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Robbins Pathology Neuro (Chpter 28,29) > Chapter 28-The CNS > Flashcards

Chapter 28-The CNS Flashcards

1
Q

What vulnerability do neurons in the brain regions show and what is meany by it

A

Selective vulnerability, as some parts of the brain are more susceptible to different agents than others due to different NT used, locations, etc

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2
Q

What is the state of cell division in mature neurons

A

Incapable

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3
Q

What is the classical sign of an acute neuronal injury and when are the seen

A

Aka red nucleus, seen 12 to 24 hours after hypoxia/ischemic event

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4
Q

What event is characterized by shrinkage of cell body, pyknosis of nuclear strength, dissapreance of nucleoli, loss of Nissl substance, and intense eosinophilia

A

Acute neuronal injury

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5
Q

Which event is characterized by red nucleus

A

Acute neuronal injury

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6
Q

What condition is characterized by reactive gliosis

A

Subacute or chronic neuronal injury/degeneration

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7
Q

What is axonal reaction and where is it commonly seen

A

Change in the cell body during regeneration of the axon, most commonly seen in the anterior horn

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8
Q

What are neuronal inclusions

A

Manifests with aging and are intracytoplasmic accumulation of complex lipids, proteins, and carbs

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9
Q

When are Cowdry bodies seen

A

Intranuclear inclusions seen in herpetic infection as a result of viral infection

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10
Q

When are Nehru bodies seen

A

Cytoplasmic inclusions seen during rabies infection

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11
Q

When are neurofibrillary tangle seen

A

Alzheimer’s

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12
Q

When are Lewi bodies seen

A

Parkinson’s

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13
Q

When is abnormal vacuolization of the perikaryon and neuronal cell process in the neurophil seen

A

CJD

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14
Q

What is the most important histopathological indicator of CNS injury

A

Gliosis

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15
Q

What is gliosis characterized by

A

Hyperplasia and hypertrophy of astrocytes

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16
Q

What protein is characteristic of astrocytes

A

Glial fibrillary acidic protein (GFAP), an intermediate filament

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17
Q

What is the function of astrocytes

A
  • Metabolic buffers and detoxifier of the brain

- Barrier function on control flow of macromolecules between the blood, CSF and brain

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18
Q

What is the morphological feature of gliosis

A

Astrocyte’s nucleus become enlarged, vesicular, and prominent nucleoli

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19
Q

What astrocyte is characterized by changes to grey matter cell with a large nucleus, pale staining central chromatin, intranuclear glycogen droplet, and a prominent nuclear membrane and nuceolus

A

Alzheimer’s type 2 astrocyte

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20
Q

Which conditions cause Alzheimer type 2 astrocyte changes

A
  • Long standing hyperammonemia in chronic liver disease
  • Wilsons disease
  • Hereditary disorders in the urea cycle
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21
Q

What proteins are contained in the Rosenthal fibers

A

Heat shock protein alphabeta-crystallin
Heat shock protein hsp27
Ubiquitin

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22
Q

Rosenthal fibers are indicative of what

A

Pilocytic astrocytoma

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23
Q

Where are Rosenthal fibers usually found

A

Regions of long standing gliosis

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24
Q

Alexanders disease is associated with mutations in which gene

A

Encoding GFAP

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25
Q

IN Alexander’s disease, where are Rostenthal fibers found

A

Periventricular, verivascular, and subpial locations

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26
Q

Corpora amylacea are commonly found in which condition

A

Aka polyglucosan bodies found in Alexanders disease

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27
Q

Lafora bodies are commonly found with which location

A

Myoclinic epilepsy

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28
Q

What is the response of microglia to injury

A
  • Proliferation
  • elongated nuclei (rod cells)
  • forming aggregates around foci of necrosis (microglial nodules)
  • congregating around cell bodies of dying neurons (neuronophagia)
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29
Q

How many neurons do oligodendrocytes usually myelinate

A

Multiple neurons as opposed to the 1 to1 of Schwann cells

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30
Q

What is a feature of acquired demyelination game disorders and leukodystrophies

A

Injury to oligodendrocytes

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31
Q

What is harbored in oligodendrocytes in progressive multifocal leukoencephalopathy

A

Viral inclusions

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32
Q

What is found in multiple system atrophy (MSA)

A

Glial cytoplasmic inclusions (aka oligodendrocytes with alpha-synuclein)

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33
Q

What is found in oligodendrocytes in multiple system atrophy (MSA)

A

Alpha-synuclein

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34
Q

Damage to ependymal cells result in what

A

Ependymal granulations, which are proliferation of sub ependymal astrocytes

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35
Q

What infectious agents in particular causes extensive ependymal injury

A

CMV

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36
Q

What is vasogenic edema

A

Increase in ECF by blood brain barrier disruption and increased Vascular permeability. This results in fluid going from intravasular compartment to intercellular spaces

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37
Q

What is cytotoxic edema

A

Increased in ECF secondary to neuronal injury, resulting in the prevention of maintaining normal membrane gradients

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38
Q

What is the physical result of edema

A

Gyro are flattened, sucking are narrowed and ventricular cavities are compressed

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39
Q

What is hydrocephalus

A

Accumulation of CSF in the ventricular system

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40
Q

What is the most common cause of hydrocephalus

A

Impaired flow and resorption of CSF

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41
Q

What is noncommunicating hydrocephalus

A

aka obstructive hydrocephalus, and the ventricular system does not communicative with the subarachnoid space

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42
Q

What usually causes a noncommunicating hydrocephalus

A

Blockage of the third ventricule due to a mass

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43
Q

What is hydrocephalus ex vacuo

A

Enlarging of the ventricular space due to loss of the brain parenchyma

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44
Q

What occurs during a subfalcine/cingulate herniation

A

Unilateral or asymmetrical expansion of the cerebral hemisphere where the circular gyrus herniated under the falx

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45
Q

What structures can become compressed during a subfalcine/cinguate herniation

A

Anterior cerebral artery and its branches

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46
Q

What occurs during a transtentorial herniation

A

Medial aspect of the temporal lobe is compressed against tentorium

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47
Q

Which structures are affected during transtentorial herniation

A
  • Third cranial nerve (pupil dilation and loss of EOM on side of lesions)
  • posterior cerebral artery and ischemia to primary visual cortex
  • Kernohan notch
  • Duret hemorrhage’s
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48
Q

What is kernohan notch

A

Contralateral cerebral puduncle is compressed, resulting in ipsilateral hemiparesis

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49
Q

What are duret hemorrhages

A

lesions in the pons and midbrain. In linear or flame shape due to tearing of veins and arteries supplying the upper brainstem

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50
Q

What accounts for the most CNS malformations and what is the most common structure involved

A

Neural tube defects, with most involving the spinal cord

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51
Q

What is spinal dysraphism

A

Aka spina bifida, which is disorganized segments of spinal cord. Associated with overlying meningeal out pouching

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52
Q

What is a myelomeningocele

A

Extension of the CNS tissue through a defect in the vertebral column

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53
Q

Where do most myelomenigoceles occur

A

Lumbosacral regions

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54
Q

What is an encephalocele

A

Brain tissue extending through a defect in the cranium (commonly the posterior fossa)

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55
Q

What is the rate of recurrent neural tube defects in subsequent pregnancies

A

4-5%

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56
Q

When does folate supplementation need to occur to be affective

A

First 28 days

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57
Q

What is an anencephaly

A

Malformation in the anterior end of the neural tube with the absence of brain and calvarium

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58
Q

During anencephaly, what is area cerebrovascusa

A

Disrupted forebrain development, resulting in disorganized brain tissue with mixed ependymal, choroid plexus, and meningothelial cells

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59
Q

What is the path of migration for those cells that are to become excitatory neurons

A

Radial migration

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60
Q

What is the migration pattern for those neurons that are to become inhibitory neurons

A

Tangential

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61
Q

Fo radial migration, which protein is secreted

A

Aka for excitatory neurons, reelin is secreted to migrating neuroblast

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62
Q

What is Lissencephaly

A

Reduction in the number of gyri

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63
Q

What is the most extreme case of lissencephaly

A

Agyria or complete lack of gyri

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64
Q

Which type of lissencephaly is associated with smooth surface

A

Type 1

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65
Q

Type 1 lissencephaly is associated with which mutation

A

Disruptions in signaling for migration and cytoskeleton motor proteins that drive migration

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66
Q

Which type of lissencephaly is associated with rough or cobblestones

A

Type 2

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67
Q

Type 2 lissencephaly is associated with what mutations

A

Genetic abnormalities that disrupt the stop signal for migration

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68
Q

What is polymicrogyria

A

Small unusually numerous irregular formed cerebral convolutions

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69
Q

What are neuronal heterotopias assoacited with

A

Epilepsy

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70
Q

What are periventricular heterotopias commonly caused by

A
  • Mutations in coding for filamin A. (Acting binding protein in assembly of meshwork)
  • Doublecortin (DCX) microtuble associated protein
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71
Q

