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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
Neuronal heterotopias
Neuron collections in wrong places along migratory pathway Along ventricular surface Associated with epilepsy
26
Bat-wing deformity
Agenesis of corpus callosum | Bat wing = misshapen lateral ventricles
27
Arnold-Chiari malformation
``` Small posterior fossa Misshapen cerebellum (midline) Tonsillar herniation Hydrocephalus Lumbar myelomeningocele ```
28
Dandy-Walker malformation
``` Enlarged posterior fossa Absent cerebellar vermis Midline cyst lined by ependyma/leptomeninges Cyst = expanded 4th ventricle Dysplasias of brainstem nuclei ```
29
Hydromyelia
Expansion of central canal of spinal cord
30
Syringomyelia
Fluid-filled cavity in neural part of spinal cord
31
Syringomyelia presentation
Destroyed anterior white commissure Dense reactive gliosis Loss of P/T in upper extremities
32
Cerebral palsy
Spasticity, dystonia, ataxia, paresis | Due to pre/perinatal insults
33
Premature infants
Intraparenchymal hemorrhage w/in germinal matrix | Supratentorial periventricular infarcts (white matter)
34
Multicystic encephalopathy
Grey and white matter ischemic damage (infarcts) | Cystic lesions through hemispheres
35
Common perinatal ischemic lesions (later)
Caudate, putamen, thalamus | Movement disorders common (choreoathetosis)
36
Displaced skull fracture
Bone is displaced into cranial cavity beyond the thickness of the bone
37
Impact to occiput or temporal regions Lower CN symptoms Orbital/mastoid hematomas
Basal skull fracture
38
Diastatic fractures
Cross suture lines
39
Concussion presentation
``` Instant neurologic dysfunction (temporary) Loss of consciousness Respiratory arrest Loss of reflexes Complete recovery (damage remains) ```
40
Cause of sudden neuro symptoms in concussion
Dysregulaton of RAS in brainstem
41
Common contusion sites
Crests of gyri (near rough bone) - Orbital ridges (frontal lobe) - Temporal lobes
42
Coup injury only
Head immobile at time of impact
43
Coup + contrecoup
Sudden deceleration
44
Plaque jaune
Old traumatic contusions Depressed, retracted, yellow Gyral crests EPILEPTIC FOCI
45
Coma shortly after trauma...think ______
Diffuse axonal injury
46
Causes of subarachnoid hemorrhage
Aneurysm AVM Trauma
47
Those at risk for subdural hemorrhage
``` Brain atrophy (elderly, AD, etc.) Infants (thin bridging veins) ```
48
Chronic traumatic encephalopathy
Repeated head trauma Atrophy Neurofibrillary tangles (frontal and temporal lobes)
49
2 main causes of CVD
Hypoxia/ischemia/infarction - embolism, thrombosis | Hemorrhage - HTN, aneurysms, AVMs
50
Global cerebral ischemia
General reduced cerebral perfusion Cardiac arrest, shock, hypotension Pyramidal and purkinje cells affected most Vegetative state, brain dead, respirator brain
51
Sickle-shaped necrosis over cerebral convexities
Watershed infarcts | - Hypotensive episodes
52
Phases of ischemic damage
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
53
Focal Cerebral Ischemia (sites)
Thalamus, basal ganglia, deep white matter | No collateral flow for deep penetrating vessels
54
Embolic infarction
Cardiac mural thrombi Tumor, fat, air MCA DISTRIBUTION
55
Thromboembolism
Carotid artery atherosclerosis
56
Thrombotic occlusions Common locations
Atherosclerotic plaque rupture Carotid bifurcation, MCA origin, basilar artery
57
Inflammatory vasculitis causes
Syphilis, TB, aspergillosis (immunocomp), CMV (immunocomp)
58
Primary angiitis of CNS
Inflammatory disorder of small/medium parenchymal/subarachnoid vessels Chronic inflammation Multinucleated giant cells Vessel wall destruction
59
Drugs to cause thrombosis/infarction
Amphetamines, heroin, cocaine
60
2 ways for reperfusion injury
Collateral circulation | Occlusion fragmentation/lysis
61
When is thrombotic therapy contraindicated?
