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Step 1 Neurology-First Aid 2017 > Neuropathology > Flashcards

Neuropathology Flashcards

1
Q

Brain lesion that causes disinhibition, deficits in concentration, orientation and judgement

A

Frontal lobe

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2
Q

Brain lesion that causes reemergence of primitive reflexes

A

Frontal lobe

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3
Q

Brain lesion that causes eyes to look toward side of lesion (ipsilateral)

A

Frontal eye fields

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4
Q

Brain lesion that causes eyes to look away from lesion

A

Paramedian pontine reticular formation

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5
Q

Brain lesion that causes impaired adduction of ipsilateral eye with nystagmus of contralateral eye with abduction

A

Medial longitudinal fasciculus

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6
Q

Internuclear ophthalmoplegia

A

Impaired adduction of ipsilateral eye with nystagmus of contralateral eye with abduction

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7
Q

Demyelinating disease that causes internuclear ophthalmoplegia

A

Multiple sclerosis

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8
Q

Brain lesion that causes agraphia, acalculia, finger agnosia, left-right disorientation

A

Dominant parietal cortex

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9
Q

Neuropsychological disorder characterized by dyscalculia, dysgraphia, finger agnosia and left-right disorientation

A

Gerstmann syndrome

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10
Q

Brain lesion that causes agnosia of the contralateral side of the world

A

Non-dominant parietal cortex

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11
Q

Syndrome characterized by agnosia of the contralateral side of the world

A

Hemispatial neglect syndrome

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12
Q

Inability to make new memories

A

Anterograde amnesia

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13
Q

Brain lesion that causes anterograde amnesia

A

Hippocampus (bilateral)

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14
Q

Brain lesion that causes tremor at rest, chorea, athetosis

A

Basal ganglia

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15
Q

Neurodegenerative conditions that cause resting tremors, chorea, and athetosis

A

Huntington and Parkinson disease

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16
Q

Brain lesion that causes contralateral hemiballismus

A

Subthalamic nucleus

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17
Q

Brain lesion that causes confusion, ataxia, ophthalmoplegia, nystagmus memory loss, confabulation and personality changes

A

Mammillary bodies (bilateral)

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18
Q

Neurological disorder characterized by confusion, ataxia, ophthalmoplegia, nystagmus memory loss, confabulation and personality changes

A

Wernicke-Korsakoff syndrome

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19
Q

Syndrome characterized by hyperphagia, hypersexuality, hyperorality

A

Kluver-Bucy syndrome

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20
Q

Brain lesion that causes Kluver-Bucy syndrome of disinhibited behavior

A

Amygdala (bilateral)

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21
Q

Syndrome characterized by paralysis of conjugate vertical gaze

A

Parinaud syndrome

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22
Q

Brain lesion that causes paralysis of conjugate vertical gaze

A

Superior colliculus

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23
Q

Viral infection that causes of Kluver-Bucy syndrome

A

HSV-1 encephalitis

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24
Q

Complications that cause Parinaud syndrome

A

Stroke, hydrocephalus, and pinealoma

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25
Q

Brain lesion that causes reduced levels of arousal and wakefulness

A

Reticular activating system (midbrain)

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26
Q

Brain lesion that causes intention tremor, limb ataxia, loss of balance toward side of lesion

A

Cerebellar hemispheres

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27
Q

Brain lesion that causes truncal ataxia and dysarthria

A

Cerebellar vermis

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28
Q

Degeneration of vermis is associated with what

A

Chronic alcohol use

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29
Q

After how many minutes does hypoxia begin to cause irreversible brain damage

A

5 minutes

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30
Q

Areas of brain most vulnerable to hypoxia

A

Hippocampus, neocortex, cerebellum, watershed areas

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31
Q

Which area of the brain is most vulnerable to ischemic hypoxia

A

Hippocampus

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32
Q

Best imaging modality to use to detect ischemia within 3-30 minutes

A

Diffusion-weighted MRI

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33
Q

Best imaging modality to use to detect ischemic changes within 6-24 hours

A

CT

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34
Q

Imaging modality used to exclude hemorrhage

A

Noncontrast CT

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35
Q

What needs to be done before tPA can be given in stroke patient

A

Exclude hemorrhage with noncontrast CT

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36
Q

Ischemic changes seen within 12-24 hours

A

Red neurons

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37
Q

Eosinophilic neuron with pyknotic nuclei

A

Red neuron

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38
Q

Ischemic changes seen after 24-72 hours

A

Necrosis plus neutrophils

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39
Q

Ischemic changes seen after 3-5 days

A

Macrophages (microglia)

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40
Q

Ischemic changes seen after 1-2 weeks

A

Reactive gliosis plus vascular proliferation

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41
Q

Ischemic changes seen after 2 weeks or more

A

Glial scar (cystic lesion with gliosis)

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42
Q

Type of necrosis seen after ischemic attack

A

Liquefactive necrosis

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43
Q

Types of ischemic strokes

A

Thrombotic, Embolic, Hypoxic

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44
Q

Type of stroke common in cardiovascular surgeries affecting watershed areas due to hypoperfusion or hypoxemia

A

Hypoxic stroke

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45
Q

Type of stroke due to a clot forming at site of infarction commonly affecting MCA or basilar and carotid bifurcation

