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Path 2 > Block 3 - CNS > Flashcards

Block 3 - CNS Flashcards

1
Q

___ aggregates are seen in Alzheimer’s, Pick disease, and progressive supranuclear palsy

Microtubule associated protein

Soluble in monomeric form

Insoluble fibrillary aggregates escape degradation and form neurofibrillary tangles

In some patients, the enzyme that cleaves the protein can be altered ( familial AD and PD)

A

tau

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2
Q

___ aggregates seen in Parkinson’s, dementia with lewy bodies, and multiple system atrophy

A

synuclein

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3
Q

____ aggregates seen in Alzheimer’s and cerebral amyloid angiopathy

A

beta-amyloid

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4
Q

___ protein aggregates seen in CJD, vCJD, FFI, and animal diseases

A

prion

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5
Q

Type of degeneration seen in alzheimer’s

A

diffuse cortical

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6
Q

type of degeneration seen in diffuse lewy body disease

A

diffuse cortical

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7
Q

degeneration seen in frontotemperol dementias

A

diffuse cortical

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8
Q

type of degeneration seen in parkinson disease

A

midbrain/brainstem

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9
Q

type of degeneration seen in progressive supranuclear palsy

A

midbrain/brainstem

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10
Q

type of degeneration seen in huntington disease

A

caudate nucleus

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11
Q

type of degeneration seen in amyotrophic lateral sclerosis

A

motor neurons

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12
Q

chromosome associated with beta-amyloid precursor protein in Alzheimer’s disease

A

21

(alzheimer’s increased in Down syndrome)

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13
Q

gene associated with presenilin 1, associated with Alzheimer’s

A

14q24.3

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14
Q

gene associated with presenilin 2, associated with Alzheimer’s

A

1q31-q42

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15
Q

gene associated with tau, associated with Alzheimer’s

A

17q21.1

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16
Q

____ plaques as seen in alzheimer’s

A

amyloid

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17
Q

____ ____ of Tau protein as seen in alzheimer’s disease

A

neurofibrillary tangles

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18
Q

Superficial cortical dark spots denote ____ deposition in Alzheimer’s

A

hemosiderin

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19
Q

Parkinson’s disease involves the deposition of ____ inclusions (Lewy bodies) with progressive loss of neurons, visible grossly in the substantia nigra.

A

synuclein

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20
Q

Parkinson’s disease involves the deposition of synuclein inclusions, called ______ _____, with progressive loss of neurons, visible grossly in the substantia nigra.

A

Lewy bodies

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21
Q

Parkinson’s disease involves the deposition of synuclein inclusions (Lewy bodies) with progressive loss of neurons, visible grossly in the ____ ____.

A

substantia nigra

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22
Q

Huntington chorea involves chromosome ___, which codifies Huntingtin, containing a polyglutamine sequence due to CAG repeats. Repeats increase with each subsequent generation, increasing the severity of the disease.

A

4p

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23
Q

Huntington chorea involves chromosome 4p, which codifies Huntingtin, containing a polyglutamine sequence due to CAG repeats. Repeats increase with each subsequent generation, increasing the severity of the disease. This phenomenon is called ___.

A

anticipation

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24
Q

Huntington’s disease shows ___ nucleus atrophy and neuronal loss. Causes significant dilation of the lateral ventricles.

A

caudate

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25
Q

amyotrophic lateral sclerosis involves the loss of ____ neurons

A

motor (upper and lower)

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26
Q

amyotrophic lateral sclerosis involves the loss of motor neurons, which appears as degeneration of the ____ tracts.

A

corticospinal

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27
Q

Anencephaly is one of the more common neural tube defects due to closure failure of the ___ ___, resulting of the cerebrum and calvarium.

A

frontal neuropore

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28
Q

Anencephaly is associated with ___ deficiency

A

folic acid

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29
Q

____ is closure failure of the frontal to caudal neuropores, exposing the spinal cord and adjacent nerve roots.

A

Craniorachischisis

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30
Q

Failure of closure of part of the spinal cord. Spinal cord/roots can protrude from the defect.

