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Neurology Flashcards

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USMLE® Step 1 flashcards
1
Q

Neural tube development

A

1) Notochord induces ectoderm to differentiate into neuroectoderm and form neural plate
2) Neural plate gives rise to neural tube and neural crest cells
3) Notochord becomes nucleus pulposus of intervertbral disc

Alar plate- dorsal, sensory
Basal plate- ventral, motor

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2
Q

Regionalization of the brain

A

PRimary vesicles: prosencephalon (forebrain), mesencephalon (midbrain), rhombencephalon (hindbrain)

Secondary vescles
-prosencephalon – telencephalon (cerebral hemispheres and lateral venticles) and diencephalon (thalamus and third ventricle)
-mesencephalon – midbrain and cerebral aqueduct
rhombencephalon –metencephalon (pons and cerebellum) and myelencephalon (medulla); both contribute to fourth ventricle

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3
Q

Neuroectoderm derivatives

A

CNS neurons, epednymal cells that line the ventricles and make CSF, oligodendroglia, astrocytes

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4
Q

Neural crest derivatives

A

PNS neurons and schwann cells

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5
Q

mesoderm derivatives

A

microglia (hematopoietic lineage)

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6
Q

Testing for neural tube defects

A

Elevated AFP in maternal serum and amniotic fliud

Elevated AChE in amniotic fluid

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7
Q

Spina bifida occulta

A

Failure of bony spinal canal to close without any structural herniation and with dura intact. AFP is normal. Least severe neural tube defect.

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8
Q

Meningocele and Meningomyelocele

A

Meningocele: meninges only herniate through bony defect
Meningomyelocele: meninges and nural tissue hernate through bony defect; associated with chiari II malformation

both have risk of ascending infection
Look like plaquode on skin

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9
Q

Holoprosencephaly

A

Not a neural tube defect. Failure of left and right hemispheres to separate. Can be caused by sonic hedgehog mutations. Associated with Patau syndrome and FAS. Associated with cleft palate/lip, cyclopia

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10
Q

Chiari II

A

Hernation of cerebellar tonsils and vermis through foramen magnum. Aqueductal sternosis and hydrocephalus as result. Meningomyelocele (neural tube defect) is associatd

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11
Q

Dandy Walker malformation

A

Agenesis of cerebellar vermis with cystic enlargement of the fourth ventricle. Associated with hydrocephalus and spina bifida

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12
Q

Syringomyelia

A

Cystic cavity in spinal cord. Typicall damages corssing anterior spinal commisurral fibers leading to cape like loss of pain and temp in upper extremities. Associated with chiari I

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13
Q

Chiari I malformation

A

Cerebellar tonsils herniate. Associated with syringomyelia. Usually asymptomatic in childhood

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14
Q

Tongue development

A

1st and second branchial arches: anterior 2/3 with sensation from V3, taste from VII
3rd and 4th arches form posterior 1/3 with sensation and taste from IX and X
Motor from X (palatoglossus) and XII (all others)

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15
Q

Nissl stain

A

Stains RER and can be used to visualize cell bodies and dendrites of neurons. Doesn’t stain axon as RER is not present: proteins are synthesized in the cell body and transported down the axon

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16
Q

Wallerian degeneration

A

In response to axonal injury, the nerve degenerates distal to the injury and the axon retracts proximally, allowing for regeneration if in the PNS. Regeneration occurs slowly over weeks to months.

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17
Q

Astrocytes

A

Functions: physical support, repair, potassium metabolism, removal of excess neurotransmitter (take up glutamate and convert to glutamine), component of BBB, reactive gliosis in response to neuronal injury, match cerebral blood flow to synaptic activity

Marker: GFAP

Derivative of neuroectoderm

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18
Q

HIV in the brain

A

Infect microglia, which fuse to form multinucleated giant cells in the CNS

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19
Q

Scwann cells

A

Each Schwann cell myelinates 1 PNS axon. Promote axonal regeneration. Derived from neural crest

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20
Q

Oligodendroglia

A

Myelinate axons of CNS. Each oligodendrocyte can myelinate many axons. Derived from neuroectoderm. Fried egg appearance

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21
Q

Types of sensory receptors: free nerve endings, meissner corpuscles, pacinian corpuscles, merkel discs, ruffini corpuscles

A

Free nerve endings: Carry pain on C fibers, temp on Adelta fibers. Located in skin and seome viscera

Meissner corpuscles: Carry fine touch and position on large myelinated fibers that adapt quickly. Found on hairless skin

Pacinian corpuscles: Carry vibration and pressure on large myelinated fibiers that adapt quickly. Found in deep skin layers, ligaments, and joints.

Merkel discs: Carry pressure, deep touch, and position on large myelinated fibers that adapt slowly. Found in fingertips and superficial skin

Ruffini corpuscles: Carry pressure information on slow adapting dendritic endings within the capsule

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22
Q

Layers of a peripheral nerve

A

Endoneurium: surrounds single nerve fibers
Perineurium: surround fasicle of nerve fibers
Epineurium: dense connective tissue surrounding entire nerve

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23
Q

Location of neurotransmitter synthesis: NE, DA, 5-HT, ACh, GABA

A 
NE: locus ceruleus of the pons
Dopamine: ventral tegmentum and substantia niagra of midbrain
5-HT: Raphe nuclei in brainstem
ACh: Basal nucleus of Meynert
GABA: nucleus accumbens
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24
Q

Components of BBB

A
  1. Tight junctions between nonfenestrated capillary endothelial cells
  2. Basement membrane
  3. Astrocyte foot processes
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25
Q

OVLT

A

Area of hypothalamus not protected by BBB that senses changes in osmolarity.

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26
Q

Area postrema

A

Area of hypothalamus not protected by BBB that responds to emetics

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27
Q

Supraoptic nucleus

A

Part of hypothalamus that makes ADH

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28
Q

PAraventricular nucleus

A

Part of hypothalamus that makes oxytocin

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29
Q

Hypothalamus: lateral, ventromedial, anterior, posterior, suprachiasmic nucleus

A

Lateral area: hunger, inhibited by leptin
Ventromedial area: Satiety, stimulated by leptin
Anterior hypothalamus: Cooling, parasympathetic
Posterior hypothalamus: Heating, sympathetic
Suprachiasmic nucleus: Circadian rhythm

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30
Q

Circadan rhythm

A

Controlled by suprachiasmic nucleus. Drives nocturnal release of ACTH, prolactin, melatonin, norepinephrin.

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31
Q

Effect of CNS depressants on sleep

A

EtOH, benzos, and barbs decrease REM sleep and delta wave sleep

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32
Q

Pathology of different sleep stages

A

Non-REM stage N2: bruxism (teeth grinding)

Non-REM stage N3: sleepwalking, night terrors, bed wetting

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33
Q

Thalamic nuclei

A

VPL: Spinothalamic tract and dorsal column relay to sensory cortex

VPM: Trigeminal and taste relat to sensory cortex

LGNL Received vision info from CNII and sends to calcarine sulcus

MGN: Reveives auditory info from sup olive and inf collicular and sends to auditory cortex

VL: Reveives motor info from basal ganglia and cerebellum and relays to motor cortex

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34
Q

Limbic system

A

Includes hippocampus, amygdala, fornix, mamillary bodies, and cingulate gyrus

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35
Q

Central pontine myelinolysis

A

Result of overly rapidly correcting hyponatremia. Results in acute paralysis, LOC, locked in syndrome

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36
Q

Pathways through basal ganglia

A

Excitatory: Cortical inputs stimulate striatum; striatum stimulates release of GABA which disinhibits the thalamus via GPi/SNr

Inhibitors: Cortical inputs stimulate striatum, disinhibits STN via GPe, STN stimulates GPI/SNr to inhibit thalamus.

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37
Q

Dopamine receptors

A

D1: stimulates excitatory pathway
D2: inhibits inhibitory pathway
both increase motion

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38
Q

Hemiballismus

A

Decreased activity of subthalamic nucleus decreasing GPint activity, increasing thalamic activity and causing flailing. Sudden wild flailing of 1 arm and sometimes ipsilateral leg due to damage to contralateral subthalamic nucleus

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39
Q

Athetosis

A

Slow writhing movements of the fingers seen with lesions to basal ganglia, such as in Huntington

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40
Q

Treatment of essential tremor

A

beta blcokers

primidone

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41
Q

Myoclonus

A

Sudden, brief, uncontrolled muscle contraction seen iwth metabolic abnormalities

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42
Q

Pathology of PD

A

Lewy bodies of alpha synuclein which have intracellular eosinophilic inclusions. Loss of dopaminergic neurons of substantia niagra

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43
Q

Neurotransmitters in HD

A

DA increased
GABA decreased
ACh decreased

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44
Q

Broca aphasia

A

Nonfluent aphasia with intact comprehension, impaired repetition.

Broca area in inferior frontal gyrus of frontal lobe

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45
Q

Wernicke aphasia

A

Fluent aphasia with impaired comprehension and repetition.

Wernicke area is in superior temporal gyrus of termporal lobe

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46
Q

Conduction aphasia

A

Poor repetition but fluent speech and intact comprehension

Can be caused by damage to arcuate fasciculus

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47
Q

Global aphasia

A

Nonfluent aphasia with impaired comprehension

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48
Q

Transcortical motor aphasia

A

Nonfluent, good comprehension, intact repetition

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49
Q

Transcortical sensory aphasia

A

Poor comprehension, fluent speech, intact repetition

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50
Q

Mixed transcortical aphasia

A

Nonfluent speech, poor comprehension, intanct repetition

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51
Q

Kluver Bucy syndrome

A

Bilateral damage to amygdalae. Disinhibited behavior with hyperphagia, hypersexuality, hyperorality. Associated with HSV-1

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52
Q

Frontal lobe lesions

A

Cause disinhibition, problems with concentration, orietnation, judgement, possible reemergence of primitive reflexes

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53
Q

Parietal-temporal cortex lesions

A

Nondominant: hemispatial neglect syndrome

Dominant: Gerstmann syndrome of agraphia, acalculia, finger agnosia, left-right disorientation

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54
Q

Reticular activating system lesions

A

Reduced levels of arousal and wakefulness

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55
Q

Wernicke-Korsakoff syndrome

A

Pathology: affects mammillary bodies

Presentation: confusion, ophthalmoplegia, ataxia, memory loss, personality changes, confabulation

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56
Q

Basal ganglia lesions

A

Resting tremor, chorea, athetosis

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57
Q

Cerebellar lesions

A

Hemisphere: Intention tremor, limb ataxia, loss of balance. Ipsilateral defects; fall toward side of lesion

Vermis: Truncal ataxia, dysarthria

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58
Q

Hippocampal lesions

A

Anterograde amnesia

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59
Q

Defects to paramedian pontine reticular formation vs frontal eye fields

A

Paramedian pontine reticular formation: eyes look away from side of lesion

Frontal eye fields: eye look toward lesion

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60
Q

Regulation of cerebral perfusion

A

Driven by Pco2. Po2 modulates only in severe hypoxemia. Increased Pco2 increases cerebral perfusion pressure. Decreased O2

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61
Q

Therapeutic hyperventilation

A

Decreases Pco2 which helps to decrease intracranial pressure in acute cerebral edema via vasoconstrtion (brain thinks it has plenty of oxygen if CO2 is low). Hypventilation in panic attacks decreases perfusion leading to fainting.

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62
Q

Cerebral perfusion pressure

A

CPP = MAP - ICP

So, hypotension or incresaed intracranial pressure can decrease cerebral perfusion

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63
Q

Presentation of MCA stroke

A

Can get contralateral paralysis of upper limb and face due to effect on motor cortex

Can get contralateral sensory loss of uppper limb and face due to effect on sensory cortex

Can get aphasia or hemineglect due to effect on ermporal lobe and frontal lobe

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64
Q

Presentation of lenticulostriate artery stroke

A

Affects striatum and internal capsule. Contralateral hemiparesis/hemiplegia. Often lacunar infarct secondary to unmanaged hypertension

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65
Q

Presentation of anterior spinal artery stroke

A

Affects lateral corticospinal tract: contralateral hemiparesis of upper and lower limbs

Affects medial lemniscus: loss of contralateral proprioception

Affects caudal medulla: ipsilateral hypoglossal dysfunction with tongue deviating ipsilaterally

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66
Q

Presentation of PICA stroke

A

Affects lateral medulla: vomiting, vertigo, nystagmus due to cerebellar dysfunction and effect on vestibular nuclei. Decreased pain and temp sensation from ipsilateral face, contralateral body due to effect on lateral spinothalamic tract. Dysphagia and hoarseness due to effect on nucleus ambiguus. Ipsilateral horner syndrome due to effect on sympathetic fibers

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67
Q

Presentation of AICA stroke

A

Affects lateral pons including middle and inferior cerebellar peduncles. Presents with vomiting, vertigo, nystagmus, face paralysis, ataxia, dysmetria

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68
Q

Presentation of PCA stroke

A

Affects occipital cortex and visual cortex. Contralateral hemianopia with macular sparing

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69
Q

Presentation of basilar artery stroke

A

Locked in syndrome. Preserved consciousness and blinking with quadriplegia and loss of mouth, face, tongue movements

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70
Q

Presentation of anterior communicating artery stroke

A

Most often due to aneurysm. Presents with visual field defect

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71
Q

Presentation of posterior communicating artery stroke

A

Saccular aneurysms. CNIII palsy.

72
Q

Saccular aneurysms

A

Also called berry aneurysms. Occur at bifurcations of circle of willis, most commonly junction of ACA and ant comm a. Rupture leads to SAH or hemorrhagic stroke. May compress optic chiasm.

73
Q

Diseases associated with saccular aneurysms

A

ADPKD, Ehlers-danlos

74
Q

Charcot-Bouchard microaneurysm

A

Associated with chronic HTN. Affects small vessels of basal ganglia and thalamus

75
Q

Central post-stroke pain syndrome

A

Neuropathic pain due to thalamic lesions. Parasthesias followed by allodynia and dysesthesia

76
Q

Epidural hematoma

A

Caused by middle meningeal artery rupture secondary to fracture. Classic lucid interval followed by expansion and transtentorial herniation with CNIII palsy. Biconvex blood collection that doesn’t cross suture lines, can cross falx, tentorium

77
Q

Subdural hematoma

A

Caused by rupture of bridging veins. Slow venous bleeed over time. Seen in eldery, alcoholics, blunt trauma, shaken baby. Crescent shaped hemorrhage that crosses suture lines. Cannot cross tentorium

78
Q

SAH

A

Caused by rupture of an aneurysm or AVM. Rapid ime course. Worst headache of life. Bloody or yellow spinal tap. Risk of vasospasms 2-3 days afterward due to blood breadown and rebleed

79
Q

Intraparenchymal hemorrhage

A

Associate dwith systemic HTN or amyloid angiopathy, vascultitis, neoplasm. Basal ganglia and internal capsule are most common locations

80
Q

Ischemic brain diesease

A

irreversible damage 5 mins of hypoxia. Hippocampus, neocorex, and cerebellum most vulnerable.

Imaging: noncontrats CT to exclude hemorrhage. Will detect ischemic changes in 6-24 hours. Diffusion-weighted MRI can detect ischemia much sooner

81
Q

Histologic changes seen after ischemic stroke

A 
12-48 hrs: red neurons
24-72 hrs: necrosis and neutrophils
3-5 days: macrophages/microglia
1-2 weeks: reactive gliosis and vascular proliferation
over 2 wks: glial scar
82
Q

CSF flow

A

Made by ependymal cells of choroid plexus. Flows from lateral ventricles through R and L interventricular foramina of monroe into 3rd ventricle, through cerebral aqueduct to 4th ventricle, into subarachnoid space through foramina of luschka (lateral) and foramen of magendie (medial). Reabsorbed by arachnoid granulations. Drains into dural venous singuses

83
Q

Idiopathic intracranial HTN

A

Also called pseudotumor cerebri. Increased ICP without apparent cause on imaging. Presents with headaches and diplopia without mental status change. Papilledema present. Increased opening pressure on LP.

Risk factors: woman of childbearing age, vitamin A excess, danazol

Treatment: weight los, acetazolamide, topiramate

84
Q

Normal pressure hydrocephalus

A

Idiopathic condition seen in elderly. CSF pressure elevated episodically. Expansion of ventricles, distorting fibers of corona radiata. Wet, wobbly, and wacky: urinary incontinence, ataxia, cognitive dysfunction

85
Q

Ex vacuo ventriculomegaly

A

Appearance of increased CSF on imaging due to decreased brain tissue. Normal ICP

86
Q

Spinal nerve naming

A

C1-C7 exit above corresponding vertebra. C8 exits below C7. Rest exit below their corresponding vertebra

87
Q

Vertebral disc herniation

A

Nucleus pulposus herniates through annulus fibrosus.

88
Q

Lower extension of spinal cord

A

Adults: lower border of L1-L2 vertebra. Subarachnoid space to loer border of S2. LP performed between L3/L4 or L4/L5

89
Q

Medial lemniscus

A

Carries sensory fibers from the nucleus gracilis and cuneatus to LGN

90
Q

Poliomyelitis

A

Destruction of anterior horns. LMN lesions only (flaccid paralysis). Replicates in oropharynx and sm intestine; then spreads to CNS by blood stream

CSF: increased WBCs and slight increased protein

91
Q

Spinal muscular atrophy

A

congenital degeneration of anterior hormones. LMN lesions only (flaccid paralysis). Floppy baby with tongue fasciculations. AR inheritance

92
Q

Multiple sclerosis effect on spinal cord

A

Demyelination mostly of white matter in cervical region. Random and asymmetric lesions

93
Q

Riluzole

A

Glutamate antagonist used to treat ALS

94
Q

Tabes dorsalis

A

Caused by tertiary syphilis. Degeneration of dorsal columns and nerve roots resulting in impaired sensation and proprioception and poor coordination. Absent DTRs, pos romberg

95
Q

Vitamin B12 def - effect on spinal cord

A

Subacute combined degeneration: demyelination of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts resulting in ataxic gait, paresthesia, impaired pos and vibration sense

96
Q

Friedreich ataxia

A

AR GAA repeat on chromosome 9. Gene encodes iron binding protein frataxin. Leads to impairment of mitochondrial function.

Degeneration of spinal tracts resultingin weakness, loss of DTRs, vibratory sense, and proprioception.

Associated with diabetes and hypertrophic cardiomyopathy. Also kyphoscoliosis

97
Q

Brown Sequard syndrome

A

Hemisection of cord. If above T1 can cause Horner syndrome as well as spinal cord findings

98
Q

Key dermatomes: C2, C3, C4, T4, T7, T10, L1, L4, S2, S3, S4

A 
C2: posterior half of skull cap
C3: high turtleneck shirt
C4: low collar shirt
T4: nipple
T7: xiphoid process
T10: umbilicus
L1: inguinal ligament
L4: kneecap
S2, S3, S4: erection and sensation of penis and anus
99
Q

Pineal gland

A

Located above the superior colliculi. Responsible for melatonin secretion and circadian rhythms

100
Q

Supeiror and inferior colliculi

A

Superior: conjugate vertical gaze
Inferior: auditory function

101
Q

Locations of cranial nerve nuclei

A

Midbrain: III and IV
Pons: V, VI, VII, VIII
Medulla: IX, X, XII
Spinal cord: XI

Latearl nuclei are sensory, originating from alar plate
Medial nuclei are motor, originating from basal plate

102
Q

Optic canal: what travels through

A

CNII, ophthalmic artery, central venous vein

103
Q

Superior orbital fissure: what passes through

A

CNIII, IV, V1, VI (all nerve to eye except optic) + opthalmic vein and sympathetic fibers

104
Q

Foramen rotundum

A

Carries CN V2

105
Q

Foramen ovale

A

Carries CN V3

106
Q

Foramen spinosum

A

Holds middle meningeal artery

107
Q

Internal auditory meatus

A

Carries CN VII and VIII

108
Q

Jugular foramen

A

Carries CN IX, X, XI, jugular vein

109
Q

Hypoglossal canal

A

Carries CN XII

110
Q

Foramen magnum

A

Carries spinal roots of CN XI, brainstem, vertebral arteries

111
Q

Function of CN IV and XI

A

IV (trochlear) innervates superior oblique

VI (abducens) innervates lateral rectus

112
Q

Sensation of tongue

A

Somatosensory for anterior 2/3: CNV
Taste for anterior 2/3: CNVII
Taste and somatosensory for posterior 1/3: CNIX
Taste from epiglottic region: CNX

113
Q

Carotid and aoritc body monitoring

A

CNIX monitors carotid body and sinus chemo and baroreceptors

CNX monitors aortic arch chemo and baroreceptors

114
Q

Nucleus solitarius

A

Visceral sensory information for CNVII, IX, X - taste, baroreceptors, gut distention

115
Q

Nucleus ambiguus

A

Motor information for pharynx, larynx, upper esophagus. CNIX, X, XI

116
Q

Dorsal motor nucleus

A

Autonomic fibers of CNX to heart, lungs, upper GI tract

117
Q

Gag reflex

A

Afferent: IX
Efferent: X

118
Q

Cavernous sinus

A

Collection of venous sinuses on either side of pituitary. Blood from eye and superficial cortex drains into cavernous sinus which drains into internal jugular vein.

CN III, IV, V1, VI pass through to get to orbit

119
Q

Cavernous sinus syndrome

A

Ophthalmoplegia, decreased corneal sensation, Horner syndrome due to pituitary tumor mass effect, fistula, cavernous sinus thrombosis affecting nerves that pass through

120
Q

Tonotopy of basilar membrane

A

Low freq heard at apex which is wide and flexible

High freq heard at base of cochlea which is thin and rigid

121
Q

Test results for conductive vs senorineural hearing loss

A

Conductive hearing loss: Rinne test abnormal with bone greater than air. Weber test localizes to affected ear

Sensorineural hearing loss: Rinne test normal wiht air greater than bone conduction. weber localizes to unaffected ear

122
Q

Noise induced hearing loss

A

Damages hair cells. Leads to loss of high freq hearing loss first

123
Q

Cholesteatoma

A

Overgrowth of desquamated keratin debris within middle ear. May errode ossicles and mastoid air cells leading to conductive hearing loss

124
Q

UMN vs LMN lesion of face

A

UMN: causes contralateral paralysis of the face. upper face is spared because it gets bilateral UMN innervation

LMN: causes ipsilateral paralysis of upper and lower face

125
Q

Muscles of mastication

A

Masseter, temporalis, and medial pterygoid close the jaw

Lateral pterygoid opens

126
Q

Aqueous humor pathway

A

Produrced by ciliary epithelium. Collected by trabecular meshwork, then goes into canal of schlemm to drain

127
Q

Refractive errors: hyperopia, myopia, astigmatism, presbyopia

A

Hyperopia: eye too short; light focused behind retina
Myopia: eye too long; light focused in front of retina
Astigmatism: abnormal curvature of cornea
Presbyopia: age-related impaired acommodation due to decreased lens elasticity