Hemorrhagic infarcts
62
Lacunar infarcts
HTN Deep penetrating arteries (arteriolar sclerosis) Basal ganglia, internal capsule, deep white matter Tissue loss + gliosis
63
Slit hemorrhages
Rupture of small penetrating vessels | Hemorrhages resorb, leaving slit-like cavity
64
Hypertensive encephalopathy
Malignant HTN Diffuse cerebral dysfunction Increased ICP Potential tonsillar herniation
65
Multi-infarct dementia
Dementia, gait issues, pseudobulbar signs
66
Causes of multi-infarct dementia
1. Cerebral atherosclerosis 2. Thrombosis/embolism 3. Chronic HTN arteriolar sclerosis
67
Binswanger disease
Multi-infarct dementia in subcortical white matter | Myelin and axon loss
68
Ganglionic hemorrhages Cause
In basal ganglia/thalamus | Via HTN
69
Lobar hemorrhages Cause
In hemispheres | Via CAA
70
Charcot-Bouchard microaneurysms
Tiny vessel hemorrhages in basal ganglia | Via chronic HTN
71
CADASIL What is it? Occurs when?
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
Saccular aneurysms Cause?
Subarachnoid hemorrhage via cerebral artery Most common intracranial aneurysm Mostly in ANTERIOR CIRCULATION Absence of smooth muscle and intimal elastic
73
Predisposing factors for saccular aneurysms
``` Polycystic kidney disease Ehlers-Danlos NF-1 Marfan syndrome Cigarette smoking HTN ```
74
"Worst headache I've ever had" | Rapid loss of consciousness
Subarachnoid hemorrhage (aneurysm)
75
Dangers of subarachnoid hemorrhage
Vasospasm --> ischemic injury Especially in basal (CoW) hemorrhage Meningeal fibrosis/scarring --> CSF block
76
2 main types of vascular malformations
AVM | Cavernous malformation
77
AVM
Pulsatile AV shunt with high blood flow Enlarged BVs with gliotic tissue in between Abnormally structures vessels
78
Cavernous malformation
Low-flow, no shunting, distended, poorly organized channels | Surrounded by old hemorrhage/infarction/calcification
79
Viruses transported along peripheral nerves
Rabies, herpes zoster
80
Local extension of infection
Air sinuses, teeth, skull, or vertebrae
81
Meningitis
Leptomeningeal inflammation (and CSF) in subarachnoid space
82
Causes of chronic meningitis
TB, spirochete (Lyme, syphilis), cryptococcus
83
Neonatal meningitis
Group B strep | E. coli
84
Elderly meningitis
Strep. pneumoniae | Listeria
85
Young adult meningitis
Neisseria meningitidis
86
Symptoms of bacterial meningitis
Headache, Photophobia, Irritability, Cloudy consciousness, Neck stiffness Cloudy/purulent CSF, increased pressure, neutrophils, increased protein, decreased glucose
87
Meningitis + septicemia --> adrenal hemorrhage + cutaneous petechiae
Waterhouse-Friderichsen syndrome
88
Symptoms of viral meningitis
Fever, stiff neck, consciousness issues CSF = lymphocytic pleocytosis, mildly elevated protein, normal glucose
89
Most common cause of viral meningitis
Enteroviruses (when pathogen identified)
90
Brain abscess
Localized necrosis w/ inflammation Collagenous capsule via BV fibroblasts Generally via bacterial infection
91
Common causes of brain abscesses
Staph. aureus, streptococci
92
CSF in brain abscess
High WBC Increased protein Normal glucose
93
Complications of brain abscess
Herniation, rupture, sinus thrombosis
94
Subdural empyema Dangers?
Infection of skull or air sinuses --> subdural space Mass effect OR thrombophlebitis of bridging veins --> infarction/edema
95
Clinical presentation of subdural empyema
Fever, headache, neck stiffness CSF like brain abscess
96
Chronic TB meningoencephalitis... CSF? Complications?
