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NEUROLOGY > Demyelination & Dementia > Flashcards

Demyelination & Dementia Flashcards

1
Q

What does it mean to have a rise in lymphocytes in CSF?

A

-indicates INFECTION or INFLAMMATION in the intracranial cavity

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2
Q

What are the 3 roles of Oligodendrocytes?

A

MYELIN production:

  1. locally confining neuronal DEPOLARIZATION
  2. protecting axons
  3. forming NODES of RANVIER
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3
Q

What are the nodes resp. for?

A

Precipitating RAPID saltatory conduction

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4
Q

Most likely group to have MULTIPLE SCLEORSIS?

A
  • Female in 20-30yr gr.

- a/w Latitude

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5
Q

Describe Multiple SCELORSIS.

A
  • demyelinating d.o characterized by distinct NEUROLOGICAL deficits, seperated in TIME
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6
Q

How to clinically dx MS?

A
  1. two DISTINCT neuro deficits occurring at diff. times
  2. neuro defect implicating site A on brain, but MRI appreciates a site B defect
  3. multiple, distinct CNS lesions on MRI
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7
Q

What is seen in the CSF of MS?

A

IgG oligoclonal bands in CSF

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8
Q

What are the clinical fts of MS?

A
  • OPTIC nerve lesions (neuritis) –unilateral
  • spinal cord lesions (MOTOR/SENSORY deficit in TRUNK or LIMBS; spasticity; bladder dysfxn)
  • brain stem lesions (CN signs; ataxia; nystagmus; internuclear ophthalmoplegia
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9
Q

Describe in 3 points the course of MS.

A
  • Acute or Insidious onset
  • relapsing and remitting course
  • neurological deficits may mount and become progressive
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10
Q

Describe plaques on cut surface.

A
  • well circumscribed/demarcated
  • irregular shape
  • glassy appearance
  • v.small to LARGE
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11
Q

Which sites are commonly affected by Plaques? (7)

A
  • CN 2
  • periventricular white matter
  • corpus callosum
  • brainstem
  • ascending and descending tracts.
  • spinal cord and cerebellum
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12
Q

What is seen Microscopically in Active plaques? (2)

A
  • active inflammation (perivascular)

- ongoing DEMYELINATION

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13
Q

What is seen in Inactive plaques?

A
  • gliosis
  • little myelinated axons
  • oligodendrocytes and axons REDUCE in no.
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14
Q

What cells are predominantly present in an Active Plaque?

A

Microglia

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15
Q

Distinguish grossly an Active plaque from an Inactive plaque.

A

Active plaque - appears YELLO/BROWN

Inactive- appears Grey-brown

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16
Q

What could be environmental factors causing MS to occurs?

A
  • latitude association
  • vit.D deficiency (inadequate sunlight exposure at high latitudes)
  • probable viral trigger (EBV)
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17
Q

Are genes a possible cause for MS?

A
  • genetic a/w HLA DRB1
  • 15x risk if first degree relative has MS
  • 150x risk if you have a Monozygotic twin
  • a/w polymorphisms in IL-2 and IL-7
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18
Q

Why is MS said to be an immune mediated disease?

A
  • histologically: lymphocytic infiltration in csf
  • oligoclonal IgG bands in csf
  • genetic linkage to HLA DRB1
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19
Q

What have animal studies proven in terms of the immune pathogenesis of MS?

A
  • T-helper 1 and 17 are actively involved in the demyelinating disease
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20
Q

What is one form of immune therapy for MS?

A

Anti B-cells therapies reduce relapses and frequencies of myelinating lesions

—-b-cells are the Ab-producing lymphocytes

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21
Q

Define Dementia

A

ACQUIRED and PERSISTENT, generalized disturbance of higher mental functions, in an otherwise alert person.

PATHOLOGICAL! —not part of ageing

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22
Q

Apart from IIary dementia; what other factors may contribute to the manifestation of dementia?