Filamin A gene is located in what location and what is the result in each gender

A

On X chromosome

  • Male-lethal
  • Female-periventrular heterotopia
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72
Q

Where is the gene for DoubleCortin protein (DCX) located

A

X chromosome

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73
Q

What is the result of a mutation in DCX gene in each gender

A

Male-lissencephaly

Female- subcortical band heterotpias

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74
Q

What is holoprosencephaly

A

Incomplete separation of the cerebral hemisphere across the midline

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75
Q

What are common malformation seen as part of a holopresencephaly

A
  • cyclopia

- arrhinencephaly (absence of olfactory cranial nerves)

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76
Q

What is holoprosencephaly associated with

A

Trisomy 13

Mutations in sonic hedgehog

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77
Q

What is the radiological finding in agenesis of corpus callosum

A

“Bat wings” as a result of misshapen lateral ventricle

-bundles of anteropoteriorly oriented white matter

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78
Q

What are the characteristics of Arnold-Chiari type 2 malformation

A
  • Small posterior fossa
  • misshapen midline cerebellum with downward vermi through foramen magnum
  • variable, but hydrocephalus and lumbar myelomeningocele
  • Caudal displacement of medulla, tectum, aqueductal stenosis
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79
Q

What are the characteristics of Chiari type 1 malformation

A

Less severe than type 2

-Low lying cerebellar tonsils extend down into the vertebral canal

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80
Q

What are the characteristics of a Dandy Walker malformation

A
  • Enlarged posterior fossa
  • Cerebellar vermis is absent or barely present
  • Large midline cyst as a expanded roofless fourth ventricle
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81
Q

What is commonly found in assoacition with Dandy-walker malformation

A

Dysplasia of the brain

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82
Q

What is Joubert syndrome

A

Hypoplasia of the cerebellar vermis with elongation of the superior cerebellar peduncles

  • altered shape of brain stem
  • molar tooth sign
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83
Q

What is the radiological sign seen in Joubert syndrome

A

“Molar tooth sign”

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84
Q

What is the common cause of Joubert syndrome

A

Mutations in genes coding primary (non-motile) cilium

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85
Q

What is hydromylia

A

Expansion of ependymal lined central canal of cord

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86
Q

What is a syringomyelia aka syrinx

A

Fluid filled cleft like cavity in the inner portion of the spinal cord

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87
Q

What is a syringobulbia

A

Fluid filled cleft like cavity extending into the brainstem

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88
Q

Syringomyelia are associated with which conditions

A
  • Chiari malformations
  • intraspinal tumors
  • traumatic injury
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89
Q

What are the clinical presentations of syrinx

A

Isolated loss of pain, temperature and in the upper extremities due to involvement of the anterior commisure

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90
Q

What is cerebral palsy

A

Nonprogressibe neurological motor deficit with ataxia, spasticity, paresis as a result of injury during the prenatal and perinatal period

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91
Q

What condition is characterized by ataxia, spasticity, paresis

A

Cerebral palsy

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92
Q

Which population is at a higher risk for intraparenchymal hemorrhage

A

Premature infants

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93
Q

Where are parenchyma hemmorages commonly occurring

A

In the terminal matrix near the junction between developing thalamus and caudate nucleus

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94
Q

Which population is at a higher risk for periventricular leukomalcias

A

Aka supratentorial periventricular white mater

Premature infants

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95
Q

How to periventricular leukomalacias present

A

Chalky yellow plaques in regions of white matter necrosis and calcification

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96
Q

What is multicystic encephalopathy

A

White and grey matter involvement in ischemic damage resulting in cystic lesions throughout the hemisphere

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97
Q

During perinatal ischemic lesions of the cerebral cortex, where is most of the damage seen

A

Depths of the sulci, resulting in thinned out gliosis gyri

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98
Q

What is ulegyria

A

Thinned out and gliotic gyri as a result of ischemic lesions

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99
Q

What is status marmoratus

A

the marble like appearance of the deep nuclei as a result of an ischemic event in the sulci of the cerebral cortex

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100
Q

What is the order of events in an ischemic event of the cerebral cortex

A
  • Ulegyria (thinned out sulci)

- Status marmoratus (marbled like appearance)

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101
Q

What are commonly seen symptoms following damage the cerebral cortex

A

Movement disorders (choreoarthetsis) due to damage to putamen, caudate, and thalamus

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102
Q

What is a displaced skull fracture

A

Fracture of the bone is displaced into the cavity greater than the thickness of the bone

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103
Q

What is a diastic fracture

A

Fractures that cross suture lines

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104
Q

Which portion of the brain is most susceptible to concussion and physical injury

A

Crests of the gyri

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105
Q

What is a coup brain injury

A

The brain injury is on the same side as the impact

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106
Q

What is a counter coup brain injury

A

Injury is on the opposite side of the impact

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107
Q

What is the common injury if the head was immobile at the time of the impact

A

Coup

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108
Q

How long does the morphological signs of brain trauma take to appear

A

24 hours

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109
Q

What are plaque Jaune and what do the signify

A

They are depressed, retracted, yellowish brown patches at the crest of the gyri, commonly at the countercoup sites of injury.

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110
Q

What is diffuse axonal injury characterized by

A

Widespread, often asymmetrical axonal swelling within hours

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111
Q

What is the stain of choice for diffuse axonal injury

A

Immunoperoxidase stains for amyloid precursor protein and alpha-synuclein

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112
Q

Why are older patients more prone to subdural hematomas following injury

A

Brain atrophy with age causes bridging veins to stretch and easy torn

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113
Q

What occurs in the first week of acute subdura hematomas

A

Lysis of the close

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114
Q

What occurs during the second week of acute subdural hematomas

A

Growth of fibroblasts from the Dural surface into the hematoma

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115
Q

What occurs during 1-3 months following acute subdural hematoma

A

Early development of the hyalinized connective tissue

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116
Q

What are the morphological signs seen in an acute subdural hematoma

A

Freshly clotted blood along the brain surface without the extension into the sulci

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117
Q

What portion of the brain are subdural hematomas commonly seen

A

Lateral cerebral hemispheres, bilateral in 10% of pts

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118
Q

What is the cause of post traumatic hydrocephalus

A

Obstruction of CSF absorption from hemorrhage into the subarachnoid space

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119
Q

What is the cause of chronic traumatic encephalopathy

A

Aka CTE or Demetria pugilistica

From repeated trauma and blows to the head

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120
Q

What do brains in patients with CTE look like

A

Atropine, enlarged ventricles, accumulation of tau proteins, involving the frontal and temporal lobe

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121
Q

How does cerebral vascular disease rank on leading causes of death

A

Third

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122
Q

What are the two forms seen in cerebrovascular disease

A
  • Hemorrhage (rupture of blood vessel in CNS)

- hypoxia, ischemia, and infarction (impaired blood flow to CNS)

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123
Q

What is released and the process of neuronal cell death

A

Excitatory amino acids (Glut), leading to NMDA receptors and the influx of calcium

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124
Q

What is the penumbra

A

At risk area that is the transition area from the necrotic tissue to normal brain tissue

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125
Q

When does global cerebral ischemia occur

A

Generalized reduction in cerebral perfusion (cardiac shock/arrest, severe hypotension

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126
Q

What are the most sensitive cells in the CNS

A

Neurons

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127
Q

What are the most sensitive neurons in the brain

A
  • Pyramidal cell layer in the hippocampus (CA1 aka Sommer sector)
  • Cerebellar purkinje cells
  • pyramidal cells in cerebral cortex
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128
Q

What is the Sommer sector

A

The hypoxic sensitive pyramidal neurons in the hippocampus

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129
Q

What are watershed infarcts

A

Areas most distant to arterial blood supply, usually between two sources of arterial blood

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130
Q

Where in the cerebral hemisphere is most sensitive to arterial blood disruptions

A

Between anterior and middle cerebral artery

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131
Q

When are border zone infants usually seen

A

Following Hypotensive episodes

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132
Q

What is the common exact location of watershed infarcts seen

A

Sickle shaped band of necrosis over the cerebral convexity just lateral to the interhemispheric fissure

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133
Q

What are the early changes (12-24 hours)seen in brain ischemia

A
  • microvasculization
  • eosinophilia of neuronal cytoplasm
  • nuclear pyknosis and karyorrhexis
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134
Q

What are the subacute changes seen in ischemic events of the brain

A

24 hour to 2 weeks

  • tissue necrosis
  • influx of macrophages
  • vascular proliferation
  • reactive gliosis
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135
Q

What is pseudolaminar necrosis

A

Neuronal loss and gliosis of the cerebral neocortex is uneven, where there is preservation of some layers, yet destruction of others

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136
Q

When does a focal cerebral inshemic event occur

A

Reduction of blood flow to a localized area of the brain due to arterial occlusion or hypoperfusion

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137
Q

What is the most common emboli leading to focal cerebral ischemia

A

Cardiac mural thrombi > atheromatous plaques in carotid

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138
Q

What are the predisposing conditions for an embolism

A
  • MI
  • Valvular disease
  • Arterial fibrillation
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139
Q

