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Neurology 2 > Robbins 27/28 > Flashcards

Robbins 27/28 Flashcards

1
Q

Red neurons

A 
Acute neuronal injury
Hypoxia/ischemia
Earliest morphologic marker of neuronal death
12-24 hours
Irreversible
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2
Q

Subacute and chronic neuronal injury

A 
Neurodegenerative diseases (ALS, AD)
Cell loss + reactive gliosis
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3
Q

Axonal reaction

A

Anterior horn cells
After motor neurons are cut or damaged
Increased protein synthesis
Central chromatolysis

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4
Q

Cowdry body

A

Herpes

Intranuclear inclusions

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5
Q

Negri body

A

Rabies

Cytoplasmic inclusions

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6
Q

CMV

A

Nuclear AND cytoplasmic inclusions

Ependymal injury

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7
Q

GFAP

A

Glial fibrillary acidic protein

Astrocytes

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8
Q

Gliosis

A

Astrocyte hypertrophy/hyperplasia
Nuclei become vesicular with prominent nucleoli
Cytoplasm becomes very eosinophilic

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9
Q

Gemistocytic astrocytes

A

Eosinophilic cytoplasm around eccentric nucleus

Ramifying processes emerge from nucleus

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10
Q

Rosenthal fibers

A 
Thick, long, eosinophilic
Within astrocyte processes
Alpha-B crystallin and HSP-27 and ubiquitin
LONG-TERM GLIOSIS
Pilocytic astrocytoma
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11
Q

Alexander disease

A

Leukodystrophy
GFAP mutation
Rosenthal fibers
Corpora amylacea

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12
Q

Microglial response to injury

A
  1. Proliferate
  2. Elongated nuclei (rod cells)
  3. Aggregate around necrosis (microglial nodules)
  4. Congregate around dying cell bodies (neuronophagia)
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13
Q

Oligodendrocyte injury/apoptosis

A

Demyelinating diseases

Leukodystrophies

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14
Q

Ependymal granulations

A

Inflammation/dilation of ventricles –>
Ependymal lining disruption +
Subependymal astrocyte proliferation

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15
Q

3 causes of increased ICP

A

General brain edema
Increased CSF
Expanding mass lesion

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16
Q

Vasogenic edema

A

BBB disruption or increased vasular permeability

Increased ECF

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17
Q

Cytotoxic edema

A

CNS cell membrane injury
Ischemia or metabolic derangement
Increased ICF

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18
Q

Cingulate herniation

A

Under falx cerebri

ACA compression

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19
Q

Uncal herniation

A 
Through tentorium
CN3 compression (oculomotor palsy)
PCA compression (visual problems)
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20
Q

Duret hemorrhages

A

Transtentorial herniation
2º hemorrhagic lesions in midbrain and pons
Midline, linear/flame-shaped lesions
Torn penetrating vessels to upper brainstem

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21
Q

Tonsillar herniation

A

Through foramen magnum

Compresses respiratory/cardiac centers in medulla

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22
Q

Microencephaly causes

A

Fetal alcohol syndrome
HIV-1 infection in utero
Chromosomal abnormalities

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23
Q

Lissencephaly

A

Reduced # of gyri (smoothed or cobblestone surface)

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24
Q

Polymicrogyria

A

Small, numerous, unusual cerebral convolutions

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25
Q

Neuronal heterotopias

A

Neuron collections in wrong places along migratory pathway
Along ventricular surface
Associated with epilepsy

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26
Q

Bat-wing deformity

A

Agenesis of corpus callosum

Bat wing = misshapen lateral ventricles

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27
Q

Arnold-Chiari malformation

A 
Small posterior fossa
Misshapen cerebellum (midline)
Tonsillar herniation
Hydrocephalus
Lumbar myelomeningocele
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28
Q

Dandy-Walker malformation

A 
Enlarged posterior fossa
Absent cerebellar vermis
Midline cyst lined by ependyma/leptomeninges
Cyst = expanded 4th ventricle
Dysplasias of brainstem nuclei
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29
Q

Hydromyelia

A

Expansion of central canal of spinal cord

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30
Q

Syringomyelia

A

Fluid-filled cavity in neural part of spinal cord

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31
Q

Syringomyelia presentation

A

Destroyed anterior white commissure
Dense reactive gliosis
Loss of P/T in upper extremities

