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Step 1 > Neurology > Flashcards

Neurology Flashcards

1
Q

What is the role of the notochord?

A

Induces overlying ectoderm to differentiate into neuroectoderm and form neural plate.

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2
Q

What is the role of the neural plate?

A

Gives rise to neural tube and neural crest cells.

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3
Q

What does the notochord become?

A

Nucleus pulposus of the intervertebral discs

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4
Q

What does the neural tube become?

A

Spinal cord and brain

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5
Q

What are the three primary vesicles and 5 secondary vesicles of the developing brain?

A

Prosencephalon (forebrain; becomes diencephalon and 2 telencephalons), Mesencephalon (midbrain), Rhombencephalon (hindbrain; becomes metencephalon and myelencephalon)

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6
Q

What does the neuroectoderm differentiate into?

A

CNS neurons, ependymal cells, oligodendroglia, astrocytes

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7
Q

What do the neural crest cells differentiate into?

A

PNS neurons, Schwann cells

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8
Q

In what week of gestation do neural tube defects occur?

A

4th week

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9
Q

What is an Arnold-Chiari malformation and what is another name for it?

A

Chiari malformation II; Herniation of cerebellar tonsils and vermis through foramen magnum with aqueductal stenosis and hydrocephalus. Often with lumbosacral myelomeningocele and paralysis below defect.

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10
Q

What is a Dandy-Walker malformation?

A

Agenesis of cerebellar vermis with cystic enlargement of 4th ventricle. Associated with hydrocephalus and spina bifida.

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11
Q

What is the role of microglia?

A

CNS phagocytes. HIV-infected microglia fuse to form multinucleated giant cells in CNS

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12
Q

Multiple sclerosis, progressive multifocal leukoencephalopathy (PML), and leukodystrophies are all a result of what?

A

Injured oligodendroglia (myelination in CNS)

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13
Q

Guillain-Barré is a result of what?

A

Destruction of Schwann cells; ascending paralysis

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14
Q

Where is NE synthesized?

A

Locus ceruleus (pons)

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15
Q

Where is DA synthesized?

A

Ventral tegmentum and SNc (midbrain)

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16
Q

Where is 5-HT synthesized?

A

Raphe nucleus (pons, medulla, midbrain)

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17
Q

Where is ACh synthesized?

A

Basal nucleus of Meynert

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18
Q

Where is GABA synthesized?

A

Nucleus accumbens

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19
Q

The BBB is formed by which 3 structures?

A

Tight junctions between non-fenestrated capillary endothelial cells, basement membrane, and astrocyte foot processes

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20
Q

What specifically makes ADH?

A

Supraoptic nucleus of the hypothalamus

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21
Q

What specifically makes oxytocin?

A

Paraventricular nucleus

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22
Q

The lateral area of the hypothalamus is responsible for what? What happens in its destruction? What is the lateral area inhibited by?

A

Hunger; Anorexia, failure to thrive; Leptin

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23
Q

The ventromedial area of the hypothalamus is responsible for what? What happens in its destruction? What is the ventromedial area stimulated by?

A

Satiety; Hyperphagia; Leptin

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24
Q

The anterior hypothalamus is responsible for what?

A

Cooling, parasympathetic. “A/C: Anterior Cooling”

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25
Q

The posterior hypothalamus is responsible for what?

A

Heating, sympathetic.

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26
Q

The suprachiasmatic nucleus of the hypothalamus is responsible for what?

A

Circadian rythm

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27
Q

Branchial clefts are derived from which embryonic layer?

A

Ectoderm

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28
Q

Branchial arches are derived from which embryonic layer?

A

Mesoderm

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29
Q

Branchial pouches are derived from which embryonic layer?

A

Endoderm

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30
Q

What does the 1st cleft develop into?

A

External auditory meatus

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31
Q

What do the 2nd-4th clefts form?

A

Temporary cervical sinuses, which are obliterated by proliferation of 2nd arch mesenchyme.

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32
Q

What does a persistent cervical sinus lead to?

A

Branchial cleft cyst within lateral neck.

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33
Q

How is bedwetting (sleep enuresis) treated?