A

Thrombotic stroke

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46
Q

Type of stroke affecting multiple vascular territories caused by a-fib, DVT with patent foramen ovale

A

Embolic stroke

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47
Q

Ischemic stroke treatment

A

tPA if within 3.5 hours and no risk of hemorrhage

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48
Q

Brief reversible episode of focal neurologic dysfunction without acute infarction resolving within 15 minutes

A

Transient ischemic attack

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49
Q

Common locations of hemorrhagic strokes

A

Thalamus, basal nuclei, pons, cerebellum

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50
Q

Clopidogrel MOA

A

ADP receptor inhibitor

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51
Q

Common blood vessel injured in epidural hematoma

A

Middle meningeal artery (foramen spinosum)

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52
Q

Area of skull damaged in epidural hematoma

A

Pterion (thinnest area of lateral skull)

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53
Q

Skull bones that meet at pterion

A

Frontal, parietal, temporal, sphenoid bones

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54
Q

CT findings in epidural hematoma

A

Biconvex (lentiform) hyperdense blood collection not crossing suture lines

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55
Q

Common finding prior to symptoms in epidural hematoma

A

Lucid interval

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56
Q

Common cause of subdural hematoma

A

Rupture of bridging veins

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57
Q

Predisposing factors for subdural hematoma

A

Brain atrophy, trauma, older age, alcoholism

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58
Q

Common cause of subdural hematoma in infants

A

Shaken baby syndrome

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59
Q

CT findings in subdural hematoma

A

Crescent-shaped hemorrhage that crosses suture lines

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60
Q

Type of herniation seen in epidural hematoma

A

Transtentorial herniation causing CN III palsy

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61
Q

Type of herniation seen in subdural hematoma

A

Midline shifting of ventricles

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62
Q

Common cause of subarachnoid hemorrhage

A

Bleeding from trauma or rupture of aneurysms

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63
Q

Types of aneurysms that commonly rupture in subarachnoid hemorrhage

A

Saccular or Berry aneurysms

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64
Q

Symptoms of subarachnoid hemorrhage

A

“Worst headache of my life”, bloody or yellow spinal tap

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65
Q

Complication of subarachnoid hemorrhage

A

Blood breakdown or rebleed cause vasospasms resulting in ischemic infarct

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66
Q

Treatment in subarachnoid hemorrhage to reduce or prevent vasospasms

A

Nimodipine

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67
Q

What genetic diseases are associated with saccular and Berry aneurysms

A

Marfan syndrome and ADPKD

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68
Q

What risk is increased in subarachnoid hemorrhages

A

Risk of developing communicating and/or obstructive hydrocephalus

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69
Q

Brain hemorrhage most commonly caused by systemic HTN

A

Intraparenchymal hemorrhage

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70
Q

Conditions associated with intraparenchymal hemorrhage

A

Amyloid angiopathy, vasculitis, neoplasms

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71
Q

Intraparenchymal hemorrhage may be secondary to what type of injury

A

Ischemic stroke

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72
Q

Common location of intraparenchymal hemorrhage

A

Basal ganglia and internal capsule

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73
Q

Condition that develops in perforating vessels as a result of HTN

A

Charcot-Bouchard microaneurysm of lenticulostriate vessels

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74
Q

Presentation of intraparenchymal hemorrhage

A

Presents with headache, nausea, vomiting, and eventually coma

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75
Q

Effects of MCA stroke to temporal lobe (Wernicke); frontal lobe (Broca)

A

Aphasia if dominant (left hemisphere)

Hemineglect if non-dominant (right hemisphere)

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76
Q

Effects of MCA stroke to frontal lobe

A

Broca’s aphasia

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77
Q

Effects of MCA stroke to motor and sensory cortices

A

Contralateral paralysis and sensory loss (face and upper limb)

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78
Q

Area of brain associated with Wernicke aphasia

A

Right superior quadrant

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79
Q

Area of brain involved in MCA stroke causing visual field deficits

A

Temporal lobe

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80
Q

Effects of Anterior cerebral stroke to motor and sensory cortices

A

Contralateral paralysis and sensory loss (lower limb)

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81
Q

Effects of Lenticulostriate artery stroke to striatum, internal capsule

A

Contralateral paralysis and/or sensory loss (face and body)

Absence of cortical signs (neglect, aphasia, visual loss)

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82
Q

Common cause of lacunar infarcts

A

Hyaline arteriosclerosis due to unmanaged hypertension

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83
Q

Effects of Anterior spinal artery stroke to Lateral corticospinal tract

A

Contralateral paralysis (upper and lower limbs)

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84
Q

Effects of Anterior spinal artery stroke to Medial lemniscus

A

Decreased contralateral proprioception

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85
Q

Effects of Anterior spinal artery stroke to Caudal medulla-hypoglossal nerve

A

Ipsilateral hypoglossal dysfunction (tongue deviates ipsilaterally)

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86
Q

Ipsilateral deviation of tongue, contralateral limb weakness, contralateral sensory loss

A

Medial medullary syndrome

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87
Q

Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting nucleus ambiguus

A

Dysphagia, hoarseness, decreased gag reflex

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88
Q

Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Vestibular nuclei

A

Vomiting, vertigo, nystagmus

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89
Q

Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting lateral spinothalamic tract, spinal trigeminal nucleus