A
  • Drugs and Toxins (ALCOHOL)
  • Vit. B1 deficiency
  • paraneoplastic syndromes
  • intracranial space occupying lesions
  • chronic hydrocephalus
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23
Q

Why is the age of onset for Alzheimer’s dementia relevant?

A
  • the LATER, the age of onset; the more rapid and severe the changes tend to be.
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24
Q

What are demographics of Alzheimer’s like?

A

female, 85+y.o

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25
Q

What is Alzheimer’s genetically a/w?

A
  • increased incidence with TRISOMY 21 (APP)

- Familial incidence (1%)

26
Q

What mutations are implicated in familial AD?

A
  • Presenilin 1 (Chr.14) &2 (Chr.1)

- point mutations in amyloid precursor protein (APP)

27
Q

What is the initial presentation of Alzheimer’s?

A
  • insidious impairment of HIGHER INTELLECTUAL fxn

- alterations in MOOD and BEHAVIOUR

28
Q

What occurs later on in Alzheimer’s?

A
  • progressive disorientation
  • MEMORY LOSS
  • aphasia (d/t severe cortical DYSFXN)
  • results in muteness, profound disability, immobility
29
Q

Which areas of the brain is primarily affected and how?

A
  • the frontal, parietal and temporal lobes will ATROPHY
  • widening of sulci and narrowing of gyri
  • ->DECREASED size and weight of the brain
30
Q

What may occur IIary to cortical loss of the brain?

A

-hydrocephalus Ex Vacuo (dilatation of the ventricles)

31
Q

What are 2 major microscopic fts of Alzheimer’s?

A
  1. Neuritic Plaques

2. Neurofibrillary tangles

32
Q

What are neurofibrillary tangles?

A
  • accumulation of INSOLUBLE microtubules in the cytoplasm of neurones
  • TAU protein is disregulated in AD and other dementias
  • microscopic changes, specifically seen in HIPPOCAMPUS and TEMPORAL lobe
33
Q

What is seen in and around the plaques?

A
  • tortuous neuritic fibres surrounding an A-beta amyloid core
  • also surrounded by astrocytes and microglia
34
Q

What is a vascular ft of AD?

A

-AMYLOID ANGIOPATHY

35
Q

Why are Down $ pts most likely to get early onset Alzheimer’s ?

A

because the APP gene is located on Chromosome 21 —extra quantity of the gene

36
Q

What is Presenilin resp. for?

A
  • metabolism of APP
37
Q

what is a/w pathological severity of AD and why?

A

-the quantity of A-beta OLIGOMERS

38
Q

What does A-Beta Oligomers do pathologically?

A
  • known to promote hyper-phosphorylation and mislocalisation of TAU (accumulation)==> neuronal dysfxn and cell death
  • brings about POST-SYNAPTIC activation of NMDA receptors==> EXCITOTOXICITY ==> NEURONAL DAMAGE
39
Q

How does Amyloid angiopathy come about?

A

occurs due to accumulation of A-beta amyloid proteins within the walls of the vessels.

40
Q

What occurs as a result of amyloid angiopathy?

A

-disruption of BBB
>serum leaking
>edema
>local hypoxia ==>excititoxicity

41
Q

What is the presentation of Lewis Bodies Dementia?

A
  • progressive dementia, with Hallucinations and FLUCTUATING levels of attention/cognition
  • memory is affect later (contrast to AD)
  • fts of Parkinsonism at onset
42
Q

What is the relation between Parkinson’s and Lewis Body Dementia?

A
  • only a small portion of those with Parkinson’s will develop Lewis Body Dementia
  • ALL with Lewis body dementia get Parkinson’s fts
43
Q

What other conditions affect the Substantia Nigra ?

A
  • idiopathic parkinson disease
  • Drugs (phenothiazines)–chlorpromazine
  • trauma …dementia Pugilistica (boxer’s dementia)
  • Multi-system Atrophy
  • progressive SUPRANUCLEAR PALSY
  • cortico-basal degeneration
44
Q

What macroscopic change is seen in the Mid brain in Lewis Body dementia?

