Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Mechanisms of Disease > Neurological Disorders > Flashcards

Neurological Disorders Flashcards

1
Q

Label the following:

Optic nerve, Lens, Anterior chamber, Posterior chamber, Sclera, Vitreous body, Cornea, Ora serrata, Iris, Retina, Uvea

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Label the following:

Bruch’s membrane, Sclera, Inner limiting membrane, Inner nuclear layer, Ganglion layer, Outer nuclear layer, Region of rods and cones, Choroid, Axon layer, Inner plexiform layer, Outer plexiform layer, Retinal pigmented epithelium, Outer limiting membrane

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Explain the pathogenesis of glaucoma

A
  • Increased intraocular pressure
  • Blocks flow through intraocular vessels in the uvea
  • Reduced blood flow deprives retina of nutrients
  • Retinal atrophy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What causes increased intraocular pressure? (2)

A
  1. Increased production of vitreous humor
  2. Decreased drainage of vitreous humor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How does glaucoma present clinically?

A
  1. Retinal damage → blurred vision and impaired dark adaptation
  2. Corneal damage → halos around light
  3. Optic nerve atrophy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Label the following:

Facial nerve (CN VII), Tympanic membrane, Stapes, Malleus, Incus, Vestibulocochlear nerve (CNVIII), Round window, Vestibular branch of CN VIII, External acoustic meatus, Tympanic cavity, Cochlear branch of CN VIII, Oval window, Vestibule, Cochlea, Internal acoustic meatus

A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is otosclerosis?

A
  • Bony growth around the oval window
  • Failure of resorption
  • Immobilization of the stapes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is transient tinnitus?

A
  • Rining in the ears not associated with diseases
  • Excessive stimulation of hair cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is persistent tinnitus?

A
  • Ringing in the ears associated with hearing loss
  • Associated with cochlear dysfunction or cranial nerve VIII dysfunction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Terminals of motor axons synapse with _____. Neurotransmitter is _____.

A
  • Sarcolemma
  • Acetylcholine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is myasthenia gravis?

A
  • Auto-antibodies against acetylcholine receptors
  • Induce aggregation and degradation of receptors
  • Antibodies can also interact with thymus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Explain the clinical presentation of myasthenia gravis

A
  1. Fluctuating weakness
  2. Diplopia (double vision) and ptosis (eyelid drooping)
  3. Decreased muscle responsiveness upon repeated stimulation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How can myasthenia gravis be treated? (3)

A
  1. Acetylcholinesterase inhibitors
  2. Immunosuppressive therapy or plasmapheresis
  3. Thymectomy for patients with thymoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

_____ is an autoimmune disease that targets components of the myelin sheath

A

Multiple sclerosis (MS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

True or false. In MS, lesions are usually softer than the surrounding tissue.

A

False. Firmer (hence sclerosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the common signs and symptoms of MS?

A
  1. Unilateral visual impairment
  2. Brainstem involvement
  3. Spinal cord lesions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What symptoms in MS are associated with damage to the brain stem? (4)

A
  1. Cranial nerve signs
  2. Ataxia
  3. Nystagmus
  4. Internuclear ophthalmoplegia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

By what mechanisms can ethanol damage the CNS? (3)

A
  1. Hepatic encephalopathy
  2. Thiamine deficiency
  3. Ethanol toxicity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is hepatic encephalopathy?

A
  • Damaged liver (by EtOH) cannot remove toxins from blood
    • elevated ammonia and pr-inflammatory cytokines
  • Glial cells in CNS respond to toxins (cerebral cortex & basal ganglia)
  • Astrocytes get enlarged nuclei and minimal reactive cytoplasm (Alzheimer Type II cells)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is Wernicke encephalopathy?

A
  • Acute thiamine deficiency associated with EtOH abuse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the symptoms of Wernicke’s encephalopathy? How is it treated?