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32
Q

Cerebral palsy

A

Spasticity, dystonia, ataxia, paresis

Due to pre/perinatal insults

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33
Q

Premature infants

A

Intraparenchymal hemorrhage w/in germinal matrix

Supratentorial periventricular infarcts (white matter)

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34
Q

Multicystic encephalopathy

A

Grey and white matter ischemic damage (infarcts)

Cystic lesions through hemispheres

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35
Q

Common perinatal ischemic lesions (later)

A

Caudate, putamen, thalamus

Movement disorders common (choreoathetosis)

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36
Q

Displaced skull fracture

A

Bone is displaced into cranial cavity beyond the thickness of the bone

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37
Q

Impact to occiput or temporal regions
Lower CN symptoms
Orbital/mastoid hematomas

A

Basal skull fracture

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38
Q

Diastatic fractures

A

Cross suture lines

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39
Q

Concussion presentation

A 
Instant neurologic dysfunction (temporary)
Loss of consciousness
Respiratory arrest
Loss of reflexes
Complete recovery (damage remains)
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40
Q

Cause of sudden neuro symptoms in concussion

A

Dysregulaton of RAS in brainstem

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41
Q

Common contusion sites

A

Crests of gyri (near rough bone)

  • Orbital ridges (frontal lobe)
  • Temporal lobes
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42
Q

Coup injury only

A

Head immobile at time of impact

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43
Q

Coup + contrecoup

A

Sudden deceleration

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44
Q

Plaque jaune

A

Old traumatic contusions
Depressed, retracted, yellow
Gyral crests
EPILEPTIC FOCI

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45
Q

Coma shortly after trauma…think ______

A

Diffuse axonal injury

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46
Q

Causes of subarachnoid hemorrhage

A

Aneurysm
AVM
Trauma

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47
Q

Those at risk for subdural hemorrhage

A 
Brain atrophy (elderly, AD, etc.)
Infants (thin bridging veins)
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48
Q

Chronic traumatic encephalopathy

A

Repeated head trauma
Atrophy
Neurofibrillary tangles (frontal and temporal lobes)

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49
Q

2 main causes of CVD

A

Hypoxia/ischemia/infarction - embolism, thrombosis

Hemorrhage - HTN, aneurysms, AVMs

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50
Q

Global cerebral ischemia

A

General reduced cerebral perfusion
Cardiac arrest, shock, hypotension
Pyramidal and purkinje cells affected most
Vegetative state, brain dead, respirator brain

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51
Q

Sickle-shaped necrosis over cerebral convexities

A

Watershed infarcts

- Hypotensive episodes

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52
Q

Phases of ischemic damage

A
  1. (12-24 hrs) Red neurons (vacuol., eosin., pyknosis)
  2. (1 day to 2 wks) Necrosis, neutrophils –> macrophages, vascular proliferation, reactive gliosis
  3. (Post 2 wks) Repair, gliosis, pseudolaminar necrosis
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53
Q

Focal Cerebral Ischemia (sites)

A

Thalamus, basal ganglia, deep white matter

No collateral flow for deep penetrating vessels

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54
Q

Embolic infarction

A

Cardiac mural thrombi
Tumor, fat, air
MCA DISTRIBUTION

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55
Q

Thromboembolism

A

Carotid artery atherosclerosis

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56
Q

Thrombotic occlusions

Common locations

A

Atherosclerotic plaque rupture

Carotid bifurcation, MCA origin, basilar artery

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57
Q

Inflammatory vasculitis causes

A

Syphilis, TB, aspergillosis (immunocomp), CMV (immunocomp)

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58
Q

Primary angiitis of CNS

A

Inflammatory disorder of small/medium parenchymal/subarachnoid vessels
Chronic inflammation
Multinucleated giant cells
Vessel wall destruction

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59
Q

Drugs to cause thrombosis/infarction

A

Amphetamines, heroin, cocaine

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60
Q

2 ways for reperfusion injury

A

Collateral circulation

Occlusion fragmentation/lysis

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61
Q

When is thrombotic therapy contraindicated?