A

Oral desmopressin acetate (DDAVP) which mimics ADH

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34
Q

How are night terrors and sleepwalking treated?

A

Benzodiazepines

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35
Q

What is the input to the VPL nucleus of the thalamus?

A

Spinothalamic and dorsal columns/medial lemniscus

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36
Q

What is the input to the VPM nucleus of the thalamus?

A

Trigeminal and gustatory pathway

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37
Q

What is the input to the LGN nucleus of the thalamus?

A

CNII (vision)

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38
Q

What is the input to the MGN nucleus of the thalamus?

A

Superior olive and inferior colliculus of tectum (hearing)

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39
Q

What is the input to the VL nucleus of the thalamus?

A

Basal ganglia, cerebellum (motor)

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40
Q

Which structures make up the limbic system? What is the limbic system responsible for?

A

Hippocampus, amygdala, fornix, mammillary bodies, and cingulate gyrus. The famous 5 F’s: Feeding, Fleeing, Fighting, Feeling, and Fucking

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41
Q

What is an intention tremor?

A

Slow, zig zag motion when pointing/extending toward a target - cerebellar dysfunction

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42
Q

What is hemiballismus?

A

Sudden, wild flailing of 1 arm +/- ipsilateral leg - contralateral subthalamic nucleus (lacunar stroke)

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43
Q

What is athetosis?

A

Slow, writhing movements; especially seen in fingers - basal ganglia (Huntington)

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44
Q

What is myoclonus?

A

Sudden, brief, uncontrolled muscle contraction

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45
Q

What is Kluver-Bucy syndrome?

A

Bilateral lesion of amygdala. Hyperorality, hypersexuality, disinhibited behavior. Associated with HSV-1.

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46
Q

What are the consequences of a right parietal-temporal cortex lesion?

A

Spatial neglect syndrome (agnosia of the contralateral side of world)

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47
Q

What are the consequences of a left parietal-temporal cortex lesion?

A

Agraphia, acalculia, finger agnosia, and L-R disorientation. Aka Gerstmann syndrome.

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48
Q

What are the consequences of a lesion to the subthalamic nucleus?

A

Contralateral hemiballismus

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49
Q

What are the consequences of a lesion to the paramedian pontine reticular formation?

A

Eyes look away from side of lesion

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50
Q

What are the consequences of a lesion to the frontal eye fields?

A

Eyes look toward lesion

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51
Q

What disease process results from correcting serum Na too fast?

A

Central pontine myelinolysis

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52
Q

What is central pontine myelinolysis?

A

A variant of the osmotic demyelination syndrome. Acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness. “Locked-in syndrome.”

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53
Q

What is conduction aphasia?

A

Poor repetition but fluent speech, intact comprehension

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54
Q

What is transcortical motor aphasia?

A

Nonfluent aphasia with good comprehension and repetition

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55
Q

What is transcortical sensory aphasia?

A

Poor comprehension with fluent speech and repetition

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56
Q

What is mixed transcortical aphasia?

A

Nonfluent speech, poor comprehension, good repetition

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57
Q

What is the most common location of a berry aneurysm?

A

Junction of anterior communicating artery and anterior cerebral artery

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58
Q

Which diseases are berry aneurysms associated with?

A

ADPKD, Ehlers-Danlos, and Marfan syndrome

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59
Q

What is a Charcot-Bouchard microaneurysm?

A

Associated with chronic HTN, affects small vessels (basal ganglia, thalamus)

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60
Q

An epidural hematoma is most commonly caused by rupture of which artery?

A

Middle meningeal artery

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61
Q

Does an epidural hematoma cross suture lines? Fax and tentorium?

A

Does not cross suture lines; can cross falx and tentorium

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62
Q

Does a subdural hematoma cross suture lines? Fax and tentorium?

A

Crosses suture lines; cannot cross falx or tentorium

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63
Q

A subdural hematoma is most commonly caused by rupture of what?

A

Rupture of bridging veins of arachnoid space

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64
Q

Which type of intracranial hemorrhage causes bloody or yellow (xanthochromatic) spinal tap?

A

Subarachnoid hemorrhage

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65
Q

Which disease process causes one to be “wet wobbly and wacky?”

