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Pathology > Pathoma: CNS > Flashcards

Pathoma: CNS Flashcards

1
Q

Incomplete closure of neural tube

A

Neural Tube Defect

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2
Q

Low folate levels prior to conception

A

Neural Tube Defect

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3
Q

Detected by high AFP levels

A

Neural Tube Defect

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4
Q

Absence of skull and brain

A

Anencephaly

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5
Q

Frog-like appearance

A

Anencephaly

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6
Q

Result in maternal polyhydramnios

A

Anencephaly

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7
Q

Failure of posterior vertebral arch to close

A

Spina bifida

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8
Q

Dimple or patch of hair overlying vertebral defect

A

Spina bifida occulta

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9
Q

Cystic protrusion of underlying tissue through vertebral defect

A

Spina bifida

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10
Q

Protrusion of meninges

A

Meningocele

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11
Q

Protrusion of meninges and spinal cord

A

Meningomyelocele

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12
Q

Congenital narrowing of channel that drains CSF from 3rd to 4th ventricle

A

Cerebral Aqueduct Stenosis

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13
Q

Most common cause of hydrocephalus in newborns

A

Cerebral Aqueduct Stenosis

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14
Q

Presents with enlarging head circumference due to dilation of ventricles

A

Cerebral Aqueduct Stenosis

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15
Q

Congenital failure of cerebellar vermis to develop

A

Dandy-Walker Malformation

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16
Q

Presents as massively dilated 4th ventricle and absent cerebellum.

A

Dandy-Walker Malformation

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17
Q

Congenital downward displacement of cerebellar vermis and tonsils through foramen magnum

A

Arnold-Chiari Malformation Type II

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18
Q

Obstruction results in hydrocephalus

A

Arnold-Chiari Malformation Type II

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19
Q

May occur in association with meningomyelocele and syringomyelia

A

Arnold-Chiari Malformation Type II

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20
Q

Cystic degeneration of spinal cord

A

Syringomyelia

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21
Q

Cause: Trauma or Arnold-Chiari malformation

A

Syringomyelia

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22
Q

Occurs at C8-T1

A

Syringomyelia

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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

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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

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25
Q

Damange to anterior motor due to poliovirus infection

A

Poliomyelitis

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26
Q

Presents with lower motor neuron signs (flaccid paralysis with muscle atrophy, fasciculations, weak muscles, impaired reflexes, and negative Babinski

A

Poliomyelitis

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27
Q

Inherited Degeneration of Anterior Motor Horn

A

Werdnig-Hoffman Disease

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28
Q

Autosomal Recessive

A

Werdnig-Hoffman Disease

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29
Q

Presents as floppy baby, with death within a few years

A

Werdnig-Hoffman Disease

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30
Q

Degenerative disorder of upper and lower motor neurons of corticospinal tract

A

ALS

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31
Q

Presents with lower motor neuron signs (flaccid paralysis with muscle atrophy, fasciculations, weak muscles, impaired reflexes, and negative Babinski

A

ALS

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32
Q

Presents with upper motor neuron signs (spastic paralysis with hyperreflexia, increased tone, positive Babinski sign

A

ALS

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33
Q

Atrophy and weakness of hands is an early sign

A

ALS

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34
Q

Lack of sensory impairment distinguishes this from syringomyelia

A

ALS

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35
Q

Most cases are sporadic arising in middle age adults

A

ALS

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36
Q

Zinc-copper superoxide dismutase mutation (SOD1) is present in some familial cases, leads to free radical injury

A

ALS

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37
Q

Degenerative disorder of cerebellum and spinal cord

A

Friedrich Ataxia

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38
Q

Presents with ataxia (cerebellum) and loss of vibratory sense and proprioception, muscle weakness in legs, and loss of deep tendon reflexes

A

Friedrich Ataxia

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39
Q

Autosomal recessive due to unstable trinucleotide repeat (GAA) in frataxin gene

A

Friedrich Ataxia

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40
Q

This mutated protein is essential for mitochondrial iron regulation

A

Friedrich Ataxia

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41
Q

Presents with iron buildup

A

Friedrich Ataxia

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42
Q

Presents in early childhood

A

Friedrich Ataxia

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43
Q

Associated with hypertrophic cardiomyopathy

A

Friedrich Ataxia

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44
Q

Inflammation of leptomeninges (pia and arachnoid)

