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Medical Neuropathology > Exam 2 Notes > Flashcards

Exam 2 Notes Flashcards

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USMLE® Step 1 flashcards
1
Q

Acquired diseases are…

A

demyelinating disease, like MS

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2
Q

what makes up the defense of the CNS

A

Meninges

Blood-brain barrier

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3
Q

What are the three routes of infection

A

Direct implantation
Local spread
Hematogenous (most common)

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4
Q

Inherited diseases are

A

dysmyelinating, due to myelin synthesis (leukodystrophies)

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5
Q

What are the three most important factors in infection

A

Number of organisms
Host immune system
Type of organism

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6
Q

Acute infection process

A

Blood enters the brain, the immune system responds.

ends in either resolution or death

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7
Q

Chronic infection

A

third possible outcome infection, happens when acute infections worsens

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8
Q

What do viruses do, what type of infection is it an example of

A

provoke a lymphocytic response and may precede inflammation to myelin proteins causing demyelination
– found in Acute and chronic infection

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9
Q

what do Fungi cause, what type of infection is it an example of

A

granulomatous inflammation, found in chronic infection

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10
Q

What is the basic definition of MS?

A

demyelinating disorder in which episodes of neurologic deficits are separated by time.

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11
Q

Is MS a white or gray matter disorder?

A

White matter.

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12
Q

Name three symptoms of MS

A

(Varied): visual loss, paralysis, sensory loss, ataxia, brainstem signs, psychiatric disorders, dementia.

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13
Q

Name the classifications of MS

A

Benign MS
Relapsing remitting MS
Secondary chronic progressive
Primary progressive

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14
Q

What factor makes certain people more predisposed to MS?

A

Living in da cold

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15
Q

What is happening in Devic’s disease?

A

Within damaged white matter, there is necrosis. Also, many pts. show antibodies to aquaporins (used in astrocytic foot process and thus in integrity of BBB)

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16
Q

Bacteria causes what? what type of infection is it an example of?

A

cause purulent reactions with polymorphonuclear leukocytes and later necrosis – Acute infection

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17
Q

what permits chronic inflammation

A

Low-toxicity organisms

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18
Q

What cell is characteristic of a granuloma

A

Macrophages in the form of a giant cell

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19
Q

What responds to infectious organisms

A

astrocytes and microglia

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20
Q

Acute disseminated encephalomyelitis (ADEM) is most common in what group of people and how deadly is it?

A

Children. Favorable outcome if treated quickly.

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21
Q

Define Osmotic demyelination syndrome

A

Degeneration of a symmetric midline path of the basis pontis. Basically a severe loss of myelin and axons.

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22
Q

What causes Progrssive multifocal leukoencephalopathy (PML)?

A

JC virus, it causes infection of oligos

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23
Q

Define demyelinating

A

loss of myelin

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24
Q

Define Dysmyelinating

A

cannot form appropriate myelin

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25
Q

The little boy with discolored finger nails had what disease?

A

Adrenoleukodystrophy (ALD) AKA Addison’s

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26
Q

Name some neurologic manifestations of ALD

A

Normal until 3-8, progressive neurologic decline
Impaired auditory discrimination
Visual disturbances

27
Q

What is one major pathological finding of Alexander’s disease?

A

Rosenthal fibers

28
Q

What are some symptoms of infantile Krabbe’s disease

A

Normal for a few months, then irritability appears, along with spasticity, neurological regression and seizures

29
Q

What are the cause of HIE in infants?

A

Placental abruption, cord accident, or cardiovascular stress during a difficult delivery.

30
Q

What area of brain damage in term infants with HIE occurs?

A

border zone(watershed area) and basal ganglia and brainstem.

31
Q

In mature infants, the basal ganglia, thalamus, and brainstem is affected more severely than other cortical region. Explain why.

A

they have higher energy demands and different neurotransmitters compared to the cortex

32
Q

Periventricular leukomalacia cause what in low birth-weight and term infants?

A

Cerebral palsy

33
Q

What pathology finding will be associated with PVL?

A

Ischemic white matter damage predominantly in the parieto-occipital lobes (watershed area).

