Exam 2 Notes

Question 1

Acquired diseases are…

Answer 1

demyelinating disease, like MS

Question 2

what makes up the defense of the CNS

Answer 2

Meninges

Blood-brain barrier

Question 3

What are the three routes of infection

Answer 3

Direct implantation
Local spread
Hematogenous (most common)

Question 4

Inherited diseases are

Answer 4

dysmyelinating, due to myelin synthesis (leukodystrophies)

Question 5

What are the three most important factors in infection

Answer 5

Number of organisms
Host immune system
Type of organism

Question 6

Acute infection process

Answer 6

Blood enters the brain, the immune system responds.

ends in either resolution or death

Question 7

Chronic infection

Answer 7

third possible outcome infection, happens when acute infections worsens

Question 8

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

Answer 8

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

Question 9

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

Answer 9

granulomatous inflammation, found in chronic infection

Question 10

What is the basic definition of MS?

Answer 10

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

Question 11

Is MS a white or gray matter disorder?

Answer 11

White matter.

Question 12

Name three symptoms of MS

Answer 12

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

Question 13

Name the classifications of MS

Answer 13

Benign MS
Relapsing remitting MS
Secondary chronic progressive
Primary progressive

Question 14

What factor makes certain people more predisposed to MS?

Answer 14

Living in da cold

Question 15

What is happening in Devic’s disease?

Answer 15

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)

Question 16

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

Answer 16

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

Question 17

what permits chronic inflammation

Answer 17

Low-toxicity organisms

Question 18

What cell is characteristic of a granuloma

Answer 18

Macrophages in the form of a giant cell

Question 19

What responds to infectious organisms

Answer 19

astrocytes and microglia

Question 20

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

Answer 20

Children. Favorable outcome if treated quickly.

Question 21

Define Osmotic demyelination syndrome

Answer 21

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

Question 22

What causes Progrssive multifocal leukoencephalopathy (PML)?

Answer 22

JC virus, it causes infection of oligos

Question 23

Define demyelinating

Answer 23

loss of myelin

Question 24

Define Dysmyelinating

Answer 24

cannot form appropriate myelin

Question 25

The little boy with discolored finger nails had what disease?

Answer 25

Adrenoleukodystrophy (ALD) AKA Addison's

Question 26

Name some neurologic manifestations of ALD

Answer 26

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

Question 27

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

Answer 27

Rosenthal fibers

Question 28

What are some symptoms of infantile Krabbe's disease

Answer 28

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

Question 29

What are the cause of HIE in infants?

Answer 29

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

Question 30

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

Answer 30

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

Question 31

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

Answer 31

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

Question 32

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

Answer 32

Cerebral palsy

Question 33

What pathology finding will be associated with PVL?

Answer 33

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

Question 34

What clinical signs are shown in degenerative diseases

Answer 34

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

Question 35

What are the 6 common pathologic factors in neurodegenerative diseases

Answer 35

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

Question 36

Where are plaques found in Alzheimers

Answer 36

In deposits outside and around the neurons

Question 37

What are Plaques

Answer 37

Beta amyloid fragments that clump together

Question 38

What are Neurofibrillary tangles

Answer 38

paired helical filaments

Question 39

What are Neurofibrillary tangles often referred to as and why

Answer 39

Ghost tangles, due to the death of the host neuron

Question 40

What do Tau deposits cause

Answer 40

Impairment of axonal transport

Question 41

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

Answer 41

Primary lesions are due to Aß deposition

Question 42

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

Answer 42

Periventricular leukomalacia (PVL)

Question 43

What is the important cause of PVL?

Answer 43

Ischemia | Involving premyelinating oligodendrocyte that populate in the white matter

Question 44

Clinical symptoms of PVL

Answer 44

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

Question 45

Where does Germinal Matrix Hemorrhage start?

Answer 45

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

Question 46

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

Answer 46

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

Question 47

Why does Kernicterus develop?

Answer 47

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

Question 48

What pathological finding are in Status Marmoratus?

Answer 48

Marbled appearance of the thalamus and basal ganglia

Question 49

What is the most common cause of Picks disease

Answer 49

Mutated tau protein

Question 50

What clinical features are shown in picks disease

Answer 50

severe atrophy of frontal and temporal lobes

Question 51

What are pick bodies

Answer 51

straight filaments and paired helical filaments

Question 52

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

Answer 52

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

Question 53

What are some of the Parkinson symptoms

Answer 53

rigidity, small step gait, slow movement, tremors

Question 54

What pathologically causes Parkinsons

Answer 54

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

Question 55

what are the 5 possible ways to acquire parkinsonism

Answer 55

Drugs, vascular, toxins, infections, trauma

Question 56

Describe dementia with Lewy bodies

Answer 56

Parkinsonism and dementia combined. causes cognitive deficits and hallucinations

Question 57

What is Huntington disease

Answer 57

Degeneration of caudate, putamen, and the cortex

Question 58

What is "anticipation" when dealing with Huntingtons

Answer 58

When the disease appears in younger ages and more severely

Question 59

what is Progressive Supranuclear Palsy (PSP)

Answer 59

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

Question 60

What are the symptoms of PSP

Answer 60

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

Question 61

What is ALS

Answer 61

Fatal degenerative disorder of upper and lower motor neurons

Question 62

ALS clinical symptoms

Answer 62

affects Middle aged individuals, Muscle weakness, Spasticity Incontinence

Question 63

What is Friedreich's ataxia (FRDA)

Answer 63

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

Question 64

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

Answer 64

relapsing inflammatory demyelinative disease that causes transverse myelitis and optic neuritis