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Neuroscience II (Exam 2) > Demyelinating Disease and Eye Pathology > Flashcards

Demyelinating Disease and Eye Pathology Flashcards

1
Q

The major prototypical disorder of demyelination is _________ .

A

Multiple Sclerosis (MS)

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2
Q

True or False. MS is more common in Men with a 3:1 ratio to Women.

A

FALSE!

2:1 (Women:Men)

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3
Q

Young woman (20-30s) presenting with eye problems should throw up which red flag?

A

Multiple Sclerosis!!!!

MS is super rare in children and after the age of 50

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4
Q

Where are people living that might be predisposed to Multiple Sclerosis?

A

Further away from the Equator (above 38 degrees)

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5
Q

__________ tends to frequently present with MS patients.

A

Optic Neuritis

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6
Q

What can be associated (but is not diagnostic) of MS in the lumbar puncture?

A

CSF oligoclonal bands and Increased CSF protein levels

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7
Q

Who is responsible for the demyelination that takes place in MS?

A

Activated Leukocytes/macrophages and injurious soluble factors

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8
Q

Which virus has a high association with infecting MS patients?

A

Epstein-Bar Virus

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9
Q

Which cytokine is increased in the blood of people with SLE or MS?

A

BAFF (B-cell activating factor)

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10
Q

There are higher BAFF frequencies in different parts of the world (particularly close to italy). BAFF will protect you against ________ but it might predispose you to __________ .

A

Falciform Malaria

MS

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11
Q

Oligoclonal bands found in some MS patients are consistent with _______ activation.

A

B-Cell

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12
Q

Elevated levels of _______ may be characterized by remissions of MS.

A

Myelin Basic Protein

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13
Q

What is Optic Neuritis?

A

Unilateral visual impairment due to involvement of the Optic Nerve (CN II)

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14
Q

How can you differentiate between neuromyelitis optica and initial MS symptoms?

A

NMO: Presents with Bilateral Optic Neuritis and Spinal Cord Demylination; EVEN MORE common in Women than classical MS; Abs are going to attack Aquaporin-4 Channel

MS: Unilater Optic Neuritis

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15
Q

Which cells are going to be present in the MS plaques?

A

T Cells and Macrophages

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16
Q

CD4+ ______ and _______ reactive against myelin (self) antigens and secrete more cytokines that are going to potentiate that response in a genetically predisposed individual.

A

Th1 and Th17

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17
Q

Experimental Autoimmune Encephalomyelitis (EAE) is a relevant ANIMAL model of MS-like disease in which demyelination and inflammation occur after immunization of animals with ______ that recognize myelin antigens.

A

T-Cells

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18
Q

Which demylinating disease is caused by rapid correction of hyponatremia in patients that are malnourished?

A

Central Pontine Mylinolysis

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19
Q

Which Aquaporin is in the brain? Which cell is it located on?

A

Aquaporin-4

Located on Astrocytes

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20
Q

ADEM (Acute disseminated encephalomyelitis) and ANHE (Acute Necrotizing hemorrhagic Encephalomyelitis) are associated with a ________ trigger.

A

Viral

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21
Q

Why are ADEM, ANHE and Central Pontine Myelinolysis considered to be monophasic?

A

They are monophasic because they are caused by a SINGLE event and that means all of the plaques are the same age!

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22
Q

________ is not monophasic because it has varying degrees of plaques.

A

Multiple Sclerosis

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23
Q

Which demyelination disease is called “locked-in” syndrome?

A

Central Pontine Myelinolysis

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24
Q

Which is more serious of a syndrome: ADEM or ANHE?

A

ANHE because it tends to be fatal with those that are afflicted

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25
Q

Which part of the brain is most commonly involved in the neurodegenerative diseases?

A

Neocortex

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26
Q

What is the prototypical disease that is going diffusely effect both cerebral cortices?

A

Alzheimer’s Disease

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27
Q

What is the neurodegenerative disease that is going to be more localized and affect 1 cerebral cortex?

A

Frontotemporal dementias (FTLDs)

28
Q

Which neurodegenerative disease is going to affect Motor Neurons?

A

Amyotrophic Lateral Sclerosis (ALS)

29
Q

Neurofibrillary Tangles are going to be present in Alzheimer’s Disease and they contain which protein?

A

Tau (typically phosphorylated)

30
Q

What is the most common cause of dementia in older patients?

A

Alzheimer’s Disease

31
Q

Progressive loss of cognitive function independent of the state of attention is known as _______ .

A

Dementia

32
Q

Why do you have an early onset of Alzheimer in Down Syndrome Patients?

A

Amyloid Precursor Protein is located on chromosome 21

33
Q

Diffuse bilateral cerebral cortical atrophy, neuritic plaques, neurofibrillary tangles, hirano bodies (aggregates of proteins) and reactive gliosis are the morphological features of _________ .

A

Alzheimer’s Disease

34
Q

Which type of hydrocephalus is associated with Alzheimer’s Disease?