A

Meningitis

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45
Q

Most commonly due to an infectious agent

A

Meningitis

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46
Q

Most common neonate meningitis causes

A

GBS, E. Coli, Listeria Monocytogenes

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47
Q

Most common children/teenager meningitis cause

A

N. Meningitidis

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48
Q

Most common adult and elderly meningitis cause

A

Streptococcus pneumoniae

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49
Q

Most common non-vaccinated infant meningitis

A

H. influenza

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50
Q

Most common viral cause of meningitis

A

Coxsackie virus

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51
Q

Most common immunocompromised cause of meningitis

A

Fungi

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52
Q

Classic triad of headache, nuchal rigidity, and fever

A

Meningitis

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53
Q

Diagnosis made by lumbar puncture

A

Meningitis

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54
Q

CSF finding: Neutrophils with decreased glucose

A

Bacterial Meningitis

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55
Q

CSF finding: Lymphocytes with normal glucose

A

Viral Meningitis

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56
Q

CSF finding: Lymphocytes with decreased glucose

A

Fungal meningitis

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57
Q

Major cause of cerebrovascular disease

A

Ischemia

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58
Q

Causes: Low perfusion, acute decrease in blood flow, chronic hypoxia, and repeated hypoglycemia

A

Global Cerebral Ischemia

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59
Q

Presents as transient confusion with prompt recovery

A

Mild Global Ischemia

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60
Q

Presents as diffuse necrosis with vegetative state

A

Severe global ischemia

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61
Q

Infarcts in watershed areas (between anterior and middle cerebral rtery

A

Moderate global ischemia

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62
Q

Damage in this results in laminar necrosis of pyramidal cortex neurons, damage to pyramidal hippocampus neurons, and damage to purkinje layer of cerebellum

A

Moderate global ischemia

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63
Q

Focal neurologic deficits lasting longer than 24 hours

A

Ischemic stroke

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64
Q

Focal neurologic deficits lasting less than 24 hours

A

Transient Ischemic Attack

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65
Q

Due to rupture of an atherosclerotic plaque

A

Thrombotic stroke

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66
Q

Usually involves the bifurcation of internal carotid and middle cerebral artery in circle of Willis

A

Thrombotic stroke

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67
Q

Results in pale infarct

A

Thrombotic stroke

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68
Q

Due to thromboemboli

A

Embolic stroke

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69
Q

Most common source is left side of heart

A

Embolic stroke

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70
Q

Usually involves the middle cerebral artery

A

Embolic stroke

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71
Q

Results in hemorrhagic infarct at periphery of cortex

A

Embolic stroke

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72
Q

Occurs secondary to hyaline arteriolosclerosis due to hypertension

A

Lacunar Stroke

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73
Q

Most involve the lenticulostriate vessels resulting in small cystic areas of infarction

A

Lacunar Stroke

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74
Q

Involvement of internal capsule leads to pure motor stroke

A

Lacunar Stroke

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75
Q

Involvement of thalamus leads to a pure sensory stroke

A

Lacunar Stroke

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76
Q

Results in liquefactive necrosis

A

Ischemic stroke

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77
Q

Presents as eosinophilic/red neurons within 12 hours of occureence

A

Ischemic stroke

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78
Q

Necrosis occurs by 24 hours

A

Ischemic stroke

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79
Q

Infiltration of neutrophils in days 1-3

A

Ischemic stroke

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80
Q

Microglial cells in days 4-7

A

Ischemic stroke

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81
Q

Gliosis in weeks 2-3

A

Ischemic stroke

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82
Q

Classically due to Charcot-Bouchard microaneurysms of lenticulostriate vessels

A

Intracerebral Hemorrhage

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83
Q

Basal ganglia is most common site

A

Intracerebral Hemorrhage

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84
Q

Presents as severe headache, flu-like, and eventual coma

A

Intracerebral Hemorrhage

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85
Q

Bleeding into brain parenchyma

A

Intracerebral Hemorrhage

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86
Q

Bleeding into subarachnoid space

A

Subarachnoid hemorrhage

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87
Q

Presents as sudden headache (Worst headache of my life) with nuchal rigidity

A

Subarachnoid hemorrhage

88
Q

Lumbar puncture shows xanthochromia due to bilirubin breakdown

A

Subarachnoid hemorrhage

89
Q

85% are due to berry aneurysm

A

Subarachnoid hemorrhage

90
Q

Most frequently located in anterior circle of Willis at branch points of anterior communicating artery