A

Hemorrhagic infarcts

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62
Q

Lacunar infarcts

A

HTN
Deep penetrating arteries (arteriolar sclerosis)
Basal ganglia, internal capsule, deep white matter
Tissue loss + gliosis

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63
Q

Slit hemorrhages

A

Rupture of small penetrating vessels

Hemorrhages resorb, leaving slit-like cavity

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64
Q

Hypertensive encephalopathy

A

Malignant HTN
Diffuse cerebral dysfunction
Increased ICP
Potential tonsillar herniation

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65
Q

Multi-infarct dementia

A

Dementia, gait issues, pseudobulbar signs

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66
Q

Causes of multi-infarct dementia

A
  1. Cerebral atherosclerosis
  2. Thrombosis/embolism
  3. Chronic HTN arteriolar sclerosis
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67
Q

Binswanger disease

A

Multi-infarct dementia in subcortical white matter

Myelin and axon loss

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68
Q

Ganglionic hemorrhages

Cause

A

In basal ganglia/thalamus

Via HTN

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69
Q

Lobar hemorrhages

Cause

A

In hemispheres

Via CAA

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70
Q

Charcot-Bouchard microaneurysms

A

Tiny vessel hemorrhages in basal ganglia

Via chronic HTN

71
Q

CADASIL

What is it?

Occurs when?

A

Cerebral autosomal dominant arteriopathy w/ subcortical infarcts and leukoencephalopathy

NOTCH3 mutation

Recurrent strokes (infarcts) and dementia

Infarcts in 40’s

Concentric thickening of media/adventitia (CNS, skin)

72
Q

Saccular aneurysms

Cause?

A

Subarachnoid hemorrhage via cerebral artery
Most common intracranial aneurysm
Mostly in ANTERIOR CIRCULATION
Absence of smooth muscle and intimal elastic

73
Q

Predisposing factors for saccular aneurysms

A 
Polycystic kidney disease
Ehlers-Danlos
NF-1
Marfan syndrome
Cigarette smoking
HTN
74
Q

“Worst headache I’ve ever had”

Rapid loss of consciousness

A

Subarachnoid hemorrhage (aneurysm)

75
Q

Dangers of subarachnoid hemorrhage

A

Vasospasm –> ischemic injury
Especially in basal (CoW) hemorrhage

Meningeal fibrosis/scarring –> CSF block

76
Q

2 main types of vascular malformations

A

AVM

Cavernous malformation

77
Q

AVM

A

Pulsatile AV shunt with high blood flow
Enlarged BVs with gliotic tissue in between
Abnormally structures vessels

78
Q

Cavernous malformation

A

Low-flow, no shunting, distended, poorly organized channels

Surrounded by old hemorrhage/infarction/calcification

79
Q

Viruses transported along peripheral nerves

A

Rabies, herpes zoster

80
Q

Local extension of infection

A

Air sinuses, teeth, skull, or vertebrae

81
Q

Meningitis

A

Leptomeningeal inflammation (and CSF) in subarachnoid space

82
Q

Causes of chronic meningitis

A

TB, spirochete (Lyme, syphilis), cryptococcus

83
Q

Neonatal meningitis

A

Group B strep

E. coli

84
Q

Elderly meningitis

A

Strep. pneumoniae

Listeria

85
Q

Young adult meningitis

A

Neisseria meningitidis

86
Q

Symptoms of bacterial meningitis

A

Headache, Photophobia, Irritability, Cloudy consciousness, Neck stiffness

Cloudy/purulent CSF, increased pressure, neutrophils, increased protein, decreased glucose

87
Q

Meningitis + septicemia –> adrenal hemorrhage + cutaneous petechiae

A

Waterhouse-Friderichsen syndrome

88
Q

Symptoms of viral meningitis

A

Fever, stiff neck, consciousness issues

CSF = lymphocytic pleocytosis, mildly elevated protein, normal glucose

89
Q

Most common cause of viral meningitis

A

Enteroviruses (when pathogen identified)

90
Q

Brain abscess

A

Localized necrosis w/ inflammation
Collagenous capsule via BV fibroblasts
Generally via bacterial infection

91
Q

Common causes of brain abscesses

A

Staph. aureus, streptococci

92
Q

CSF in brain abscess

A

High WBC
Increased protein
Normal glucose

93
Q

Complications of brain abscess

A

Herniation, rupture, sinus thrombosis

94
Q

Subdural empyema

Dangers?