Pleocytosis, HIGH HIGH PROTEIN, little reduced/normal glucose Hydrocephalus via arachnoid fibrosis Obliterative endarteritis --> arterial occlusion --> infarction
97
Chronic meningitis Immunocompromised Macrophages filled with organisms Few/no granulomas
Avium-intracellulare
98
Neurosyphilis
Tabes dorsalis + paresis Mood alterations (grandeur) - NAPOLEAN Dementia Ataxia, loss of pain/temp, lightning pains in back and backs of legs, no DTRs
99
Neuroborreliosis (Lyme disease)
Meningitis + facial nerve palsies (Bell's) + polyneuropathies + encephalopathy
100
Viral CNS infection after LATENCY
Herpes zoster | PML (JC polyomavirus)
101
Polio-like spinal cord paralysis | Viral encephalitis
West Nile
102
Viral encephalitis causes (9)
``` West Nile HSV-1 HSV-2 Herpes Zoster CMV Poliomyelitis Rabies HIV JC Polyomavirus ```
103
``` Viral encephalitis Child/young adult Altered mood, memory, behavior Temporal lobes Perivascular infiltrates Cowdry A viral inclusions in neurons/glia ```
HSV-1 encephalitis
104
Painful vesicular skin eruption of dermatomal pattern
``` Herpes Zoster (Shingles) Re-activation ```
105
Elderly | Persistent pain via not-painful stimuli
Postherpetic neuralgia (Herpes zoster)
106
Fetus Periventricular necrosis/calcification Microcephaly Enlarged CNS cells with inclusions (nuclear and cytoplasmic)
CMV encephalitis
107
Gastroenteritis Aseptic meningitis Flaccid paralysis + hyporeflexia Respiratory compromise, death
Poliomyelitis (secondary CNS invasion) | - Anterior horn motor neurons of spinal cord
108
``` 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
Rabies encephalitis
109
Microglial nodules Multinucleated giant cells Necotic foci with reactive gliosis Cognitive dysfunction
HIV encephalitis (chronic microglial inflammation)
110
Immunosuppressed Demyelination at multiple foci Progressive
PML (JC polyomavirus) | - Via Re-activation w/ immunosuppression
111
Immunocompromised Hemorrhagic infarction --> sepsis Vasculitits
Mucormycosis or Aspergillosis (fungal meningoencephalitis)
112
Fungal causes of parenchymal granulomas/abscesses
Candida, Cryptococcus
113
HIV immunosuppression Ring-enhancing lesions (multiple) near gray-white junction and in deep gray nuclei Tachyzoites and bradyzoites in necrotic foci
Cerebral toxoplasmosis
114
Ring-enhancing lesions
CNS lymphoma TB Fungal infections
115
Dives into still water Necrotic encephalitis Rapidly progressive/fatal
Naegleria
116
Mad Cow Disease
Variant Creutzfeldt-Jakob Disease
117
Prion disease without spongiform features
Fatal Familial Insomnia
118
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
Multiple sclerosis
119
Pathogenesis of MS Viral roll?
Auto-immune Th1 and Th17 cells react against myelin antigens EBV (or other) infection activates self-reactive T cells
120
Bilateral optic neuritis | Anti-aquaporin antibodies
Neuromyelitis optica
121
Viral infection or immunization Headache, lethargy, coma Rapid progression Type of disease?
Acute Disseminated Encephalomyelitis Demyelinating
122
Recent URI Perivenular demyelniation throughout CNS Disseminated necrosis of white and gray matter Hemorrhage, fibrin, neutrophils Type of disease?
Acute Necrotizing Hemorrhagic Encephalomyelitis Demyelinating
123
Correction of hyponatremia Rapidly evolving quadriplegia "Locked-in syndrome" Type of disease
Central pontine myelinolysis Demyelinating
124
Neurodegenerative diseases
Progressive loss of neurons with functional relationships | Accumulation of protein aggregates
125
3 reasons for protein aggregation in neurodegenerative diseases
Mutated protein Peptide of larger protein Unexplained alteration of normal protein
126
Cerebral cortex degenerative diseases
Alzheimer's | FTLD
127
Basal ganglia/brainstem degenerative diseases
``` Parkinson Progressive supranuclear palsy Corticobasal degeneration MSA Huntington Spinocerebellar ataxias ```
128
Motor neuron degerative diseases
ALS | Spinal bulbar muscular atrophy
129
A-beta inclusions
Alzheimer's
130
Tau inclusions
Alzheimer's FTLD (behavioral, language dysfunctions) Parkinson's (hypokinetic movement) Progressive supranuclear palsy (Park. + eye movements) Corticobasal degeneration (Park. + asymmetric movements)
131
TDP-43
FTLD | ALS
132
FUS
FTLD | ALS
133
Alpha-synuclein
Parkinson's | MSA
134
Poly-glutamine (CAG)
Huntington Spinocerebellar ataxia Spinal bulbar muscular atrophy
135
Presenilin mutation
Alzheimer's | Gamma-secretase makes more A-beta 42
136
When does tau aggregate?
