Pathoma: CNS Flashcards
(217 cards)
1
Q
Incomplete closure of neural tube
A
Neural Tube Defect
2
Q
Low folate levels prior to conception
A
Neural Tube Defect
3
Q
Detected by high AFP levels
A
Neural Tube Defect
4
Q
Absence of skull and brain
A
Anencephaly
5
Q
Frog-like appearance
A
Anencephaly
6
Q
Result in maternal polyhydramnios
A
Anencephaly
7
Q
Failure of posterior vertebral arch to close
A
Spina bifida
8
Q
Dimple or patch of hair overlying vertebral defect
A
Spina bifida occulta
9
Q
Cystic protrusion of underlying tissue through vertebral defect
A
Spina bifida
10
Q
Protrusion of meninges
A
Meningocele
11
Q
Protrusion of meninges and spinal cord
A
Meningomyelocele
12
Q
Congenital narrowing of channel that drains CSF from 3rd to 4th ventricle
A
Cerebral Aqueduct Stenosis
13
Q
Most common cause of hydrocephalus in newborns
A
Cerebral Aqueduct Stenosis
14
Q
Presents with enlarging head circumference due to dilation of ventricles
A
Cerebral Aqueduct Stenosis
15
Q
Congenital failure of cerebellar vermis to develop
A
Dandy-Walker Malformation
16
Q
Presents as massively dilated 4th ventricle and absent cerebellum.
A
Dandy-Walker Malformation
17
Q
Congenital downward displacement of cerebellar vermis and tonsils through foramen magnum
A
Arnold-Chiari Malformation Type II
18
Q
Obstruction results in hydrocephalus
A
Arnold-Chiari Malformation Type II
19
Q
May occur in association with meningomyelocele and syringomyelia
A
Arnold-Chiari Malformation Type II
20
Q
Cystic degeneration of spinal cord
A
Syringomyelia
21
Q
Cause: Trauma or Arnold-Chiari malformation
A
Syringomyelia
22
Q
Occurs at C8-T1
A
Syringomyelia
23
Q
Presents as sensory loss of pain and temperature with sparing of fine touch and position sense in uppper extremities (Cape-like distribution)
A
Syringomyelia
24
Q
Expansion leads to damage of LMN’s of anterior horn (muscle atrophy) and damage of lateral horn of hypothalamospinal tract (Horner syndrome)
A
Syringomyelia
25
Damange to anterior motor due to poliovirus infection
Poliomyelitis
26
Presents with lower motor neuron signs (flaccid paralysis with muscle atrophy, fasciculations, weak muscles, impaired reflexes, and negative Babinski
Poliomyelitis
27
Inherited Degeneration of Anterior Motor Horn
Werdnig-Hoffman Disease
28
Autosomal Recessive
Werdnig-Hoffman Disease
29
Presents as floppy baby, with death within a few years
Werdnig-Hoffman Disease
30
Degenerative disorder of upper and lower motor neurons of corticospinal tract
ALS
31
Presents with lower motor neuron signs (flaccid paralysis with muscle atrophy, fasciculations, weak muscles, impaired reflexes, and negative Babinski
ALS
32
Presents with upper motor neuron signs (spastic paralysis with hyperreflexia, increased tone, positive Babinski sign
ALS
33
Atrophy and weakness of hands is an early sign
ALS
34
Lack of sensory impairment distinguishes this from syringomyelia
ALS
35
Most cases are sporadic arising in middle age adults
ALS
36
Zinc-copper superoxide dismutase mutation (SOD1) is present in some familial cases, leads to free radical injury
ALS
37
Degenerative disorder of cerebellum and spinal cord
Friedrich Ataxia
38
Presents with ataxia (cerebellum) and loss of vibratory sense and proprioception, muscle weakness in legs, and loss of deep tendon reflexes
Friedrich Ataxia
39
Autosomal recessive due to unstable trinucleotide repeat (GAA) in frataxin gene
Friedrich Ataxia
40
This mutated protein is essential for mitochondrial iron regulation
Friedrich Ataxia
41
Presents with iron buildup
Friedrich Ataxia
42
Presents in early childhood
Friedrich Ataxia
43
Associated with hypertrophic cardiomyopathy
Friedrich Ataxia
44
Inflammation of leptomeninges (pia and arachnoid)
Meningitis
45
Most commonly due to an infectious agent
Meningitis
46
Most common neonate meningitis causes
GBS, E. Coli, Listeria Monocytogenes
47
Most common children/teenager meningitis cause
N. Meningitidis
48
Most common adult and elderly meningitis cause
Streptococcus pneumoniae
49
Most common non-vaccinated infant meningitis
H. influenza
50
Most common viral cause of meningitis
Coxsackie virus
51
Most common immunocompromised cause of meningitis
Fungi
52
Classic triad of headache, nuchal rigidity, and fever
Meningitis
53
Diagnosis made by lumbar puncture
Meningitis
54
CSF finding: Neutrophils with decreased glucose
Bacterial Meningitis
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CSF finding: Lymphocytes with normal glucose
Viral Meningitis
56
CSF finding: Lymphocytes with decreased glucose
Fungal meningitis
57
Major cause of cerebrovascular disease