A

Infection of skull or air sinuses –> subdural space

Mass effect OR thrombophlebitis of bridging veins –> infarction/edema

95
Q

Clinical presentation of subdural empyema

A

Fever, headache, neck stiffness

CSF like brain abscess

96
Q

Chronic TB meningoencephalitis…

CSF?

Complications?

A

Pleocytosis, HIGH HIGH PROTEIN, little reduced/normal glucose

Hydrocephalus via arachnoid fibrosis
Obliterative endarteritis –> arterial occlusion –> infarction

97
Q

Chronic meningitis
Immunocompromised
Macrophages filled with organisms
Few/no granulomas

A

Avium-intracellulare

98
Q

Neurosyphilis

A

Tabes dorsalis + paresis
Mood alterations (grandeur) - NAPOLEAN
Dementia
Ataxia, loss of pain/temp, lightning pains in back and backs of legs, no DTRs

99
Q

Neuroborreliosis (Lyme disease)

A

Meningitis + facial nerve palsies (Bell’s) + polyneuropathies + encephalopathy

100
Q

Viral CNS infection after LATENCY

A

Herpes zoster

PML (JC polyomavirus)

101
Q

Polio-like spinal cord paralysis

Viral encephalitis

A

West Nile

102
Q

Viral encephalitis causes (9)

A 
West Nile
HSV-1
HSV-2
Herpes Zoster
CMV
Poliomyelitis
Rabies
HIV
JC Polyomavirus
103
Q 
Viral encephalitis
Child/young adult
Altered mood, memory, behavior
Temporal lobes
Perivascular infiltrates
Cowdry A viral inclusions in neurons/glia
A

HSV-1 encephalitis

104
Q

Painful vesicular skin eruption of dermatomal pattern

A 
Herpes Zoster (Shingles)
Re-activation
105
Q

Elderly

Persistent pain via not-painful stimuli

A

Postherpetic neuralgia (Herpes zoster)

106
Q

Fetus
Periventricular necrosis/calcification
Microcephaly
Enlarged CNS cells with inclusions (nuclear and cytoplasmic)

A

CMV encephalitis

107
Q

Gastroenteritis
Aseptic meningitis
Flaccid paralysis + hyporeflexia
Respiratory compromise, death

A

Poliomyelitis (secondary CNS invasion)

- Anterior horn motor neurons of spinal cord

108
Q 
Local paresthesias around bite wound
Pain with slightest touch, violent motor responses
Afraid to drink liquid
Intense cerebral edema
Neuronal degeneration
Flaccid paralysis, respiratory failure

Round/oval cytoplasmic inclusions in hippocampus pyramidal cells and cerebellar purkinje cells

A

Rabies encephalitis

109
Q

Microglial nodules
Multinucleated giant cells
Necotic foci with reactive gliosis
Cognitive dysfunction

A

HIV encephalitis (chronic microglial inflammation)

110
Q

Immunosuppressed
Demyelination at multiple foci
Progressive

A

PML (JC polyomavirus)

- Via Re-activation w/ immunosuppression

111
Q

Immunocompromised
Hemorrhagic infarction –> sepsis
Vasculitits

A

Mucormycosis or Aspergillosis (fungal meningoencephalitis)

112
Q

Fungal causes of parenchymal granulomas/abscesses

A

Candida, Cryptococcus

113
Q

HIV immunosuppression
Ring-enhancing lesions (multiple) near gray-white junction and in deep gray nuclei
Tachyzoites and bradyzoites in necrotic foci

A

Cerebral toxoplasmosis

114
Q

Ring-enhancing lesions

A

CNS lymphoma
TB
Fungal infections

115
Q

Dives into still water
Necrotic encephalitis
Rapidly progressive/fatal

A

Naegleria

116
Q

Mad Cow Disease

A

Variant Creutzfeldt-Jakob Disease

117
Q

Prion disease without spongiform features

A

Fatal Familial Insomnia

118
Q

Separate episodes of neurologic deficits
Optic neuritis
HLA-DR2-MHC
Well circumscribed lesions of T cells and macrophages
Oligoclonal bands of IgG in CSF
Lesions are firmer than surrounding tissue
Depressed, glassy, irregularly shaped plaques

A

Multiple sclerosis

119
Q

Pathogenesis of MS

Viral roll?

