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Flashcards in Pathoma: CNS Deck (217):
1

Incomplete closure of neural tube

Neural Tube Defect

2

Low folate levels prior to conception

Neural Tube Defect

3

Detected by high AFP levels

Neural Tube Defect

4

Absence of skull and brain

Anencephaly

5

Frog-like appearance

Anencephaly

6

Result in maternal polyhydramnios

Anencephaly

7

Failure of posterior vertebral arch to close

Spina bifida

8

Dimple or patch of hair overlying vertebral defect

Spina bifida occulta

9

Cystic protrusion of underlying tissue through vertebral defect

Spina bifida

10

Protrusion of meninges

Meningocele

11

Protrusion of meninges and spinal cord

Meningomyelocele

12

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

Cerebral Aqueduct Stenosis

13

Most common cause of hydrocephalus in newborns

Cerebral Aqueduct Stenosis

14

Presents with enlarging head circumference due to dilation of ventricles

Cerebral Aqueduct Stenosis

15

Congenital failure of cerebellar vermis to develop

Dandy-Walker Malformation

16

Presents as massively dilated 4th ventricle and absent cerebellum.

Dandy-Walker Malformation

17

Congenital downward displacement of cerebellar vermis and tonsils through foramen magnum

Arnold-Chiari Malformation Type II

18

Obstruction results in hydrocephalus

Arnold-Chiari Malformation Type II

19

May occur in association with meningomyelocele and syringomyelia

Arnold-Chiari Malformation Type II

20

Cystic degeneration of spinal cord

Syringomyelia

21

Cause: Trauma or Arnold-Chiari malformation

Syringomyelia

22

Occurs at C8-T1

Syringomyelia

23

Presents as sensory loss of pain and temperature with sparing of fine touch and position sense in uppper extremities (Cape-like distribution)

Syringomyelia

24

Expansion leads to damage of LMN's of anterior horn (muscle atrophy) and damage of lateral horn of hypothalamospinal tract (Horner syndrome)

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

55

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

59

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

62

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

64

Focal neurologic deficits lasting less than 24 hours

Transient Ischemic Attack

65

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

73

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

Lacunar Stroke

74

Involvement of internal capsule leads to pure motor stroke

Lacunar Stroke

75

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

85

Bleeding into brain parenchyma

Intracerebral Hemorrhage

86

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

95

Collection of blood underneath dura

Subdural Hematoma

96

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

103

Compression of anterior cerebral artery leads to infarction

Subfalcine herniation

104

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