Ischemia
58
Causes: Low perfusion, acute decrease in blood flow, chronic hypoxia, and repeated hypoglycemia
Global Cerebral Ischemia
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Presents as transient confusion with prompt recovery
Mild Global Ischemia
60
Presents as diffuse necrosis with vegetative state
Severe global ischemia
61
Infarcts in watershed areas (between anterior and middle cerebral rtery
Moderate global ischemia
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Damage in this results in laminar necrosis of pyramidal cortex neurons, damage to pyramidal hippocampus neurons, and damage to purkinje layer of cerebellum
Moderate global ischemia
63
Focal neurologic deficits lasting longer than 24 hours
Ischemic stroke
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Focal neurologic deficits lasting less than 24 hours
Transient Ischemic Attack
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Due to rupture of an atherosclerotic plaque
Thrombotic stroke
66
Usually involves the bifurcation of internal carotid and middle cerebral artery in circle of Willis
Thrombotic stroke
67
Results in pale infarct
Thrombotic stroke
68
Due to thromboemboli
Embolic stroke
69
Most common source is left side of heart
Embolic stroke
70
Usually involves the middle cerebral artery
Embolic stroke
71
Results in hemorrhagic infarct at periphery of cortex
Embolic stroke
72
Occurs secondary to hyaline arteriolosclerosis due to hypertension
Lacunar Stroke
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Most involve the lenticulostriate vessels resulting in small cystic areas of infarction
Lacunar Stroke
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Involvement of internal capsule leads to pure motor stroke
Lacunar Stroke
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Involvement of thalamus leads to a pure sensory stroke
Lacunar Stroke
76
Results in liquefactive necrosis
Ischemic stroke
77
Presents as eosinophilic/red neurons within 12 hours of occureence
Ischemic stroke
78
Necrosis occurs by 24 hours
Ischemic stroke
79
Infiltration of neutrophils in days 1-3
Ischemic stroke
80
Microglial cells in days 4-7
Ischemic stroke
81
Gliosis in weeks 2-3
Ischemic stroke
82
Classically due to Charcot-Bouchard microaneurysms of lenticulostriate vessels
Intracerebral Hemorrhage
83
Basal ganglia is most common site
Intracerebral Hemorrhage
84
Presents as severe headache, flu-like, and eventual coma
Intracerebral Hemorrhage
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Bleeding into brain parenchyma
Intracerebral Hemorrhage
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Bleeding into subarachnoid space
Subarachnoid hemorrhage
87
Presents as sudden headache (Worst headache of my life) with nuchal rigidity
Subarachnoid hemorrhage
88
Lumbar puncture shows xanthochromia due to bilirubin breakdown
Subarachnoid hemorrhage
89
85% are due to berry aneurysm
Subarachnoid hemorrhage
90
Most frequently located in anterior circle of Willis at branch points of anterior communicating artery
Subarachnoid hemorrhage
91
Associated with Marfan syndrome and autosomal dominant polycystic kidney disease
Subarachnoid hemorrhage
92
Collection of blood between dura and skull
Epidural Hematoma
93
Clasically due to fracture of temporal bone with rupture of middle meningeal artery
Epidural Hematoma
94
Lens-shaped lesion on CT
Epidural Hematoma
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Collection of blood underneath dura
Subdural Hematoma
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Due to tearing of bridging veins that lie between the dura and arachnoid
Subdural Hematoma
97
Crescent-shaped lesion on CT
Subdural Hematoma
98
Presents with progressive neurologic signs
Subdural Hematoma
99
Displacement of brian tissue due to mass effect or increased intracranial pressure
Herniation
100
Displacement of cerebellar tonsils into foramen magnum
Tonsillar herniation
101
Compression of brain stem leads to cardiopulmonary arrest
Tonsillar herniation
102
Displacement of cingulate gyrus under falx cerebri
Subfalcine herniation
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Compression of anterior cerebral artery leads to infarction
Subfalcine herniation
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Displacement of temporal lobe uncus under the tentoriumcerebelli
Uncal herniation
105
Compression of cranial nerve III leads to eye moving down and out with dilated pupil
Uncal herniation
106
Compression of posterior cerebral artery leads to infarction of occipital lobe with contralateral homonymous hemianopsia
