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D&T2 Exam 1 > Central Nervous System Degenerative Diseases > Flashcards

Central Nervous System Degenerative Diseases Flashcards

1
Q

What characterizes a Neurodegenerative disease

A
  • progress loss of neurons

- typically affecting groups of neurons with functional relationships even though they are not next to each other.

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2
Q

what is the pathologic process commonly seen in neurodegenerative diseases

A

accumulation of protein aggregates

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3
Q

Name 2 cerebrocortical Degenerations disease

A

Alzheimer disease

-frontotemporal lobar degenerations Picks disease

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4
Q

Alzheimer is a progressive loss of neurons where in the brain? what exactly happens? This process results in what?

A
  • decreased choline acetyltransferase and acetylcholine in cerebral cortex
  • Dementia
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5
Q

If a patient were to walk into the office, how would you diagnose them with Alzheimer disease

A

Clinical and brain imaging ID

-brain tissue for definitive diagnosis

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6
Q

pathogenesis

A

the manner of development of a disease

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7
Q

pathogenesis of Alzheimer disease

A

Amyloid precursor protein

  • transmembrane
  • can be cleaved to form ABeta peptide
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8
Q

What are 2 proteins associated with Alzheimer disease

A

ABeta 40 and 42

Tau

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9
Q

what are some general characteristics of Abeta peptide

A
  • aggregates easily
  • forms beta-pleated sheets of amyloid
  • resistant to breakdown
  • Neurotoxic
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10
Q

what can be stained to find ABeta

A

congo red

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11
Q

what are the abnormal chromosomes in Alzheimer

A

1, 14, 19, 21

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12
Q

what protein is on chromosome 21

A

Amyloid precursor protein

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13
Q

Besides Alzheimer disease being associated with chromosome 21, what other disease is associated with it ? When do people with this get Alzheimer

A

Down Syndrome

-3rd or 4th decade

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14
Q

What get mutated on chromosome 14 and 1 for Alzheimer

A

Chromosome 14: Presenilin 1

Chromosome 1: Presenilin 2

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15
Q

what happens when Presenilin 1 and Presenilin 2 get mutated in Alzheimer Disease

A

increases the amounts of Abeta peptide via gama-secretase complex

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16
Q

What protein is associated with chromosome 19

A

ApoE

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17
Q

What does ApoE E4 isoform promote

A

ABeta generation and deposition

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18
Q

4 brain morphology of Alzheimer disease

A
  • atrophy of cerebral cortex
  • Thinned Gyri and Widened Sulci
  • Cortical Gray matter thinned
  • dilation of lateral ventricles
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19
Q

Hydrocephalus ex vacuo

A

dilation of lateral ventricles

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20
Q

what are 3 things seen under the microscope for Alzheimer Disease

A

Neuritic plaques
Neurofibrillary tangles
cerebral amyloid angiopathy

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21
Q

what is in a neuritic plaque

A

central amyloid Abeta42 peptide core with clear halo

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22
Q

where in the brain are neuritic plaques found

A

hippocampus
amygdala
neocortex
spares: motor and sensory cortices

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23
Q

Another name for Bielschowsky stain

A

silver staining

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24
Q

what are nuerofibrillary tangles

A

bundles of filaments in cytoplasm

-paired helical filaments (tau)

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25
Q

what does Neurofibrillary tangles look like on a Silver stain? what type of cell are they found on?