A

pallor of the substantia Nigra

45
Q

What is seen microscopically in Lewis Body Dementia at the Substantia Nigra?

A
  • loss of pigmented neurons
  • reactive gliosis
  • remaining neurons contain Lewis Bodies ( eosinophilic bodies with a DENSE core and pale halo)
  • aggreagates of alpha-synuclein and ubiquitin
46
Q

What demographics for Huntington’s disease? 1

A

ages 35-60

47
Q

What are the clinical fts. of Huntington’s Disease?

A 
- triad of Emotional, Cognitive and Motor Disturbance 
>chorea, myoclonus
>clumsiness, slurred speech
> depressed. irritable and apathetic
LATER DEVELOPS DEMENTIA
48
Q

Why is genetic counselling important in Huntington’s Disease?

A
  • because it is an AUTOSOMAL DOMINANT inherited disease, which has a late disease onset at about 35-50y.o
  • by the time, most people would have already have kids
49
Q

Explain the genetic pathogenesis in Huntington’s Disease.

A
  • mutation of the Huntingtin gene on Chromosome 4p
  • mutant gene of CAG repeats form
  • DISEASE occurs when >35 repeats occur!
50
Q

What is seen macroscopically in Huntington’s?

A
  • atrophy of the BASAL ganglia (Caudate-esp. , and Putamen nuclei)
  • cortical atrophy occurs later on
51
Q

What is seen microscopically in Huntington’s?

A
  • neuronal atrophy of striatal neurones in basal ganglia ==> loss of INHIBITORY inhibition of motor activity> CHOREA
  • Pronounced astrocytic gliosis
52
Q

What is Fronto-Temporal Dementia?

A
  • aka Pick’s Disease
  • progressive dementia beginning MID-LIFE (between 50-60s)
  • progressive change in CHARACTER and SOCIALdeterioration
    >impairment of INTELLECT, MEMORY and LANGUAGE
53
Q

What are the symptoms of Pick’s Disease?

A
  • a/w frontal and temporal lobes
  • speech and comm. problems
  • personality and behavioural changes
  • change in EATING habits
  • reduced attention span
54
Q

Describe the progress of FTD?

A
  • rapidly progressive illness
  • may last between 2-10years
  • mean length of illness (7years)
55
Q

How diff. is the degree of Atrophy in Pick’s?

A
  • MUCH BIGGER than AD
  • extreme atrophy of cerebral cortex in the F AND T lobes
  • so severe that the brain weighs <1 kg
56
Q

What is seen Microscopically in Picks?

A
  • pick’s cells (Swollen Neurones)
  • intracytoplasmic filamentous INCLUSIONS known as PICK’S
  • pick’s cells are enriched with TAU, Ubiquitin and TDP-43
57
Q

How does Multi-infarct (VASCULAR) dementia come about?

A
  • deteriotration in mental functioning d/t CUMULATIVE damage to the BRAIN through hypoxia/anoxia as a result of MULTIPLE BLOOD CLOTS in blood vessels. > large areas of cell death and damage = DEMENTIA (need 50-100ml of brain loss for dementia to occur)
58
Q

What are the demographic in this disease?

A
  • 60y.o Men
  • also in middle-aged hypertensives
  • –note: those aware of THEIR mental deficits are prone to DEPRESSION and ANXIETY
59
Q

How to distinguish Vascular dementia from AD?

A
  • MID has an ABRUPT onset
  • stepwise progression—as further strokes occur down the line
  • hx of HTN or STROKE
  • evidence seen on CT or MRI
60
Q

What is seen morphologically with Vascular Dementia ?

A
  • large vessel infarcts
  • scattered throughout hemispheres
  • atheroma of large vessel arteries
  • small vessel infarcts (rarer) —d/t longstnding HTN
61
Q

Which genes are involved in the early onset of AD?

A
  • Presinilin 1, 2

- APP

62
Q

What gene prediposes you rrisk of AD in general?

A
  • Apoprotein E allele E4

NEUROLOGY (36 decks)

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