A

Decreased pain and temperature from contralateral body, ipsilateral face

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90
Q

Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Sympathetic fibers

A

Ipsilateral Horner syndrome

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91
Q

Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Inferior cerebellar peduncle

A

Ataxia, dysmetria

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92
Q

Artery specific to Nucleus ambiguus effects

A

PICA

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93
Q

Artery supplying Inferior cerebellar peduncle

A

PICA

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94
Q

Syndrome characterized by Ataxia, dysmetria; Ipsilateral Horner syndrome; Decreased pain and temperature on contralateral body and ipsilateral face; Vomiting, vertigo, nystagmus; Dysphagia, hoarseness, decreased gag reflex

A

Lateral Medullary syndrome

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95
Q

Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Facial nucleus

A

Face paralysis, decreased lacrimation, salivation, and taste from anterior 2/3 of tongue

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96
Q

Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Vestibular nuclei

A

Vomiting, vertigo, nystagmus

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97
Q

Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Spinothalamic tract, spinal trigeminal nucleus

A

Decreased pain and temperature from contralateral body and ipsilateral face

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98
Q

Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Sympathetic fibers

A

Ipsilateral Horner syndrome

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99
Q

Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Middle and Inferior cerebellar peduncles

A

Ataxia, dysmetria

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100
Q

Syndrome characterized by Face paralysis, decreased lacrimation, salivation, and taste from anterior 2/3 of tongue; Vomiting, vertigo, nystagmus; Decreased pain and temperature from contralateral body and ipsilateral face; Ataxia, dysmetria

A

Lateral pontine syndrome

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101
Q

Facial nucleus affects are specific to a lesion of what artery

A

AICA

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102
Q

Effects of Basilar artery stroke to Pons, medulla, lower midbrain

A

Completely paralyzed but RAS spared, therefore preserved consciousness

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103
Q

Condition in which patient is fully aware and can only move their eyes

A

Locked-in syndrome

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104
Q

Effects of Basilar artery stroke to Corticospinal and corticobulbar tracts

A

Quadriplegia with loss of voluntary facial, mouth and tongue movements

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105
Q

Effects of Basilar artery stroke to Ocular cranial nerve nuclei and PPRF

A

Loss of horizontal, but not vertical eye movements

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106
Q

Effects of Posterior cerebellar artery stroke to Occipital lobe

A

Contralateral hemianopia with macular sparing

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107
Q

Initial paresthesias followed in weeks to months by allodynia and dysesthesia due to thalamic lesions in 10% of stroke patients

A

Central post-stroke pain syndrome

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108
Q

Lesion to inferior frontal gyrus of frontal lobe in which patient has non-fluent speech but intact comprehension

A

Broca’s aphasia

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109
Q

Lesion to arcuate fasciculus with intact comprehension and fluent paraphrasic speech

A

Conduction aphasia

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110
Q

Lesion to arcuate fasciculus affecting Broca and Wernicke areas with impaired comprehension and non-fluent speech

A

Global aphasia

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111
Q

Lesion to superior temporal gyrus of temporal lobe with impaired comprehension and non-sensical fluent speech

A

Wernicke’s aphasia

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112
Q

Lesion affecting frontal lobe with sparing of Broca’s area with intact comprehension and non-fluent speech

A

Transcortical motor aphasia

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113
Q

Lesion affecting watershed areas with sparing of Broca’s, Wernicke’s and arcuate fasciculus with impaired comprehension and non-fluent speech

A

Transcortical, mixed aphasia

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114
Q

Lesion affecting temporal lobe with sparing of Wernicke’s area with impaired comprehension and fluent speech

A

Transcortical sensory aphasia

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115
Q

Most common site of Berry aneurysms

A

Junction of anterior communicating artery and ACA

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116
Q

Diseases associated with berry aneurysms

A

ADPKD and Ehlers-Danlos syndrome

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117
Q

Risk factors for berry aneurysms

A

older age, HTN, smoking, race

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118
Q

Race at increased risk of berry aneurysms

A

African-Americans

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119
Q

Symptoms of Anterior communicating artery compression aneurysm

A

Bitemporal hemianopia (optic chiasma compression)

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120
Q

Symptom of posterior communicating artery compression

A

Ipsilateral CN III palsy (mydriasis, ptosis, “down and out” eye

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121
Q

Disorder of recurrent seizures

A

Epilepsy

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122
Q

Continuous or recurring seizures that may result in brain injury lasting 5-30 minutes

A

Status epilepticus

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123
Q

Seizure with impaired consciousness

A

Complex partial seizure

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124
Q

Seizure with motor, sensory, autonomic, psychic auras with preserved consciousness