A
  1. Psychotic symptoms
  2. Ophthalmoplegia
  • Reversible with thiamine supplementation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is Korsakoff syndrome? How is it treated?

A
  • Chronic thiamine deficiency due to ethanol abuse
  • Irreversible
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the symptoms of Korsakoff syndrome? They are typically associated with a lesion to what area of the brain?

A
  • Short term memory problems
  • Confabulation
  • Associated with lesion to thalamus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

_____ dysfunction is seen in 1% of chronic alcoholics

A

Cerebellar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

How does cerebellar damage present clinically in patients with chronic ethanol toxicity?

A
  • Truncal ataxia
  • Unsteady gate
  • Nystagmus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Cerebral edema is caused by_____

A
  • Accumulation of excess fluid within brain parenchyma
  • Caused by excess fluid leakage from blood vessels or CNS cellular damage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Explain the vasogenic pathway of cerebral edema

A
  • BBB disruption and and increased vascular permeability
  • Allows fluid to move from within vasculature to within parenchymal space
28
Q

Explain the cytotoxic pathway of cerebral edema

A
  • Secondary to cell membrane injury (neuron, glia, endothelium)
  • Generalized hypoxia/ischemia
  • Ionic homeostasis disruption
29
Q

Limited to no blood flow to a specific area of the brain is known as ______. If sustained, it will result in _____.

A
  • Focal cerebral ischemia
  • Cerebral infarct
30
Q

Explain the normal cellular response to cerebral edema

A
  1. Neuronal stress (red neurons)
  2. Macrophages and reactive gliosis clean up damage + neovascularization
  3. Repair - removal of tissue, loss of architecture, gliosis
31
Q

What are prions?

A

Abnormal forms of proteins (PrP)

32
Q

What is Creutzfeldt-Jakob disease?

A
  • Prion disease that results in neurodegeneration
  • Scrapie in sheep and goats
33
Q

True or false. Oftentimes in CJD the disease progresses so rapidly that no noticeable atrophy is seen on gross examination of the brain.

A

True

34
Q

What is the average survival for someone with CJD?

A

7 months

35
Q

What are the symptoms of CJD? (3)

A
  1. Changes in memory/behavior
  2. Dementia
  3. Startle myoclonus
36
Q

What is bovine spongiform encephalopathy?

A
  • Variant of CJD
  • Mad cow disease
  • Triggered by ingestion of prions from contaminated beef or blood transfusions
37
Q

Extracellular aggregates of PrPSC are known as ____. They are typically found in the _____.

A
  • Kuru plaque
  • Cerebellum
38
Q

What stain can be used to detect Kuru plaques?

A
  • Periodic acid-Schiff
  • Congo Red
39
Q

_______ is the loss of lower motor neurons in the brain, brainstem, and spinal cord

A

Amyotrophic lateral scerlosis (ALS)

40
Q

“Amyotrophic” refers to what in ALS?

A
  • Muscular paralysis with absence of atrophy
  • Hypertonia (rigidity) and exaggerated deep muscle tendon flexes
41
Q

What does “lateral sclerosis” refer to in ALS?

A
  • Degeneration of corticospinal tracts
  • Produces upper and lower motor neuron paralysis in extremities
42
Q

In ALS, which surface of the spinal cord is damaged?

A

Anterior surface; has motor neuron roots

43
Q

In 25% of cases of familial ALS, what gene is mutated and what type of mutation is it?

A
  • Copper-zinc superoxide dismutase (SOD1)
  • Gain-of-function mutation
44
Q

Aside from SOD1, mutations in what other genes can lead to familial ALS? (3)

A
  1. Dynactin (retrograde transport)
  2. VAMP-associated protein B (regulation of vesicle transport)
  3. Alsin (regulates endosomal trafficking)
45
Q

What is the consequence of a mutation in SOD1?

