Krafts Diseases and Pearls Flashcards Preview

Hailey - Neuro > Krafts Diseases and Pearls > Flashcards

Flashcards in Krafts Diseases and Pearls Deck (87):
1

cells of CNS

neurons
astrocytes
oligodendrocytes
microglia
ependymal
(last 4: glial)

2

astrocytes Fx

"cell that does the most"
most important glial cells
star shaped, long processes (rosenthal processes)

big responders to injury
provide glucose for brain, ONLY source

3

oligodendrocytes Fx

myelin

4

microglia Fx

macrophages

5

ependymal cell fx

produce CSF
line ventricles

6

stain used to show astrocytes and their processes

silver stain

7

astrocyte vs oligodendrocyte

1. astrocytes bigger
2. astrocytes open chromatin pattern
3. ogd chromatin black dot
4. ogd in lacunae/space

8

ependymal cells have

cilia (motion)
microvilli (absorption)

9

when acute CNS injury, think

red neurons

10

associate red neurons with

acute injury
loss of blood flow
"red is dead"

11

inclusion bodies are

reactions of neurons to injury
by axonal spheroids
accumulations within nucleus or cytoplasm of neurons

12

cowdry a

herpes
"owl's eye"

13

negri

rabies

14

neurofibrillary tangles

AD
silver stain, see accumulation of amyloid

15

lewy bodies

PD

16

gliosis

astrocyte proliferation
*important sign of infection
is like scarring in the brain

17

gemistocytic astrocytes

happy, make more proteins

18

inclusions in astrocytes*

rosenthal fibers (processes of astrocytes, thick, tumor, metabolic disease, nonspecific injurious process going on)

corpora amylacea (pearl like, light blue, due to aging)

19

microglial nodules

dead tissue, foreign substance that needs to be chewed up by microglia

20

genetic metabolic disease shows up when...

birth*
missing enzyme that primarily affects CNS --> build up --> lose function

21

genetic metabolic diseases

neuronal storage disease (storage disorders in CNS neurons)
leukodystrophies (affect white matter)

22

acquired metabolic diseases

vitamin deficients
metabolic and toxic disturbances
later in life*

23

NSDs

diagnose by clinical exam, labs, common look of neuron

1. Neuronal ceroid lipofuscinoses: accumulation of lipofusin --> neuronal dysfxn. blindness, motor probe, seizures

2. Tay-Sachs Disease: Ashkenazi Jews, deficiency in HEX-A, hexosaminidase A --> breaks down ganglioside (GM2) (a "food" for neurons). baby seems normal at birth, doesn't meet development milestones (6-8mo), motor impair, paralysis, loss near fxn, death by age 4, cherry red spots

24

Tay-Sachs Disease

-Ashkenazi Jews
-deficiency in HEX-A
-hexosaminidase A --> breaks down ganglioside (GM2 type, a "food" for neurons)
-baby seems normal at birth, doesn't meet development milestones (6-8mo)
-cherry red spots (macula sticks out)*
-motor impair
-paralysis
-loss neurological fxn
-death by age 4

25

cherry-red spots

whole retina contains neurons stuffed with gangliosides that are white, so entire retina becomes palsied, white ---> makes macula more red

26

Leukodystrophies

myelin abnormalitis (ogds, not neurons)

1. Krabbe Disease
-galactosylceramidase deficiency
-accumulation of galactocerebrosie --> TOXIC to OGDs --> loss of myelin --> axons conduct poorly
-globoid cells: fat macrophages stuffed full of the toxic metabolite, released as OGD dies ****does see in other disorders
-motor

27

difference between NSDs and LDs?

LDs: deterioration of motors skills, spasticity, hypotonia, ataxia (less cognitive issues)

28

Krabbe Disease hallmarks

1. globoid cells
2. motor
3. galactosylceramidase deficiency --> accum galactrocerebroside (TOXIC)

29

B1 deficiency

Thiamine deficiency
-chronic alcoholism, starvation
--> Wernicke/Korsakoff

30

First stage B1 deficiency (in CNS)

Wernicke encephalopathy
mammillary bodies starved --> hemorrhage, necrosis, eaten up by microglia
EARLY AND REVERSIBLE (give thiamine)
1. confusion
2. ophthalmoplegia
3. ataxia

31

Second stage B1 deficiency

Korsakoff syndrome
mammillary bodies atrophied
NOT REVERSIBLE
1. Wernicke encephalopathy
2. memory disturbances
3. confabulation

32

B2 deficiency

cobalamin
needed to make DNA
anemia
subacute combined degeneration of spinal cord

33

Subactue combined degeneration of SC

B12 deficiency
motor and sensory tracts loss myelin (ascending and descending)
numbness, ataxia, weakness REVERSIBLE
paraplegia IRREVERSIBLE
*distribution dorsal column, and dosrsolateral columns