Uncal herniation
107
Rupture of paramedian artery leads to Duret hemorrhage
Uncal herniation
108
inherited mutations in enzymes necessary for production or maintenance of myelin
Leukodystrophies
109
Due to deficiency of arylsulfatase (autosomal recessive)
Metachromatic leukodystrophy
110
Most common leukodystrophy
Metachromatic leukodystrophy
111
Sulfatides can't be degraded and accumulate in lysosomes of oligodendrocytes
Metachromatic leukodystrophy
112
Autosomal recessive deficiency of galactocerebrosidase
Krabbe disease
113
Accumulates in macrophages
Krabbe disease
114
Impaired addition of coenzyme A to long-chain fatty acids (X-linked defect)
Adrenoleukodystrophy
115
Accumulation of fatty acids damages adrenal glands and white matter of brain
Adrenoleukodystrophy
116
Autoimmune destruction of CNS myelin and oligodendocytes
Multiple Sclerosis
117
Most common chronic CNS disease of young adults, more commonly seen in women
Multiple Sclerosis
118
Associated with HLA-DR2
Multiple Sclerosis
119
More commonly see in regions away from equator
Multiple Sclerosis
120
Presents with relapsing neurologic deficits with periods of remission
Multiple Sclerosis
121
Presents with blurred vision in one eye and internuclear opthalamoplegia
Multiple Sclerosis
122
Presents with vertigo and scanning speech mimicking drunkeness
Multiple Sclerosis
123
Presents with hemiparesis or unilateral loss of sensation, lower extremity loss of sensation or weakness, and bowel, bladder & sexual dysfunction
Multiple Sclerosis
124
Diagnosis is made by MRI and lumbar puncture
Multiple Sclerosis
125
MRI reveals plaques and lumbar puncture shows increased lymphocytes, IG's with oligoclonal IgG bands, and myelin basic protein
Multiple Sclerosis
126
Treatment of acute attacks is high-dose steroids
Multiple Sclerosis
127
Treatment long-term is interferon beta
Multiple Sclerosis
128
Due to slow progressing, persistent infection of brain by measles virus
Subacute Sclerosing Pancencephalitis
129
Infection occurs in infancy with neurologic signs years later
Subacute Sclerosing Pancencephalitis
130
Characterized by viral inclusions within neurons of gray matter and oligodendrocytes of white matter
Subacute Sclerosing Pancencephalitis
131
JC virus infection of oligodendrocytes
Progressive multifocal leukoencephalopathy
132
Immunosuppression leads to reactivation of latent virus
Progressive multifocal leukoencephalopathy
133
Presents with progressive neurologic signs (Visual loss, weakness, dementia) leading to death
Progressive multifocal leukoencephalopathy
134
Focal demyelination of the pons
Central pontine myelinolysis
135
Due to rapid intravenous correction of hyponatremia
Central pontine myelinolysis
136
Occurs in severely malnourished patients (alcoholics and liver disease)
Central pontine myelinolysis
137
Classically presents as acute bilateral paralysis (locked in syndrome) with only exception being the eyes
Central pontine myelinolysis
138
Degeneration of cortex
Dementia
139
Degeneration of brainstem and basal ganglia
Movement disorders
140
Most common cause of dementia
Alzheimer Disease
141
Presents as slow-onset memory loss (short-term first) and progressive disorientation
Alzheimer Disease
142
Loss of learned motor skills and language
Alzheimer Disease
143
Changes in behavior and personality
Alzheimer Disease
144
Most cases are sporadic and seen in elderly
Alzheimer Disease
145
Risk of this increases with age
Alzheimer Disease
146
ApoE4 is associated with increased risk
Alzheimer Disease
147
ApoE2 is associated with decreased risk
Alzheimer Disease
148
Seen in presenilin 1 and presenilin 2 mutations
Early-Onset Alzheimer Disease
149
Seen in Down syndrome
Early-Onset Alzheimer Disease
150
Cerebral atrophy with narrowing of the gyri, widening of sulci, dilation of ventricles
Alzheimer Disease
151
Neuritic plaques of A-Beta amyloid with entangled neuriti cprocesses
Alzheimer Disease
152
APP on chromosome 21 is the source of protein to be turned into Beta amyloid
Alzheimer Disease
153
Presents with neurofibrillary tangles: aggregates of hyperphosphorylated tau protein
Alzheimer Disease
154
Diagnosis of exclusion
Alzheimer Disease
155
Diagnosis confirmed upon autopsy
Alzheimer Disease
156
Multifocal infarction and injury due to HTN, atherosclerosis, or vasculitis
Vascular Dementia
157
2nd most common cause of dementia
Vascular Dementia
158
Degenerative disease of frontal and temporal cortex
Pick Disease
159
Characterized by round aggregates of tau protein