Hyperphosphorylated
137
Cause of degeneration in Alzheimer's
A-beta aggregates cause inflammatory response from microglia and astrocytes (including cytokine mediators) Inflammatory cascades also hyperphosphorylate tau
138
Forgetfulness, memory issues Language, math, learned motor deficits Incontinent, mute, unable to walk Death by ______
Alzheimer's Pneumonia (intercurrent disease)
139
Personality, behavior, language (aphasia) alterations Later, memory loss Eventually global dementia (before 65 often)
Frontotemporal lobar degeneration
140
FTLD symptoms | Smooth cellular inclusions
Pick disease
141
Two forms of FTLD-TDP
C9orf72 (familial) | TDP-43 (ALS-like symptoms)
142
Classic Parksonsonism symptoms
``` Diminished facial expression (masked facies) Stooped posture Slowed voluntary movements Festinating gait Cog-wheel rigidity Pill-rolling resting tremor ```
143
Tremor Rigidity Bradykinesia (slowed movements)
CENTRAL TRIAD of Parkinsonism
144
Autosomal dominant Parkinson's
Alpha-synuclein, Lewy bodies LRRK2 gene mutation
145
Autosomal recessive Parkinson's
Mitochondrial-related proteins (DJ-1, PINK1, parkin)
146
Pallor of the substantia nigra (and locus ceruleus)
Parkinson's (loss of dopaminergic neurons)
147
Parkinsonian symptoms Hallucinations Frontal lobe signs (conscious thought, etc.)
Dementia with Lewy Bodies
148
Truncal rigidity Difficulty with eye movements Parkinsonian symptoms Tau inclusions in brainstem/cerebellum
Progressive supranuclear palsy Brainstem/cerebellum focus
149
Truncal rigidity Jerking movements of limbs Difficulty performing asked actions (apraxia) Balooned neurons in cortex, tau inclusions
Corticobasal degeneration Cortical focus
150
Alpha-synuclein in oligodendrocytes Parkonsonian symptoms Ataxia Orthostatic hypotention
Multiple system atrophy
151
Why is it called MSA?
Degeneration of 3 white matter tracts: - Striatonigral - Olivopotocerebella - ANS
152
``` 40s-50s Jerky, hyperkinetic, chorea movements Bradykinesia Rigidity Writhing movements of extremities Poly-glutamine repeats in HTT gene ```
Huntington's
153
Cause of chorea in Huntington's
Loss of GABAergic neurons in striatonigral system | - Thus, loss of cortical inhibition of skeletal muscle contractions
154
Causes of death in Huntington's
Intercurrent infection | Suicide
155
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
Friedreich Ataxia
156
Child Recurrent sinopulmonary infections, difficulty walking Dysarthria, eye movement abnormalities Red/purple clusters/lesions on conjunctiva and skin T-cell leukemias
Ataxia-Telangiectasia
157
Spastic paralysis | Flaccid paralysis
ALS
158
Superoxide dismutase mutation
ALS
159
C9orf72 mutation
ALS (motor neurons) + FTLD (behavior, language)
160
``` Weakness of hands Dropping objects Cramping/spacticity of arms and legs Fasciculations Recurrent pulmonary infections ```
ALS
161
Variants of ALS
Progressive muscular atrophy (LMNs mostly) Primary lateral sclerosis (UMNs mostly) Progressive bulbar palsy (phonation/chewing mostly)
162
Progressive bulbar palsy
ALS with issues in nucleus ambiguus and hypoglossal nucleus
163
Kennedy disease
LMN degeneration Atrophy of tongue and dysphagia Androgen insensitivity, gynecomastia, testicular atrophy
164
Baby Deficiency of beta-galactosidase Stiffness, weakness Die young
Krabbe disease
165
Deficiency of arylsulfatase A Cerebroside sulfate (sulfatides) accumulation Dyes cause shift of absorbance spectrum
Metachromatic leukodystrophy
166
X-linked (mostly young men) Behavioral changes Adrenal insufficiency Elevated very long chain FA's (VLCFAs) in serum
Adrenoleukodystrophy
167
GFAP gene mutation
Alexander disease
168
eIF2B gene mutation
Vanishing white matter leukoencephalopathy
169
``` Recurrent episodes of acute neuro dysfunction Cognitive changes Weakness Stroke-like episodes Lactic acidosis MTTL1 mutation ```
MELAS (mitochondrial encephalopathy, lactic acidosis, stroke-like episodes)
170
``` Infant Lactic acidemia Feeding problems Seizures Extraocular palsies Weakness with hypotonia Spongiform brain and BV proliferation ```
Leigh syndrome
171
``` Psychotic symptoms Ophthalmoplegia Hemorrhagic foci Necrosis of mamillary bodies and ventricular walls Hemosideran-laden macrophages ```
Wernicke encephalopathy (thiamine deficiency)
172
Short term memory loss Confabulation Alcoholic/gastric disorders
Korsakoff syndrome (thiamine deficiency)
173
Bilateral numbness, tingling, ataxia of LEs Progression to spastic weakness Eventual complete paraplagia Starts where?
Vitamin B12 deficiency (subacute combined degeneration of spinal cord) Mid-thoracic level, then extends up and down
174
Degeneration of retinal ganglion cells | Makes moonshine
Methanol toxicity

Neurology 2 (22 decks)

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