A

meningomyelocele

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31
Q

Meningomyelocele is associated with ___ deficiency.

A

folic acid

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32
Q

Meninges and/or brain herniate through a mesodermal defect. Usually occipital, secondly frontoethmoidal.

A

encephalocele

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33
Q

fluid accumulation in the central canal of spinal cord.

A

hydromyelia

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34
Q

downward herniation of cerebellar tonsils through the foramen magnum. Asymptomatic or neck pain, lower CN palsies, sleep apnea, and sudden death. Cerebellar atxia and long tract signs. Syringomyelia is common.

A

chiari malformation type 1

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35
Q

downward herniation of tonsils and brainstem. Highly associated with lumbosacral myelomeningocele and hydrocephalus.

A

chiari malformation type 2

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36
Q

failure to divide the brain into hemispheres. Associated with mutations: SHH, SIC2, SIX3, TGIF1

A

holoprocencephaly

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37
Q

holoprocencephaly is associated with abnormalities of which chromosome?

A

trisomy 13

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38
Q

agenesis of the ___ ___ is total or partial disruption of cerebral interhemispheric axonal migration across the midline during development. Asymptomatic or seizures and cognitive impairment.

A

corpus callosum

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39
Q

Agenesis of the corpus callosum shows remnants of ____ matter bundles called bundles of Probst.

A

white

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40
Q

Common incidental finding on imaging or autopsy that involves the failure of the two halves of the septum pellusidum go fuse at the midline. Typically asymptomatic.

A

cavum septum pellusidum

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41
Q

cerebral cortical defects are due to impairment of ____ migration and cortical differentiation.

A

neuroblast

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42
Q

____ is the absence of normal convolutions on the brain, “smooth brain.” Seen in neuronal migration disorders.

A

lissencephaly

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43
Q

autosomal dominant syndrome of defective neuronal migration that results in lissencephaly

A

miller-dieker syndrome

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44
Q

chromosomal abnormality for miller-dieker syndrome

A

17p13.3

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45
Q

x linked syndrome associated with defective neuronal migration, resulting in lissencephaly

A

filamin A and doublecortin (DCX)

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46
Q

autosomal recessive syndrome associated with defective neuronal migration, resulting in lissencephaly

A

Walker-warburg syndrome

47
Q

2 genes associated with Walker-warburg syndrome

A

POMT1 and POMT2

48
Q

group of conditions of various etiologies that show nodules of abnormally placed gray matter. Arrest of neurons during migration, which stay around ventricles and white matter. Asymptomatic or seizures.

A

heterotopias

49
Q

disruption of normal gyration (too many, too small). Diffuse, focal, bilateral, or unilateral. Seizures and severe psychomotor retardation. Results from fetal infection, prenatal hypoxia, metabolic disease, or genetics.

A

polymicrogyria

50
Q

The least severe degree of CNS destruction from stroke during gestation. any fluid-filled cavity in the fetal or neonatal brain. A thin membrane may separate the cavity from the lateral ventricle or the subarachnoid space.

A

porencephaly

51
Q

Moderately severe degree of CNS destruction from stroke during gestation. ongenital clefts in the cerebral mantle.

A

schizencephaly

52
Q

the most severe degree of CNS degradation from stroke during gestation. erebral hemispheres are replaced by a thin-walled, fluid-filled cyst. The aqueduct is usually atretic, and increased fluid pressure causes the cyst (and the head) to enlarge.

A

hydranencephaly

53
Q

Mycobacterial infection commonly seen in the brains of AIDS patients.

A

TB

54
Q

Fungal infection that begins as a pulmonary infection and spreads hematogenous to the brain in immunosuppressed patients. Angioinvasion leads to infarcts. Fibrin and hyphae are seen in the artery wall.