A

Subarachnoid hemorrhage

91
Q

Associated with Marfan syndrome and autosomal dominant polycystic kidney disease

A

Subarachnoid hemorrhage

92
Q

Collection of blood between dura and skull

A

Epidural Hematoma

93
Q

Clasically due to fracture of temporal bone with rupture of middle meningeal artery

A

Epidural Hematoma

94
Q

Lens-shaped lesion on CT

A

Epidural Hematoma

95
Q

Collection of blood underneath dura

A

Subdural Hematoma

96
Q

Due to tearing of bridging veins that lie between the dura and arachnoid

A

Subdural Hematoma

97
Q

Crescent-shaped lesion on CT

A

Subdural Hematoma

98
Q

Presents with progressive neurologic signs

A

Subdural Hematoma

99
Q

Displacement of brian tissue due to mass effect or increased intracranial pressure

A

Herniation

100
Q

Displacement of cerebellar tonsils into foramen magnum

A

Tonsillar herniation

101
Q

Compression of brain stem leads to cardiopulmonary arrest

A

Tonsillar herniation

102
Q

Displacement of cingulate gyrus under falx cerebri

A

Subfalcine herniation

103
Q

Compression of anterior cerebral artery leads to infarction

A

Subfalcine herniation

104
Q

Displacement of temporal lobe uncus under the tentoriumcerebelli

A

Uncal herniation

105
Q

Compression of cranial nerve III leads to eye moving down and out with dilated pupil

A

Uncal herniation

106
Q

Compression of posterior cerebral artery leads to infarction of occipital lobe with contralateral homonymous hemianopsia

A

Uncal herniation

107
Q

Rupture of paramedian artery leads to Duret hemorrhage

A

Uncal herniation

108
Q

inherited mutations in enzymes necessary for production or maintenance of myelin

A

Leukodystrophies

109
Q

Due to deficiency of arylsulfatase (autosomal recessive)

A

Metachromatic leukodystrophy

110
Q

Most common leukodystrophy

A

Metachromatic leukodystrophy

111
Q

Sulfatides can’t be degraded and accumulate in lysosomes of oligodendrocytes

A

Metachromatic leukodystrophy

112
Q

Autosomal recessive deficiency of galactocerebrosidase

A

Krabbe disease

113
Q

Accumulates in macrophages

A

Krabbe disease

114
Q

Impaired addition of coenzyme A to long-chain fatty acids (X-linked defect)

A

Adrenoleukodystrophy

115
Q

Accumulation of fatty acids damages adrenal glands and white matter of brain

A

Adrenoleukodystrophy

116
Q

Autoimmune destruction of CNS myelin and oligodendocytes

A

Multiple Sclerosis

117
Q

Most common chronic CNS disease of young adults, more commonly seen in women

A

Multiple Sclerosis

118
Q

Associated with HLA-DR2

A

Multiple Sclerosis

119
Q

More commonly see in regions away from equator

A

Multiple Sclerosis

120
Q

Presents with relapsing neurologic deficits with periods of remission

A

Multiple Sclerosis

121
Q

Presents with blurred vision in one eye and internuclear opthalamoplegia

A

Multiple Sclerosis

122
Q

Presents with vertigo and scanning speech mimicking drunkeness

A

Multiple Sclerosis

123
Q

Presents with hemiparesis or unilateral loss of sensation, lower extremity loss of sensation or weakness, and bowel, bladder & sexual dysfunction

A

Multiple Sclerosis

124
Q

Diagnosis is made by MRI and lumbar puncture

A

Multiple Sclerosis

125
Q

MRI reveals plaques and lumbar puncture shows increased lymphocytes, IG’s with oligoclonal IgG bands, and myelin basic protein

A

Multiple Sclerosis

126
Q

Treatment of acute attacks is high-dose steroids

A

Multiple Sclerosis

127
Q

Treatment long-term is interferon beta

A

Multiple Sclerosis

128
Q

Due to slow progressing, persistent infection of brain by measles virus

A

Subacute Sclerosing Pancencephalitis

129
Q

Infection occurs in infancy with neurologic signs years later

A

Subacute Sclerosing Pancencephalitis

130
Q

Characterized by viral inclusions within neurons of gray matter and oligodendrocytes of white matter