Pick Disease
160
Presents with behavioral and language symptoms early
Pick Disease
161
Degenerative loss of dopaminergic neurons in substantia nigra of basal ganglia
Parkinson Disease
162
Rare cases related to MPTP exposure
Parkinson Disease
163
Tremor
Rigidity
Akinesia/Bradykinesia
Postural instability/Shuffling Gait
Parkinson Disease
164
Loss of pigmented neurons in substantia nigra
Parkinson Disease
165
Eosinophilic inclusions of alpha-synuclein (Lewy bodies)
Parkinson Disease
166
Usually the cause of early-onset dementia
Parkinson Disease
167
Degeneration of GABAergic neurons in caudate nucleus of basal ganglia
Huntington Disease
168
Autosomal dominant disorder of CAG trinucleotide repeat of gene on chromosome 4
Huntington Disease
169
Further expansion of repeats during spermatogenesis leads to anticipation
Huntington Disease
170
Presents with chorea that progresses to dementia and depression
Huntington Disease
171
Suicide is common cause of death
Huntington Disease
172
Increased CSF resulting in dilated ventricles and stretching of nerves
Normal Pressure Hydrocephalus
173
Presents as triad of urinary incontinence, gait instability and dementia
(Wet, Wacky, Wobbly)
Normal Pressure Hydrocephalus
174
Lumbar puncture improves symptoms
Normal Pressure Hydrocephalus
175
Treatment is ventriculoperitoneal shunting
Normal Pressure Hydrocephalus
176
Degenerative disease due to prion protein
Spongiform Encephalopathy
177
Disease arises with conversion to a Beta-pleated conformation
Spongiform Encephalopathy
178
Damage to neurons and glial cells characterized by intracellular vacuoles
Spongiform Encephalopathy
179
Most common spongiform encephalopathy
Creutzfeldt-Jakob Disease
180
Presents as rapidly progressive dementia with ataxia and startle myoclonus
Creutzfeldt-Jakob Disease
181
Periodic sharp waves are seen on EEG
Creutzfeldt-Jakob Disease
182
Related to exposure to bovine spongiform encephalopathy
Variant Cruejtzfeldt-Jakob Disease
183
Inherited form of prion disease characterized by severe insomnia and exaggerated startle response
Familial Fatal Insomnia
184
Most common primary malignant CNS tumor in adults
Glioblastoma Multiforme
185
Malignant, high grade tumor of astrocytes
Glioblastoma Multiforme
186
Arises in cerebral hemisphere and crosses the corpus callosum to form a butterfly lesion
Glioblastoma Multiforme
187
Characterized by regions of necrosis surrounded by tumor cells (Pseudopalisading) and endothelial cell proliferation
Glioblastoma Multiforme
188
Tumor cells are GFAP positive
Glioblastoma Multiforme
189
Benign tumor of arachnoid cells
Meningioma
190
Most common benign CNS tumor in adults
Meningioma
191
More commonly seen in women due to being estrogen sensitive
Meningioma
192
Present as seizures
Meningioma
193
Findings: Round mass attached to dura, whorled pattern and possible psammoma bodies
Meningioma
194
Benign tumor of Schwann cells
Schwannoma
195
Involves cranial or spinal nerves, most frequently CN VIII at cerebellopontine angle
Schwannoma
196
Presents as loss of hearing and tinnitus
Schwannoma
197
Tumor cells are S-100 positive
Schwannoma
198
Bilateral forms of this are seen in neurofibromatosis type 2
Schwannoma
199
Malignant tumor of oligodendrocytes
Oligodendroglioma
200
Calcified tumor in white matter usually in frontal lobe
Oligodendroglioma
201
Presents as seizures
Oligodendroglioma
202
Fried egg appearance of cells on biopsy
Oligodendroglioma
203
Benign tumor of astrocytes
Pilocytic astrocytoma
204
Most common CNS tumor in kids
Pilocytic astrocytoma
205
Imaging reveals cystic lesion with a mural nodule
Pilocytic astrocytoma
206
Biopsy shows Rosenthal fibers and eosinophilic granular bodies
Pilocytic astrocytoma
207
Tumor cells are GFAP positive
Pilocytic astrocytoma
208
Malignant tumor derived from granular cells of cerebellum in kids
Medulloblastoma
209
Small round blue cells with Homer-Wright rosettes
Medulloblastoma
210
Metastasis of this to cauda equina is termed drop metastasis
Medulloblastoma
211
Malignant tumor of ependymal cells in kids
Ependymoma
212
Most commonly arises in 4th ventricle and may presents with hydrocephalus
Ependymoma
213
Primary finding are perivascular pseudorosettes
Ependymoma
214
Tumor that arises from epithelial remnants of Rathke's pouch
Craniopharyngioma
215
Presents as a supratentorial mass in a child or young adult
Craniopharyngioma
216
May compress the optic chiasm leading to bitemporal hemianopsia
Craniopharyngioma
217
Calcifications are commonly seen on imaging (derived from tooth-like tissue)
Craniopharyngioma