A

Aspergillosis

55
Q

Infection that spreads hematogenously from the lung in immunosuppressed patients. Abscesses, thick & slimy meninges, and parenchymal cysts. Yeast with thick capsules invade macrophages.

A

cryptococcosis

56
Q

RNA virus that replicates in the muscle and migrates to the CNS via centripetal axonal transport –> salivary and lacrimal glands. Neurons with cytoplasmic Negri bodies.

A

Rabies

57
Q

Herpes virus causes necrosis of the ____ lobes, called necrotizing panencephalitis. Causes severe memory disturbances and other deficits

A

temporal

58
Q

Diffuse white matter damage caused by JC virus. Shows small, round, confluent white matter lesions (demyelination).

A

Progressive multifocal leukoencephalopathy (PML)

59
Q

JC virus goes latent in which two organs?

A

kidneys and tonsils (B cells)

60
Q

Infection in immunosuppressed patients, especially pregnant women transplacentally to fetus, that encysts and remains dormant in the CNS. Reactivation due to immunosuppression. Shows multifocal areas of necrosis and inflammation, bradyzoite cysts and tachyzoites.

A

toxoplasmosis

61
Q

Cystic infection that reactivates in immunosuppressed patients.

A

Toxoplasmosis

62
Q

Most common CNS parasitic infection and a leading cause of epilepsy. Tapeworms develop cysts in any organ. Inflammation follows the parasite’s death and eventual calcification

A

Neurocysticercosis

63
Q

___ hematoma occurs between the dura and the skull.

Associated with skull fracture.

Progressive mass effect, so patient may have a lucid intervan with subsequent mental decline.

Biconcave (lens) shaped and does not cross suture lines.

A

Epidural (extradural)

64
Q

____ hematoma occurs between the dura and the brain.

Associated with trauma and injury to bridging veins, which may be more common in elderly patients due to fragility of the veins. Increased risk with anticoagulation and alcoholism.

Progress quickly.

May become chronic

Banana shaped and may cross suture lines.

A
65
Q

___ hemorrhage between the arachnoid membrane and CNS parenchyma.

Commonly caused by ruptured aneurysms, AVM, or cortical contusion.

“Worse headache of my life.”

High fatality due to hemorrhage and vasospasm.

Blood can be seen in the CSF on LP or anywhere that CSF is present.

A

Subarachnoid

66
Q

Aneurysms are commonly seen at the ____ of an artery

A

bifurcation

67
Q

_____ hemorrhage is located within the cortex.

Related to HTN (basal ganglia or thalamus), metastases, or vascular lesions

Hemorrhage after CNS infarct.

Presents as sudden headache, loss of function, weakness, and hemiplegia

A

Parenchymal

68
Q

Traumatic brain injury can lead to progressive brain atrophy and cortical ___ deposition in deep sulci

A

Tau

69
Q

Cerebral contusions usually occur in the ___ gyri and ___ lobe.

Can cause diffuse axonal and vascular rinjury.

A

supraorbital

temporal

70
Q

Cerebral contusions can present with ____ lesions, where the brain hits the skull on the opposite side from the trauma.

A

countercoup

71
Q

diffuse axonal and vascular injury, typically caused by a ____ contusion.

A

cerebral

72
Q

____ herniation involved the movement of part of the temporal lobe under the free edge of the tentorium, compressing the midbrain and the oculomotor nerve (ipsilateral palsy). This is usually caused by a rapidly expanding middle cranial fossa epidural, subdural, or infratemporal lobe hematoma.

A

uncal

73
Q

In ____ herniation, the part of both the cerebral hemispheres are squeezed through a notch in the tentorium cerebelli.

A

Diencephalic/central

74
Q

_____ herniation is the most common form of intracranial herniation and occurs when brain tissue is displaced under the falx cerebri. The cingulate gyrus is herniated under the falx, and if progression occurs, other areas of the frontal lobe are involved.