A

Normal pressure hydrocephalus

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66
Q

How many spinal nerves do we have?

A
  1. 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, 1 cocygeal
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67
Q

What are the common sites of a vertebral disc herniation?

A

Posterolaterally at L4-L5 or L5-S1

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68
Q

Where is a lumbar puncture performed?

A

Between L3 and L5 (cauda equina)

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69
Q

What are characteristics of an UMN lesion?

A

Spastic paralysis, Babinski +, weakness, hyperreflexia, more tone, clasp knife spasticity

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70
Q

What are characteristics of an LMN lesion?

A

Weakness, atrophy, hyporeflexia, fasciculations, low tone, flaccid paralysis

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71
Q

What medication can modestly increase survival of ALS patients?

A

Riluzole - decreases presynaptic glutamate release

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72
Q

A complete occlusion of the anterior spinal artery would spare which portion of the spinal cord?

A

The dorsal columns and Lissauer tracts

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73
Q

What is Tabes dorsalis?

A

Degeneration/demyelination of dorsal columns and roots, caused by tertiary syphilis. Exam will demonstrate Argyll Robertson pupils, Charcot joints, shooting pain, absence of DTRs, and positive Romberg.

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74
Q

What are Argyll Robertson pupils?

A

Small bilateral pupils that further constrict to accommodation and convergence, but not to light. Seen in Tabes dorsalis.

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75
Q

What is a positive Romberg sign?

A

Pt stands up with feet together and can only keep balance with their eyes open.

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76
Q

A VitaminB12 or VitE deficiency will affect which portions of the spinal cord?

A

Dorsal column, lateral corticospinal tracts, and spinocerebellar tracts.

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77
Q

What is Werdnig-Hoffman disease?

A

Congenital degeneration of anterior horns of spinal cord –> LMN lesion. Floppy baby with marked hypotonia and tongue fasciculations. Mutations in the survival motor neuron (SMN1) gene - involved in assembly of snRNPs (spliceosome).

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78
Q

What is the inheritance pattern of Freidreich ataxia?

A

Autosomal recessive

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79
Q

What is the inheritance pattern of Werdnig-Hoffman disease?

A

Autosomal recessive

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80
Q

Mutations in the survival motor neuron (SMN1) gene are associated with what disease?

A

Werdnig-Hoffman disease

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81
Q

Freidreich ataxia is do to what molecular process?

A

Trinucleotide repeat disorder (GAA) on chromosome 9 in gene that encodes frataxin (iron binding protein). Leads to impairment of mitochondrial functioning.

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82
Q

What are the clinical findings of Freidreich ataxia?

A

Muscle weakness, loss of DTRs, vibratory sense, proprioception. Staggering gait, falls, nystagmus, dysarthria, pes cavus, hammer toes, hypertrophic cardiomyopathy (cause of death).

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83
Q

How does Freidreich ataxia present in childhood?

A

Kyphoscoliosis

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84
Q

What is Brown-Séquard syndrome and what are its findings?

A

Hemisection of spinal cord. Ipsilateral UMN signs below level of lesion, ipsilateral LMN at level of lesion, ipsilateral loss of tactile, vibration, proprioreception sense 1-2 levels below level of lesion, contralateral pain and temperature loss below level of lesion.

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85
Q

What is the landmark of dermatome C2?

A

Posterior half of a skull “cap”

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86
Q

What is the landmark of dermatome C3?

A

High turtleneck shirt

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87
Q

What is the landmark of dermatome C4?

A

Low collared shirt

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88
Q

What is the landmark of dermatome T4?

A

Nipple

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89
Q

What is the landmark of dermatome T7?

A

Xiphoid process

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90
Q

What is the landmark of dermatome T10?

A

Belly button

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91
Q

What is the landmark of dermatome L1?

A

Inguinal ligament

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92
Q

What is the landmark of dermatome L4?

A

Kneecaps

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93
Q

What is the landmark of dermatome S2, S3, S4?

A

Erection and sensation of penile and anal zones

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94
Q

The biceps reflex is associated with which nerve root?

A

C5

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95
Q

The triceps reflex is associated with which nerve root?