34

Toxins affect CNS

carbon monoxide
methanol
ethanol

35

CO

confusion, disoriented -->
delirium -->
coma

goes to CNS first*
particularly: hippocampus, parking cells, cortex
high affinity for Hb (won't allow proper O2 delivery)
pink discoloration of tissues**

36

Methanol CH3OH

30-100ml fatal
4ml cause blindness
prefers to go to ganglionic cells in retina

37

Ethanol CH3CH2OH

prefers to go to cerebellum (middle --> ataxia)
acute effects REVERSIBLE
ataxia, nystagmus
Bergmann gliosis
--> cerebellar atrophy

38

Bergmann gliosis

proliferation of astrocytes collect where purine cells should be

39

Peripheral Nerve Diseases

neuropathies
1. Guillian-Barre Syndrome
2. Hansen's Disease
3. VZV
4. Charcot-Marie-Tooth Disease
5. Peripheral Neuropathy in Diabetes
6. Neuropathies associated with malignancy

40

Guillian-Barre Syndrome

= Acute Inflammatory Demyelination Polyradiculoneuropathy

-ascending parasthesia
-flu-like illness prior
-immune-mediated (T cells and macrophages cause segmental demyelination*** after having attacked virus)
-resolves over time
-plasmaphoresis: remove ab, seem to get better
-respiratory therapy if paralysis into lungs

41

clinical course of Guillian-Barre

symmetric ascending paralysis > rapid-onset weakness > loss deep tendon reflexes > loss of sensation > elevated CSF protein > 2-5% mortality from respiratory paralysis, autonomic instability, cardiac arrest

42

Hansen's Disease

slowly progressive infection of skin and nerves
Mycobacterium leprae
most of world is naturally immune
endemic in poor tropical countries
disabling deformities

1. tuberculoid leprosy (less severe, skin disorder, nerve degeneration - anesthesia, ulcers)

2. lepromatous leprosy (more sever, skin, nerves, eye, mouth, tests, hands, feet - immune system doesn't respond well to kill pathogen)

43

Tuberculoid leprosy

nice T cell response to make enough granulomas, decrease pathogen
Mycobacterium leprae
skin rash*

44

Lepromatous leprosy

leonine facies*
autoamputation*
Mycobacterium leprae
immune system doesn't respond as well

45

VZV

common viral inf of PNS
dormant in DRG, cord, or brainstem
neuronal destruction --> pain
"dew drop on a rose petal"
dermatomes
multinucleate giant cells

46

most common hereditary neuropathy

Hereditary Motor and Sensory Neuropathy Type 1 = Charcot-Marie-Tooth disease

47

Charcot-Marie-Tooth disease

de-myelination then re-myelination*
muscle loss, sensory loss but pain intact**
childhood/early adulthood
autosomal dominant
pes cavus (high arch), foot drop, hammer toes, muscle atrophy
normal life span

48

Peripheral Neuropathy in Diabetes

too much sugar in blood > glucose stick to peripheral nerves > irreversible connections with cells
autonomic neuropathy (difficult bladder control) or asymmetric neuropathy (involve one nerve)
*symm M and S neuropathy in distal
decreased pain sensation > ulcers, injury > no heal > amputations
*stocking glove distribution

49

Neuropathies associated with malignancy

1. neoplasms: metastatic, mononeuropathy (push on nerve)
eg. lung pressing on brachial plexus, obturator palsy from pelvic neoplasms, cranial nerve palsies from brain tumors

2. paraneoplastic effects: look like hormone effects, polyneuropathy (tumor production of substances/antibodies that start reacting with nerves)
eg. small cell lung cancer, plasma cell malignancies

50

Skull fractures

Linear
Depressed
Diastatic
Basal

51

Linear skull fracture

most COMMON
STRAIGHT crack
usually not serious

52

Depressed skull fracture

bone displaced INWARD
comminuted (in pieces)
can damage brain

53

Diastatic skull fracture

across a SUTURE
suture widens
usually in children

54

Basal skull fracture

more force required
distant hematoma (battle sign behind ear*, raccoon eyes*)
CSF drainage from nose or ear*

55

frontal bone fracture

patient unconscious, couldn't stop self from falling on face

56

Parenchymal injuries

1. Concussion
2. Second-impact Syndrome
3. Chronic traumatic encephalopathy
4. Direct Parenchymal injury
5. Diffuse axonal injury
6. Traumatic vascular injury

57

Concussion

altered consciousness from head injury due to change in momentum of head (stretching and snapping back of brain)

Sx: amnesia, confusion, HA, visual disturb, N/V, dizzi

58

Second-impact Syndrome

rare
CATASTROPHIC: brain swells rapidly
second concussion before first healed

59

Chronic traumatic encephalopathy

multiple conconsions
life changing effect on person/personality
progressive degenerative disease
histologically looks like AD: tau protein