A

subfalcine

75
Q

____ ____ can be drilled in the skull to relieve intracranial pressure

A

Burr holes

76
Q

A ____ herniation is characterized by the descent of the cerebellar tonsils through the foramen magnum, which compresses the medulla against the clivus/odontoid process. It is described as “coning” as the brain tissue is squeezed down through the foramen like being squeezed into a cone.

A

tonsillar

77
Q

____ ___ phenomenon occurs with uncal herniation, which causes contralateral pupillary dilation and ipsilateral oculomotor nerve weakness.

A

Kernohan’s notch

78
Q

Secondary ___ hemorrhages in the brainstem occur after uncal herniation, due to overstretching of the vessels.

A

Duret

79
Q

Brief episodes of CNS symptoms lasting <1 hr, due to ischemia and thromboembolism.

A

TIA (transient ischemic attack)

80
Q

Abrupt onset of CNA symptoms lasting >24 hours, due to tissue infarct and necrosis from thromboembolism.

A

Stroke

81
Q

____ injury occurs when an infarcted area of the cortex bleeds after treatment to reestablish blood flow.

A

Reperfusion

82
Q

Dural based tumors in adults. Slow growing with good prognosis. Women > men.Increased in neurofibromatosis and post-radiation. May grow through bone without being histologically malignant.

Well-circumscribed. Uniform tumor cells grow in whorls with no evidence of malignancy.

A

Meningioma

83
Q

Ill-defined, infiltrating tumor. Occur anywhere in the CNS but more commonly supratentorial. Cause seizures, headaches, and focal signs. The tendency to become malignant.

MRI shows ill-defined hyperdensity (hypercellular) with higher water content than normal white matter.

A

astrocytoma

84
Q

Most frequent glial tumor in adults. >supratentorial. Highly malignant.

Present as seizures, headaches, focal deficits.

Hypercellular, especially at rim.

Cells have marked atypia and mitosis.

A

Glioblastoma

85
Q

benign tumor in adults. More common in peripheral nerves, but within the CNS, more common in vestibular branch of CN 8 (cerebellopontine angle).

sporadic = solitary

Neurofibromatosis 2 = bilateral

Well circumscribed

A

schwannoma

86
Q

Well-circumscribed tumor, typically intraventricular or in the spinal cord, centrally placed. Any age. Causes hydrocephalus, increased ICP, and atxia.

A

ependymoma

87
Q

_____ frequently form perivascular “pseudo rosettes.” Centered by blood vessels and tumor cells arrange around them.

A

ependymoma

88
Q

Most frequent glial tumor in children. Occur mostly in the cerebellum, but also in the optic nerve, thalamus, hypothalamus, and supratentorially. Well circumscribed, solid and cystic. Present with increased ICP and cerebellar signs (ataxia).

Spindle cells with dense, fibrillary cytoplasm. Low cellularity and characteristic Rosenthal fibers.

A

pilocytic astrocytoma

89
Q

Common malignant tumor in children, <15yo. Typically in the posterior fossa, vermis. Present with ataxia and signs of increased ICP. Tendency to disseminate along the subarachnoid space (drop metastases).

Mt: SHH, WSH

Solid, typically in the midline.

Markedly cellular, frequently with “rosettes.” Some neuroblasts within these tumors form nodules of more differentiated cells, called neurocites.