A

C7

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96
Q

The patellar reflex is associated with which nerve root?

A

L4

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97
Q

The achilles reflex is associated with which nerve root?

A

S1

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98
Q

Primitive reflexes are inhibited by what in neurologically intact adults?

A

Mature/developing frontal lobe

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99
Q

What is the moro reflex?

A

“Hang on for life” reflex - abduct/extend limbs when startled, then draw together

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100
Q

What is the rooting reflex?

A

Movement of head toward one side if cheek or mouth is stroked

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101
Q

What is the sucking reflex?

A

Sucking response when roof of mouth is touched

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102
Q

What is the palmar reflex?

A

Holding on to finger

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103
Q

What is the plantar reflex?

A

Dorsiflexion of large toe and fanning of rest with plantar stimulation. Babinski sign if positive in adults.

104
Q

What is the galant reflex?

A

Stroking along one side of the spine while newborn is in ventral suspension causes lateral flexion (face down) of lower body toward stimulated side

105
Q

What is the pineal body responsible for?

A

Melatonin secretion and circadian rhythms

106
Q

What is the superior colliculus responsible for?

A

Conjugate vertical gaze

107
Q

What is the inferior colliculus responsible for?

A

Auditory

108
Q

What is Parinaud syndrome?

A

Paralysis of conjugate vertical gaze due to lesion in superior colliculi

109
Q

Which cranial nerve is responsible for mastication?

A

Trigeminal

110
Q

Which cranial nerve is responsible for taste from epiglottic region?

A

Vagus

111
Q

Which cranial nerve is responsible for taste and somatosensation from posterior 1/3 of tongue?

A

Glossopharyngeal

112
Q

Which cranial nerve is responsible for somatosensation of the anterior 2/3 of tongue?

A

Trigeminal

113
Q

Which cranial nerve is responsible for swallowing and salivation?

A

Glossopharyngeal

114
Q

The nuclei of which cranial nerves reside in the medulla?

A

IX, X, XII

115
Q

The nuclei of which cranial nerves reside in the pons?

A

V, VI, VII, VIII

116
Q

The nuclei of which cranial nerves reside in the midbrain?

A

III, IV

117
Q

The nuclei of which cranial nerves reside in the spinal cord?

A

XI

118
Q

What are some etiologies for Horner syndrome?

A

Pancoast tumor, Brown-Sequard syndrome, late-stage syringomyelia

119
Q

The arcuate nucleus of the hypothalamus is responsible for what?

A

Secretion of DA, GHRH, and gonadotropin-releasing hormone

120
Q

The paraventricular nucleus of the hypothalamus is responsible for what?

A

Secretion of ADH, CTRH, oxytocin, and thyrotropin-releasing hormone.

121
Q

Where is the chemoreceptor trigger zone located?

A

The dorsal surface of the medulla at the caudal end of the 4th ventricle

122
Q

What is the mnemonic to remember clinical reflexes?

A

S1,2 “buckle my shoe” (Achilles)
L3,4 “kick the door” (Patellar)
C5,6 “pick up sticks” (Biceps)
C7,8 “lay them straight” (Triceps)

L,2 Cremaster
S3,4 Anal wink ;)

123
Q

What is the most common pineal gland mass?

A

Germinona

124
Q

What is Parinaud syndrome?

A

Paralysis of upward gaze, ptosis, and pupillary abnormalities. Due to direct compression of the pretectal region of midbrain.

125
Q

What is Wallenberg syndrome and what does it lead to?

A

Lateral medullary syndrome. Contralateral loss of pain and temperature sensations along with ipsilateral paralysis of CNs V, IX, X, XI

126
Q

What does Medial medullary syndrome cause?

A

Contralateral spastic paralysis, contralateral sensory loss of vibration and proprioception, and ipsilateral flaccid paralysis of the tongue (CN XII)

127
Q

What is the nucleus solitarius (vagal nucleus) responsible for?

A

Visceral sensory info (taste, baroreceptors, gut distention) - VII, IX, X

128
Q

What is the nucleus ambiguus (vagal nucleus) responsible for?