Sx: progressive dementia, poor judgement, apathy depression, memory loss, confusion, aggression

60

Direct Parenchymal injury

laceration (eg. knife wound)
contusion (bruising)
coup/contrecoup injury

61

Diffuse Axonal injury

rotational acceleration, torque side to side > shearing of axons
injury to axons in deep white matter
coma (can exist)
eg. shaken baby syndrome, boxing

62

Direct parencymal vs concussion

direct parenchymal: can point to lesion in one localized area

63

Traumatic vascular injury

epidural hematoma
subdural hematoma
subarachnoid hemorrhage

64

epidural hematoma

above dura
SURGICAL EMERGENCY
rupture middle meningeal artery*
contours are smooth: lens on MRI/CT "tough mother, dura is so tough"

ex: baseball hit to the temple, lucid period*

65

subdural hematoma

between dura and arachnoid
shearing of cerebral veins*
contours are not smooth

ex: elderly patient falls, ripped veins as as they run through dura. 1. acute (hours) numbness one side, 2. chronic (months) numb and personality changes

66

subarachnoid hemorrhage

in arachnoid space
NEUROSURGICAL EMERGENCY
actual bleed cerebral arteries*
contusions, ruptured berry aneurysms*
(40% ACA, 20% internal carotid, 34% MCA branches, 4% PCA)

ex: "worst headache I have ever had"

67

ICP increased in

brain swelling/inflammation
hematoma
rumors
hydrocephalus
increased CSF

68

cerebral edema

1. vasogenic
cause: leaky vessels/inc vascular permeability
space between cells
localized or generalized

2. cytotoxic
cause: cell membrane injury (hypoxia or metabolic damage > messes channels in/out of cells)
space within cells: cells r swole

often occur together whenever cerebral edema

69

local vasogenic cerebral edema tx

1. steroid: dexamethasone (reduce water permeability of tight junctions)
2. mannitol: osmotic diuretic, will draw water out of CSF into blood

70

cytotoxic tx

no known treatment

71

hydropcephalus

accumulation of excessive CSF within ventricular system

infancy > head enlarges (sutures aren't closed)
adulthood > ventricles expand, increased ICP

72

Five kinds hydrocephalus

1. communicating
2. non-communicating
3. ex vacuo
4. increased CSF production
5. normal pressure

73

Non-communicating

blockage in one way circulation
eg. congenital malformation, mass, collection of blood/hematoma

74

Communicating

block exit of CSF into sinus
eg. resolving meningitis

75

Craniopharygioma

blocks CSF, pituitary oma, rare

76

Ex vacuo

no block in ventricular system
degenerative
BRAIN ATROPHY > RESULTING HYDROCEPHALUS
dilated ventricular system

77

Choroid Plexus Papilloma

increased CSF production?, rare

78

Normal pressure

Triad: wet, wobbly, wacky
1. urinary incontinence
2. magnetic gait
3. dementia
REVERSIBLE
no cortical atrophy, ventricles enlarge
can be confused with AD
*something blocked CSF by some arachnoid blockage, CSF couldn't get out, then somehow, pressure normalizes*

79

How differentiate normal pressure hydrocephalus from AD?

Dementia is different. NP H the gait is unusual, patient doesn't really care about incontinence issues (frontal lobe changes, apathy)

80

Tx hydrocephalus

goal: reduce fluid volume and pressure
1. surgery, shunt to drain CSF
2. decrase CSF production with acetazolamide (CA inhibitor) or furosemide (NKCC inhibitor)

81

critical enzyme: carbonic anhydrase

converst CO2 and water to H ion and bicarbonate
block CA > reduce HCO3 > less transport of Cl and Na > less production of CSF

SE: reduced stomach acid production, more sodium release, increased urine output, many

82

increased ICP

mass effect (edema, tumor, hematoma)
compromised blood flow to brain > pain, papilledema, decreased consciousness
feared outcome: herniation

83

common herniations

1. subfalcine hernation
2. transtentorial hernation
3. tonsilar hernation

84

herniation Sx

focal neurological symptoms
brainstem symptoms: respiratory and cardiac arrest, coma
often fatal

85

subfalcine herniation

cingulate gyrus pushed under falx cerebri
compresses ACA

86

transtentorial uncinate hernation

uncus through tentorium
compresses:
-CN III (fixed, dilated pupil, ophthalmoplegia)
-brainstem (rip blood vessels, duret hemorrhages) FATAL
-PCA (ischemia in 1* visual cortex)
-contralateral cerebral peduncle (hemiparesis)

87

tonsilar hernation

cerebellar tonsils through foramen magnum
compresses medulla (compromises vital resp and cardiac centers)
LIFE THREATENING