Nervous system diseases III

Question 1

What is dementia?

Answer 1

associated with progressive decline of brain functioning that effects quality of life

Question 2

What type of disease is dementia?

Answer 2

a syndrome

Question 3

What is the most common type of dementia?

Answer 3

alzheimers

Question 4

What is alzheimers?

Answer 4

progressive cognitive decline

Question 5

What is an early symptom of Alzheimers?

Answer 5

memory lapse

Question 6

What are mid-stage symptom of Alzheimers?

Answer 6

worsening memory problems

increasing confusion and disorientation

obsessive and repetitive disorientation

obsessive and repetitive behaviours

changes in mood

Question 7

What are more mid-stage symptom of Alzheimers?

Answer 7

sleep disturbance

hallucinations

aphasia

Question 8

What are later symptom of Alzheimers?

Answer 8

weight loss

incontinence

difficulty moving

gradual loss of speech

dysphagia

Question 9

Neuropathology of Alzheimer’s disease

Answer 9

Senile plaques
->Extracellular deposition of β-amyloid peptide

Neurofibrillary tangles
-> Intracellular
-> Phosphorylated tau protein
-> Tau – microtubule associated protein

Neuroinflammation

Massive loss of neurons and synapses in the hippocampus & cortex

Question 10

Alzheimer’s disease
Risk factors

Answer 10

• Age (risk doubles every 5 years after 65 years old)
• Downs syndrome
• Cardiovascular disease
• Head injuries
• Apolipoprotein (APOE) on xsome 19 – APOE ε4

Question 11

Early Onset Dementia

Answer 11

3 gene mutations that affect breakdown of APP

Amyloid precursor protein (APP) on xsome 21

Presenilin 1 (PSEN1) on xsome 14

Presenilin 2 (PSEN2) on xsome 1

Question 12

Brain atrophy in Alzheimer’s disease
Treatment

Answer 12

• No cure
• Acetylcholinesterase inhibitors – increase Ach levels

Question 13

Frontotemporal dementia (FTD) (Pick’s disease)

Answer 13

• Rare form of dementia
• Shrinking of the frontal and temporal lobes of the brain
• Tends to occur at a younger age than AD (40 - 65 years) but also later in life

Question 14

Frontotemporal dementia (FTD) (Pick’s disease) - Causes

Answer 14

Intracellular depositions of abnormal form of tau
protein inside neurons

Degeneration of neurons, microvacuoles formation, and astrocytosis

Question 15

Frontotemporal dementia (FTD) (Pick’s disease)
Symptoms

Answer 15

Dramatic changes in personality
eg. Inappropriate social behaviour

Language disturbance
- Difficulty using and understanding language
- Hesitant speech

Spatial skills and memory remain intact

Question 16

Frontotemporal dementia (FTD) (Pick’s disease)- Treatment

Answer 16

• No cure or specific treatment
• Medications used to manage symptoms
• Antidepressants
• Anti-psychotics
• Therapies

Question 17

Vascular Dementia - Cause

Answer 17

• Reduced blood flow to the brain
• Sub-cortical vascular dementia: narrowing of blood vessels in the brain
• Single-infarct dementia: stroke cuts of blood supply to part of the brain (blood clot)
• Multi-infarct dementia: multiple strokes

Question 18

Vascular Dementia - Symptoms

Answer 18

• Confusion
• Short-term memory problems
• Wandering & getting lost in familiar places
• Losing bladder or bowel control
• Difficulty following instructions

Question 19

Vascular Dementia
Treatment

Answer 19

• No treatment to reverse brain damage
• Prevention of future strokes
• Address risk factors

Question 20

Parkinson’s disease

Answer 20

Motor system disorder

Question 21

Parkinson’s disease - cause

Answer 21

Loss of dopamine-producing neurons in the substantia nigra

Question 22

Parkinson’s disease - Symptoms

Answer 22

• Tremor
• Rigidity – muscle stiffness
• Slowed movement (bradykinesia)
• Postural instability
• impaired balance and coordination
• Speech changes

Question 23

Parkinson’s disease Treatment

Answer 23

No cure

Levodopa
- Enters brain and converted to dopamine
- Combined with carbidopa to prevent premature conversion

Deep brain stimulation
- Control dyskinesia (involuntary movements)

Question 24

Huntington’s disease

Answer 24

Motor system disorder

Question 25

Huntington’s disease - Cause

Answer 25

Familial hereditary disease (autosomal dominant ) Huntingtin gene mutation Abnormally long version of huntingtin protein - Expansion of CAG repeats (normally 10-35; mutation 36 – 120 repeats) Smaller, toxic fragments - bind together and accumulate in neurons Loss of huntingtin protein's DNA repair function - Accumulation of DNA damage in neurons? Striatum and cerebral cortex particularly affected - Co-ordinate movement and control thinking and emotions