A

Motor innervation of pharynx, larynx, and upper esophagus - IX X, XI

129
Q

What is the dorsal motor nucleus (vagal nucleus) responsible for?

A

Sends autonomic (parasympathetic) fibers to heart, lungs, and upper GI - CN X

130
Q

Where is the cavernous sinus located?

A

On either side of the pituitary

131
Q

Which nerves pass through the cavernous sinus?

A

CN III, IV, V1, V2, and VI and postganglionic sympathetic fibers en route to the orbit (and internal carotid artery)

132
Q

What are the clinical manifestations of cavernous sinus syndrome?

A

Ophthalmoplegia and decreased corneal and maxillary sensation with normal visual acuity. CN VI commonly affected as well.

133
Q

In a CN V motor lesion, does the jaw deviate toward or away lesion?

A

Toward; due to unopposed force from the opposite pterygoid muscle

134
Q

In CN X lesion, what sign can be seen in the mouth?

A

Uvula deviates away from side of lesion. Weak side collapses and uvula points away.

135
Q

What is the clinical manifestation of a CN XI lesion?

A

Weakness turning head to contralateral side of lesion (SCM muscle), and shoulder droop on ipsilateral side of lesion (trapezius)

136
Q

What is the clinical manifestation of a CN XII lesion?

A

Tongue deviates toward side of lesion

137
Q

What does the Rinne test test?

A

Tests air to bone conduction (place tuning fork behind ear then in front of ear and ask pt when they stop hearing)

138
Q

What does the Weber test test?

A

Differentiates between conductive and sensorineural hearing loss (place tuning fork on top of head)

139
Q

If a patient has conductive hearing loss, what happens in the Rinne and Weber tests?

A

Rinne: Abnormal (bone>air)
Weber: Localizes to affected ear

140
Q

If a patient has sensorineural hearing loss, what happens in the Rinne and Weber tests?

A

Rinne: Normal (air>bone)
Weber: Localizes to unaffected ear

141
Q

Is the forehead paralyzed in a facial UMN lesion?

A

No - bilateral UMN innervation of forehead

142
Q

Is the forehead paralyzed in a facial UMN lesion?

A

Yes

143
Q

What is the treatment of a facial nerve palsy?

A

Corticosteroids

144
Q

What are the 3 muscles of mastication?

A

Masseter, temporalis, and medial pterygoid

145
Q

What is hyperopia?

A

Eye too short for refractive power

146
Q

What is myopia?

A

Eye too long for refractive power

147
Q

What is astigmatism?

A

Abnormal curvature of cornea resulting in different refractive power at different axes

148
Q

What is presbyopia?

A

Decrease in focusing ability during accommodation due to sclerosis and decreased elasticity

149
Q

What are the usual causes of retinitis?

A

CMV, HSV, HZV

150
Q

What are the two types of diabetic retinopathy?

A

Proliferative and non-proliferative

151
Q

Describe non-proliferative diabetic retinopathy

A

Damaged capillaries leak blood –> lipids and fluid seep into retina –> hemorrhages and macular edema.

152
Q

Describe proliferative diabetic retinopathy

A

Chronic hypoxia results in new blood vessel formation with resultant traction on retina.

153
Q

What is the treatment for non-proliferative diabetic retinopathy?

A

Glucose control and macular laser

154
Q

What is the treatment for proliferative diabetic retinopathy?

A

Peripheral retinal photocoagulatoin, anti-VEGF injections

155
Q

Define glaucoma

A

Optic disc atrophy with characteristic cupping, usu with increased IOP and progressive peripheral visual field loss

156
Q

What is open angle glaucoma associated with?

A

Age, African-American race, family hx. Painless.

157
Q

What is secondary open angle glaucoma caused by?

A

Blocked trabecular meshwork from WBCs (uveitis), RBCs, retinal elements

158
Q

What is primary closed/narrow angel glaucoma?

A

Forward pushing of lens against iris blocks trabecular network, fluid buildup and increased IOP

159
Q

What is secondary closed/narrow angel glaucoma?

A

Hypoxia from retinal disease induces vasoproliferation in iris which contracts angle

160
Q

What does papilledema indicate?

