Nervous system diseases III Flashcards

1
Q

What is dementia?

A

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

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2
Q

What type of disease is dementia?

A

a syndrome

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3
Q

What is the most common type of dementia?

A

alzheimers

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4
Q

What is alzheimers?

A

progressive cognitive decline

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5
Q

What is an early symptom of Alzheimers?

A

memory lapse

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6
Q

What are mid-stage symptom of Alzheimers?

A

worsening memory problems

increasing confusion and disorientation

obsessive and repetitive disorientation

obsessive and repetitive behaviours

changes in mood

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7
Q

What are more mid-stage symptom of Alzheimers?

A

sleep disturbance

hallucinations

aphasia

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8
Q

What are later symptom of Alzheimers?

A

weight loss

incontinence

difficulty moving

gradual loss of speech

dysphagia

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9
Q

Neuropathology of Alzheimer’s disease

A

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

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10
Q

Alzheimer’s disease
Risk factors

A
  • Age (risk doubles every 5 years after 65 years old)
  • Downs syndrome
  • Cardiovascular disease
  • Head injuries
  • Apolipoprotein (APOE) on xsome 19 – APOE ε4
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11
Q

Early Onset Dementia

A

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

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12
Q

Brain atrophy in Alzheimer’s disease
Treatment

A
  • No cure
  • Acetylcholinesterase inhibitors – increase Ach levels
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13
Q

Frontotemporal dementia (FTD) (Pick’s disease)

A
  • 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
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14
Q

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

A

Intracellular depositions of abnormal form of tau
protein inside neurons

Degeneration of neurons, microvacuoles formation, and astrocytosis

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15
Q

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

A

Dramatic changes in personality
eg. Inappropriate social behaviour

Language disturbance
- Difficulty using and understanding language
- Hesitant speech

Spatial skills and memory remain intact

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16
Q

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

A
  • No cure or specific treatment
  • Medications used to manage symptoms
  • Antidepressants
  • Anti-psychotics
  • Therapies
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17
Q

Vascular Dementia - Cause

A
  • 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
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18
Q

Vascular Dementia - Symptoms

A
  • Confusion
  • Short-term memory problems
  • Wandering & getting lost in familiar places
  • Losing bladder or bowel control
  • Difficulty following instructions
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19
Q

Vascular Dementia
Treatment

A
  • No treatment to reverse brain damage
  • Prevention of future strokes
  • Address risk factors
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20
Q

Parkinson’s disease

A

Motor system disorder

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21
Q

Parkinson’s disease - cause

A

Loss of dopamine-producing neurons in the substantia nigra

22
Q

Parkinson’s disease - Symptoms

A
  • Tremor
  • Rigidity – muscle stiffness
  • Slowed movement (bradykinesia)
  • Postural instability
  • impaired balance and coordination
  • Speech changes
23
Q

Parkinson’s disease Treatment

A

No cure

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

Deep brain stimulation
- Control dyskinesia (involuntary movements)

24
Q

Huntington’s disease

A

Motor system disorder

25
Q

Huntington’s disease - Cause

A

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

26
Q

Huntington’s disease Symptoms - Early

A

Memory lapses

Stumbling and clumsiness

Mood swings

27
Q

Huntington’s disease Symptoms - Advanced

A

Uncontrolled jerking (“Huntington’s Chorea”)

Slurred speech

Slow or rigid movements

Personality changes

Breathing and swallowing problems

28
Q

Huntington’s disease Symptoms - Treatment

A

No cure

Medications to manage
symptoms

29
Q

Cerebral Aneurysm
Characteristics

A
  • 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
30
Q

Cerebral Aneurysm - Symptoms

A

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

31
Q

Cerebral Aneurysm Treatment

A

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

32
Q

Two types of CNS infections

A

Leptomeningitis & Encephalitis

33
Q

Leptomeningitis & Encephalitis

A

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

34
Q

Leptomeningitis & Encephalitis - Causes

A

Meningitis: bacterial or viral infections

Encephalitis: viral infections e.g., HSV, enteroviruses

35
Q

Symptoms of meningitis

A

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

36
Q

Symptoms of encephalitis

A

Sudden fever, headache, vomiting

Increased sensitivity to light

Stiff neck, confusion and impaired judgment

Drowsiness

Weak muscles, unsteady gait

Irritability

37
Q

Severe symptoms of encephalitis

A

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

38
Q

Leptomeningitis & Encephalitis
Treatment

A

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

39
Q

Creutzfeldt-Jakob Disease (CJD)
Characteristics

A

Rare, degenerative, invariably fatal brain disorder

Spongiform encephalopathy

40
Q

Creutzfeldt-Jakob Disease (CJD) - Cause

A

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

41
Q

Creutzfeldt-Jakob Disease (CJD) - Treatment

A

None available

Death usually as a result of respiratory infections

42
Q

Creutzfeldt-Jakob Disease (CJD)
Types

A

Sporadic

Variant

Familial

43
Q

Creutzfeldt-Jakob Disease (CJD) - Sporadic

A

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

44
Q

Creutzfeldt-Jakob Disease (CJD) - Variant

A

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

45
Q

Creutzfeldt-Jakob Disease (CJD) - Familial

A

Very rare genetic condition

Mutation causes prions to form in the brain during adulthood

46
Q

Creutzfeldt-Jakob Disease (CJD) – Initial symptoms

A

Neurological symptoms
- Problems with walking, balance & co- ordination
- Slurred speech
- Numbness
- Dizziness
- Visual problems
- Hallucinations

Psychological symptoms
- Severe depression
- Anxiety
- Irritability
- Insomnia
- Withdrawing

47
Q

Creutzfeldt-Jakob Disease (CJD) – Advanced symptoms

A

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

48
Q

Kuru

A

Rare and fatal brain disorder

49
Q

When was kuru epidemic?

A

Epidemic levels during the 1950s-60s

50
Q

Where is Kuru relevant?

A

Fore people in the highlands of New Guinea

51
Q

Kuru - Causes

A

Transmissible spongiform encephalopathy (prion protein)

Ritualistic cannibalism

52
Q

Kuru Symtoms

A

Cerebellum affected

Unsteady gait, tremors, and slurred speech

Behavioural & mood changes often present