Question 26

Huntington’s disease Symptoms - Early

Answer 26

Memory lapses Stumbling and clumsiness Mood swings

Question 27

Huntington’s disease Symptoms - Advanced

Answer 27

Uncontrolled jerking ("Huntington's Chorea") Slurred speech Slow or rigid movements Personality changes Breathing and swallowing problems

Question 28

Huntington’s disease Symptoms - Treatment

Answer 28

No cure Medications to manage symptoms

Question 29

Cerebral Aneurysm Characteristics

Answer 29

- Ballooning/bulge in the wall of a blood vessel in the brain - Caused by a weakness in the blood vessel wall, usually where it branches

Question 30

Cerebral Aneurysm - Symptoms

Answer 30

Unruptured - Depends on size and rate of growth - loss of feeling in the face or visual problems After rupture - Sudden agonising headache - Stiff neck, vomiting & loss of consciousness

Question 31

Cerebral Aneurysm Treatment

Answer 31

Unruptured - Only treated if high risk Surgery - Endovascular coiling - fill the aneurysm with tiny platinum coils - Neurosurgical clipping - tiny metal clip clamped on aneurysm Ruptured - Nimodipine - reduce risk of disruption to brain blood supply (cerebral ischaemia) - Clipping or coiling

Question 32

Two types of CNS infections

Answer 32

Leptomeningitis & Encephalitis

Question 33

Leptomeningitis & Encephalitis

Answer 33

Inflammatory conditions of the brain (encephalitis) and meninges of brain & spinal cord (meningitis)

Question 34

Leptomeningitis & Encephalitis - Causes

Answer 34

Meningitis: bacterial or viral infections Encephalitis: viral infections e.g., HSV, enteroviruses

Question 35

Symptoms of meningitis

Answer 35

High fever Severe & persistent headache Stiff neck, nausea and vomiting Confusion, sleepiness, and difficulty waking up Increased sensitivity to light Infants: irritability or fatigue, lack of appetite, fever

Question 36

Symptoms of encephalitis

Answer 36

Sudden fever, headache, vomiting Increased sensitivity to light Stiff neck, confusion and impaired judgment Drowsiness Weak muscles, unsteady gait Irritability

Question 37

Severe symptoms of encephalitis

Answer 37

loss of consciousness, seizures, muscle weakness, or sudden severe dementia

Question 38

Leptomeningitis & Encephalitis Treatment

Answer 38

Bacterial infections: antibiotics Viral infections: antiviral medications Anticonvulsants to prevent or treat seizures Corticosteroids to reduce brain swelling & inflammation Sedatives may be needed for irritability or restlessness Medications for fever and headache

Question 39

Creutzfeldt-Jakob Disease (CJD) Characteristics

Answer 39

Rare, degenerative, invariably fatal brain disorder Spongiform encephalopathy

Question 40

Creutzfeldt-Jakob Disease (CJD) - Cause

Answer 40

Abnormal, infectious protein = prion (no nucleic acid) Prions accumulate at high levels in the brain & cause irreversible damage to neurons No diagnostic test - confirmed by brain biopsy or autopsy

Question 41

Creutzfeldt-Jakob Disease (CJD) - Treatment

Answer 41

None available Death usually as a result of respiratory infections

Question 42

Creutzfeldt-Jakob Disease (CJD) Types

Answer 42

Sporadic Variant Familial

Question 43

Creutzfeldt-Jakob Disease (CJD) - Sporadic

Answer 43

Normal brain protein changes abnormally ("misfolds") and turns into a prion

Question 44

Creutzfeldt-Jakob Disease (CJD) - Variant

Answer 44

Likely caused by consuming meat from a cow with bovine spongiform encephalopathy (BSE, or "mad cow" disease)

Question 45

Creutzfeldt-Jakob Disease (CJD) - Familial

Answer 45

Very rare genetic condition Mutation causes prions to form in the brain during adulthood

Question 46

Creutzfeldt-Jakob Disease (CJD) – Initial symptoms

Answer 46

Neurological symptoms - Problems with walking, balance & co- ordination - Slurred speech - Numbness - Dizziness - Visual problems - Hallucinations Psychological symptoms - Severe depression - Anxiety - Irritability - Insomnia - Withdrawing

Question 47

Creutzfeldt-Jakob Disease (CJD) – Advanced symptoms

Answer 47

Neurological symptoms - Loss of physical co-ordination - Muscle twitches and spasms - Loss of bladder & bowel control - Blindness - Dysphagia - Loss of speech Psychological symptoms - Severe memory loss - Confusion - Agitation - Aggression - Paranoia - Appetite loss

Question 48

Kuru

Answer 48

Rare and fatal brain disorder

Question 49

When was kuru epidemic?

Answer 49

Epidemic levels during the 1950s-60s

Question 50

Where is Kuru relevant?

Answer 50

Fore people in the highlands of New Guinea

Question 51

Kuru - Causes

Answer 51

Transmissible spongiform encephalopathy (prion protein) Ritualistic cannibalism

Question 52

Kuru Symtoms

Answer 52

Cerebellum affected Unsteady gait, tremors, and slurred speech Behavioural & mood changes often present