A

Increased intracranial pressure

161
Q

How does the eye look with CN III damage/compression?

A

Eyes look down and out, ptosis, pupillary dilation, loss of accommodation

162
Q

How does the eye look with CN IV damage?

A

Eyes move upward and head tilt toward side of lesion (because the superior oblique muscle rotates eye inward)

163
Q

How does the eye look with CN VI damage?

A

Medially directed eye that cannot abduct (lateral rectus lesion)

164
Q

Describe the pupillary light reflex

A

Light in either retina sends a signal via CN II to pretectal nuclei in midbrain that activates bilateral Edinger-Westphal nuclei and both pupils constrict

165
Q

What is an afferent pupillary defect?

A

When there is less bilateral pupillary constriction when light is shone in affected eye relative to unaffected eye. Due to optic nerve damage or severe retinal injury.

166
Q

What is CN III composed of?

A

Motor and parasympathetic fibers

167
Q

If the upper left visual field is blind, where is lesion? (left upper quadrantic anopia)

A

Right Meyer loop (temporal lobe)

168
Q

If the lower left visual field is blind, where is lesion? (left lower quadranti anopia)

A

Right dorsal optic radiation (parietal lobe)

169
Q

If only the left center visual field is blind, where is the lesion?

A

Left macula

170
Q

If the left visual field is blind sparing the macula, where is the lesion?

A

PCA infarct of contralateral side

171
Q

Where is the brain does the Meyer loop travel?

A

Loops around inferior horn of lateral ventricle

172
Q

Where in the brain does the dorsal optic radiation travel?

A

Internal capsule

173
Q

Internuclear ophthalmoplegia (INO) occurs when what tract is damaged (usu with MS)?

A

MLF

174
Q

What is the role of the MLF in terms of vision?

A

Allows for crosstalk between CN VI and CN III nuclei - coordinates both eyes to move in same horizontal direction.

175
Q

In INO, what is seen?

A

With a left MLF lesion, the right eye looks laterally but the left medial rectus cannot contract and left eye stays looking straight. Right eye will have nystagmus because CN VI overfires to try to stimulate CN III. Convergence is normal.

176
Q

An orbital floor fracture will damage what nerve?

A

Infraorbital nerve (continuation of maxillary nerve).

177
Q

Atrophy of cortex leads to what disease process?

A

Dementia

178
Q

Atrophy of basal ganglia leads to what kind of diseases?

A

Movement disorders

179
Q

What is the neurotransmitter abnormality in Alzheimer’s?

A

Low ACh; due to decreased choline acetyltransferase activity in the nucleus basalis

180
Q

What are the hallmarks of Alzheimers?

A

Senile plaques composed on extracellular beta amyloid, and neurofibrillary tangles composed of intracellular hyperphosphorylated tau protein

181
Q

Early onset Alzheimers is due to which altered proteins?

A

APP (Chr 21), presenilin-1 (Chr 14), and presenilin-2 (Chr 1)

182
Q

Late onset Alzheimers is due to which altered protein?

A

ApoE4 (Chr 9)

183
Q

Which protein expression is protective against Alzheimers?

A

ApoE2 (Chr 19)

184
Q

Are there focal neurological deficits in Alzheimers?

A

No! Diffuse process

185
Q

What is another name for Pick disease?

A

Frontotemporal dementia

186
Q

What is the hallmark of Pick disease?

A

Pick bodies: spherical tau protein aggregates

187
Q

What is Lewy body dementia and what is its hallmark?

A

Initially dementia and visual hallucinations followed by Parkinsononian features; alpha-synuclein defect

188
Q

What is seen on EEG with Creutzfeldt-Jakob disease (CJD)?

A

Spike wave complexes

189
Q

What builds up in the brain in CJD?

A

Prions (beta pleated sheets resistant to proteases)

190
Q

What is the most common cause of dementia in the elderly?

A

Azheimer

191
Q

What is the second most common cause of dementia in the elderly?

A

Multi-infarct

192
Q

What is a very vulnerable area of the brain to infarct?

A

Hippocampus

193
Q

Where is the defect in restless leg syndrome (RLS)?

A

Dopaminergic pathway

194
Q

What diseases is RLS associated with?

A

CKD and DM

195
Q

What is the treatment of RLS?

A

DA agonists (ropinirole, pramipexole)

196
Q

Which HLA type is MS associated with?

A

HLA DR2

197
Q

What are the CSF findings in a patient with MS?

A

Increased lymphs, increased IgG with oligoclonal IgG bands, and myelin basic protein

198
Q

What is the gold standard for diagnosis of MS?

A

MRI - periventricular plaques

199
Q

What are three treatment options for MS?

A

Beta-interferon, immunosuppression, and natalizumab.

200
Q

What is acute inflammatory demyelinating polyradiculopathy?

A

Autoimmune condition that destroys Schwann cells. (Guillain-Barre) Results in ascending weakness/paralysis.

201
Q

Which bug causes subacute sclerosing panencephalitis?

A

Measles

202
Q

Lesions of the subthalamic nucleus usually cause what?

A

Contralateral hemiballismus

203
Q

Homovanillic acid is a metabolite of what?

A

DA

204
Q

Low CSF hydroxyindole-acetic acid has been associated with what?

A

Impulsive, destructive behaviors

205
Q

Elevated CSF levels of 14-3-3 protein are helpful for diagnosing what?

A

CJD

206
Q

Progressive multifocal leukoencephalopathy (PML) is associated with which virus?

A

JC virus

207
Q

Increased risk of PML is seen with which medication?

A

Natalizumab

208
Q

What is acute disseminated (postinfectious) encephalomyelitis?

A

Multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations

209
Q

What is Metachromatic Leukodystrophy and what is its inheritance pattern?

A

An autosomal recessive lysosomal storage disease, most commonly due to arylsulfatase A deficiency. Buildup of sulfatides leads to impaired production of myelin sheath.

210
Q

What are the findings of Metachromatic Leukodystrophy?

A

Central and peripheral demyelination with ataxia, dementia.

211
Q

What is Charcot-Marie-Tooth?

A

A group of inherited (usu autosomal dominant) progressive nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or myelin sheath

212
Q

What is Krabbe disease?

A

Autosomal recessive lysosomal storage disease due to deficiency of galactocerebrosidase. Buildup of galactocerebroside and psychosine in macrophages destroys myelin sheath.

213
Q

What are the findings of Krabbe disease?

A

Peripheral neuropathy, developmental delay, optic atrophy, globoid cells

214
Q

What is adrenoleukodystrophy (ALD)? What’s its inheritance pattern?

A

X-linked genetic disorder usu in males. Disrupts metabolism of very-long-chain fatty acids –> excessive buildup in nervous system, adrenal gland, and testes. Can lead to long-term coma/death and adrenal crisis.

215
Q

What is the difference between simple and complex seizure?

A

Consciousness (lose it in complex)

216
Q

What is status epilepticus?

A

Continuous seizure for >30min or recurrent seizures without regaining consciousness between seizures for >30min. Medical emergency.

217
Q

What is a proper treatment for cluster headaches?

A

Sumatriptan

218
Q

What is a proper treatment for migraine headaches?

A

Abortive therapies (triptans, NSAIDs), Prophylactic therapies (propranolol, topiramate, CCBs, amitriptyline)

219
Q

What is the difference between peripheral and central vertigo?

A

Peripheral is more common and involves the inner ear. Central is a brainstem or cerebellar lesion.

220
Q

What is Sturge-Weber syndrome? What are all of its manifestations? *this is a long one…

A

Congenital non-inherited developmental anomaly of neural crest derivatives due to activating mutation of GNAQ gene. Affects small blood vessels –> port wine stain of face in CN V1/V2 distribution; ipsilateral leptomeningeal angioma –> seizures/epilepsy; intellectual disability; episcleral hemangioma –> increased IOP –> early onset glaucoma.

221
Q

What are all the manifestations of tuberous sclerosis? (Hint: use mnemonic HAMARTOMAS)

A

Hamartomas in CNS and skin, Angiofibromas, Mitral regurgitation, Ash-leaf spots, cardiac Rhabdomyoma, (Tuberous sclerosis), autosomal dOminant, Mental retardation, renal Angiomyolipoma, Seizures, Shagreen patches.

222
Q

Patients with tuberous sclerosis have increased incidence of what?

A

Subependymal astrocytomas and ungual fibromas

223
Q

What are the clinical findings of NF1?

A

Cafe-au-lait spots, Lisch nodules, neurofibromas in skin, optic gliomas, pheochromocytomas.

224
Q

What is the cause and inheritance pattern of NF1?

A

Mutation NF1 tumor suppressor gene - neurofibromin, a negative regulator of Ras) on chr 17; autosomal dominant

225
Q

What are the clinical findings of von Hippel-Lindau disease?

A

Cavernous hemangiomas in skin, mucosa, organs; bilateral renal cell carcinomas; hemangioblastoma in retina, brain stem, cerebellum; and pheochromocytomas

226
Q

What is the cause and inheritance pattern of von Hippel-Lindau?

A

Autosomal dominant; mutated VHL tumor suppressor gene on chr 3, which results in a constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors

227
Q

About what percent of brain tumors are primary?

A

50%

228
Q

“Pseudopalisading” is a buzzword for which brain tumor?

A

Glioblastoma multiforme

229
Q

A meningioma arises from which cells?

A

Arachnoid cells

230
Q

Which type of brain tumor occurs more commonly in females?

A

Meningioma (expresses estrogen R)

231
Q

Which brain tumor can produce EPO and lead to polycythemia?

A

Hemangioblastoma

232
Q

Schwannomas are usually found where?

A

Cerebellopontine angle

233
Q

Which brain tumor is S-100 positive?

A

Schwannoma

234
Q

Bilateral acoustic schwannomas are found in what disease?

A

NF-2

235
Q

“Chicken-wire capillary pattern” is a buzzword for what brain tumor?

A

Oligodendroglioma

236
Q

“Rosenthal fibers” are a buzzword for what brain tumor?

A

Pilocytic astrocytoma

237
Q

Which two brain tumors are GFAP positive?

A

Glioblastoma and Pilocytic astrocytoma

238
Q

“Homer-Wright rosettes” are a buzzword for what brain tumor?

A

Medulloblastoma

239
Q

What passes through the foramen magnum?

A

Spinal roots of CN XI, brain stem, vertebral arteries

240
Q

What passes through the foramen rotundum?

A

CN V2 (maxillary)

241
Q

What passes through the foramen ovale?

A

CN V3 (mandibular)

242
Q

What passes through the jugular foramen?

A

CNs IX, X, XI, jugular vein

243
Q

What passes through the cribriform plate?

A

CN I olfactory bundles

244
Q

What passes through the optic canal?

A

CN II, ophthalmic artery, central retinal vein

245
Q

What passes through the superior orbital fissure?

A

CNs III, IV, V1, VI, ophthalmic vein, sympathetic fibers

246
Q

What passes through the foramen spinosum?

A

Middle meningeal artery and vein

247
Q

What passes through the internal acoustic meatus?

A

CNs VII and VIII

248
Q

What passes through the hypoglossal canal?

A

CN XII

249
Q

What is pseudobulbar palsy?

A

Caused by neuro disorders such as MS; Dysarthria, dysphagia, dysphonia, and impaired movement of tongue and facial muscles

250
Q

What is Eaten-Lambert syndrome and what disease process is it associated with?

A

An autoimmune disorder of the NMJ seen primarily in patients with small cell lung cancer

251
Q

Conduction aphasia is a result of a lesion where?

A

Arcuate fasciculus (insula)

252
Q

Transcortical motor aphasia is due to a lesion where?

A

Near Broca area

253
Q

Transcortical sensory aphasia is due to a lesion where?

A

Near Wernicke area

254
Q

Which nerves are unmyelinated?

A

Group C nerve fibers: Autonomic postganglionic nerves, afferent neurons that conduct heat sensation, afferent neurons that transmit slow-onset dull, burning or visceral pain, and first order bipolar sensory neurons of olfaction

255
Q

What are the consequences of a left frontal lobe lesion?

A

Apathy and depression

256
Q

What are the consequences of a right frontal lobe lesion?

A

Disinhibited behavior

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