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CNS Pathology > Dementia & Degenerative Disorders > Flashcards

Dementia & Degenerative Disorders Flashcards

1
Q

What often causes dementia and degenerative disorders?

A

Accumulation of protein which damages neurons within gray matter

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2
Q

Degeneration of what structure causes dementia?

A

Cortex

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3
Q

Degeneration of what structures causes movement disorders?

A

Brainstem and Basal ganglia

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4
Q

What is the MCC of dementia?

A

Alzheimer’s disease

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5
Q

clinical features of Alezheimer’s disease

A

Slow onset memory loss (begins with short term memory loss and progresses to long term memory loss) and progressive disorientation

Loss of learned motor skills and language

Changes in behavior and personality

Patients become mute and bedridden; infection is a common cause of death

Focal neurologic deficits are not seen in early disease

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6
Q

What genes are associated with an increased risk of Alzheimer’s? Decreased risk?

A

E4 allele of apolipoprotein E is associated with an increased risk

E2 allele of APOE is associated with a decreased risk

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7
Q

When is early onset AD often seen?

A

Familial cases

Down syndrome

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8
Q

What are the morphologic features of Alzheimer’s?

A

Cerebral atrophy

Neuritic plaques

Neurofibrillary tangles

Loss of cholingergic neurons in the nucleus basalis of Meynert

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9
Q

How does cerebral atrophy appear?

A

Narrowing of the gyri, widening of the sulci and dilation of the ventricles

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10
Q

What are the neuritic plaques composed of?

A

Extracellular core comprised of Aß amyloid with entangled neuritic processes

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11
Q

How is Aß amyloid formed?

A

It is derived from APP

Normally APP is processed by alpha-secretase to form an alpha product which is degradeable. However in AD patients, beta-secretase cleaves APP into Aß amyloid which cannot be degraded thus depositing as] plaques.

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12
Q

What chromosome is APP coded on?

A

Chromosome 21

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13
Q

What are neurofibrillary tangles?

A

Intracellular aggregates of fibers composed of hyperphosphorylated tau protein

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14
Q

What is tau protein?

A

Microtubule associated protein

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15
Q

How is AD diagnosed?

A

Presumptive diagnosis made by clinical and pathological correlation.

Definitive diagnosis made by histology at autopsy

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16
Q

What is vascular dementia?

A

Multifocal infarction and injury due to HTN, athersclerosis, or vasculitis

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17
Q

What is Pick disease?

A

Degenerative disease of the frontal and temporal cortex; spares the parietal and occipital lobes

18
Q

How is Pick disease histologically characterized?

A

Characterized by round aggregates of tau protein (Pick bodies) in neurons of the cortex

19
Q

What are the symptoms of Pick disease?

A

Behavioral and language symptoms arise early

Eventually progresses to dementia

20
Q

What is Parkinson’s?

A

Degenerative loss of dopaminergic neurons in the substantia nigra of the basal ganglia

21
Q

What neural pathway are the dopaminergic neurons of the substantia nigra an essential part of?

A

Nigrostriatal pathway of the basal ganglia.

The basal ganglia coordinates movement using dopamie sent from the substantia nigra

22
Q

What are the clinical features of Parkinson’s?

A

“TRAP”

Tremor - pill rolling tremor at rest; disappears with movement

Rigidity - cogwheel rigidity in the extremities

Akinesia/bradykinesia - slowing of voluntary movement; expressionless face

Postural instability and shuffling gait

23
Q

Histo of Parkinson’s

A

Loss of pigmented neurons in th substantia nigra

Round eosinophilic inclusions of alpha-synuclein (Lewy bodies) in affected neurons

24
Q

What is a common feature of late stage Parkinson?

A

Dementia

25
Q

What is Lewy body dementia?

A

Suggested if early onset dementia is seen in Parkinsons

Characterized by dementia, hallucinations and parkinsonian features

26
Q

What is seen on the histology of Lewy body dementia?

A

Cortical Lewy bodies

27
Q

What is Huntington’s disease?

A

Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia

28
Q

What genetic disorder is found in Huntington’s?

A

Autosomal dominant disorder on chromosome 4 characterized by expanded trinucleotide (CAG) in the huntington gene

Further expansion of repeats during spermatogenesis leads to anticipation (earlier age of presentation with each generation)

29
Q

Clinical presentation of Huntingtons

A

Chorea (random muscular movement) that can progress to dementia and depression

Average age of presentation = 40

30
Q

What is normal pressure hydrocephalus?

A

Increased CSF resulting in dilated ventricles

31
Q

What is the triad of normal pressure hydrocephalus?

A

Urinary incontinence

Gait instability

Dementia

32
Q

The increased CSF in normal pressure hydrocephalus stretches which structure causing the triad of symptoms?

A

Corona radiata

33
Q

What procedure improves the symptoms of normal pressure hydrocephalus?

A

Lumbar puncture

34
Q

What is spongiform encephalopathy?

A

Degenerative disease due to prion protein

35
Q

What form of PrP is normal and which is pathogenic?

A

PRPc is an alpha helical configuration of PRP normally expressed in the CNS

PRPsc is a beta-pleated configuration that is converted from the normal PRPc. This form is pathogenic.

36
Q

How does PRPsc cause damage?

A

It is not degradeable and further converts normal protein into the pathologic form.

It accumulates in the neurons nad causes damage to the neurons and glial cells characterized by intracellular vacuoles (spongy degeneration)

37
Q

What is the MC form of spongiform encephalopathy?

A

Creutzfeldt-Jakob disease

(CJD)

38
Q

Clinical presentation of CJD

A

Rapidly progressive dementia associated with ataxia and myoclonus

39
Q

What is seen on the EKG of Creutzfeldt-Jakob disease patients?

A

Spike-wave complexes

40
Q

Variant CJD is a special form of the disease that is related to exposure to what?

A

Bovine spongiform encephalopathy (“mad cow”)

CNS Pathology (11 decks)

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