A

Subacute Sclerosing Pancencephalitis

131
Q

JC virus infection of oligodendrocytes

A

Progressive multifocal leukoencephalopathy

132
Q

Immunosuppression leads to reactivation of latent virus

A

Progressive multifocal leukoencephalopathy

133
Q

Presents with progressive neurologic signs (Visual loss, weakness, dementia) leading to death

A

Progressive multifocal leukoencephalopathy

134
Q

Focal demyelination of the pons

A

Central pontine myelinolysis

135
Q

Due to rapid intravenous correction of hyponatremia

A

Central pontine myelinolysis

136
Q

Occurs in severely malnourished patients (alcoholics and liver disease)

A

Central pontine myelinolysis

137
Q

Classically presents as acute bilateral paralysis (locked in syndrome) with only exception being the eyes

A

Central pontine myelinolysis

138
Q

Degeneration of cortex

A

Dementia

139
Q

Degeneration of brainstem and basal ganglia

A

Movement disorders

140
Q

Most common cause of dementia

A

Alzheimer Disease

141
Q

Presents as slow-onset memory loss (short-term first) and progressive disorientation

A

Alzheimer Disease

142
Q

Loss of learned motor skills and language

A

Alzheimer Disease

143
Q

Changes in behavior and personality

A

Alzheimer Disease

144
Q

Most cases are sporadic and seen in elderly

A

Alzheimer Disease

145
Q

Risk of this increases with age

A

Alzheimer Disease

146
Q

ApoE4 is associated with increased risk

A

Alzheimer Disease

147
Q

ApoE2 is associated with decreased risk

A

Alzheimer Disease

148
Q

Seen in presenilin 1 and presenilin 2 mutations

A

Early-Onset Alzheimer Disease

149
Q

Seen in Down syndrome

A

Early-Onset Alzheimer Disease

150
Q

Cerebral atrophy with narrowing of the gyri, widening of sulci, dilation of ventricles

A

Alzheimer Disease

151
Q

Neuritic plaques of A-Beta amyloid with entangled neuriti cprocesses

A

Alzheimer Disease

152
Q

APP on chromosome 21 is the source of protein to be turned into Beta amyloid

A

Alzheimer Disease

153
Q

Presents with neurofibrillary tangles: aggregates of hyperphosphorylated tau protein

A

Alzheimer Disease

154
Q

Diagnosis of exclusion

A

Alzheimer Disease

155
Q

Diagnosis confirmed upon autopsy

A

Alzheimer Disease

156
Q

Multifocal infarction and injury due to HTN, atherosclerosis, or vasculitis

A

Vascular Dementia

157
Q

2nd most common cause of dementia

A

Vascular Dementia

158
Q

Degenerative disease of frontal and temporal cortex

A

Pick Disease

159
Q

Characterized by round aggregates of tau protein

A

Pick Disease

160
Q

Presents with behavioral and language symptoms early

A

Pick Disease

161
Q

Degenerative loss of dopaminergic neurons in substantia nigra of basal ganglia

A

Parkinson Disease

162
Q

Rare cases related to MPTP exposure

A

Parkinson Disease

163
Q

Tremor
Rigidity
Akinesia/Bradykinesia
Postural instability/Shuffling Gait

A

Parkinson Disease

164
Q

Loss of pigmented neurons in substantia nigra

A

Parkinson Disease

165
Q

Eosinophilic inclusions of alpha-synuclein (Lewy bodies)

A

Parkinson Disease

166
Q

Usually the cause of early-onset dementia

A

Parkinson Disease

167
Q

Degeneration of GABAergic neurons in caudate nucleus of basal ganglia

A

Huntington Disease

168
Q

Autosomal dominant disorder of CAG trinucleotide repeat of gene on chromosome 4

A

Huntington Disease

169
Q

Further expansion of repeats during spermatogenesis leads to anticipation

A

Huntington Disease

170
Q

Presents with chorea that progresses to dementia and depression

A

Huntington Disease

171
Q

Suicide is common cause of death

A

Huntington Disease

172
Q

Increased CSF resulting in dilated ventricles and stretching of nerves

A

Normal Pressure Hydrocephalus

173
Q

Presents as triad of urinary incontinence, gait instability and dementia
(Wet, Wacky, Wobbly)