Which artery territory is most at risk for an embolism

A

Middle cerebral artery (off of internal artery)

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140
Q

What are thrombolism occlusion most commonly associated with

A

-Athersclerosis, hypertension, diabetes, and plaque ruptures

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141
Q

What are the most commonly locations for a thrombolism

A

Carotid bifurcation, origin of MCA, basilar artery

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142
Q

What are the common causes of infectious vasculitis that leads to thrombi

A
  • TB
  • syphilis
  • immunosuppression
  • opportunistic infections
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143
Q

What is the correlation between polyarteritis nodosa and infarcts of the brain

A

A noninfectious Vasculitis causing an infarct

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144
Q

How do secondary hemmorages of the brain occur and there are their characteristics

A
  • Occurs due to ishechmia-reperfusion injury due to collateral flow or fragmentation of intravascular occlusive material
  • Results in petechial hemorrhage
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145
Q

In nonhemorrhagic infarcts, what is the extent of appearance changes in the first 6 hours

A

No changes

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146
Q

What is the extent of appearance changes in nonhemorrhagic infarcts at time of 48 hours

A

looks pale, soft, swollen, and corticomedulary Junction becomes indistinct

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147
Q

Following an nonhemorrhagic infarct, what is the microscopic appearance after 12 hours

A

Ischemic neuronal changes with both cytotoxic and vasogenic edema

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148
Q

Following an nonhemorrhagic infarct, what is the microscopic appearance after 48 hours

A

Neutrophilic emigration increases, then falls off

-monocyte derived cells become the dominate cell type until week 3

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149
Q

Following an nonhemorrhagic infarct, what is the microscopic appearance of reactive astrocytes

A

Can be seen 1 week following the infarct

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150
Q

Which conditions increases the risk for hemorrhagic infarcts

A

Carcinoma
Localized infections
Anything leading to hypercoagability

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151
Q

What is a main cause of lacunar infarcts

A

Hypertension

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152
Q

What are the characteristics of a lacunar infarct

A

Cave like infarct less that 15 mm wide

-Usually the deep vessels of the brain

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153
Q

What is the location of lacunar infarcts from most common to lesser

A 
1-Lenticular nucleus 
2-thalamus 
3-internal capsule
4-deep white matter
5-caudate nucleus
6-pons
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154
Q

What is etat crible

A

Widening of the perivascular space without tissue infarction

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155
Q

What condition causes slit hemorrhages

A

Hypertension

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156
Q

What are the characteristics of slit hemorrhages

A

Small hemorrhages surrounded by focal tissue destruction and pigment laden macros

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157
Q

What is a acute hypertensive encephalopathy

A

Malignant hypertension causes cerebral dysfunction, which causes headaches, confusion, vomiting, and convulsions

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158
Q

What is binswanger disease

A

Brain injury due to lack of blood flow that is preferentially the subcortical white matter with myelin and axon loss

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159
Q

Which population is at the highest risk for intraparenchymal hemorrhages

A

Middle to late adult life, peaking at 60 years old

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160
Q

What are the 2 main causes of ganglionic and lobar hemorrhages

A

Hypertension

Cerebral amyloid hemorrhages

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161
Q

What is the main risk factors assoacited with deep brain parenchyma hemorrhages

A

Hypertension

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162
Q

Which deep structure hemorrhages are commonly damaged as a result of hypertension

A

Deep white/grey matter
Brainstem
Cerebellum

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163
Q

What are Charcot Bouchard microaneurysms and where are they commonly occurring

A

Hypertension caused minute aneurysms, commonly seen in the basal ganglia

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164
Q

What is the most common location of a hypertensive intraparenchymal hemorrhage

A

Putamen (50 to 60% of cases)

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165
Q

Which condition is most commonly associated with lobar hemorrhages

A

Cerebral amyloid angiopathy (CAA)

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166
Q

The presence of which allele increases the risk of bleeding due to CAA

A

Epsilon 2 or 4

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167
Q

CAA is usually restricted to which vessels

A

Leptomeningeal and cerebral cortical arterioles and capillaries

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168
Q

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is associated with mutations in which gene

A

Notch3

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169
Q

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a disease of dysfunction in which structures

A

Vascular smooth muscle

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170
Q

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) results in which events

A

Repeat strokes

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171
Q

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) will result in which finding in the arteries of the CNS

A

Concentric thickening of media and adventitia, loss of smooth muscle cells, basophilic, PAS positive deposits

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172
Q

What is the most common cause of clinically significant subarachnoid hemorrhage

A

Rupture of a saccular “berry” aneurism in a cerebral artery

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173
Q

What is the most common intracranial aneurysm

A

Saccular, aka Berry’s

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174
Q

Where are most saccular aneurysms located

A

About 90% in the anterior portion of circulation

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175
Q

When is a rupture of an aneurysm most likely to occur

A

Acute increases in intracranial pressure, especially in the 5th decade of life

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176
Q

What is the most common type of clinically significant vascular malformation

A

Arteriovenous malformations (high pulsating blood flow)

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177
Q

What is fox-alajouanine malformation

A

Venous angiomatous of the spinal cord, most commonly in the lumbosacral region. It is associated with ischemic injury to spinal cord and progressive neuro symptoms

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178
Q

Which population is more likely to develop a vascular malformation

A

Males are twice as likely

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179
Q

What is the most common site for arterialvenous malformations in the brain

A

Middle cerebral artery, in particular their posterior branches

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180
Q

What is the most common cause of bacterial meningitis in neonates

A

Strep B

E. Coli

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181
Q

What is the most common cause of meningitis in older individuals

A
  • Step pneumonia

- listeria

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182
Q

What is the most common cause of meningitis in adults

A

Neisseria meningitidis

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183
Q

What are the symptoms of meningitis

A
  • irritations and neuro impairment
  • headache
  • photophobia
  • stiff neck
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184
Q

What is the typical spinal tap result from someone with bacterial meningitis

A

Increased protein concentration

Decreased glucose concentration

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185
Q

What is the Waterhouse Friderichsen syndrome

A

Meningitis associated septicemia with hemorrhagic infarction of the adrenal gland

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186
Q

Where is the purulent exudate commonly found in meningitis caused by H. Influenza

A

Basal

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187
Q

Where is the purulent exudate commonly found in meningitis caused by pneumococcal bacteria

A

Cerebral convexities

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188
Q

What is the condition of chronic adhesive arachnoiditis

A

Pneumococcal meningitis infections lead to capsular polysaccharide production resulting in a gelatinous exudate that promotes fibrosis

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189
Q

What are the CSF finding in aseptic meningitis

A
  • lymphocytic pleocytosis (increased amount)
  • Moderate protein elevation
  • Normal glucose
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190
Q

When can an asymptomatic like meningitis arise without there actually being an infection of any kind

A

Rupture of a epidermis cyst into the subarachnoid space or introduction of a chemical irritant. There will still be neutrophils in the CSF, along with normal glucose and slightly elevated protein levels

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191
Q

What is a brain abscess

A

Necrosis of brain tissue along with inflammation, commonly due to a bacterial infection

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192
Q

What are predisposing conditions for a brain abcess

A

Acute bacterial endocarditis
Congenital heart defects with right to left shunting
Loss of pulmonary filtration of organisms
Chronic pulmonary sepsis (example: bronchiectasis)
Systemic infection (usually due to immunosuppression)

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193
Q

What is the CSF concentration in patients with a brain abscess

A

High white count
Increased protein concentration
Normal glucose levels

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194
Q

What are the most common causes of brain abscess

A

Staphy and streptococci

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195
Q

What leads to a subdural empyema

A

Infections of the skull bones or air sinuses that spread to the subdural space

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196
Q

What is the process of complications following a subdural empyema

A

Produces a mass effect or thrombophlebitis of the bridging veins that cross the subdural space, resulting in venous occlusion and infarction

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197
Q

What is the CSF concentration of subdural empyema

A

Similar to brain abcess with:

  • High white count
  • Normal glucose
  • Elevated protein
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198
Q

What are extramural abscesses associated with

A

Osteomyelitis, usually arising from an sinisitis or surgical procedure

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199
Q

What are the clinical presentations of a patient with TB meningitis

A

Headache, malaise, mental confusion, vomiting

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200
Q

What is the CSF profile of TB

A

Pleocytosis of monocytes, sometimes neutrophils are included
Elevated protein
Reduced or normal glucose

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201
Q

What is the most serious complication associated with chronic TB meningitis

A
  • Arachnoid fibrosis producing hydrocephalus
  • obliterating endarteritis resulting in arterial occlusion
  • infarction of brain
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202
Q

What is the most common pattern of TB involvement in the brain

A

Meningoencephalitis

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203
Q

What are the contaminates seen in the arachnoid space during TB meningitis

A

-gelatinous or fibrinous exudate involving the base of the brain

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204
Q

What are the clinical presentations of Neurosyphilis

A
  • Menigovascular syphilis
  • paretic neurosyphilis
  • tabes dorsalis (loss of coordinated movement)
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205
Q