A

flame-shaped in pyramidal neurons

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26
Q

What is cerebral amyloid angiopathy

A

deposition of amyloid Abeta40 in walls of small meningeal and cortical arteries

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27
Q

Gliosis

A

nonspecific reactive change of glial cells in response to damage to the central nervous system

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28
Q

what is the age group for Alzheimer Disease

A

greater than 50 years

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29
Q

what are the first subtle changes in Alzheimer disease

A
  • loss of higher cortical function

- changes in mood and behavior

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30
Q

what are later clinical changes in Alzheimer disease

A

disorientation
memory loss
aphasia

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31
Q

aphasia

A

loss of ability to understand or express speech

can’t perform ADL’s

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32
Q

How is Alzheimer disease treated

A

some drugs can help with symptomatic improvement

-no treatment stops progression of disease

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33
Q

what is frontotemporal lobar degenerations

A

heterogeneous set of disorders associated with focal degeneration of frontal and/or temporal lobes

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34
Q

what age group does frontotemporal lobar degenerations hit

A

younger than 65 years of age

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35
Q

how is Frontotoemporal lobar degenerations distinguished from Alzheimer’s disease

A

alterations in personality, behavior, and language preceding dementia

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36
Q

what proteins are associated with frontotemporal lobar degenerations

A

tau or TDP43

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37
Q

FTLD-Tau is also called what

A

Pick disease

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38
Q

what age range does pick disease usually present

A

40-65 years

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39
Q

Where on the brain does Pick Disease usually occur? what areas are usually preserved?

A
  • selective lobar atrophy of anterior frontal and temporal lobes ( knife-edge atrophy)
  • posterior 2/3 of superior temporal gyrus, parietal and occipital lobes are preserved
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40
Q

In what layers of the brain are neurons lost in Pick Disease

A

outer 3 layers of cortex

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41
Q

Surviving neurons in Pick disease are called what and have what characteristics?

A

Pick bodies

- tau

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42
Q

Pick bodies usually occur where in the brain

A

cerebral cortex in cortical layer II

dentate gyrus of hippocampus

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43
Q

Name 3 basal ganglia degenerations

A

Parkinson disease
Parkinsonism
Huntington Disease

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44
Q

What happens in the brain in Parkinson disease

A 
depigmentation
neuronal loss
gliosis of pigmented nuclei 
-damage pathway from substantia nigra to corpus striatum 
-decrease dopamine in corpus striatum
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45
Q

how are neurons lost in Parkinson disease

A

apoptosis or necrosis

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46
Q

what is the genetic link for Parkinson disease

A

mutation in gene for alpha-synuclein on chromosome 4q

-affect its folding/alter its structure

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47
Q

What would the gross pathology look like for Parkinson’s

A

depigmented substantia nigra and locus ceruleus

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48
Q

what are 3 things on microscopic level for Parkinson disease

A
  • Lewy Body
  • gliosis of substantia nigra and locus ceruleus
  • loss of dopaminergic neurons
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49
Q

Lewy bodies also have what in them

A

melanin granules

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50
Q

After what age does Parkinson occur

A

after 50

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51
Q

what are some clinical features for Parkinsons

A

cog-wheel rigidity

  • resting tremor
  • Bradykinesia
  • Gait, festinating
  • slow, difficult speech
52
Q

what are treatment options for Parkinsons

A

Pharaceuticals
deep brain stimulation
pallidotomy

53
Q

what is the prognosis for Parkinsons disease

A

Poor

54
Q

Patients with Parkinson disease also have what

A

dementia

hallucinations and frontal signs

55
Q

where are lewy bodies found in brain

A

medulla
midbrain
cortex

56
Q

what are 3 causes of Parkinsonism

A

Postencephalitic Parkinsonism - after Spanish influenza 1915-1918

  • drug side-effect
  • trauma
57
Q

what drugs might cause Parkinsonism

A

Phenothiazines
reserpine
MPTP

58
Q

How is Huntington Disease inherited

A

autosomal dominant
complete penetrance
delayed appearance

59
Q

what gene and chromosome is messed up in Huntington disease

A

HD gene
short arm of chromosome 4
-CAG repeats

60
Q

where do loss of neurons occur for Huntington disease

A

cuadate nucleus
putamen
variable cerebrocortical atrophy

61
Q

at what age does Huntington present

A

20-50 years

62
Q

what are symptoms of Huntington’s disease

A

dementia and psychotic

choreiform movements

63
Q

what is correlated with an earlier onset of Huntington Disease

A

longer CAG repeats

64
Q

Spinocerebellar degnerations usually occur where

A

cerebeullum, brainstem, spinal cord, peripheral nerves

65
Q

Name a spinocerebellar degneration disease

A

Friedrich ataxia

66
Q

How is Friedrich ataxia inherited? what goes wrong genetically?