A

Simple partial seizure

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125
Q

Seizures affecting single area of brain, often preceded by auras

A

Partial seizures

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126
Q

Lobe commonly involved with partial seizures

A

Medial temporal lobe

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127
Q

Seizure characterized by blank stare and no postictal confusion

A

Absence seizure

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128
Q

Seizure characterized by quick, repetitive jerks

A

Myoclonic seizure

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129
Q

Seizure characterized by alternating stiffening and movement

A

Tonic-clonic seizure

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130
Q

Seizure characterized by 3 Hz spike-and-wave discharges

A

Absence seizure

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131
Q

Seizure characterized by stiffening

A

Tonic seizure

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132
Q

Seizure characterized by “drop” to floor and commonly mistaken for fainting

A

Atonic seizure

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133
Q

Common causes of seizures in children

A

Genetic, febrile seizures, trauma, congenital metabolic

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134
Q

Common causes of seizures in adults

A

Tumor, trauma, stroke, infection

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135
Q

Common causes of seizures in elderly

A

Stroke, tumor, trauma, metabolic, infectioin

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136
Q

Repetitive, brief, unilateral headaches with severe periorbital pain with lacrimation and rhinorrhea

A

Cluster headache

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137
Q

Treatment for acute cluster headaches

A

Sumatriptan, 100% O2

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138
Q

Prophylactic treatment for cluster headaches

A

Verapamil (CCB)

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139
Q

Unilateral headache that last 15 min to 3 hours, is repetitive and may present with Horner syndrome

A

Cluster headache

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140
Q

Unilateral headache with pulsating pain, nausea, photophobia and phonophobia; possible aura lasting 4 to 72 hours

A

Migraine headache

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141
Q

Cause of migraine

A

Irritation of CN V, meninges or blood vessels

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142
Q

Substances released from blood vessels causing migraine

A

Substance P, calcitonin gene-related peptide and vasoactive particles

143
Q

Acute treatment for migraine

A

NSAIDs, triptans, dihydroergotamine

144
Q

Prophylactic treatment for migraines

A

Lifestyle changes, beta-blockers, CCBs, amitryptaline, topiramate, valproate

145
Q

Bilateral headache with steady pain and constant lasting 4 to 6 hours

A

Tension headache

146
Q

Treatment for tension headaches

A

Analgesics, NSAIDs, acetaminophen; (amitriptyline for chronic pain)

147
Q

Repetitive, unilateral, shooting pain in the distribution of CN V lasting less than 1 minute

A

Trigeminal neuralgia

148
Q

Treatment for trigeminal neuralgia

A

Carbamazepine

149
Q

Restlessness and intense urge to move; cannot sit still

A

Akathisia

150
Q

Common causes of akathisia

A

Neuroleptics use or Parkinson disease

151
Q

Extension of wrists causes flapping motion

A

Asterixis

152
Q

Basal ganglia lesion causing slow, snake-like, writhing movements, especially in fingers

A

Athetosis

153
Q

Basal ganglia lesion causing sudden jerky, purposeless movements

A

Chorea

154
Q

Type of chorea seen in acute rheumatic fever and Huntington disease

A

Sydenham chorea

155
Q

Sustained, involuntary muscle contractions like torticollis, writer’s cramp or blepharospasm

A

Dystonia

156
Q

High-frequency tremor with sustained posture worsened with movement or when anxious

A

Essential tremor

157
Q

Patients with essential tremors often self-medicate with which substance

A

Alcohol

158
Q

Treatment for essential tremors

A

Primidone or non-selective beta-blockers like propranolol

159
Q

Contralateral subthalamic lesion causing sudden, wild flailing of 1 arm; maybe ipsilateral leg

A

Hemiballismus

160
Q

Cerebellar dysfunction causing slow, zigzag motion when pointing/extending toward a target

A

Intention tremor

161
Q

Sudden, brief, uncontrolled muscle contraction

A

Myoclonus

162
Q

Substantia nigra lesion causing uncontrolled movement of distal appendages alleviated by intentional movement

A

Resting tremor

163
Q

Disease causing intention tremor characterized by “pill-rolling tremor” at rest

A

Parkinson disease

164
Q

Signs and symptoms of Parkinson disease

A

Tremor at rest, Rigidity, Akinesia, Postural instability, Shuffling gait (TRAP)

165
Q

Description of Lewy bodies

A

Made of alpha-synuclein

166
Q

Intracellular eosinophilic inclusions seen in Parkinson disease made of alpha-synuclein

A

Lewy bodies

167
Q

Cause of movement disorders in Parkinson disease

A

Loss of dopaminergic neurons of substantia nigra pars compacta

168
Q

Illegal street drug contaminant that can cause Parkinson disease

A

MPTP

169
Q

Autosomal dominant trinucleotide repeat in Huntington disease

A

CAG

170
Q

Chromosome affected in Huntington disease

A

Chromosome 4

171
Q

Brain structure affected in Huntington disease

A

Atrophy of caudate and putamen with ex vacuo ventriculomegaly

172
Q

Cause of neuronal death in Huntington disease

A

NMDA-R binding and glutamate excitotoxicity

173
Q

Neurotransmitter changes in Huntington disease

A

Increased dopamine

Decreased GABA, ACh

174
Q

Signs and symptoms of Huntington disease

A

Chorea, Dementia, Athetosis, Depression, Aggression (C DADA)