What is meningovascular neurosyphilis

A

Chronic meningitis involving the base of the brain, and sometimes obliterating endarteritis with perivascular inflammation

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206
Q

What is paretic neurosyphilis

A

Invasion of the brain resulting in damage to the frontal lobe with loss of neurons, proliferation of microglia, gliosis, and iron deposits

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207
Q

What is tabes dorsalis

A

Loss of sensory axons in the dorsal roots, resulting in ataxia, loss of pain, joint damage, “lightning pains”, and loss of deep tendon reflexes

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208
Q

What are the general symptoms seen in patients with Arbor virus encephalopathy

A

Seizures, confusion, delirium, stupor, coma, reflex asymmetries and ocular palsies

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209
Q

What are the characteristics of the brain changes seen in Arboviruses

A

Perivascular accumulation of lymphocytes, neuronophagia (engulfing of necrotic neural debris, and microglial nodules

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210
Q

HSV 1 encephalitis occurs in which age group

A

Children and young adults

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211
Q

HSV 1 encephalitis will present with which symptoms

A

-alterations in mood, memory and behavior

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212
Q

Which mutation population are at a higher risk for HSV1 encephalitis

A

TLR3 signaling mutations

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213
Q

Where does meningitis caused by HSV 1 begin

A

Inferior and medial regions of Temporal lobe, and the orbital gyri of frontal lobes

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214
Q

What is found in the neurons and glia during an HSV 1 encephalitis

A

-Cowdry type A inclusion bodies

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215
Q

Which population is commonly affected by CMV and what does it cause

A

Subacute encephalitis in immunocompromised individuals

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216
Q

Where does the CMV tend to accumulate and what is the result

A

Accumulates in the paraventricular subependymal regions of the brain, where it causes severe hemorrhaging and necrosis of the ventricles and choroid plexus

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217
Q

Where is the target location for polio virus

A

Anterior horns of the motor neuron

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218
Q

How does the CSF present with poliomyelitis

A

Same as aseptic meningitis

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219
Q

Which infection is characterized by the presence of Negri bodies

A

Rabies infection

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220
Q

What is the pattern of infection during a rabies infection

A

Ascending, starting with the peripheral nerves of the wound, and moves into the CNS (1 to 3 months)

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221
Q

What is the diagnostic clinical symptoms leading to rabies

A

-Local paresthesias around the wound, along with malaise, fever, headache

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222
Q

What are the clinical symptoms of a severe and late infection of rabies

A

CNS excitability with extreme movements and exaggeration of pain on even the slightest of touches

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223
Q

What is the only CNS cell type that contains both targets for HIV and what are they

A

Microglia contain both CCR5 and CXCR4

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224
Q

What is immune reconstitution inflammatory syndrome and what infection is it associated with

A

Patients with AIDS that have received treatment, deteriorate due exacerbation of symptoms from opportunistic infections

225
Q

With HIV encephalitis, what are the histological findings

A

Widely distributed microglial nodules with multinucleated giant cells

226
Q

What is progressive multifocal leukoencephalopahy (PML) caused by

A

JC virus

227
Q

How does the JC virus cause PML

A

JC virus targets the oligodendrocytes, leading to demyelination

228
Q

Which patient population is at risk for PML caused by JC virus

A

Immunosuppressed

229
Q

What are the histological characteristics of Progressive Multifocal Leukoencephalopathy (PML)

A

Patches of irregular, ill defined white matter injury, usually demyelination in the subcortical area

230
Q

What are the characteristics of the lesions in PML

A

Scattered laden macrophages with enlarged oligodendrocyte nuclei containing glassy amphophilic viral inclusions

231
Q

What are the clinical signs of subacute sclerosing panencephalitis (SSPE)

A

Cognitive decline, spasticity of limb, and seizures

232
Q

Subacute sclerosing panencephalitis is commonly seen months to years after which initial infection

A

Measles

233
Q

How is SSLE characterized in the brain

A

Gliosis, degeneration of myelin, viral inclusions in the oligodendrocyte nuclei

234
Q

What do the viral inclusions in SSLE contain

A

Nucleocapsids characteristic of measles, with the measles virus antigen being present

235
Q

What are the three main forms of fungal infection in the CNS

A
  • Chronic meningitis
  • Vasculitis
  • parenchymal invasion
236
Q

Which structures are affected during cryptococcal meningitis

A

Basal leptomeninges, which can obstruct the foramine of Luschka and Magendie causing hydrocephalus

237
Q

Which infection is characterized by “soap bubbles”

A

Cryptococcal infection

238
Q

What are the clinical presentations of an infection with malaria

A

Reduction in cerebral blood flow, ataxia, seizures, coma, and long term cognitive deficits

239
Q

What is the conformational change that is seen in prions that lead to pathogenesis

A

Change from the alpha helix isoform (PrPc) to beta sheet (PrPsc)

240
Q

Mutations in which gene have shown in familiar forms of prion diseases

A

PRNP

241
Q

Which codon number and amino acids influence the chances of obtaining a prion disease

A

Homozygous for either Met or Val at codon 129

242
Q

Mutations in which gene are responsible for CJD

A

PRNP

243
Q

What is a clinical symptom of CJD

A

Startle myoclonus (involuntary muscle jerking upon sudden stimulation)

244
Q

What is the distinguishing feature of vCJD

A

No alteration in PNRP, only 129 codons affected, but has the precedence of cortical plaques surrounded by halo of spongiform change

245
Q

What is fatal familial insomnia (FFI) caused by

A

Mutations in the PRNP gene leading to an asparagine to an aspartate change at residue 178 along with MET at 129

246
Q

What disease is caused by a PRNP mutation to an aspartate at 178 along with a methionine are 129

A

FFI

247
Q

What disease is caused by a PRNP mutation with an aspartate change at 178 with a valine at 129

A

CJD

248
Q

What is the change in pathology that separates FFI from other prion diseases

A

Neuronal loss and reactive gliosis in the anterior ventral and dorsomedial thalami nuclei, along with the inferior olivary nuclei

249
Q

What are leukodystrophies

A

Inherited disorders that affect synthesis or turnover of myelin components

250
Q

What is the most common demyelination disorder

A

Multiple sclerosis

251
Q

What is MS characterized by

A

Autoimmune demyelination due to white matter lesions

252
Q

What is the rare of cases of MS between genders

A

Women are twice as likely to acquire it

253
Q

What is the normal clinical cycle involved in MS

A

Relapsing and remitting episodes of impaired neurological function, followed by a time of partial recovery

254
Q

How is MS correlated with genetics

A

Very with 15x more likely if a dizygotic twin with 150x more likely with monozygotic

255
Q

Which genetic component is relegated with in increase in MS

A

DRB1*1501, with each acquired copy increasing the risk by 3x

256
Q

What is the immune mechanism of MS

A

Initiated by TH1 and TH17, followed by the production of IFNgamma and subsequent inflammation, macrophage and lymphocyte recruitment follow

257
Q

Where do the plaques in MS generally tend to accumulate

A

Adjacent to the lateral ventricles, optic nerve/chiasm, brain stem

258
Q

During MS, what are the characteristics of an active plaque

A

Ongoing myelin breakdown associated with abundant macrophage containing PAS debris

259
Q

During MS, what are the characteristics of an inactive plaque

A

Little to no myelin found, reduced number of oligodendrocytes, but an increased gliosis and astrocyte proliferation

260
Q

What are shadow plaque indicative of

A

During MS, where there is partial and incomplete regeneration of myelin from the surviving oligodendrocytes

261
Q

What is the common initial clinical manifestation of MS

A

Unilateral visual impairment due to involvement of the optic nerve

262
Q

What is the make up of the CSF during MS

A
  • Mild elevation of protein

- IgG levels elevated

263
Q

What are the clinical presentations of Neuromyelitis optica (NMO)

A

Synchronous bilateral optic neuritis and spinal cord demyelination

264
Q

Which gender is more commonly affected by Neuromyelitis optica (NMO)

A

Females

265
Q

What is the characterizing feature of Neuromyelitis optica

A

Antibodies against aquaporin 4

266
Q

What is present in the CSF during NMO

A

White cells, including neutrophils

267
Q

What are the clinical symptoms of acute disseminated encephalomyelitis

A

Monophasic demyelination disease characterized by rapid onset of headache, lethargy, and coma

268
Q

What is the ultimate outcome of acute disseminated encephalomyelitis

A

Death in 20%, the rest recover completely

269
Q

When does acute disseminated encephalomyelitis occur

A

Week or 2 after viral infection or viral immunization

270
Q

What is are the characteristics of acute necrotizing hemorrhagic encephalomyelitis

A

CNS demyelination affecting young adults and children, resulting in death or severe deficits