A

Auto-immune
Th1 and Th17 cells react against myelin antigens

EBV (or other) infection activates self-reactive T cells

120
Q

Bilateral optic neuritis

Anti-aquaporin antibodies

A

Neuromyelitis optica

121
Q

Viral infection or immunization
Headache, lethargy, coma
Rapid progression

Type of disease?

A

Acute Disseminated Encephalomyelitis

Demyelinating

122
Q

Recent URI
Perivenular demyelniation throughout CNS
Disseminated necrosis of white and gray matter
Hemorrhage, fibrin, neutrophils

Type of disease?

A

Acute Necrotizing Hemorrhagic Encephalomyelitis

Demyelinating

123
Q

Correction of hyponatremia
Rapidly evolving quadriplegia
“Locked-in syndrome”

Type of disease

A

Central pontine myelinolysis

Demyelinating

124
Q

Neurodegenerative diseases

A

Progressive loss of neurons with functional relationships

Accumulation of protein aggregates

125
Q

3 reasons for protein aggregation in neurodegenerative diseases

A

Mutated protein
Peptide of larger protein
Unexplained alteration of normal protein

126
Q

Cerebral cortex degenerative diseases

A

Alzheimer’s

FTLD

127
Q

Basal ganglia/brainstem degenerative diseases

A 
Parkinson
Progressive supranuclear palsy
Corticobasal degeneration
MSA
Huntington
Spinocerebellar ataxias
128
Q

Motor neuron degerative diseases

A

ALS

Spinal bulbar muscular atrophy

129
Q

A-beta inclusions

A

Alzheimer’s

130
Q

Tau inclusions

A

Alzheimer’s
FTLD (behavioral, language dysfunctions)
Parkinson’s (hypokinetic movement)
Progressive supranuclear palsy (Park. + eye movements)
Corticobasal degeneration (Park. + asymmetric movements)

131
Q

TDP-43

A

FTLD

ALS

132
Q

FUS

A

FTLD

ALS

133
Q

Alpha-synuclein

A

Parkinson’s

MSA

134
Q

Poly-glutamine (CAG)

A

Huntington
Spinocerebellar ataxia
Spinal bulbar muscular atrophy

135
Q

Presenilin mutation

A

Alzheimer’s

Gamma-secretase makes more A-beta 42

136
Q

When does tau aggregate?

A

Hyperphosphorylated

137
Q

Cause of degeneration in Alzheimer’s

A

A-beta aggregates cause inflammatory response from microglia and astrocytes (including cytokine mediators)

Inflammatory cascades also hyperphosphorylate tau

138
Q

Forgetfulness, memory issues
Language, math, learned motor deficits
Incontinent, mute, unable to walk

Death by ______

A

Alzheimer’s

Pneumonia (intercurrent disease)

139
Q

Personality, behavior, language (aphasia) alterations
Later, memory loss
Eventually global dementia (before 65 often)

A

Frontotemporal lobar degeneration

140
Q

FTLD symptoms

Smooth cellular inclusions

A

Pick disease

141
Q

Two forms of FTLD-TDP

A

C9orf72 (familial)

TDP-43 (ALS-like symptoms)

142
Q

Classic Parksonsonism symptoms

A 
Diminished facial expression (masked facies)
Stooped posture
Slowed voluntary movements
Festinating gait
Cog-wheel rigidity
Pill-rolling resting tremor
143
Q

Tremor
Rigidity
Bradykinesia (slowed movements)

A

CENTRAL TRIAD of Parkinsonism

144
Q

Autosomal dominant Parkinson’s

A

Alpha-synuclein, Lewy bodies

LRRK2 gene mutation

145
Q

Autosomal recessive Parkinson’s

A

Mitochondrial-related proteins (DJ-1, PINK1, parkin)

146
Q

Pallor of the substantia nigra (and locus ceruleus)

A

Parkinson’s (loss of dopaminergic neurons)

147
Q

Parkinsonian symptoms
Hallucinations
Frontal lobe signs (conscious thought, etc.)