A 
autosoma recessive 
GAA repeat
protein deficiency (Frataxin)
67
Q

who usually gets Friedreich’s ataxia

A

late childhood/pre-adolescence

68
Q

what goes wrong in Friedreich’s ataxia

A

degeneration of spinocerebellar tracts, posterior columns, pyramidal tract, peripheral nerves

69
Q

what are clinical symptoms for Friedriech’s ataxia

A

pes cavus

kyphoscholiosis

70
Q

Define motor neuron disease

A

degeneration of both upper and lower motor neurons

71
Q

where does loss of upper motor neurons occur

A

cerebral Motor cortex

72
Q

where does degeneration of corticospinal tracts occur

A

lateral spinal cord ( lateral sclerosis)

73
Q

where does loss of lower motor neurons occur

A

brain stem and spinal cord ( anterior)

74
Q

Name two motor neuron diseases

A

amyotrophic lateral scleriosis

spinal muscular atrophy

75
Q

what does the gross anatomy look like for ALS

A

thinned ventral roots of spinal cord

76
Q

what does ALS look like under a microscope

A
  • upper motor neuron destruction leads to degeneration of myelin in corticospinal tract
  • decrease neuron in anterior horn throughout length of spinal cord, loss of anterior root myelinated fibers
  • skeletal muscles show neurogenic atrophy
77
Q

What are clinical symptoms for ALS

A
  • upper motor paralysis of extremities

- lower motor neuron signs

78
Q

what are some upper motor signs for ALS

A 
weakness
- increased reflexes
spasticity 
-pseuodbulbar 
- babinski sign
79
Q

pseuodbulbar

A

Inappropriate involuntary laughing and crying due to a nervous system disorder

80
Q

what are lower motor neuron sign for ALS

A 
weakness
decreased reflexes
atrophy 
fasciculation 
muscle denervation
81
Q

what gender is more prone to ALS? age?

A

men usually over 50

82
Q

what is the prognosis for ALS

A

50% 5-year survivial

83
Q

what is the usual cause of death for ALS

A

bronchopenumonia secondary to muscle paralysis

84
Q

what used for treatment of ALS

A

symptomatic and riluzole ( glutamate antagonist)

85
Q

Another name for Spinal muscular atrophy type 1

A

Werdnig-Hoffman disease

86
Q

how is Werdnig-Hoffman disease inherited

A

autosomal recessive - deletions of survival motor neuron gene, chromosome 5

87
Q

what goes wrong in Werdnig-Hoffman disease

A
  • loss of motor neuron in anterior horn
  • atrophy of anterior spinal roots and peripheral motor nerves
  • denervation and atrophy of skeletal muscle groups
88
Q

clinical presentation for spinal muscular atrophy

A

congenital hypotonia (“floppy baby”)

89
Q

what is the most common autoimmune inflammatory, demyelinating disorder

A

Multiple sclerosis

90
Q

what is MS

A

episodes of neurologic deficits separated in time, attributable to white matter lesions separated in space
-plaques in white matter ( adjacent to lateral ventricles, optic nerves, chiasm, brain stem, cerebellum, and spinal cord)

91
Q

who is most likely to get MS

A

any age 20-40 most common

Females

92
Q

how is MS an autoimmune disease

A

initiated by Th1 and Th17 which react against myelin Ag ( cellular immune response)