271
Q

When does acute necrotizing hemorrhagic encephalomyelitis occur

A

Following a recent upper respiratory infection

272
Q

What are the characteristics of pathogenesis of Central Pontine Myelinolysis

A

Acute disorder with demyelination of Basis Portis and pontine integument

273
Q

When does central pontine myelitis occur

A

2 to 6 days after rapid correction of hyponatremia, but possible with other electrolyte disturbances

274
Q

What disease is osmotic demyelination disorder

A

Central pontine myelinolysis

275
Q

What is the characteristics of damage to cells in central pontine myelinolysis

A
  • Absence of inflammation, neurons and axons well preserved

- All lesions are at the same stage of myelin loss and reaction

276
Q

What are the clinical symptoms seen in central pontine myelinolysis

A

Rapidly evolving quadriplegia

-“locked in syndrome”(fully conscious but unresponsive”)

277
Q

What is the pathological process that is common across most of the neurodegenerative diseases

A

Accumulation of protein aggregates aka proteinopathy

278
Q

What is the location that tau protein will accumulate

A

Produced intracellular lay and stay Extracellular

279
Q

Where does the protein AB in Alzheimer disease (AD) accumulate

A

Neuropil

280
Q

What is the critical initiation event for the development of Alzheimer disease

A

Accumulation of AB protein

281
Q

Which genomic abnormality and condition is associated with AB protein and Alzheimer disease

A

APP, which is located on chromosome 21, so Down syndrome is commonly associated with AD

282
Q

What is the result if the APP is cleaved at the alpha secretaries site

A

Soluble fragment is produced and non amyloidosis portion

283
Q

What is the result when the APP in AD is cleaved at the beta secretary site

A

Generates the AB protein that forms the plaque

284
Q

How do the different cleavage site for APP protein differ in size

A

AB 42 is the protein that starts the formation of plaques

285
Q

In the cleavage of APP, what are the components of gamma secretary complex

A
  • presenilin
  • nicastrin
  • Pen-2
  • Aph-1
286
Q

In AD, what is the location of PS1

A

Chromosome 14

287
Q

In AD, what is the location of PS2

A

Chromosome 1

288
Q

What is the process of tau becoming an inclusion

A

There is the development of tangles, which causes a shift to somatic-dendritic distribution, hyperphosphorylated and can no longer bind to microtubules

289
Q

Which pathogenesis of AD correlates well with the degree of dementia

A

Number of neurofibrillary tangles

290
Q

During the course of AD, where in the brain is the effect seen

A

Cortical atrophy and widening of the cerebral sulci in the frontal, temporal, and parietal lobes

291
Q

What is hydrocephalus ex vacuo

A

The widening/enlargement of the ventricles as a result of brain atrophy

292
Q

Where are plaques during AD normally seen

A

Hippocampus, amygdala, neocortex

293
Q

What are diffuse plaques and where are they found

A

Deposition of AB plaques found in the basal ganglia and cerebral cortex that have the amyloid characteristics but lack the surrounding neurotic processes

294
Q

What are the characteristics of the neurofibrillary tangles

A

Tau containing bundles that are in pyramidal neurons that have an elongated flame shape or rounded “globose” tangles

295
Q

What is an almost invariable accompany of AD

A

Cerebral amyloid angiopathy, which are AB40 plaques

296
Q

What is the common terminal event that will kill a patient with AD

A

Intercurrent disease, usually pneumonia

297
Q

What are the FTLDs locations of damage

A

Aka frontotemporal lobar degenerations, so the frontal and temporal lobes

298
Q

How are FTLDs distinguished from Alzheimer’s disease

A

Alterations in personality, behavior and language (aphasia) proceeds memory loss

299
Q

Picks disease is classified under which neurodegenerative disease

A

FTLD-tau

300
Q

What is the diagnostic feature for Pick disease and subsequent FTLD-tau

A

-Pick bodies, which are smooth contoured inclusions, along with severe atrophy of the frontotemporal lobes

301
Q

IN FTLD, which layers are generally seen to have the most severe neuron loss

A

Outer three layers

302
Q

What is the most common genetically caused FTLD-TDP

A

Expansion of hexanucleotide repeat in the 5’ UTR of C9orf72

303
Q

Which diseases are associated with mutations in the 5’ UTR of C9orf72

A

ALS

FTLD-TDP

304
Q

Which of the FTLD genetically caused conditions are not also associated with ALS

A

Gene encoding progranulin

305
Q

What is the result of mutations in the gene coding progranulin in FTLD-TDP

A

Loss of function at progranulin, which gets cleaved to help contain inflammation

306
Q

Which morphology is strongly associated with FTLD-TDP

A

Presence of needle like NII’s and progranulin mutations

307
Q

What is the general cause of Parkinson’s disease

A

Loss of dopaminergic neurons in the substantia nigra

308
Q

What are the clinical symptoms of Parkinson Disease

A

Diminished facial expressions (aka masked facies), stooped posture, slowing of voluntary movement, festinating gait posture, rigidity and “pill rolling” tremor

309
Q

What is the triad of Parkinsonism

A

Tremor, rigidity, and bradykinesia

310
Q

Acute Parkinsonian syndrome with destruction of neurons in the substantia nigra can can be elicited by ingestion of which compound

A

MPTP with use of illegal meperidine

311
Q

What is the diagnostic hallmark of Parkinson disease

A

Lewy body

312
Q

What is the location of gene mutation in Parkinson Disease leading to alpha-synuclein aggregation

A

4q21

313
Q

Where do Lewy bodies first appear in PD

A

Medulla and then ascend

314
Q

In Parkinson Disease, what is the inheritance pattern of mitochondrial dysfunction

A

Autosomal recessive

315
Q

Mitochondrial dysfunction in Parkinson Disease is assoacited wth mutations in genes coding which proteins

A

DJ-1
PINK1
Parkin

316
Q

What is the inheritance of mutations in LRRK2 causing parkinson disease

A

Autosomal dominant

317
Q

What is the mutation that causes autosomal dominant Parkinson disease

A

LRRK2

318
Q

What is the characteristic finding of parkinson disease

A
  • Pallor of the substantia nigra and locus ceruleus

- Lewy body

319
Q

What are the clinical presentations of progressive supranuclear palsy (PSP)

A

Parkinson Disease symptoms plus:

  • truncal rigidity
  • disequilibrium
  • difficulty with voluntary eye movements
  • Nuchal dystonia
  • pseudobulbar palsy
320
Q

What gender is at a higher risk for developing progressive supranuclear palsy

A

Men 2x likely

321
Q

What is a pathological hallmark of progressive suprafacial palsy

A

Tau-containing inclusions in neurons and glia

322
Q

What are the symptoms of corticobasal degeneration (CBD)

A

Parkinson disease symptoms plus:

  • extrapyramidal rigidity
  • jerking of limbs
  • impaired higher cortical function (aka apraxia)
323
Q

How is CBD and PSP differentiated from one another in the location of tau

A

CBD-cerebral cortical

PSP-brainstem and deep grey matter

324
Q

What are the morphological features of corticobasal degeneration

A
  • “Ballooned” neurons (aka neuronal achromatia)
  • Atrophy of motor and premotor
  • Severe loss of neurons and gliosis
325
Q

What is multiple system atrophy (MSA) characterized by

A

Cytoplasmic inclusions of alpha-synuclein in oligodendrocytes

326
Q

What distinguished multiple system atrophy (MSA) from other neurodegerative diseases

A

Observed in glial cells and degeneration of white matter tracts without the presence of inclusions in neurons

327
Q

What are the three tracts involved in Multiple System Atropy (MSA) and the result f their degeneration.

A

1-striatonigral circuit (Parkinsonism)
2-olivopontocerebellar circuit (ataxia)
3- ANS (orthostatic hypotension)

328
Q

What is the hereditarabilty of Huntington disease

A

Autosomal dominant

329
Q

What are the classical presentation signs for Huntington disease

A

Progressive movement disorders and dementia

330
Q

What is the general cause of Huntington disease

A

Degeneration of striatal neurons

331
Q

What are the repeats seen in Huntington Disease

A

CAG repeats coding for a poly glut sequence

332
Q

What is the location and name of the gene associated with Huntington Disease

A

Huntington protein coded by HTT on chromosome 4p16.3

333
Q

When is the repeat sequence in Huntington Disease usually occuring and what is the term used for it

A

Anticipation- repeat sequence is expanded during spermatogenesis, so the onset is early

334
Q

What is the morphology of the brain in Huntington Disease

A

Brain is small and shows atrophy of the striatal neurons in the caudate nucleus and frontal lobe

335
Q

What is the direction of pathological changes seen in Huntington disease

A

Medial to lateral in caudate

Dorsal to ventral in putamen

336
Q

Which set of neurons are especially prone to injury in Huntington Disease

A

Medium sized spiny neurons that primarily use gamma-aminobutyric acid at the NT, but also enkephalin, dynorphin, and substance P

337
Q

Which set of neurons seem to be spared in Huntington Disease

A
  • diaphorase positive neurons with nitric oxide synthase

- large cholinesterase-positive neurons

338
Q

What is choreoathetosis

A

Increased motor output leading to jerky movements

339
Q

What aspect of Huntington disease is associated with CAG repeats

A

The onset of the disease, so the longer the sequence the early the disease onset

340
Q

Patients with Huntington disease have a higher risk of which condition

A

Suicidal thoughts

341
Q

What are the heritability of spinocerebellar ataxias (SBA)

A

Autosomal dominant

342
Q

What are the spinal cerebellar ataxias associated with poly glutamate (CAG) repeats

A

1,2,3 (machado-Jones disease), 6,7 (includes visual impairment), 17, dentatorubropallidoluysian atrophy (DRPLA)

343
Q

What are the spinocerebellar ataxias associated with expansion of non coding region repeats

A

8,10,12,31,36

344
Q

What is the heritability of Fredreich Ataxia

A

Autosomal recessive

345
Q

What are the general clinical presentations of Friedreich ataxia

A

Progressive ataxia, spasticity, weakness, sensory neuropathy, and cadiomyopathy,

346
Q

What disease is charactertized by gait ataxia, followed by hand clumsiness and dysarthria in the first decade of life. Loss of deep tendon reflexes (extensor plantar reflex present). Loss of joint position and vibration sense.