A

Normal Pressure Hydrocephalus

174
Q

Lumbar puncture improves symptoms

A

Normal Pressure Hydrocephalus

175
Q

Treatment is ventriculoperitoneal shunting

A

Normal Pressure Hydrocephalus

176
Q

Degenerative disease due to prion protein

A

Spongiform Encephalopathy

177
Q

Disease arises with conversion to a Beta-pleated conformation

A

Spongiform Encephalopathy

178
Q

Damage to neurons and glial cells characterized by intracellular vacuoles

A

Spongiform Encephalopathy

179
Q

Most common spongiform encephalopathy

A

Creutzfeldt-Jakob Disease

180
Q

Presents as rapidly progressive dementia with ataxia and startle myoclonus

A

Creutzfeldt-Jakob Disease

181
Q

Periodic sharp waves are seen on EEG

A

Creutzfeldt-Jakob Disease

182
Q

Related to exposure to bovine spongiform encephalopathy

A

Variant Cruejtzfeldt-Jakob Disease

183
Q

Inherited form of prion disease characterized by severe insomnia and exaggerated startle response

A

Familial Fatal Insomnia

184
Q

Most common primary malignant CNS tumor in adults

A

Glioblastoma Multiforme

185
Q

Malignant, high grade tumor of astrocytes

A

Glioblastoma Multiforme

186
Q

Arises in cerebral hemisphere and crosses the corpus callosum to form a butterfly lesion

A

Glioblastoma Multiforme

187
Q

Characterized by regions of necrosis surrounded by tumor cells (Pseudopalisading) and endothelial cell proliferation

A

Glioblastoma Multiforme

188
Q

Tumor cells are GFAP positive

A

Glioblastoma Multiforme

189
Q

Benign tumor of arachnoid cells

A

Meningioma

190
Q

Most common benign CNS tumor in adults

A

Meningioma

191
Q

More commonly seen in women due to being estrogen sensitive

A

Meningioma

192
Q

Present as seizures

A

Meningioma

193
Q

Findings: Round mass attached to dura, whorled pattern and possible psammoma bodies

A

Meningioma

194
Q

Benign tumor of Schwann cells

A

Schwannoma

195
Q

Involves cranial or spinal nerves, most frequently CN VIII at cerebellopontine angle

A

Schwannoma

196
Q

Presents as loss of hearing and tinnitus

A

Schwannoma

197
Q

Tumor cells are S-100 positive

A

Schwannoma

198
Q

Bilateral forms of this are seen in neurofibromatosis type 2

A

Schwannoma

199
Q

Malignant tumor of oligodendrocytes

A

Oligodendroglioma

200
Q

Calcified tumor in white matter usually in frontal lobe

A

Oligodendroglioma

201
Q

Presents as seizures

A

Oligodendroglioma

202
Q

Fried egg appearance of cells on biopsy

A

Oligodendroglioma

203
Q

Benign tumor of astrocytes

A

Pilocytic astrocytoma

204
Q

Most common CNS tumor in kids

A

Pilocytic astrocytoma

205
Q

Imaging reveals cystic lesion with a mural nodule

A

Pilocytic astrocytoma

206
Q

Biopsy shows Rosenthal fibers and eosinophilic granular bodies

A

Pilocytic astrocytoma

207
Q

Tumor cells are GFAP positive

A

Pilocytic astrocytoma

208
Q

Malignant tumor derived from granular cells of cerebellum in kids

A

Medulloblastoma

209
Q

Small round blue cells with Homer-Wright rosettes

A

Medulloblastoma

210
Q

Metastasis of this to cauda equina is termed drop metastasis

A

Medulloblastoma

211
Q

Malignant tumor of ependymal cells in kids

A

Ependymoma

212
Q

Most commonly arises in 4th ventricle and may presents with hydrocephalus

A

Ependymoma

213
Q

Primary finding are perivascular pseudorosettes

A

Ependymoma

214
Q

Tumor that arises from epithelial remnants of Rathke’s pouch

A

Craniopharyngioma

215
Q

Presents as a supratentorial mass in a child or young adult

A

Craniopharyngioma

216
Q

May compress the optic chiasm leading to bitemporal hemianopsia

A

Craniopharyngioma

217
Q

Calcifications are commonly seen on imaging (derived from tooth-like tissue)

A

Craniopharyngioma

Pathology (36 decks)

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