93
Q

MS occurs more commenly where in the world

A

temperate latitudes

94
Q

what happens in active plaque of MS

A

myelin breakdown
fewer oligodendrocytes
macrophages
lymphocytes, plasma cells

95
Q

what happens in inactive plaque

A

little or no myelin

96
Q

what are shadow plaques of MS

A

border between normal and affected white matter

-not sharply circumscribed

97
Q

What are common signs and symptoms in MS from the brain stem

A

ataxia, nystagmus, internuclear opthalmoplegia

98
Q

What are common signs and symptoms in MS from the spinal cord

A

motor and sensory impairment, spasticity, bladder dysfunction

99
Q

what is a common sign and symptom for MS

A

unilateral vision impairment from optic nerve involvement

intranuclear ophthalmoplegia: pt looks left, left eye moves to left with nystagmus, right eye stationary

100
Q

what are CSF values for MS

A

elevated protein
moderate lymphocytes and neutrophils
gamma Globulin increased

101
Q

what is wrong in Neuromyelitis optica

A

Abs to aquaporin-4

areas of demylination show loss of aquaporin-4

102
Q

what are clinical symptoms for Neuromyelitis optica

A

bilateral optic neuritis

prominant spinal cord involvment

103
Q

what is another name for central pontine myelinolysis

A

osmotic demylinaton syndome

104
Q

what causes central pontine myelinolysis

A

rapid correction of hyponatremia

acute hyponatermia causes cerebral edema

105
Q

what is the pathogenesis of central pontine myelinolysis

A

adapt to loss of electrolytes

after correction brain shrinks

106
Q

clinical symptoms of central pontine myelinollysis

A

dysarthria , lethargy
partially reversable
appear 2-6 days following rapid correction

107
Q

what part of the braine does central pontine myelinolysis impact

A

symmetrical loss of myelin- diffuse or in brain stem, pontine tegmentum

108
Q

what patients are at risk for central pontine myeliniolysis

A

alcoholics
liver transplant
electrolyte imbalance

109
Q

BeriBeri is associated with what vitamin

A

Thiamine B1

110
Q

B1 deficiency may cause ? if it goes untreated what can it cause

A

Wernicke Encephalopathy

Korsakoff ( memory disturbances)

111
Q

what type of patients are at risk for beriberi

A

alcoholics

Gastric disorders

112
Q

Thiamine deficiency effects what part of the brain

A

MAMMILLARY bodies

3 and 4 ventricles

113
Q

in thiamine deficiency memory disturbances are caused by lesions in what areas

A

medial dorsal nucleus of thalamus

114
Q

what are clinical features of B12 deficiency

A

ataxia
parasthesias of lower extremities
spastic weakness of paraplegia

115
Q

what and which axons are impacted by B12 deficiency

A

degeneration of axons
- ascending tract
descending pyramidal tract
subacute combined degeneration of spinal cord

116
Q

hypoglycemia resembles what

A

hypoxia

117
Q

what areas are impacted by hypoglycemia

A

large pyramidal neurons of ctx
hippocampus
cerebellum

118
Q

hyperglycemia is seen in what type of patients

A

uncontrolled diabets M.

119
Q

hyperglycemia can cause what and precaution treatment is taken

A

dehyrdation

rehydration may cause cerebral edema, SLOW rehydration

120
Q

hepatic encephalopathy have cellular response primarly in what? and what astrocytes are seen? what happens to neurons

A

glial

  • alzheimer type II astrocytes seen in hyperammonemia
  • no abnormalities of neurons
121
Q

carbon monoxide poisening impacts what part of the brain

A

bilateral necrosis of globus pallidus

demyelination

122
Q

how does methanol impact the brain

A
  • degeneration of retinal ganglion cells
  • selective bilateral putamenal necrosis
  • focal white matter necrosis
123
Q

clinical presentation for methanol

A

altered mental status

124
Q

what is the pathology for ethanol

A

atrophy and loss of granule cells in anterior vermis

advance cases: loss of PUrkinje cells with astrocytes

125
Q

pathology of radiation

A

coagulative necrosis effecting all elements of white matter.

D&T2 Exam 1 (22 decks)

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