A

Fredreich ataxia

347
Q

In Fredreich ataxia, what is the effect on reflexes

A

Deep tendon are lost, but extensor plantar remains

348
Q

Most individuals with friedrich ataxia develop which conditions

A

Pes cavus

Kyphoscoliosis

349
Q

What is the relation of friedreich ataxia and CV

A

Increased risk of cardiomyopathy with arrhythmias and congestive heart failure. Heart may be large with pericardial adhesions

350
Q

What is the cause of Fredreich ataxia

A

GAA trinucleotide repeat in the first intron on chromosome 9q13

351
Q

Which protein is mutated in Friedreich ataxia and what is its function

A

-frataxin which assembles iron into complex 1 and 2

352
Q

What are the morphological findings in Friedreich ataxia

A

Loss of axons and gliosis in posterior columns, corticospinal tracts and spinocerebellar tracts

353
Q

Which neuron are degenerated in friedreich ataxia

A
  • Spinal column (Clarke column)
  • brain stem (CN 8,10,12)
  • cerebellum (dentate nucleus)
  • Betz cells of motor cortex
354
Q

What is the inheritabilty of ataxia-telangiectasia

A

Autosomal recessive

355
Q

What are the clinical symptoms of ataxia-telangiectasia

A

Early in childhood, shows ataxia/dyskenetic Syndrome, followed by telangiectasia of conjunctiva and skin, along with immunodeficiency

356
Q

What is the protein mutated in ataxia-telangiectasia and what is its location

A

Ataxia-telangiectasia mutated (ATM) on 11q22-q23

357
Q

What is the role of ataxia-telangiectasia mutated (ATM) gene

A

Codes a protein used in fixing double stranded DNA breaks

358
Q

Which disease shows enlargement of nucleases to 2 to 5 times the size (aka amphicytes), while the LNs, thymus and gonads are hypoplastic

A

Ataxia-telangiectasia

359
Q

What is generally the first symptom in patients with ataxia-telangiectsia

A

Recurrent sinopulmonary infections (due to immunosuppression) and unsteadiness in gait

360
Q

Which condition is normally developed in ataxia-telangiastia

A

Lymphoid neoplasms, in particular T cell leukemias

361
Q

What is the pathogenesis of amyotrophic lateral sclerosis (ALS)

A

Loss of upper and lower motor neurons in the cerebral cortex with evidence of protein accumulation

362
Q

What is the inheritability of amyotrophic lateral sclerosis (ALS)

A

Autosomal dominant

363
Q

What is the most common form of amyotrophic lateral sclerosis

A

A4V mutation

364
Q

What is the characteristic course of illness in the most common form of amyotrophic lateral sclerosis

A

A4V mutation with rapid and rarely involvement of upper motor neurons

365
Q

Which mutation commonly gives rise to ALS and FTLD

A

C9orf72

366
Q

What is the morphology seen in ALS

A

Anterior roots in spinal cord are atrophied due to loss of lower motor nerves

367
Q

Which disease contains Bunina bodies, which are remnants of autophagy vacuoles

A

ALS

368
Q

What is the result of losing upper motor neurons in ALS

A

Loss of the corticospinal tracts

369
Q

What are the early symptoms seen in amyotrophic lateral sclerosis

A

Weakness of hands (dropping things), cramping/spasticity of arms and legs

370
Q

What is the commonly recurring infection and what is the cause

A

Recurrent pulmonary infections due to loss of respiratory muscles

371
Q

What is progressive musclular atrophy

A

Uncommon ALS cases that have lower motor neurons predominating

372
Q

What is primary lateral sclerosis

A

ALS cases where the upper motor neurons predominate

373
Q

What are the last motor neurons that are involved in ALS

A

Ocular eye motor neurons

374
Q

What is progressive bulbar palsy

A

Aka bulbar ALS

-degeneration of the lower brainstem Cranial motor nerves early and very rapidly

375
Q

What is the name of Kennedy Disease

A

Aka spinal and bulbar Muscular Atrophy

376
Q

What is the clinical presentation of Kennedy disease

A

Distal limb amyotrophy and bulbar signs, such as atrophy of the tongue and dysphasia
-Androgen insensitivity

377
Q

What is commonly seen as the result of the androgen insensitivity seen in Kennedy Disease

A
  • gynecomastia
  • testicular atrophy
  • oligospermia
378
Q

What is Kennedy Disease caused by

A

X linked poly Glutamine repeats

379
Q

What are the clinical presentations of spinal muscular atrophy

A

Marked loss of lower motor neurons resulting in progressive weakness

380
Q

What is the most severe type of spinal muscular atrophy

A

SMA type 1, aka Werdnig-Hoffman disease

381
Q

What is the course of illness with Type 1 SMA

A

Onset within the first year and death within 2

382
Q

What is the course of illness in Type 3 SMA

A

Aka Kugelberg-Welander disease

-Presents in late childhood or adolescence with motor disabilities

383
Q

What is the severity of spinal muscle atrophy related to

A

The level of SMN protein on chromosome 5q

384
Q

What is the function of the SMN protein that correlates with SMA

A

Assembly of the spliceosome

385
Q

What is the inheritability of neuronal storage diseases

A

Autosomal recessive

386
Q

What are the enzyme mutations unable to do in neuronal storage diseases

A

Catabolize:

  • sphongolipids
  • mucopolysaccharides
  • mucolipids
387
Q

In neuronal storage diseases, what is usually accumulating in the cell

A

The missing enzyme’s substrate

388
Q

What are leukodystrophies

A

Mutations in genes that code for enzymes that are involved in the myelin synthesis or catabolism

389
Q

What is the inheritability of leukodystrophies

A

Autosomal recessive

390
Q

What is the general result of leukodystrophies

A

White matter deterioration leading to ataxia, loss of motor, spasticity, hypotonia

391
Q

Which portion of the brain is more affected by defects in mitochondria

A

Grey matter (due to high ATP in neurons)

392
Q

What are the general results from neuronal storage diseases

A

Loss of cognitive function and seizures

393
Q

What are certified lipofuscinoses

A

Disorders in which lipid pigments accumulate in neurons

394
Q

What is the result of ceroid lipofuscinoses

A

Blindness, cognitive and motor deteriation, and seizures

395
Q

What relative age groups are prone to leukodystrophies

A

Younger ages

396
Q

Which genetic metabolic disease does Krabbe disease fall under

A

Leukodystrophies

397
Q

What is the inheritability of Krabbe disease

A

Autosomal recessive

398
Q

What is the enzyme causing Krabbe disease

A

Deficiency in galactocerebroside Beta-galactosidase (aka galactosylcerimidase)

399
Q

What product building up in Krabbe disease

A

Galactocerebroside is not proven down, so shunted into the toxic form of galactosylsphingosine

400
Q

What is the clinical presentation of Krabbe disease

A

Rapid progression of motor signs of stiffness and weakness

401
Q

When does Krabbe disease present and what is the survival rate

A

Presents between 3 and 6 month, leads to death by age 2

402
Q

What does the brain morphologically look like in Krabbe disease

A

Loss of myelin and oligodendrocytes in the CNS and peripheral nerves

403
Q

What is the diagnostic feature of Krabbe disease

A

Engorged macrophages (aka globose cells) in the brain parenchyma and blood vessels

404
Q

What is the inheritability of metachromatic leukodystrophy

A

Autosomal recessive

405
Q

What is the deficiency in metachromatic leukodystrophy

A

Lysosomal enzyme arylsulfatase A

406
Q

What is the product building up in metachromatic leukodystrophy

A

Sulfatides, especially cerebroside sulfate

407
Q

What is the pathology of metachromatic leukodystrophy

A

Sulfatides build up, leading to inhibition of oligodendrocyte differentiation, also eliciting an immuno response from the microglia