175
Q

When does anticipation occur

A

Spermatogenesis

176
Q

Most common cause of dementia in elderly

A

Alzheimer disease

177
Q

Who is at risk of developing early onset dementia

A

Down syndrome patients

178
Q

Cause of Alzheimer disease in Down syndrome

A

Amyloid precursor protein is located on chromosome 21

179
Q

What are neurofibrillary tangles

A

Hyperphosphorylated tau protein

180
Q

What are senile plaques

A

Beta-amyloid core

181
Q

Location of neurofibrillary tangles

A

Intracellular

182
Q

How is A-beta (amyloid-beta) made

A

Cleavage of amyloid precursor protein (APP)

183
Q

Which Apo protein is associated with decreased risk of sporadic form of Alzheimer’s

A

ApoE2

184
Q

Location of senile plaques

A

Extracellular space

185
Q

Gross findings in Alzheimer disease

A

Widespread cortical atrophy, especially hippocampus with narrowing of gyri and widening of sulci

186
Q

Microscopic findings in Alzheimer disease

A

Intracellular neurofibrillary tangles and extracellular senile plaques

187
Q

How many copies of APP gene are found in Down syndrome

A

3 copies on chromosome 21

188
Q

Familial form of Alzheimer’s is associated with which proteins

A

Presenelin-1 and presenelin-2

189
Q

Which Apo protein is associated with sporadic form of Alzheimer’s

A

ApoE4

190
Q

Gross findings in frontotemporal dementia

A

Frontotemporal degeneration

191
Q

Microscopic findings in frontotemporal dementia

A

Inclusions of hyperphosphorylated tau

192
Q

Appearance of tau protein in frontotemporal dementia

A

Round Pick bodies

193
Q

Signs and symptoms of frontotemporal dementia

A

Changes in personality and behavior or aphasia; possible movement disorders

194
Q

Microscopic findings in Lewy body dementia

A

Intracellular Lewy bodies primarily in cortex

195
Q

Signs and symptoms of Lewy body dementia

A

Dementia and visual hallucinations, parkinsonian features

196
Q

Cause of vascular dementia

A

Multiple arterial infarcts and or chronic ischemia

197
Q

Second most common cause of dementia in elderly

A

Vascular dementia

198
Q

Signs and symptoms of vascular dementia

A

Step-wise decline in cognitive ability with late-onset memory impairment

199
Q

Cause of vascular dementia

A

HTN, atherosclerosis or vasculitis

200
Q

Image findings in vascular dementia

A

Multiple cortical and or subcortical infarcts

201
Q

Rapidly progressive dementia with myoclonus

A

Creutzfeldt-Jacob disease

202
Q

Common findings in Creutzfeldt-Jacob disease

A

Periodic sharp waves on ECG and increases 14-3-3 protein in CSF

203
Q

Common gross and microscopic findings in Creutzfeldt-Jacob disease

A

Spongiform cortex and prions

204
Q

What are prions made of

A

Beta-pleated sheet resistant to proteases

205
Q

Increased ICP with no apparent cause

A

Idiopathic ICP

206
Q

Another name of idiopathic ICP

A

Pseudotumor cerebri

207
Q

Risk factors associated with pseudotumor cerebri

A

Female gender, obesity, vitamin A excess, tetracycline, danazol

208
Q

Signs and symptoms of pseudotumor cerebri

A

Headache, diplopia, papilledema with no mental status change

209
Q

Lumbar puncture findings in pseudotumor cerebri

A

Increased opening pressure with headache relief

210
Q

Treatment for pseudotumor cerebri

A

Weight loss, acetazolamide, topiramate

211
Q

Treatment for refractory pseudotumor cerebri

A

Repeat lumbar puncture, CSF shunt placement, or optic nerve sheath fenestration surgery

212
Q

Common findings in communicating hydrocephalus

A

Increased ICP, papilledema and herniation

213
Q

Common cause of communicating hydrocephalus

A

Arachnoid scarring post-meningitis

214
Q

Common symptoms in normal pressure hydrocephalus

A

Urinary incontinence, ataxia, cognitive dysfunction and magnetic gait (feet appear stuck on floor)

215
Q

Who is at risk for normal pressure hydrocephalus

A

Elderly

216
Q

Image findings in normal pressure hydrocephalus

A

Expansion of ventricles

217
Q

Common findings in normal pressure hydrocephalus

A

Episodic elevated CSF pressure and no increased subarachnoid space volume

218
Q

Appearance of increased CSF on imaging but actually due to decreased brain tissue and neuronal atrophy with normal ICP

A

Ex vacuo ventriculomegaly

219
Q

Acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness secondary to osmotic changes

A

Osmotic demyelination syndrome

220
Q

Other name for osmotic demyelination syndrome

A

Central pontine myelinolysis

221
Q

Complication of osmotic demyelination syndrome

A

Locked-in syndrome

222
Q

Common cause of osmotic demyelination syndrome

A

Iatrogenic from overly rapid correction of hyponatremia

223
Q

Result of correcting hypernatremia too quickly

A

Cerebral edema/herniation

224
Q

Autoimmune inflammation and demyelination of oligodendrocytes in CNS

A

Multiple sclerosis

225
Q

Most common clinical course of multiple sclerosis

A

Relapsing and remitting

226
Q

Population commonly affected by multiple sclerosis

A

Caucasian women in 20s and 30s living farther from equator

227
Q

Symptoms of MS

A

Scanning speech, Intention tremor, Incontinence, Internuclear ophthalmoplegia, Nystagmus, Hemiparesis, Hemisensory symptoms