A

Dementia with Lewy Bodies

148
Q

Truncal rigidity
Difficulty with eye movements
Parkinsonian symptoms
Tau inclusions in brainstem/cerebellum

A

Progressive supranuclear palsy

Brainstem/cerebellum focus

149
Q

Truncal rigidity
Jerking movements of limbs
Difficulty performing asked actions (apraxia)
Balooned neurons in cortex, tau inclusions

A

Corticobasal degeneration

Cortical focus

150
Q

Alpha-synuclein in oligodendrocytes
Parkonsonian symptoms
Ataxia
Orthostatic hypotention

A

Multiple system atrophy

151
Q

Why is it called MSA?

A

Degeneration of 3 white matter tracts:

 - Striatonigral
 - Olivopotocerebella
 - ANS
152
Q 
40s-50s
Jerky, hyperkinetic, chorea movements
Bradykinesia
Rigidity
Writhing movements of extremities
Poly-glutamine repeats in HTT gene
A

Huntington’s

153
Q

Cause of chorea in Huntington’s

A

Loss of GABAergic neurons in striatonigral system

- Thus, loss of cortical inhibition of skeletal muscle contractions

154
Q

Causes of death in Huntington’s

A

Intercurrent infection

Suicide

155
Q

Child - gait ataxia, hand clumsiness, dysarthria
Loss of DTRs except extensor plantar reflex
Impaired joint/vibratory sense, and/or P/T
Arrhythmias, congestive heart failure
Diabetes

A

Friedreich Ataxia

156
Q

Child
Recurrent sinopulmonary infections, difficulty walking
Dysarthria, eye movement abnormalities
Red/purple clusters/lesions on conjunctiva and skin
T-cell leukemias

A

Ataxia-Telangiectasia

157
Q

Spastic paralysis

Flaccid paralysis

A

ALS

158
Q

Superoxide dismutase mutation

A

ALS

159
Q

C9orf72 mutation

A

ALS (motor neurons) + FTLD (behavior, language)

160
Q 
Weakness of hands
Dropping objects
Cramping/spacticity of arms and legs
Fasciculations
Recurrent pulmonary infections
A

ALS

161
Q

Variants of ALS

A

Progressive muscular atrophy (LMNs mostly)
Primary lateral sclerosis (UMNs mostly)
Progressive bulbar palsy (phonation/chewing mostly)

162
Q

Progressive bulbar palsy

A

ALS with issues in nucleus ambiguus and hypoglossal nucleus

163
Q

Kennedy disease

A

LMN degeneration
Atrophy of tongue and dysphagia
Androgen insensitivity, gynecomastia, testicular atrophy

164
Q

Baby
Deficiency of beta-galactosidase
Stiffness, weakness
Die young

A

Krabbe disease

165
Q

Deficiency of arylsulfatase A
Cerebroside sulfate (sulfatides) accumulation
Dyes cause shift of absorbance spectrum

A

Metachromatic leukodystrophy

166
Q

X-linked (mostly young men)
Behavioral changes
Adrenal insufficiency
Elevated very long chain FA’s (VLCFAs) in serum

A

Adrenoleukodystrophy

167
Q

GFAP gene mutation

A

Alexander disease

168
Q

eIF2B gene mutation

A

Vanishing white matter leukoencephalopathy

169
Q 
Recurrent episodes of acute neuro dysfunction
Cognitive changes
Weakness
Stroke-like episodes
Lactic acidosis
MTTL1 mutation
A

MELAS (mitochondrial encephalopathy, lactic acidosis, stroke-like episodes)

170
Q 
Infant
Lactic acidemia
Feeding problems
Seizures
Extraocular palsies
Weakness with hypotonia
Spongiform brain and BV proliferation
A

Leigh syndrome

171
Q 
Psychotic symptoms
Ophthalmoplegia
Hemorrhagic foci
Necrosis of mamillary bodies and ventricular walls
Hemosideran-laden macrophages
A

Wernicke encephalopathy (thiamine deficiency)

172
Q

Short term memory loss
Confabulation
Alcoholic/gastric disorders

A

Korsakoff syndrome (thiamine deficiency)

173
Q

Bilateral numbness, tingling, ataxia of LEs
Progression to spastic weakness
Eventual complete paraplagia

Starts where?

A

Vitamin B12 deficiency (subacute combined degeneration of spinal cord)

Mid-thoracic level, then extends up and down

174
Q

Degeneration of retinal ganglion cells

Makes moonshine

A

Methanol toxicity

Neurology 2 (22 decks)

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