408
Q

Histologically, what is diagnostic about metachromatic leukodystrophy

A

Macrophages with vacuolated cytoplasms that are scattered in the white matter and contain crystalloid structures composed of sulfatides

409
Q

What is the inheritability of Adrenoleukodystrophy

A

X linked recessive

410
Q

What mutation is associated with Adrenoleukodystrophy

A

ABC protein D1 (aka ABCD1)

411
Q

What is the result of the mutation seen in Adrenoleukodystrophy

A

Loss of ABCD1, which transports molecules into the peroxisome

412
Q

What is the pathology of Adrenoleukodystrophy

A

Inability to catabolism very long chain fatty acids (VLCFA) within the peroxisomes

413
Q

What are the clinical presentations of Adrenoleukodystrophy seen in males

A

Progressive loss of myelin and adrenal insufficiency (atrophy of adrenal cortex)

414
Q

What is adrenomyeloneurophathy

A

Allergic disorder in adults with progressive peripheral nerve disorder

415
Q

What is the mutation seen in Pelizaeus-Merzbacher disease

A

Myelin formation proteins

416
Q

What is the mutation seen in Alexander disease

A

GFAP

417
Q

What is the mutation in vanishing white matter leukoencephalopathy

A

elF2B

418
Q

What is the condition of heteroplasmy

A

Mixture of normal and abnormal mitochondria

419
Q

In mitochondrial disorders, what can usually be seen

A

CNS targets damage in neurons and grey matter
Increased lactate levels
Decreased cytochrome C oxidase

420
Q

What are the clinical presentation of Mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes (MELAS)

A
  • Recurrent episodes of acute neurological function
  • Cognitive changes
  • Muscle weakness
  • lactic acidosis
421
Q

The stoke like effects seen with MELAS are irreversible or reversible

A

Reversible

422
Q

What is the most common mutation observed in MELAS

A

Gene encoding mito tRNA leucine (MTTL1)

423
Q

How is Myoclonic epilepsy and ragged red fibers (MERRE) transmitted

A

Maternally

424
Q

What are the clinical symptoms of Myoclonic epilepsy and ragged red fibers (MERRE)

A

Myoclonus, seizures, and myopathy (with red ragged muscle fibers), ataxia

425
Q

What is the mutation causing Myoclonic epilepsy and ragged red fibers (MERRE)

A

tRNA other than Leucine

426
Q

What is the age usually affected with Leigh syndrome

A

Infancy, most die within 1 to 2 years

427
Q

What are the clinical symptoms of Leigh syndrome

A

Lactic acidemia, loss of psychomotor development, feeding issues, seizures, extraocular palsy, weakness with hypotonia

428
Q

What are the histological findings in Leigh syndrome

A

Spongiform appearance with proliferation of blood vessels

429
Q

What is the cause of Wernicke encephalopathy

A

Thiamine deficiency

430
Q

What is the prognosis of Wernicke encephalopathy

A

Reversible if given thiamine shortly after signs

431
Q

What are the clinal presentations of Wernicke encephalopathy

A

Acute psychomotor and ophthalmoplegia symptoms

432
Q

What is the cause of Korsakoff syndrome

A

Bilateral destruction of the mammillary bodies

433
Q

What is the clinical presentation of Korsakoff syndrome

A

Loss of short term memory and confabulation

434
Q

What vitamin deficiency can lead to Korsakoff syndrome

A

Thiamine

435
Q

Which conditions can predispose a patient to Korsakoff syndrome

A

Gastric disorders, carcinomas, chronic gastritis, or persistent vomiting (anything that decreases absorption)

436
Q

Lesions on which area of the brain seem to correlate best with memory disturbance seen in Korsakoff syndrome

A

Dorsomedial nucleus of the thalamus

437
Q

What is the cause of subacute combined degeneration of the spinal cord

A

Vitamin B12

438
Q

What are the lesions in subacute combined degeneration of the spinal cord due to

A

Defect in myelin formation due to a lack of B12

439
Q

What are the clinical symptoms of subacute combined degeneration of the spinal cord

A

Bilateral symmetrical numbness, tingling, and ataxia of lower extremities

440
Q

How does the lesion in subacute combined degeneration of the spinal cord differ in early compared to late

A

Starts out at the mid thoracic level, but then spreads to the distal ends of both the ascending and descending tracts

441
Q

Which regions of the brain are prone to damage due to hypoglycemia

A

Large pyramidal neurons in the cerebral cortex

CA1 in the hippocampus

442
Q

What are the changes seen in the brain as a result of hepatic failure

A

Astrocytes with enlarged nuclei and minimal cytoplasm (aka Alzheimer type 2 cells) appear in the cerebral cortex and basal ganglia

443
Q

How does carbon monoxide cause damage to the brain

A

CO binds to the heme in cytochrome C oxidase

444
Q

What area of the brain is prone to damage by carbon monoxide

A

-Kayers 3 and 5 of the cerebral cortex, Sommer sector of hippocampus

445
Q

What is the affect of methanol on the brain

A

Causes retinal ganglion cells to degenerate and cause blindness

446
Q

What part of the brain is affected by chronic alcohol use

A

Vermis of the cerebellum

447
Q

Where do the majority of childhood CNS tumors arise and what is the percent

A

70% in posterior fossa

448
Q

Where do the majority of adult CNS tumors arise and what is the percent

A

70% in the cerebral hemispheres above tentorium

449
Q

What are the most common group of brain tumors

A

Gliomas (includes astrocytes, oligodendrocytes and ependymomas)

450
Q

What is the most common primary brain tumor in adults and what is the percentage

A

Infiltrating astrocytomas (80%)

451
Q

Where do most of the astrocytomas occur

A

The cerebral hemispheres

452
Q

What are the common clinical presentations of infiltrating astrocytomas

A

Seizures, headaches, focal neuro defects at site of infiltration

453
Q

What grade are diffuse astrocytomas

A

2/4

454
Q

What grade are anaplastic astrocytomas

A

3/4

455
Q

What grade are glioblastomas

A

4/4

456
Q

What are the mutations seen in the classic subtype glioblastoma

A
  • PTEN
  • deletions of chromosome 10
  • Amplification of EGFR
  • 9.21 (CDKNA —> p16/INK4a)
457
Q

What mutations are seen with proneural type of glioblastoma

A
  • TP53
  • isocitrate dehydrogenase genes (IDH1 and 2)
  • upregulation of PDGRRA
458
Q

What mutations are present in the neural type glioblastomas

A

NEFL
GABRA1
SYT1
SLC12A5

459
Q

What mutations are seen in mesenchymal type glioblastomas

A

NF1 on chromosome 17
Decreased NF1
Increased TNF and NFKB

460
Q

Which mutations are said to be present in the majority of primary glioblastomas

A

80-90%:

  • activate RAS/PI3 kinase
  • inactivate 53
461
Q

In higher grade astrocytomas, what gene is assoacited with a better prognosis and which one with a worse prognosis

A

Good prognosis-mutated IDH1 (R123H)

Bad prognosis-WT IDH1

462
Q

What is the general prognosis with patients with a glioblastoma

A

Not very good

463
Q

What age group does pilocytic astrocytomas generally occur

A

Children and young adults

464
Q

What is the most common location for a pilocytic astrocytoma

A

cerebellum

465
Q

What is the distinguishing feature of pilocytic astrocytoma from the other astrocytomas

A

Generally benign and tend to lack mutations in TP53

466
Q

What is the location that pleomorphic xanthoastrocytoma is generally found

A

Temporal lobe of children/young adults

467
Q

What is the distinguishing feature of pleomorphic xanthoastrocytoma

A
  • abundant reticulum deposits
  • chronic infiltration of inflammation
  • absence of necrosis or mitotic activity
468
Q

What is the grade of a pilocytic astrocytoma

A

Low (1/4)

469
Q

What is the grade of pleomorphic xanthoastrocytoma

A

Lower (2/4)

470
Q

What area of the brain is prone to oligodendroglioma

A

Cerebral hemispheres, especially the white matter

471
Q

What are the most common mutations in oligodendrogliomas

A

Isocitrate dehydrogenase genes (IDH 1 and 2)-most

Deletions of 1p and 19q

472
Q

In oligodendrocytes, which combination of deletions is correlated with a better prognosis

A

Tumors with codeletions in 1p/19q, while loss of just one is chemo resistant

473
Q

What are the characteristics of anaplastic oligodendrogliomas

A

High cell density, nuclear anaplasia, detectable mitotic activity, and necrosis

474
Q

What is a common clinical symptom is seen in cerebral palsy

A

Choreoathetosis

475
Q

What are ependymomas

A

Tumors arising next to the ependyma-lined ventricular system. Including the central canal of spinal cord

476
Q

When do ependymomas occur in children, and what is the common location

A

In the first 2 decades of life, in the fourth ventricle

477
Q

When do ependymomas occur in adults and what is the common location

A

Commonly seen in patients with neurofibromatosis type 2 (NF2) and seen in the spinal cord