228
Q

Charcot triad

A

Scanning speech, Intention tremor, Nystagmus (SIN)

229
Q

Findings in MS

A

Increased IgG levels and myelin basic protein in CSF

230
Q

Diagnosis of MS

A

Oligoclonal bands on MRI, paraventricular plaques with preservation of axon and multiple white matter lesions

231
Q

Paraventricular plaques

A

Areas of oligodendrocyte loss and reactive gliosis

232
Q

Treatment for MS to slow progression and modify disease

A

Beta-interferon slows progression, glatiramer and natalizumab

233
Q

Acute flair treatment for MS

A

IV steroids

234
Q

Symptomatic treatment for neurogenic bladder in MS

A

Catheterization, muscarinic antagonists

235
Q

Symptomatic treatment for spasticity in MS

A

Baclofen, GABA receptor antagonist

236
Q

Symptomatic treatment for pain in MS

A

TCAs, anticonvulsants

237
Q

Autoimmune condition that destroys Schwan cells causing inflammation and demyelination of peripheral nerves and motor fibers

A

Guillain-Barre syndrome

238
Q

Type of paralysis seen in Guillain-Barre syndrome

A

Symmetric, ascending paralysis beginning in lower extremities

239
Q

Prognosis of Guillain-Barre syndrome

A

Almost all patients survive with recovery in weeks to months

240
Q

CSF findings in Guillain-Barre syndrome

A

Increased CSF protein that may cause papilledema

241
Q

Bacteria associated with Guillain-Barre syndrome

A

Campylobacter jejuni

242
Q

Treatment for Guillain-Barre syndrome

A

Respiratory support, plasmapheresis, IV immunoglobulins

243
Q

Multifocal inflammation and demyelination after infection or vaccination with rapidly progressive multifocal neurological symptoms and altered mental status

A

Acute disseminated encephalomyelitis

244
Q

Disease also known as hereditary motor and sensory neuropathy

A

Charcot-Marie-Tooth disease

245
Q

Typical inheritance pattern of Charcot-Marie-Tooth disease

A

Autosomal dominant

246
Q

Mechanism of Charcot-Marie-Tooth disease

A

Defective proteins involved in structure and function of peripheral nerves or myelin sheath

247
Q

Typical findings associated with Charcot-Marie-Tooth disease

A

Foot deformities (pes cavus, hammer toe), lower extremity weakness and sensory deficits

248
Q

Inheritance pattern for Krabbe disease

A

Autosomal recessive

249
Q

Defect in Krabbe disease

A

Galactocerebrosidase deficiency

250
Q

Mechanism of Krabbe disease

A

Galactocerebroside and psychosine buildup destroy myelin

251
Q

Findings in Krabbe disease

A

Peripheral neuropathy, developmental delay, optic atrophy, globoid cells

252
Q

Inheritance pattern for metachromatic leukodystrophy

A

Autosomal recessive

253
Q

Defect in metachromatic leukodystrophy

A

Arylsulfatase A deficiency

254
Q

Mechanism of metachromatic leukodystrophy

A

Buildup of sulfatides impairs production and destroys myelin

255
Q

Findings in metachromatic leukodystrophy

A

Central and peripheral demyelination with ataxia and dementia

256
Q

Most common leukodystrophy

A

Metachromatic leukodystrophy

257
Q

Demyelinating disease of CNS seen in reactivation of latent JC virus infection that is rapidly progressive and usually fatal

A

Progressive multifocal leukoencephalopathy

258
Q

Drugs that increase risk of progressive multifocal leukoencephalopathy

A

Natalizumab and rituximab

259
Q

Population affected by progressive multifocal leukoencephalopathy

A

AIDS patients

260
Q

Inheritance pattern of adrenoleukodystrophy

A

X-linked

261
Q

Mechanism of adrenoleukodystrophy

A

Disruption of very-long-chain fatty acid metabolism increases fatty acids damaging adrenal glands, testes and nervous system

262
Q

Complications of adrenoleukodystrophy

A

Long-term coma and death in adrenal crisis

263
Q

What is the defect in adrenoleukodystrophy

A

Cannot add coenzyme A to long-chain fatty acids

264
Q

Sturge-Weber syndrome inheritance

A

Congenital, non-inherited

265
Q

Mechanism of Sturge-Weber syndrome

A

Mutation in GNAQ gene causes developmental anomaly in neural crest cell derivatives

266
Q

Sturge-Weber syndrome acronym for symptoms

A

STURGE: Sporadic, Tram track calcifications, Unilateral, Retardation, GNAQ, Glaucoma, Epilepsy

267
Q

Genetic defect of tuberous sclerosis

A

TSC1/TSC2 mutation on chromosome 16

268
Q

Inheritance pattern of tuberous sclerosis

A

Autosomal dominant, variable expression

269
Q

Common findings in tuberous sclerosis

A 
HAMARTOMAS: 
Hamartomas in CNS and skin
Angiofibromas 
Mitral regurgitation 
Ash-leaf spots
Rhabdomyoma
Tuberous sclerosis
autosomal dOminant 
Mental retardation
Angiomyolipoma
Seizures, Shagreen patches
270
Q

Inheritance pattern for NF1

A

Autosomal dominant, 100% penetrance

271
Q

Mutation in NF1

A

NF1 tumor suppressor gene on chromosome 17

272
Q

Function of NF1

A

Codes for neurofibromin

273
Q

Function of neurofibromin

A

Negative regulator of RAS

274
Q

Signs and symptoms in NF1

A

Café-au-lait spots, cutaneous neurofibromas, optic gliomas, pheochromocytoma, Lisch nodules

275
Q

Pigmented iris hamartomas

A

Lisch nodules

276
Q

Inheritance patter for NF2

A

Autosomal dominant

277
Q

Mutation in NF2

A

NF2 tumor suppressor on chromosome 22

278
Q

Signs and symptoms in NF2

A

Bilateral acoustic schwannomas, juvenile cataracts, meningiomas, and ependymomas

279
Q

NF2 affects what

A

2 ears, 2 eyes, and 2 parts of brain

280
Q

Inheritance pattern for von Hippel-Lindau disease

A

Autosomal dominant

281
Q

Mutation in von Hippel-Lindau disease

A

Deletion of VHL gene on chromosome 3

282
Q

Findings in von Hippel-Lindau disease

A 
HARP:
Hemangioblastomas - retina, brain stem, spine cerebellum
Angiomatosis - skin, mucosa, organs
Renal cell carcinoma - bilateral
Pheochromocytoma
283
Q

Common, highly malignant brain tumor that can cross corpus callosum

A

Glioblastoma

284
Q

Cell of origin in glioblastoma

A

Astrocyte

285
Q

Microscopic findings in glioblastoma

A

GFAP positive, “pseudopalisading” pleomorphic tumor cells around central area of necrosis

286
Q

Mass with “fried egg” cells in frontal lobe white matter and “chicken wire” capillary pattern presenting with seizures

A

Oligodendroglioma

287
Q

Benign brain tumor with dural attachment, spindle cells in whirled pattern and psammoma bodies presenting with seizures

A

Meningioma

288
Q

Cerebellar tumor associated with von Hippel-Lindau syndrome with thin-walled capillaries presenting with polycythemia

A

Hemangioblastoma

289
Q

Cell of origin in meningioma

A

Arachnoid cell

290
Q

S-100 positive tumor at the cerebellopontine angle localized to CN VIII

A

Schwannoma

291
Q

Cell of origin in schwannoma

A

Schwan cells

292
Q

Cell of origin in hemangioblastoma

A

Blood vessel

293
Q

Most common adult tumor

A

Meningioma

294
Q

Well circumscribed, GFA positive tumor in posterior fossa with eosinophilic, corkscrew fibers

A

Astrocytoma

295
Q

Most common malignant brain tumor in childhood

A

Medulloblastoma

296
Q

Most common benign CNS tumor in childhood

A

Astrocytoma

297
Q

Cell of origin in astrocytoma

A

Glial cell

298
Q

Cell of origin in medulloblastoma

A

Neuroectodermal

299
Q

Brain tumor in cerebellum causing non-communicating hydrocephalus with Homer-Wright rosettes and small blue cells on histology

A

Medulloblastoma

300
Q

Malignant brain tumor in 4th ventricle causing hydrocephalus with perivascular rosettes and basal ciliary bodies near the nucleus on histology

A

Ependymoma

301
Q

Most common childhood supratentorial tumor

A

Craniopharyngioma

302
Q

Cell of origin in ependymoma

A

Ependymal cells

303
Q

Cell of origin in craniopharyngioma

A

Remnants of Rathke pouch

304
Q

Brain tumor causing bitemporal hemianopia with calcification and cholesterol crystals in motor oil-like fluid within tumor on histology

A

Craniopharyngioma

305
Q

Brain tumor causing vertical gaze palsy, obstructive hydrocephalus, increased beta-HCG production in boys

A

Pinealoma

306
Q

Artery compressed with cingulate herniation under falx cerebri

A

Anterior cerebral artery

307
Q

Complications of transtentorial herniation

A

Caudal displacement of brain stem rupturing paramedian basilar artery branches leading to Duret hemorrhages

308
Q

Complications of Uncal herniation to ipsilateral CNIII

A

Blown pupil - down and outward gaze

309
Q

Complications of Uncal herniation to ipsilateral PCA

A

Contralateral homonymous hemianopia with macular sparing

310
Q

Complications of Uncal herniation to contralateral crus cerebri

A

Ipsilateral paresis

311
Q

Complications of cerebellar herniation into the foramen magnum

A

Coma and death when brain stem compressed

312
Q

UMN sign lesions

A

Weakness, Up reflexes, tone, & Babinski; spastic paresis, clasp knife spasticity

313
Q

LMN sign lesions

A

Weakness, atrophy, fasciculations, Down reflexes, tone; flaccid paralysis

314
Q

Congenital degeneration of anterior horns of spinal cord causing “floppy baby”

A

Werdnig-Hoffmann disease

315
Q

Motor nerves affected in Werdnig-Hoffmann disease

A

LMN’s

316
Q

Symptoms in Werdnig-Hoffmann disease

A

Symmetric weakness, hypotonia and tongue fasciculations

317
Q

Inheritance pattern of Werdnig-Hoffmann disease

A

Autosomal recessive

318
Q

Degeneration of anterior horns of spinal cord causing asymmetric weakness

A

Poliomyelitis

319
Q

Combined UMN and LMN deficits from loss of cortical and spinal cord neurons presenting with asymmetric limb weakness, fasciculations and eventual atrophy

A

Amyotrophic lateral sclerosis (ALS)

320
Q

Other name for amyotrophic lateral sclerosis

A

Lou Gehrig disease

321
Q

Enzyme defective in familial ALS

A

Superoxide dismutase I

322
Q

Treatment for ALS

A

Riluzole

323
Q

Presentation of complete occlusion of anterior spinal artery

A

Below lesion: UMN deficits and loss of pain and temp

Level of lesion: LMN deficits

324
Q

Sensory and Motor tracts affected in complete occlusion of anterior spinal artery

A

Cortical spinal tract below lesion - UMN deficits
Spinothalamic tract below lesion - pain and temp
Anterior horn at level of lesion - LMN deficits

325
Q

Artery that supplies anterior spinal artery at T8 spinal level

A

Artery of Adamkiewicz

326
Q

Lesion that caused by tertiary syphilis resulting in demyelination of dorsal columns and roots presenting with absence of DTRs and positive Romberg sign

A

Tabes dorsalis

327
Q

Patient with Argyll Robertson pupils and poor coordination most likely has what defect of spinal cord

A

Demyelination of dorsal columns and roots

328
Q

Lesion causing bilateral loss of pain and temp in cape-like distribution and associated with Chiari I malformation

A

Syringomyelia

329
Q

Area of spinal cord affected by syringomyelia

A

Anterior white commissure of spinothalamic tract

330
Q

Sensory and motor tracts affected in patient with ataxic gait, paresthesia and impaired position/vibration sense with vitamin B12 deficiency

A

Spinocerebellar tracts, lateral Corticospinal tracts and Dorsal columns

331
Q

Person with compression of spinal roots from L2 and below is at risk for what complications

A

Loss of bladder and anal sphincter control, radicular pain, saddle anesthesia

332
Q

Motor nerves affected in cauda equina syndrome

A

LMNs

333
Q

Cauda equina syndrome

A

Radicular pain, absent knee and ankle reflex, loss of bladder and anal sphincter control

334
Q

Mechanism of poliomyelitis

A

Poliovirus replicates in oropharynx and small intestine then spreads to bloodstream and CNS

335
Q

Area of CNS affected by poliomyelitis

A

Anterior horn of spinal cord causing LMN death

336
Q

Diagnosis of patient presenting with fever, malaise, headache, nausea, LMN signs with increased WBCs and slight protein increase in CSF

A

Poliomyelitis

337
Q

Symptoms present with damage to oculosympathetic pathway

A

Miosis, ptosis, anhydrosis

338
Q

Horner syndrome

A

Miosis, ptosis, anhydrosis

339
Q

Presentation of hemisection of spinal cord

A

Ipsilateral loss of all sensation at lesion level
Ipsilateral LMN signs at lesion level
Ipsilateral UMN signs below lesion
Ipsilateral loss of fine touch and proprioception below lesion
Contralateral pain and temp loss below lesion
Horner syndrome if lesion above T1

340
Q

Brown-Sequard syndrome

A

Lesion affecting hemisection of spinal cord

341
Q

Inheritance pattern of Friedreich ataxia

A

Autosomal recessive

342
Q

Defect in Friedreich ataxia

A

Trinucleotide GAA repeat on chromosome 9

343
Q

Mechanism of Friedreich ataxia

A

Defective frataxin gene impairs mitochondria leading to degeneration of spinal cord tracts

344
Q

Cause of death in Friedreich ataxia

A

Hypertrophic cardiomyopathy

345
Q

Presentation of Friedreich ataxia in childhood

A

Kyphoscoliosis with staggering gait, falling, diabetes, pes cavus, hammer toes, nystagmus and dysarthria

346
Q

Presentation of CN V motor lesion

A

Jaw deviates toward side of lesion from unopposed pterygoid muscle on opposite side

347
Q

Presentation of CN X lesion

A

Uvula deviates away from side of lesion

348
Q

Presentation of CN XI lesion

A

Contralateral weakness turning head and ipsilateral shoulder droop

349
Q

Presentation of CN XII lesion

A

Tongue deviates to side of lesion

350
Q

Motor neuron affect with CN XII lesion

A

LMN

351
Q

Motor neuron lesion causing ipsilateral paralysis of upper and lower muscles of face with taste loss to anterior 2/3 of tongue and hyperacusis

A

LMN lesion

352
Q

Cause of LMN lesion causing ipsilateral paralysis of upper and lower muscles of face

A

Destruction of facial nucleus or CN VII anywhere along its path

353
Q

Cause of UMN lesion causing lower facial paralysis

A

Contralateral destruction of motor cortex or connection between motor cortex and facial nucleus

354
Q

Motor neuron lesion causing contralateral paralysis of lower face muscles with sparing of forehead

A

UMN lesion

Step 1 Neurology-First Aid 2017 (5 decks)

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