A

Hydrocephalus Ex Vacuo

35
Q

Neuritic Plaques contain ______ while neurofibrillary tangles contain _______ .

A

AB amyloid plaques

Tau protein

36
Q

_______ protein has been seen in PTSD, CTE, Frontotemporal Dementia, Parkinson-Related diseases and SSPE.

A

Tau

37
Q

Brain Iron levels are important to monitor in Alzheimer Patients that have the APOE _______ risk allele. _______ brain iron levels may be useful in slowing down the disease process.

A

E4

Lowering

38
Q

How long is the clinical course of Alzheimers?

A

~ 10 years

39
Q

Death in Alzheimer Patients can be due to ______ .

A

Supervening Infection (Pneumonia or Sepsis)

DVT

40
Q

Which APOE allele is going to protect pts from alzheimer?

Which APOE is going to increase your risk?

A

E2 (Protect)

E4 (Increase Risk)

41
Q

Personality changes (frontal lobe), behavior chages and aphasia (wernicke’s) at presentation rather than declining cognitive abilities is associated with _______ .

A

Frontotemporal Lobar Degenerations

42
Q

What is an example of a Frontotemporal Lobar Degeneration with Tau neuronal depositions?

A

Pick Disease

** You are going to “Pick” the Frontal and Temporal lobes while sparing the occipital and parietal lobes

43
Q

Why does the body sequester Iron in inflammatory diseases?

A

The best guess is that the body is trying to fend off certain infections, especially those caused by bacteria (H. Influenzae), that require IRON for their pathogenicity!

44
Q

What are the three proteins that can manifest in Frontotemporal Lobar Degeneration disorders?

A
  1. FTLD-Tau
  2. FTLD-TDP
  3. FTLD-FUS
45
Q

What are the “Big Four” neurodegenerative diseases?

A
  1. Alzheimer’s Disease
  2. Parkinson Disease
  3. Huntingtons Disease
  4. ALS
46
Q

What is Ballismus?

A

Violent involuntary rapid and irregular movement

47
Q

Which disease is classically known as the Hypokinesia disease?

A

Parkinson Disease

48
Q

Which disease is classically known as the Hyperkinesia disease?

A

Huntingtons Disease

49
Q

What is the typical treatment for Parkinson Disease?

A

Administration of L-Dopa

50
Q

How can you differentiate Classic Parkinson Disease from the Atypical Hyokinesic Movement disorders?

A

The Atypical Disorders DO NOT respond well to L-Dopa administration!

51
Q

What is the central triad for Parkinson Disease?

A
  1. Tremor
  2. Rigidity
  3. Bradykinesia
52
Q

Diminished facial expression, stooped posture, slowing voluntary movements, festinating gait, rigidty and tremor are all clinical presentations for ________ .

A

Parkinson Disease

53
Q

A pathogenic agent (carrying alpha synuclein) can gain access to the GI system, invade neurons in the Enteric Nervous System, and then travel, through a retrograde fashion, to the CNS through the preganglionic vagal fibers to destroy the dopaminergic neurons in the substantia nigra pars compacta. This is known as the ________ hypothesis.

A

Braak

54
Q

Which protein is associated with Parkinson Disease?

A

Alpha synuclein

55
Q

The severity of Parkinson Disease is proportional to the loss of ________ neurons.

A

Dopaminergic

56
Q

Which tauopathy is going to have tau inclusions in neurons and glial cells and seen more commonly in Males (age 40-50s)?

A

Progressive Supranuclear Palsy

57
Q

________ is known as a sporadic disorder that affects a number of different systems in the brain and is characterized by cytoplasmic inclusions of alpha-synuclein in oligodendrocytes.

A

Multiple System Atrophy (MSA)

58
Q

The Striatonigral circuit (results in parkinsonism), Olivopontocerebellar circuit (results in ataxia) and autonomic nervous system (results in orthostatic hypotension) are systemic manifestations that are classically presented in ________ .

A

Multiple System Atrophy (MSA)

59
Q

Which anatomical structure is affected in Huntingtons Disease?

A

Caudate Nucleus (degeneration)

60
Q

Describe “anticipation” as it relates to Huntingtons disease.

A

You are going to have more CAG repeats if the disease is passed on through paternal inheritance.

The replication of CAG repeats occurs during Spermatogenesis!!!!!

61
Q

Which gene is mutated in Huntingtons disease?

A

Huntingtin Gene

62
Q

What do Patients with Freidrichs Ataxia typically die of?

A

Cardiac Disease

63
Q

When you use the term “motor neuron disease” which disease are you referring to?

A

ALS

64
Q

Which neurodegenerative disease is going to destroy the pyramidal motor system?

A

ALS

65
Q

Which disease is going to typically present at age 40, have generalized muscle weakness and atrophy and can progress to lose the ability to initiate and control virtually all motor movement?

A

ALS

Neuroscience II (Exam 2) (14 decks)

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