A

medulloblastoma

90
Q

Steroid myopathy involves (proximal/distal) muscle weakness

A

proximal

91
Q

Steroid myopathy preferentially affects (type 1/type 2) muscle fibers

A

type 2

92
Q

Dermatomyositis preferentially affects the (proximal/distal) muscles

autoimmune, women

Elevated CK
Underlying malignancy

Heliotrope erythema, Gottron sign, mechanics hands

A

proximal

93
Q

autoantibodies associated with dermatomyositis

1 = gottron papules and heliotrope rash

2 = arthritis and skin rash, interstitial lung disease

3 = paraneoplastic/juvenile

A

Anti-Mi2 (against helicase)

Anti-Jo1 (against histidyl t-RNA synthetase)

Anti-P155/P140

94
Q

Lymphocyte-mediated muscle injury leading to muscle pain and weakness

A

polymyositis

95
Q

polymyositis preferentially affects (proximal/distal) muscles, symmetrically

A

Proximal

96
Q

Durg-induced myopathy

high CK

A

Toxic/vacuolar

97
Q

Inherited myopathy (AD or AR) that can be due to defects in several cellular enzymes

Scattered fibers show more subsarcolemmal mitochondria on NADH staining, indicating increased proliferation

Typical “ragged-red fibers”

A

Mitochondrial myopathy

98
Q

X-linked (male) progressive muscle weakness

Floppy baby or asymptomatic at birth

High CK

A

Duchenne musculodystrophy

99
Q

Duchene MD favors (proximal/distal) muscles

A

proximal

100
Q

Mild dystrophy that also presents with heart disease, typically around 12yo.

A

Becker Muscular dystrophy

101
Q

Sarcoglycanopathy favors (proximal/distal) muscles

Intolerance to exercise/cramps

A

proximal (limb girdle)

102
Q

Spongiform encephalopathies are a group of progressive, lethal diseases that involve the accumulation of abnormal ___ protein in the CNS and the formation of ___ plaques.

A

prion

amyloid

103
Q

Prion PrPc protein is associated with Chromosome ___. Expressed in all cells, particularly neurons. Decrease in size of a-helices, and increase in size of b-sheets causes the protein to become resistant to digestion and liable to form aggregates.

A

20

104
Q

Rapidly progressive dementia presentiing in the 7th decade of life. Also shows myoclonus. Progressive and fatal in months.
Increased signal in the basal ganglis and cortex.

Vauolization of the gray matter neutrophil (sponge-like)

Progressive neuronal loss and reactive gliosis

PrP deposition as amyloid plaques

A

sporadic Creutxfeldt-Jakob disease (CJD)

105
Q

sporadic Creutxfeldt-Jakob disease (CJD) shows vacuolization of (gray/white) matter

A

gray

106
Q

sporadic Creutxfeldt-Jakob disease (CJD) involves ___ protein deposition in the form of amyloid plaques

A

PrP

107
Q

PrP protein disease that appears in younger patients in the UK. Initially as sensory and psychiatric disturbances and unsteadiness. Slowly progressive dementia to akinetic mutism. Death around 14 months.

Plaques and vacuolization

Spongiform degeneration in the basal ganglia and thalamus

PrPsc also found in follicular dendritic cells of tonsil, LN, spleen, thymus and GALT (all immune tissues)

A

variant Creutxfeldt-Jakob disease (CJD)

108
Q

Prion protein diseases are transmissible but are not ____.

A

contagious

109
Q

•Destruction of myelin in CNS or peripheral nerves, with relative preservation of axons

Multifocal with lesions of different ages

Autoimmune ; women > men

Increased incidence with increased latitude

  • Variable presentation with weakness, paresthesia, sensory loss of one or more limbs, optic neuritis, diplopia, incoordination, vertigo
  • Others with loss of vision, dysarthria, disturbances of micturition, painful muscle spasms, seizures
  • Usually associated with oligoclonal bands of immunoglobulins on electrophoresis of CSF
A

Multiple sclerosis

110
Q

Classify the MS:

–multiple acute attacks, each followed by clinical improvement

A

relapsing remitting

111
Q

Classify the MS:

–after years of RRMS, the patient enters a stage with no recovery between attacks

A

secondary progressive

112
Q

Classify the MS:

progressive disease without episodes of recovery

A

primary progressive

113
Q

Classify the MS:

repeated acute attacks superimposed on progressive disease without episodes of recovery

A

relapsing progresssive

Path 2 (6 decks)

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