34
Q

What clinical signs are shown in degenerative diseases

A

progressive loss of neurologic function (dementia, loss of movement control), and pathologically: loss of neurons

35
Q

What are the 6 common pathologic factors in neurodegenerative diseases

A

Deposition of fibrillar protein in matrix, Mitochondrial dysfunction, Excitotoxicity, Decreased proteasome activity, Decreased autophagy, and cell membrane alterations

36
Q

Where are plaques found in Alzheimers

A

In deposits outside and around the neurons

37
Q

What are Plaques

A

Beta amyloid fragments that clump together

38
Q

What are Neurofibrillary tangles

A

paired helical filaments

39
Q

What are Neurofibrillary tangles often referred to as and why

A

Ghost tangles, due to the death of the host neuron

40
Q

What do Tau deposits cause

A

Impairment of axonal transport

41
Q

What is the current opinion on how tau accumulates in Alzhiemers disease

A

Primary lesions are due to Aß deposition

42
Q

Liquefaction, phagocytosis, cavitation, and gliosis are infacts-like process that evolve with which disease?

A

Periventricular leukomalacia (PVL)

43
Q

What is the important cause of PVL?

A

Ischemia

Involving premyelinating oligodendrocyte that populate in the white matter

44
Q

Clinical symptoms of PVL

A

Spastic diplegia or tetraplegia. Visual impairment, cognitive deficits, and seizure

45
Q

Where does Germinal Matrix Hemorrhage start?

A

The germinal matrix is the layer under the ependymal lining of the ventricle. Hemorrhage starts between the thalamus and caudate

46
Q

Why do patient surviving large grade IV hemorrhage often develop hydrocephalus?

A

Clots or gliosis of the aqueduct and subarachnoid space by clots and fibrous tissue and from obliteration of the foramina of Luschka.

47
Q

Why does Kernicterus develop?

A

Inability of immature liver to conjugate billirubin, which crossess the BBB and enters neurons.

48
Q

What pathological finding are in Status Marmoratus?

A

Marbled appearance of the thalamus and basal ganglia

49
Q

What is the most common cause of Picks disease

A

Mutated tau protein

50
Q

What clinical features are shown in picks disease

A

severe atrophy of frontal and temporal lobes

51
Q

What are pick bodies

A

straight filaments and paired helical filaments

52
Q

What are the 4 prominent protein storage diseases, and which proteins are associated with them

A

Parkinsons: synuclein
Huntingtons: Huntingtin
PSP: Tau
ALS: ubiquitin/TDP-43

53
Q

What are some of the Parkinson symptoms

A

rigidity, small step gait, slow movement, tremors

54
Q

What pathologically causes Parkinsons

A

alpha-synuclein is deposited in the neuronal body, forming Lewy bodies

55
Q

what are the 5 possible ways to acquire parkinsonism

A

Drugs, vascular, toxins, infections, trauma

56
Q

Describe dementia with Lewy bodies

A

Parkinsonism and dementia combined. causes cognitive deficits and hallucinations

57
Q

What is Huntington disease

A

Degeneration of caudate, putamen, and the cortex

58
Q

What is “anticipation” when dealing with Huntingtons

A

When the disease appears in younger ages and more severely

59
Q

what is Progressive Supranuclear Palsy (PSP)

A

accumulation of tau and neuropil in pallidum, subthalamic nucleus, red nucleus, substantia nigra, oculomotor nucleus, medulla and dentate nucleus

60
Q

What are the symptoms of PSP

A

postural instability and early falls, vertical gaze palsy, mild cognitive changes, progressive axial rigidity and bulbar palsy

61
Q

What is ALS

A

Fatal degenerative disorder of upper and lower motor neurons

62
Q

ALS clinical symptoms

A

affects Middle aged individuals, Muscle weakness, Spasticity Incontinence

63
Q

What is Friedreich’s ataxia (FRDA)

A

Most frequent inherited ataxia, autosomal recessive disorder, begins usually before age 20 with ataxia

64
Q

what is Neuromyelitis optica-NMO (Devic’s disease)

A

relapsing inflammatory demyelinative disease that causes transverse myelitis and optic neuritis

Medical Neuropathology (10 decks)

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