid angiopathy
Beta amyloid plaques-dence depsits of protein and cellular material that accumulate outside and around nerve cells
Neurofibrillary tangles-twisted fibres built up in the nerve cell
Temporal cortex, hippocampus, amygdala

Question 15

Amyloid Presursor Protein

Answer 15

Membrane protein that sits in the membrane and extends outward. Neuronal growth survival and repair
Enzymes cut of fragments of this (they go cray) creating B-amyloids which are sticky forms of plaque.

Question 16

NT Abnormalities-Alziehmers

Answer 16

Decreased chamical messengers in brain especially AcH (cortex and hippocampus). Nucelin in brain stem-parietal, frontal and hippocmapus via AcH

Question 17

Cholinergic Agents (Cholinerase inhibitors)

Answer 17

Donepezil (Aricept)
Rivastigmine (exelon)
Galantamine (reminyl)
Tacrine (USA only)

Question 18

Non-Cholinergic Agents

Answer 18

-memantine, a gluatmate receptor modulator
Amyloid vaccine, secretase inhibitor, anti-amyloid agents, drugs that lower cholesterol.
Unproven treatments-estrogens, NSAIDs, vasodilators, propentofylline
Nondrug appreoactes-increase education, intellectual stimulation, excersize, diet, red wine.
PET Scan

Question 19

Dementia with Lewy Bodies

Answer 19

Fluctuating cognition with pronounced variation in attention and alterness
recurrent visual hallucinations
Parkinsonian features (rigidity or stiffness in muscles, slowness and poverty of movements, tremor)
Treatment with cholinesterase inhibitors, antipsychotic drugs (to control behavioural problems and agitation)
Loss of pigmented (dopamine-containing) neurons in the substantial nigra in parkinsons and dementia with lewy bodies.

Question 20

Frontotemporal Dementia (Picks)

Answer 20

Female preponderance and at younger age than AD.
Focal frontal and temporal atrophy
disinhibition, apathy, perservation, mental regidity and affective symptoms.
Tau and gene on chromosome 17
Neurodegerative condition: ALS or Lou Gehrig’s
No curative treatment
Pick bodies contain tau protein

Question 21

Vascular Dementia

Answer 21

10-15%
Stroke by stroke-progressive focal loss of function
hypertension, diabetes, increased cholesterol, smoking
may be concurrently with neurodegerative dementia
CT scan may show multiple areas of cerebral infarction
Treatment of hypertension and other vascular risk factors.