A

Unfolded protein response (UPR) by misfolded SOD1

46
Q

ALS pathogenesis is possible by what mechanisms? (5)

A
  1. SOD1 mutation
  2. ALtered axonal transport
  3. Neurofilament abnormalities
  4. Glutamate toxicity (increases intracellular calcium)
  5. Protein aggregates (Bunia bodies) in the cytoplasm
47
Q

Explain the clinical presentation of ALS

A
  • Loss of motor neurons → hand weakness; arm and leg spasticity/cramping
  • Eventually → muscle strength and bulk decrease; Fasciculations
48
Q

Death in ALS usually results from involvement of ______

A

Respiratory muscles

49
Q

Parkinson’s disease is described as degeneration of _____

A

Substantia nigra (SN) in the basal ganglia

50
Q

What are the symptoms of Parkinson’s disease? (3)

A
  1. Tremor
  2. Rigidity
  3. Bradykinesia
51
Q

True or false. Patients with Parkinson’s disease are not responsive to L-DOPA.

A

False. They are responsive

52
Q

Describe the pathology of parkinson’s disease

A
  • SN pallor (B); loss of pigmented neurons
  • Degeneration of dopaminergic neurons
  • Lewy bodies (eosinophilic cytoplasmic inclusions; α-synuclein fibers
53
Q

What is the normal function of α-synuclein?

A
  • Lipid binding protein associated with synapses
  • Activates melanin production in neurons
54
Q

Familial PD is associated with gain of function mutations in ____ (4) or loss of function mutation in ______.

A

GoF:

  1. Leucine-rich repeat kinase 2 (LRRK2)
  2. α-synuclein
  3. DJ-1
  4. PINK1

LoF:

  1. Parkin (associated with juvenile form)
55
Q

Of the genes associated with familial PD, which ones respond to stress responses (UPR, ROS)?

A
  1. α-synuclein
  2. DJ-1
56
Q

Of the genes associated with familial PD, which one if mutated leads to defective proteasome function?

A

Parkin

57
Q

Of the genes associated with familial PD, which ones if mutated result in altered mitochondrial function?

A
  1. DJ-1
  2. PINK1
58
Q

What is MPTP? What disorder can it cause?

A
  • Byproduct of synthetic opiod production (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine)
  • Common contaminant in street drugs
  • Causes Parkinsonian disorder
59
Q

Explain the mechanism by which MPTP induces Parkinsonian disorder

A
  • Converted to MPP+ in astrocytes
  • Inhibits complex I of electron transport chain
  • Reduced ATP production and oxygen metabolism
  • Increased ROS generation
  • Selectively targets dopaminergic neurons (mechanism unknown)
60
Q

What is dementia?

A

Impairment of memory and other cortical functions, while alertness is preserved

61
Q

What is Alzheimer Disease?

A
  • Most common cause of dementia (>50%)
  • Presence of specific amyloid plaques and neurofibrillary tangles (NFTs)
  • Synaptic loss and presence of reactive astrocytosis and microglial proliferation
62
Q

How long is the disease course of Alzheimer Disease after diagnosis?

A

5-10 years

63
Q

What is the first sign of Alzheimer diseases?

A

Impaired learning and recall of recent memories

64
Q

What are amyloid plaques?

A
  • Extracellular amyloid accumulation
  • Cerebral cortex and blood vessel walls (meningeal and cerebral)
65
Q

What is reactive astrocytosis?

A
  • Increased size and number of astrocytes in response to traumatic injury
  • Synthesis and release of cytokines
  • Induce migration of immune cells to CNS
  • Decreased glutamate
66
Q

What are neurofibrillary tangles (NFTs)?

A
  • Paired helical filaments containing hyperphosphorylated tau protein
  • Not specific for AD
67
Q

What proteins are associated with Alzheimer Disease (3)?

A
  1. Presenilins (amyloid processing)
  2. Apolipoprotein E4 (LDL binding receptor)
  3. Amyloid β

Mechanisms of Disease (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions