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FA note cards > Neuro and Special Senses > Flashcards

Neuro and Special Senses Flashcards

1
Q

Neuroepithelia in neural tube become what?

A

CNS neurons, ependymal cells, oligodendrocytes, astrocytes

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2
Q

What do neural crest cells become in the brain?

A

PNS neurons and Schwann cells

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3
Q

What does the mesoderm become in the brain?

A

Microglia

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4
Q

Risks for neural tube defects?

A

Maternal diabetes as well as low folic acid intake

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5
Q

Labs of NTD?

A

Elevated AFP and AChE as confirmatory

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6
Q

What is holoprosencephaly and risk factors?

A

Failure of left and right hemispheres to separate

Seen in trisomy 13 and fetal alcohol syndrome

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7
Q

Clinical presentation of holoprosencephaly?

A

Moderate form has cleft lip/palate, most severe form results in cyclopia
MRI: monoventricle and fusion of basal ganglia

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8
Q

Chiari I malformation

A

Ectopia of cerebellar tonsils
Asymptomatic; HA in adulthood
Assoc w/ syringomyelia

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9
Q

Chiari II malformation

A

Herniation of low-lying cerebellar vermis and tonsils through foramen magnum with aqueductal stenosis -> hydrocephalus
Assoc w/ LS meningomyelocele

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10
Q

Dandy-Walker syndrome

A

Agenesis of cerebellar vermis with cystic enlargement of 4th ventricle
Assoc w/ noncommunicating hydrocephalus, spina bifida

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11
Q

Syringomyelia

A 
Cystic cavity (syrinx) in central canal of spinal cord 
AWC damaged first: cape-like loss of pain and temp (fine touch intact)
Expansion into AMH (weakness); expansion into LH: Horners
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12
Q

Tongue sensation

A

-1st and 2nd branchial arches form anterior 2/3 (sensation via CN V3, taste via CN VII)
-3rd and 4th branchial arches form posterior 1/3 (sensation and taste mainly via CN IX,
extreme posterior via CN X)

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13
Q

Tongue motor innervation

A

-CN XII to hyoglossus
(retracts and depresses tongue), genioglossus (protrudes tongue), and styloglossus (draws sides of tongue upward to create a trough for swallowing)
-CN X to palatoglossus (elevates posterior tongue during swallowing)

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14
Q

Astrocytes

A

Derived from neuroectoderm. Astrocyte marker: GFAP

Physical support, repair, EC K+ buffer, removal of excess NT, part of BBB, glycogen fuel reserve buffer

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15
Q

Microglia

A

mesodermal, mononuclear origin

Phagocytic scavenger cells of CNS

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16
Q

multinucleated giant cells in CNS of HIV patients

A

fused microglia

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17
Q

Schwann cells

A

Derived from neural crest

Each Schwann cell myelinates only 1 PNS axon

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18
Q

Oligodendrocytes

A

Derived from neuroectoderm
Can myelinate many axons
“Fried egg” on histology

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19
Q

What is the BBB formed by?

A

Tight junctions between nonfenestrated capillary endothelial cells
Basement membrane
Astrocyte foot processes

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20
Q

Function of hypothalamus

A

TAN HATS

Thirst, Adeno and Neuro hypophysis (pituitary), Hunger, ANS, Temperature, Sex

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21
Q

Lateral area of hypothalamus

A

Hunger
(No lateral area: shrink laterally)
Stimulated by ghrelin,
inhibited by leptin

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22
Q

Ventromedial area of hypothalamus

A

Satiety (destruction: hyperphagia)

Stimulated by leptin

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23
Q

Anterior hypothalamus

A

Cooling, parasympathetic

A/C: anterior/cooling

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24
Q

Posterior hypothalamus

A

Heating, sympathetic

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25
Q

Suprachiasmatic nucleus of hypothalamus

A

Circadian rhythm

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26
Q

Supraoptic and paraventricular nuclei of hypothalamus

A

Synthesize ADH and oxytocin

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27
Q

At what stage of the sleep cycle does bruxism occur?

A

N2 of non-REM sleep

Delta on EEG

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28
Q

Thalamus

A

Major relay for all ascending sensory information except olfaction

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29
Q

Ventral Postero- Lateral nucleus of thalamus

A

Input from spinothalamic and dorsal columns/ medial lemniscus
Vibration, Pain, Pressure, Proprioception, Light touch, Temp

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30
Q

Ventral postero- Medial nucleus of thalamus

A

Input from trigeminal and gustatory pathway

Face sensation, taste

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31
Q

Lateral geniculate nucleus of thalamus

A

Input from CN II
Vision; goes to calcirine sulcus
(lateral = light)

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32
Q

Medial geniculate nucleus

A

Input from superior olive and inferior colliculus of tectum
Hearing; goes to auditory cortex of temporal lobe
(medial = music)

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33
Q

Ventral lateral nucleus

A

Input from basal ganglia and cerebellum

Motor; goes to motor cortex

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34
Q

What is the limbic system

A

Collection of neural structures involved in emotion, long-term memory, olfaction, behavior modulation, ANS function (the five Fs)

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35
Q

Mesocortical dopaminergic pathway

A

Decreased activity -> negative symptoms

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36
Q

Mesolimbic dopaminergic pathway

A

Increased activity: positive symptoms

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37
Q

Nigrostriatal dopaminergic pathway

A

Decreased activity: extrapyramidal sx

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38
Q

Tuberoinfundibular dopaminergic pathway

A

Decreased activity: increased prolactin and decreased libido

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39
Q

Lateral lesions of cerebellum

A

Affect voluntary movement of extremities (Limbs tend to fall toward ipsilateral side

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40
Q

Medial lesions of cerebellum

A

Involvement of Midline structures

Bilateral motor deficits

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41
Q

Purpose of basal ganglia

A

Voluntary movements and making postural adjustment

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42
Q

Location of ACh synthesis

A

Basal nucleus of Meynert

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43
Q

Location of dopamine synthesis

A

Ventral tegmentum, SNc (pars compacta)

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44
Q

Location of GABA synthesis

A

Nucleus accumbens

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45
Q

Location of Norepinephrine synthesis

A

Locus ceruleus

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46
Q

Location of Serotonin synthesis

A

Raphe nucleus

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47
Q

Ascending spinal tracts

A

Dorsal and Spinothalamic

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48
Q

Function of dorsal column

A

Pressure, vibration, fine touch, proprioception

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49
Q

Where does the dorsal column cross?

A

In the medulla

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50
Q

Where is the first synapse of the dorsal column nerves?

A

Nucleus gracilis or cuneatus (in the ipsilateral medulla)

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51
Q

Function of spinothalamic tract

A

Lateral: pain, temperature
Anterior: crude touch, pressure

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52
Q

Where does the spinothalamic tract cross?

A

anterior white commissure

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53
Q

Where is the first synapse of the spinothalamic tract nerves?

A

Ipsilateral gray matter of the spinal cord

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54
Q

Where is the second synapse of the dorsal and spinothalamic tracts?

A

VPL of the thalamus

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55
Q

Function of lateral corticospinal tract

A

Voluntary movement of contralateral limbs

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56
Q

Where is the first synapse of the lateral corticospinal tract?

A

Cell body of anterior horn (spinal cord)

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57
Q

Where does the lateral corticospinal tract cross?

A

most fibers decussate at caudal medulla (pyramidal decussation)

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58
Q

What spinal level does the cremasteric reflex test?

A

L1, L2

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59
Q

What spinal level does the anal wink reflex test?

A

S3, S4

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60
Q

Clinical significance of lesion in the frontal lobe

A

Disinhibition and deficits in concentration, orientation, judgment; reemergence of primitive reflexes

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61
Q

Clinical significance of lesion in the frontal eye fields

A

Eyes look toward lesion

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62
Q

Clinical significance of lesion in the paramedian pontine reticular formation

A

Eyes look away from lesion

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63
Q

Clinical significance of lesion in the medial longitudinal fasciculus

A

Internuclear ophthalmoplegia (can occur in MS)

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64
Q

Clinical significance of lesion in the dominant parietal cortex

A

Agraphia, acalculia, finger agnosia, left-right disorientation
(Gerstmann syndrome)

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65
Q

Clinical significance of lesion in the nondominant parietal cortex

A

Agnosia of the contralateral side of the world

Hemispatial neglect syndrome

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66
Q

Clinical significance of lesion in the hippocampus (bilateral)

A

Anterograde amnesia: inability to make new memories

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67
Q

Clinical significance of lesion in the basal ganglia

A

May result in tremor at rest, chorea, athetosis

Parkinson and Huntington

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68
Q

Clinical significance of lesion in the subthalamic nucleus

A

Contralateral hemiballismus (type of chorea)

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69
Q

Clinical significance of lesion in the mammillary bodies (bilateral)

A

Wernicke-Korsako syndrome: Confusion, Ataxia, Nystagmus, Ophthalmoplegia, memory loss (anterograde and retrograde amnesia), confabulation, personality changes

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70
Q

Clinical significance of lesion in the amygdala (bilateral)

A

Klüver-Bucy syndrome: disinhibited behavior

HSV-1 encephalitis

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71
Q

Clinical significance of lesion in the superior colliculus

A

Parinaud syndrome: paralysis of conjugate vertical gaze (rostral interstitial nucleus also involved)
(Stroke, hydrocephalus, pinealoma)

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72
Q

Clinical significance of lesion in the reticular activating system (midbrain)

A

Reduced levels of arousal and wakefulness

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73
Q

Clinical significance of lesion in the cerebellar hemisphere

A

Intention tremor, limb ataxia, loss of balance; damage to cerebellum: ipsilateral deficits; fall toward side of lesion

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74
Q

Clinical significance of lesion in the cerebellar vermis

A

Truncal ataxia, dysarthria

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75
Q

Time to irreversible brain damage and areas most vulnerable

A

5 minutes

hippocampus, neocortex, cerebellum, watershed areas

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76
Q

Epidural hematoma

A

Middle meningeal artery
2° to skull fracture
Lucid interval
CT shows biconvex (lentiform/lens), hyperdense blood collection not crossing suture lines

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77
Q

Subdural hematoma

A

Bridging veins; 2° to frontal trauma
Crescent-shaped hemorrhage that crosses suture lines
Can cause midline shift; progressive neurologic signs

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78
Q

Subarachnoid hemorrhage

A

Rapid time course; worst HA of life

Bloody or yellow spinal tap

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79
Q

Intraparenchymal hemorrhage

A

Usually due to HTN

Typically occurs in basal ganglia and internal capsule (Charcot-Bouchard microaneurysm of lenticulostriate vessels)

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80
Q

Middle cerebral artery lesion in a stroke will affect what areas of the brain

A 
Motor and sensory cortices: upper limb and face
Temporal lobe (Wernicke area)
Frontal lobe (Broca area)
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81
Q

Middle cerebral artery lesion in a stroke will have what sx?

A

Contralateral paralysis and sensory loss—face and upper limb.
Aphasia if in dominant (usually left) hemisphere. Hemineglect if lesion affects nondominant (usually right) side

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82
Q

Anterior cerebral artery lesion in a stroke will affect what areas of the brain

A

Motor and sensory cortices—lower limb

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83
Q

Anterior cerebral artery lesion in a stroke will have what sx?

A

Contralateral paralysis and sensory loss—lower limb

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84
Q

Lenticulo- striate artery lesion in a stroke will affect what areas of the brain

A

Striatum, internal capsule

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85
Q

Lenticulo- striate artery lesion in a stroke will have what sx?

A

Contralateral paralysis and/or sensory loss—face and body. Absence of cortical signs

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86
Q

Anterior spinal artery lesion in a stroke will affect what areas of the brain

A

Lateral corticospinal tract.
Medial lemniscus.
Caudal medulla—hypoglossal nerve

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87
Q

Anterior spinal artery lesion in a stroke will have what sx?

A

Medial medullary syndrome
Contralateral paralysis—upper and lower limbs.
Decreased contralateral proprioception.
Ipsilateral hypoglossal dysfunction (tongue deviates ipsilaterally)

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88
Q

Posterior inferior cerebellar artery lesion in a stroke will affect what areas of the brain

A

Lateral medulla: Nucleus ambiguus (CN IX, X, XI) Vestibular nuclei
Lateral spinothalamic tract, spinal trigeminal nucleus
Sympathetic fibers
Inferior cerebellar peduncle

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89
Q

Posterior inferior cerebellar artery lesion in a stroke will have what sx?

A

Lateral medullary (Wallenberg) syndrome
Dysphagia, hoarseness, ↓ gag reflex
Vertigo, nystagmus, ↓ pain and temperature sensation
from contralateral body,
ipsilateral face
Ipsilateral Horner syndrome Ataxia, dysmetria

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90
Q

Anterior inferior cerebellar artery lesion in a stroke will affect what areas of the brain

A 
Lateral pons: Facial nucleus
Vestibular nuclei: Spinothalamic tract, spinal
trigeminal nucleus
Sympathetic fibers
Middle and inferior cerebellar peduncles
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91
Q

Anterior inferior cerebellar artery lesion in a stroke will have what sx?

A

Lateral pontine syndrome
Paralysis of face
Vertigo, nystagmus, vomiting, decreased pain and temperature sensation
from contralateral body,
ipsilateral face
Ipsilateral Horner syndrome Ataxia, dysmetria

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92
Q

Basilar artery lesion in a stroke will affect what areas of the brain

A

Pons, medulla, lower midbrain
Corticospinal and corticobulbar tracts
Ocular cranial nerve nuclei, paramedian pontine reticular formation

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93
Q

Basilar artery lesion in a stroke will have what sx?

A

“Locked-in syndrome.”

Loss of horizontal, but not vertical, eye movements

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94
Q

Posterior cerebral lesion in a stroke will affect what areas of the brain

A

Occipital lobe

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95
Q

Posterior cerebral lesion in a stroke will have what sx?

A

Contralateral hemianopia with macular sparin

96
Q

Central post-stroke pain syndrome

A

Neuropathic pain due to thalamic lesions

97
Q

Broca aphasia

A

Expressive aphasia
Able to comprehend but not speak fluently
Broca area in inferior frontal gyrus of frontal lobe. Patient
appears frustrated, insight intact

98
Q

Wernicke aphasia

A

Receptive aphasia
Able to speak fluently but not understand others
Wernicke area in superior temporal gyrus of temporal lobe

99
Q

Conduction aphasia

A

Can be caused by damage to arCuate fasciculus.

100
Q

Global aphasia

A

Cannot speak or understand

Arcuate fasciculus; Broca and Wernicke areas affected

101
Q

Transcortical motor aphasia

A

Able to comprehend but not speak fluently

Affects frontal lobe around Broca area, but Broca area is spared

102
Q

Transcortical sensory aphasia

A

Able to speak fluently but not understand others

Affects temporal lobe around Wernicke area, but Wernicke area is spared

103
Q

Transcortical, mixed aphasia

A

Broca and Wernicke areas and arcuate fasciculus remain intact; surrounding watershed areas affected

104
Q

Most common location of a berry aneurysm

A

bifurcations in the circle of Willis; most common is junction of ACom and ACA

105
Q

Cluster HA

A

Unilateral; 15m-3h
Brief, repetitive
Periorbital pain w/ lacrimation and rhinorrhea

106
Q

Treatment and prophylaxis for cluster HA?

A

Acute: sumatriptan, 100% O2 Prophylaxis: verapamil

107
Q

Tension HA

A

Bilateral; >30m, constant
Steady pain
No photophobia or phonophobia
No aura

108
Q

Migraine

A

Unilateral; 4-72h
Pulsating pain with
nausea, photophobia, or phonophobia; “aura”

109
Q

Treatment and prophylaxis for tension HA?

A

Analgesics, NSAIDs, acetaminophen; amitriptyline for chronic pain

110
Q

Treatment and prophylaxis for migraine HA?

A

Acute: NSAIDs, triptans, dihydroergotamine
Prophylaxis: lifestyle changes, β-blockers, calcium channel blockers, amitriptyline, topiramate, valproate

111
Q

Akathisia

A

Restlessness and intense urge to move

Seen in neuroleptic use or Parkinsons

112
Q

Asterixis

A

Extension of wrists causes “flapping” motion

Hepatic encephalopathy, Wilsons

113
Q

Athetosis

A

Slow, snake-like, writhing movements; especially seen in the fingers
Basal ganglia lesion

114
Q

Chorea

A

Sudden, jerky, purposeless movements

Basal ganglia lesion

115
Q

Dystonia

A

Sustained, involuntary muscle contractions

Writer’s cramp, blepharospasm, torticollis

116
Q

Essential tremor

A

High-frequency tremor with sustained posture
(eg, outstretched arms); worse with movement or anxious
Decreases w/ alcohol
Treat w/ non-selective β-blockers

117
Q

Hemiballismus

A

Sudden, wild flailing of 1 arm +/− ipsilateral leg

Contralateral subthalamic nucleus lesion

118
Q

Intention tremor

A

Slow, zigzag motion when pointing/extending toward a target

Cerebellar dysfunction

119
Q

Myoclonus

A

Sudden, brief, uncontrolled muscle contraction

Common in metabolic abnormalities such as renal and liver failure

120
Q

Resting tremor

A

Uncontrolled movement of distal appendages
Substansia nigra lesion
“pill rolling”

121
Q

Parkinson disease sx

A

Tremor (pill-rolling tremor at rest)
Rigidity (cogwheel)
Akinesia (or bradykinesia) Postural instability
Shuffling gait

122
Q

Parkinson disease etiology

A

Loss of dopaminergic neurons (ie, depigmentation) of substantia nigra pars compacta
Lewy bodies: composed of α-synuclein (intracellular eosinophilic inclusions)

123
Q

Huntington disease sx

A

Symptoms manifest between ages 20 and 50: chorea, athetosis, aggression, depression, dementia (sometimes mistaken for substance abuse)

124
Q

Huntington disease etiology

A

AD(CAG) repeat on chromosome 4
Atrophy of caudate and putamen with ex vacuo ventriculomegaly
↑ dopamine
↓ GABA and ACh
Neuronal death via NMDA-R binding and glutamate excitotoxicity

125
Q

Alzheimer disease risk factors

A

Down syndrome: APP on 21
ApoE2: ↓ risk of sporadic form
ApoE4: ↑ risk of sporadic form
APP, presenilin-1: familial forms (10%) with earlier onset

126
Q

Alzheimer disease histology

A

Widespread cortical atrophy
Narrowing of gyri and widening of sulci
Senile plaques in gray matter: extracellular β-amyloid core; may cause amyloid angiopathy
Neurofibrillary tangles
Loss of cholinergic neurons in nucleus basalis of Meynert

127
Q

Frontotemporal dementia (Pick disease) sx

A

Early changes in personality and behavior (behavioral variant), or aphasia (primary progressive aphasia)
May have associated movement disorders

128
Q

Frontotemporal dementia (Pick disease) histology

A

Frontotemporal lobe degeneration

Inclusions of hyperphosphorylated tau (round Pick bodies) in the cortex

129
Q

Lewy body dementia histology

A

Intracellular Lewy bodies primarily in cortex

130
Q

Vascular dementia

A

Due to multiple arterial infarcts and/or chronic ischemia

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

131
Q

Creutzfeldt-Jakob disease

A

Rapidly progressive (w to mo) dementia with myoclonus (“startle myoclonus”)
Periodic sharp waves on EEG
↑ 14-3-3 protein in CSF
Spongiform cortex

132
Q

Idiopathic intracranial HTN (pseudotumor cerebri) cause and risk factors

A

Increased ICP w/ no cause

Risk factors: female gender, obesity, vitamin A excess, tetracycline, danazol

133
Q

Idiopathic intracranial HTN (pseudotumor cerebri) sx?

A

HA, diplopia (CN VI palsy), no change in mental status. Papilledema on fundoscopy
LP: ↑ opening pressure and HA relief

134
Q

Idiopathic intracranial HTN (pseudotumor cerebri) treatment

A

weight loss, acetazolamide, topiramate, invasive procedures for refractory cases (eg, repeat LP, CSF shunt, optic nerve sheath fenestration)

135
Q

Communicating hydrocephalus

A

Decreased CSF absorption by arachnoid granulations

Increased ICP, papilledema, herniation

136
Q

Normal pressure hydrocephalus

A

Affects the elderly; idiopathic
Does not result in increased subarachnoid space volume
Expansion of ventricles distorts fibers of corona radiata: triad of urinary incontinence, ataxia, and cognitive dysfunction (wet, wobbly, wacky)
Characteristic magnetic gait (feet appear stuck to floor)

137
Q

Noncommunicating hydrocephalus

A

Structural blockage of CSF circulation within ventricular system

138
Q

Ex vacuo ventriculomegaly

A

Appearance of increased CSF but actually decreased brain tissue

139
Q

Osmotic demyelination syndrome (central pontine myelinolysis)

A

Acute paralysis, dysarthria, dysphagia, diplopia, LOC; “Locked in”
Massive axonal demyelination in pontine white matter
Caused by overly rapid correction of hyponatremia (Low to high; your pons will die)

140
Q

Multiple sclerosis cause

A

Autoimmune inflammation and demyelination of CNS

141
Q

Lhermitte phenomenon

A

Neck flexion precipitates sensation of electric shock running down spine (seen in MS and chemo)

142
Q

Multiple sclerosis disease course

A

Most often affects women in their 20s and 30s; more common in Caucasians living farther from equator; HLA-DR2
Relapsing and remitting

143
Q

MS sx

A

Optic neuritis (sudden loss of vision resulting in Marcus Gunn pupils), INO, hemiparesis, hemisensory symptoms, bladder/bowel dysfunction

144
Q

Charcot triad of MS

A

Scanning speech
Intention tremor (also Incontinence and Internuclear ophthalmoplegia)
Nystagmus

145
Q

MS histology and labs

A

Increased IgG and MBP in CSF
Oligoclonal bands
Periventricular plaques

146
Q

Treatment of MS

A

Disease-modifying therapies (β-interferon, glatiramer, natalizumab)
Acute: IV steroids

147
Q

Treatment for acute inflammatory demyelinating plyradiculopathy

A

(Guillain-Barre most common subtype)

plasmapheresis and IV ig

148
Q

Charcot-Marie-Tooth disease cause

A

Also known as hereditary motor and sensory neuropathy (HMSN)

defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath

149
Q

Charcot-Marie-Tooth disease sx

A

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

150
Q

Krabbe disease cause

A

AR lysosomal storage disease due to deficiency of galactocerebrosidase: destruction of myelin sheath; galatocerebrosidase build up in macrophages

151
Q

Krabbe disease sx

A

Peripheral neuropathy, developmental delay, optic atrophy, globoid cells

152
Q

Metachromatic leukodystrophy

A

AR lysosomal storage disease: arylsulfatase deficiency; build up of myelin

153
Q

Progressive multifocal leukoencephalopathy

A

Demyelination of CNS due to destruction of oligodendrocytes

Assoc w/JC virus

154
Q

Adrenoleukodystrophy

A

X-linked
Disrupts metabolism of very-long-chain fatty acids d/t impaired addition of coenzyme A
FA accumulates and damages adrenal glands and white matter

155
Q

Sturge-Weber syndrome (encephalotrigeminal angiomatosis) cause

A

Congenital, noninherited, developmental anomaly of neural crest derivatives due to somatic mosaicism for an activating mutation in one copy of the GNAQ gene

156
Q

Sturge-Weber syndrome (encephalotrigeminal angiomatosis) sx

A
  • port-wine stain of the face
  • ipsilateral leptomeningeal angioma: seizures/ epilepsy; intellectual disability
  • episcleral hemangioma: increased IOP -> early glaucoma
157
Q

Tuberous sclerosis cause

A

TSC1/TSC2 mutation on chromosome 16. Autosomal dominant, variable expression

158
Q

Tuberous sclerosis sx

A 
HAMARTOMAS: Hamartomas in CNS and skin
Angio fibromas
Mitral regurgitation
Ash-leaf spots
cardiac Rhabdomyoma (Tuberous sclerosis)
autosomal dOminant
Mental retardation (intellectual disability)
renal Angiomyolipoma
Seizures
Shagreen patches
Increased subependymal giant cell astrocytomas and ungual fibromas
159
Q

Neurofibromatosis type I (von Recklinghausen disease) cause

A

Mutation in NF1 tumor suppressor gene on chromosome 17: codes for neuro bromin, a negative regulator of RAS
Autosomal dominant, 100% penetrance

160
Q

Neurofibromatosis type I (von Recklinghausen disease) sx

A

Café-au-lait spots, cutaneous neurofibromas, optic gliomas, pheochromocytomas, Lisch nodules (pigmented iris hamartomas)

161
Q

Neurofibromatosis type II cause

A

Mutation in NF2 tumor suppressor gene on chromosome 22

Autosomal dominant

162
Q

Neurofibromatosis type II sx

A

bilateral acoustic schwannomas, juvenile cataracts, meningiomas, and ependymomas

163
Q

von Hippel-Lindau disease cause

A

Deletion of VHL gene on chromosome 3p

Autosomal dominant

164
Q

von Hippel-Lindau disease sx

A

HARP: Hemangioblastomas (high vascularity with hyperchromatic nuclei) in retina, brain stem, cerebellum, spine
Angiomatosis (cavernous hemangiomas in skin, mucosa, organs)
bilateral Renal cell carcinomas
Pheochromocytomas

165
Q

Glioblastoma multiforme

A 
Adult primary brain tumor
grade IV astrocytoma
highly malignant, cerebral hemispheres, butterfly
Astrocyte origin, GFAP ⊕
"Pseudopalisading”
166
Q

Oligodendroglioma

A 
Adult primary brain tumor
Rare, slow growing
Frontal lobes; “Chicken-wire” capillary pattern
“Fried egg” cells
Often calcified
167
Q

Meningioma

A

Adult primary brain tumor
occurs near surfaces of brain and in parasagittal region; dural attachment
may present with seizures or focal neurologic signs
Arachnoid cell origin; whorled; psammoma

168
Q

Hemangioblastoma

A

Adult primary brain tumor
Cerebellar; assoc w/ VHL
Can produce erythropoietin: 2° polycythemia
Blood vessel origin

169
Q

Schwannoma

A

Adult primary brain tumor
Classically at cerebellopontine angle
Localized to CN VIII in internal acoustic meatus → vestibular schwannoma
Schwann cell origin: S-100 ⊕

170
Q

Pilocytic (low-grade) astrocytoma

A

Childhood 1° brain tumor
Well circumscribed, found in posterior fossa
Glial cell origin, GFAP ⊕ Rosenthal fibers- eosinophilic, corkscrew fibers

171
Q

Medulloblastoma

A

Childhood 1° brain tumor
Most common malignant brain tumor in childhood
Involves cerebellum
Can compress 4th ventricle, causing noncommunicating hydrocephalus; drop metastases
Homer-Wright rosettes, small blue cells

172
Q

Ependymoma

A

Childhood 1° brain tumor
Found in 4th ventricle; hydrocephalus
Ependymal cell origin; perivascular rosettes

173
Q

Craniopharyngioma

A

Childhood 1° brain tumor
Most common childhood supratentorial tumor
Derived from remnants of Rathke pouch
Calcification is common
Cholesterol crystals found in “motor oil”—like fluid within tumor

174
Q

Pinealoma

A

Childhood 1° brain tumor
Tumor of pineal gland
Parinaud syndrome (compression of tectum → vertical gaze palsy)
Precocious puberty in males

175
Q

Sx of CN V motor lesion

A

Jaw deviates toward side of lesion due to unopposed force from the opposite pterygoid muscle

176
Q

Sx of CN X lesion

A

Uvula deviates away from side of lesion

Weak side collapses and uvula points away

177
Q

Sx of CN XI lesion

A

Weakness turning head to contralateral side of lesion (SCM)

Shoulder droop on side of lesion (trapezius)

178
Q

Sx of CN XII lesion

A

LMN lesion

Tongue deviates toward side of lesion

179
Q

Facial nerve LMN lesion

A

paralysis of upper and lower muscles of facial expression, hyperacusis, loss of taste sensation to anterior tongue

180
Q

Middle ear bones name and function

A

Ossicles (malleus, incus, stapes); conduct and amplify sound from eardrum to inner ear

181
Q

Conductive hearing loss

A 
Abnormal Rinne (bone > air)
Weber to affected ear
182
Q

Sensorineural hearing loss

A 
Normal Rinne (air > bone)
Weber to unaffected ear
183
Q

Noise-induced hearing loss

A

Damage to stereociliated cells in organ of Corti

Loss of high-frequency hearing first

184
Q

Presbycusis

A

Aging-related sensorineural hearing loss (higher frequencies) due to destruction of hair cells at cochlear base

185
Q

Cholesteatoma

A

Overgrowth of desquamated keratin debris within middle ear space
erode ossicles, mastoid air cells -> conductive hearing loss

186
Q

Conjunctivitis

A

Inflammation of the conjunctiva; redeye

187
Q

Hyperopia

A

“farsightedness”
Eye too short for refractive power of cornea and lens -> light focused behind retina
Correct with convex (converging) lenses

188
Q

Myopia

A

“nearsightedness”
Eye too long for refractive power of cornea and lens -> light focused in front of retina
Correct with concave (diverging) lens

189
Q

Astigmatism

A

Abnormal curvature of cornea: different refractive power at different axes
Correct with cylindrical lens

190
Q

Presbyopia

A

Aging-related impaired accommodation

191
Q

Cataract

A

opacification of lens

192
Q

Glaucoma

A

Optic disc atrophy with characteristic cupping

Elevated IOP

193
Q

Uveitis

A

Innflammation of uvea

194
Q

Age-related macular degeneration

A

Causes distortion (metamorphopsia) and eventual loss of central vision (scotomas)

195
Q

Miosis

A

Constriction, PSNS:
1st neuron: Edinger-Westphal nucleus to ciliary ganglion via CN III
2nd neuron: short ciliary nerves to sphincter pupillae muscles

196
Q

Mydriasis

A

Dilation, sympathetic

197
Q

Marcus Gunn pupil

A

Afferent pupillary defect: optic nerve damage or severe retinal injury.
Decreased bilateral pupillary constriction when light is shone in affected eye relative to unaffected eye

198
Q

CN III damage: motor

A

ptosis, “down and out” gaze

199
Q

CN III damage: PSNS

A

diminished or absent pupillary light reflex, “blown pupil” often with “down-and-out” gaze

200
Q

CN IV damage

A

Eye moves upward, particularly with contralateral gaze

201
Q

CN VI damage

A

Medially directed eye that cannot abduct

202
Q

Cavernous sinus syndrome

A

variable ophthalmoplegia, decreased corneal sensation, Horner syndrome and occasional decreased maxillary sensation
CN VI is most susceptible to injury

203
Q

Internuclear ophthalmoplegia

A

Lesion in medial longitudinal fasciculus: conjugate horizontal gaze palsy
When CN VI nucleus activates ipsilateral lateral rectus, contralateral CN III nucleus does not stimulate medial rectus to contract
Seen in MS

204
Q

Suvorexant

A

Orexin (hypocretin) receptor antagonist

For insomnia

205
Q

Ramelteon

A

Melatonin receptor agonist, binds MT1 and MT2 in suprachiasmatic nucleus
For insomnia

206
Q

Drugs used in Huntington disease

A

Tetrabenazine and reserpine: inhibit VMAT

Haloperidol: D2 receptor antagonist

207
Q

Riluzole

A

Treatment for ALS that modestly increases survival by decreasing glutamate excitotoxicity via an unclear mechanism

208
Q

Memantine

A

NMDA-r antagonist

For Alzheimer

209
Q

MOA of local anesthetics

A

Block Na+ channels by binding to specific receptors on inner portion of channel

210
Q

Baclofen

A

Activates GABAb receptors at spinal cord level, inducing skeletal muscle relaxation.

211
Q

Cyclobenzaprine

A

Centrally acting skeletal muscle relaxant

Structurally related to TCAs, similar anticholinergic side effects

212
Q

Pentazocine

A

κ-opioid receptor agonist and μ-opioid receptor weak antagonist or partial agonist
Analgesia for moderate to severe pain

213
Q

Butorphanol

A

κ-opioid receptor agonist and μ-opioid receptor partial agonist; produces analgesia

214
Q

Tramadol

A

Very weak opioid agonist; also inhibits 5-HT and norepinephrine reuptake

215
Q

Frog-like appearance of a fetus

A

anencephaly

216
Q

What is impaired in anencephaly?

A

Amniotic fluid swallowing: polyhydraminos

217
Q

Cerebral aquaduct stenosis

A

Stenosis of channel that drains CSF from 3rd into 4th ventricle; accumulates in ventricular space

218
Q

Most common cause of hydrocephalus in a newborn

A

Cerebral aquaduct stenosis

219
Q

Histology of acute neuronal injury

A

Red neurons; 12-24hrs

Cell body shrinkage, pyknosis, loss of Nissle bodies, eosinophilia

220
Q

Axonal reaction

A

Increased protein synthesis, axonal sprouting, peripheral displacement of nucleus

221
Q

Astrocyte injury

A

Gliosis; nuclei prominent and vesicular with expansion into cytoplasm

222
Q

Alzheimer type II astrocyte

A

Gray matter cell with large nucleus and pale chromatin

Seen in longstanding hyper-ammonia: liver dz, Wilson’s, urea cycle disorder

223
Q

Werdnig-Hoffman Disease

A

AR; Degeneration of AMH

224
Q

Amyotrophic Lateral Sclerosis

A

Degenertive disorder with UMN (lateral corticospinal tract) and LMN (AMH)
Atrophy and b/l hand weakness - early signs
NO sensory loss
SOD mutation in familial cases (most are spontaneous)

225
Q

Friedreich Ataxia

A

Degeneration of cerebellum and spinal cord
Loss of vibration and proprioception, LE weakness, ↓ DTRs
GAA repeat; frataxin gene regulates iron in mitochondria
Assoc w/ hypertrophic cardiomyopathy

226
Q

Most common cause of meningitis in adults and elderly

A

S. pneumo

227
Q

Infarct of pyramidal neurons in the cerebral cortex leads to what

A

(layer 3, 5, 6)

Laminar necrosis

228
Q

Lacunar stroke

A

2° to hyaline arteriolosclerosis

Involved lentriculstriae vessels (from the MCA)

229
Q

Pure motor stroke

A

Internal capsule

230
Q

Pure sensory stroke

A

thalamus

231
Q

What kind of stroke leads to liquefactive necrosis?

A

Ischemic

232
Q

When do red neurons appear in a stroke

A

12-24 hours

233
Q

Intracerebral hemorrhage

A

Into brain parenchyma; usually basal ganglia
Rupture of Charcot-Bouchard of lentriculstriae vessels
2° to HTN

234
Q

Subarachnoid hemorrhage

A

Bleed into subarachnoid space; only cause of blood on bottom of brain
Xanthochromia on LP
Berry aneurysm, usually at branch points of ACA

235
Q

Subacute Sclerosing Panencephalitis

A

D/t measles

Viral inclusion in neurons and oligodendrocytes

236
Q

Excessive correction of hypernatremia

A

Cerebral edeama or herniation (from high to low, your brain will blow)

FA note cards (16 decks)

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