478
Q

What is the common location for an ependymoma if there is a mutation in chromosome 22

A

Spinal cord

479
Q

What chromosome is commonly mutated if there is a ependymoma

A

22 (due to association with NF2)

480
Q

Which tumors commonly for perivascular pseudorosettes

A

Aka tumor cells around vessels

-Ependymoma

481
Q

Expression of which gene is normally expressed in most ependymomas

A

GFAP

482
Q

What is the location that myxopapillary ependymomas develop

A

Film terminale of the spinal cord

483
Q

What is contained in the myxoid areas in myxopapillary ependymomas

A

Neutral and acidic mucopolysaccharides

484
Q

What are the typical clinical location progression in ependymomas

A

Obstruction of the fourth ventricle commonly leading to hydrocephalus

485
Q

Which location of ependymomas have the worst prognosis

A

Posterior fossa

486
Q

What are the characteristics of the subependymomas

A

Slow growing nodules attached to ventricular lining and protrudes into the ventricle. Most commonly found in the lateral and fourth ventricle and are asymptomatic

487
Q

Chorioid plexus papillomas most commonly occur in which patients

A

CHildren

488
Q

What are the characteristics of the colloid cyst of the third ventricle

A

Non-neoplastic enlarging cyst in young adults. Causes noncommunicating hydrocephalus

489
Q

What is the most common neuronal tumor of the CNS

A

Gangliogliomas

490
Q

How do gangliogliomas typically present

A

Superficial lesions with seizures

491
Q

Where are gangliogliomas commonly found

A

temporal lobe

492
Q

Which tumor is said to have “floating neurons”

A

Dysembryopplastic neuroepithelial tumor

493
Q

What is the most common poorly differentiated neoplasm

A

Medulloblastoma

494
Q

What is the location and population where medulooblastomas are seen

A

Cerebellum in children

495
Q

Which form of medulloblastoma has the best prognosis

A

WNT type

496
Q

Which medulloblastoma group has the worst prognosis

A

Group 3

497
Q

What are the characteristics of the WNT type of medduloblastoma

A
  • Mutations in WNT
  • monosomy of chromosome 6
  • nuclear expression of Beta-catenin
498
Q

What are the characteristics of the SHH type medulloblastoma

A
  • Mutations in SHH
  • modular desmoplastic histology
  • MYCN amplification
499
Q

Which patient group is WNT group medulloblastoma seen in

A

Older children

500
Q

Which patient group is SHH group medulloblastoma seen in

A

Infants or young adults

501
Q

Which patient group is group 3 medulloblastoma seen in

A

Infants and children

502
Q

What are the characteristics of group 3 medulloblastoma

A
  • MYC amplification

- isochromosome i17q alteration

503
Q

What are the characteristics of group 4 medulloblastoma

A
  • chromosome i17q alterations

- MYCN amplification

504
Q

Which expression marker is found in most cells in medulloblastoma

A

Ki-67

505
Q

Which condition causes formation of the HomerWright rosettes

A

Medulloblastoma

506
Q

What does the medulloblastoma modular desmoplastic variant show

A

Stroma response marked with reticulum depositis forming “pale islands”

507
Q

What does the medulloblastoma large cell variant show

A

Large nuclei, frequent mitosis and apoptosis

508
Q

How do medulloblastomas normally disseminate

A

Through the CSF, such as to the cuada equina which is known as a “drop metastasis”

509
Q

What is the prognosis of Atypical teratoid/Rhbdoid tumor

A

All before age 5 and survival is less than a year

510
Q

What is the location and rate of spread in atypical teratoid/rhabdoid tumor

A

Malignancy in the posterior fossa and supratentrial compartments

511
Q

What is the cause of atypical/rhabdoid tumors

A

Alterations in chromosome 22, in particular the hSNF5/INI1 coding for the INI1 protein

512
Q

Which patient population are primary CNS lymphomas seen

A

AIDs patients

513
Q

What are the characteristics of primary CNS tumors

A
  • B cell lymphomas
  • Multifocal, but rarely metastasize
  • Rapid and aggressive
514
Q

What is the morphological finding that is typical of a primary CNS Tumor

A

-Cells are separated from one another and form a “hooping” pattern

515
Q

What is the location of primary brain germ cell tumors

A

Along the midline, commonly in the pineal and suprasellar regions

516
Q

Which patient population is commonly affected by primary brain germ cell tumors

A

90% in first two decades

-More common in Japan

517
Q

Metastasis of which germ cell location is commonly seen in the CNS

A

Gonadal tumor referred to as a germinoma in the CHS

518
Q

Which tumor markers will be found in the CSF during a germ cell tumor

A
  • Alphafetoprotein

- Beta-HCG

519
Q

With regards to pineal parenchymal tumors, which form tends to affect children more

A

High grade tumors (aka pineoblastomas)

520
Q

With regards to pineal parenchymal tumors, which form tends to affect adults more

A

Low grade tumors aka pineocytomas

521
Q

How does the pineoblastoma commonly spread

A

Aggressively through the CSF space

522
Q

What is the typical aggressiveness of meningiomas

A

Benign tumors

523
Q

Where do meningiomas typically form

A

Meningothelial cells on the external surface of the brain, within the ventricular system and choroid plexus

524
Q

What predisposes a patient to having a meningiomas

A

Prior radiation to the head and neck decades before

525
Q

What is the typical mutation and gene affected meningiomas

A

Chromosome 22q with the gene NF2 coding for merlin

526
Q

Which condition is commonly seen to have a meningioma

A

NF2

527
Q

In meningiomas with NF2 mutations, what is the common mutation

A

TNF receptor assoacited factor 7 (TRAF7)

528
Q

Which mutation is assoacited with higher graded meningiomas

A

NF2, loss of chromosome 22, and chromosome instability

529
Q

What is the characteristic when meningiomas grow en plaqu

A

Sheetlike fashion over the dura

530
Q

What are the characteristics that define an atypical meningioma

A

4 or more mitosis per higher power field microscopy or at least 3 atypical features

531
Q

What histological finding is consistent with an atypical meningioma

A

Clear cell and chordoided

532
Q

What is the aggressiveness of an anaplastic meningioma

A

Aggressive malignant sarcoma

533
Q

What are the characteristics of the papillary meningiomas and rhabdoid meningiomas

A

High propensity to reoccur

534
Q

Who is likely to get spinal meningiomas and what kind is it

A

10:1 females and are usually psommataous

535
Q

What condition should be considered if meningiomas are present at multiple sites

A

Neurofibramatosis type 2

536
Q

Which receptors are commonly expressed on meningiomas

A

Progesterone, so grow rapidly during pregnancy

537
Q

What kind of cancer is the most common metastatic lesion in the brain

A

Carcinomas

538
Q

What are the five kind of primary sites of metastatic lesions in the brain

A
  • lung
  • Breast
  • melanoma
  • kidney
  • GI
539
Q

What are paraneoplastic syndromes

A

Immune response against tumor antigens that cross react to the CNS

540
Q

Subacute cerebellar degeneration is associated with damage of which cells

A

Purkinje cells, gliosis and mild chronic inflammatory cells

541
Q

Which antibodies are present in subacute cerebellar degeneration

A

PCA1, which cross reacts with purkinje cells

542
Q

What population does subacute cerebellar degeneration occur

A

Women with ovarian, uterine, and breast carcinomas

543
Q

What does limbic encephalitis usually cause in the case of a physician

A

Search for a malignancy somewhere else

544
Q

What does the ANNA-1 antibody in limbic encephalitis cross react with

A

Neuronal nuclei

545
Q

Which tumor is ANNA-1 antibody assoacited with

A

Small cell carcinoma

546
Q

What antibodies are associated with limbic encephalitis

A

ANNA-1
NMDA
VGKC complex

547
Q

What does the NMDA antibody in limbic encephalitis cross react with

A

Hippocampal neurons

548
Q

Which tumor is associated with the NMDA antibody

A

Ovarian tertomas

549
Q

What does the VGKC antibody in limbic encephalitis cross react with

A

Aka voltage gated potassium channel in peripheral nervous system

550
Q

Eye movement disorders, especially opsoclonus, are associated with which tumor

A

Neuroblastoma

551
Q

Cowden syndrome is associated with which mutation

A

PTEN mutation affecting PI3K pathway

552
Q

Li-Fraumeni Syndrome is associated with which mutations

A

TP53

553
Q

Which tumor is Chowdrey syndrome associated with

A

Dysplasia gangliogliocytoma

554
Q

Which tumor is Li-fraumeni associated with

A

Medulloblastomas

555
Q

What tumor is turbot syndrome associated with

A

Medulloblastomas

556
Q

Which mutation is seen in Turcot syndrome

A

APC

557
Q

Which tumor is associated with Gorlin syndrome

A

Medulloblastoma

558
Q

Which mutation is associated with Gorlin syndrome

A

Mutations in PTCH gene

Robbins Pathology Neuro (Chpter 28,29) (5 decks)

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