128
Q

Glaucoma

A

Opitc disc atrophy with cupping. Elevated IOP, progressive peripheral vision loss

129
Q

Open angle glaucoma

A

Painless and more common. Associated with age, african america, famiy history. Can be secondary to blocked trabecular meshwork with WBCs, RBCs, retinal elements

130
Q

Tetrodotoxin

A

Toxin found in puffer fish. Binds to NA+ channels, inhibiting influx

131
Q

Morphine effect on ion channels

A

Binds to mu receptors and causes G protein coupled acivation of K+ channels to increase K+ efflux, hyperpolarizing post-synatpic neurons and terminating pain transmission

132
Q

Kinesin

A

MT associated motor protein that does anterograde transport of intracellular vesicles and organelles toward plus end of MTs.

133
Q

Valproate during pregnancy

A

Increases risk of neural tube defects because inhibits folic acid absorption

134
Q

Length constant vs time constant

A

Length constant: How far long an axon an impulse can propagate. Increased by myelin so that conduction occurs faster.

Time constant: How long it takes for a change in membrane potential to occur. Reduced by myelination so that conduction occurs faster.

135
Q

POMC

A

Polypeptide precursor that goes through enzymatic modification to produce beta endorphins, ACTH, and MSH

136
Q

Tetrahydrobiopterin

A

BH4. Cofactor in synthesis of serotonin, DOPA, and tyrosine

137
Q

CSF findings in MS

A

Increased protein in CSF - oligoclonal bands diagnostic

138
Q

Treatment of MS

A

Disease modifying therapies: beta-interferon, natalizumab

Acute flares: IV steroids

139
Q

Treatment of Guillan Barre

A

Respiratory support. Plasmapheresis. IVIG

140
Q

Acute disseminated encephalomyelitis

A

Multifocal periventricular inflammation and demyelination after infection or certain vaccinations. Commonly seen after measles or VZV

141
Q

Charcot-Marie-Tooth disease

A

Progressive hereditary nerve disorders due to defective protein production. AD inheritance. Associated with scoliosis and foot deformities

142
Q

Krabbe disease

A

AR lysosomal storage disease due to def of galactocerebrosidase. Causes galactocerebroside to build up and destroy myelin. Presents with peripheral neuropathy, developmental delay, optic atrophy, globoid cells

143
Q

Metachromatic leukodystrophy

A

AR lysosomal storage disease. Arylsulfatase A def. Central and peripheral demyelination with ataxia and dementia

144
Q

Progressive multifocal leukoencephalopathy

A

Demyelination of CNS due to oligodendrocyte destruction. Associated with JC virus - seen in AIDS patients. Radpily progressive and fatal.

145
Q

Adrenoleukodystrophy

A

X-linked genetic disorder. Disupted metabolism of VLCFA causing build up in NS, adrenal, testes.

146
Q

Positional testing for nystagmus

A

Delayed horizontal nystagmus suggests peripheral (inner ear) vertigo

Immediate nystagmus in any direction suggests central vertigo

147
Q

Sturge-Weber syndrome

A

Congenital anomaly of neural crest derivatives due to acivating GNAQ mutation. Affects capillaries leading to port-wine stain on face. Ipsilateral leptomeningeal angioma causes seizures. Intellectual disability. Episcleral hemangioma leads to elevated IOP and early onset glaucoma

148
Q

Tuberous sclerosis

A 
Hamartomas in CNS and skin.
Angiofibromas
Mitral regurg
Ash-leaf spots
Cardiac rhabdomyoma
mental retardation
renal angiomylolipoma
seizures
shagreen patches
Autosomal dominant inheritance
149
Q

Cancers seen with von hippel lindau

A

hemangioblastomas of retina, brain stem, cerebellum, and spine
angiomatosis
bilateral renal cell carcinoma
pheochromocytoma

150
Q

Glioblastoma multiforme pathology

A

Found in cerebral hemispheres and can cross the corpus callosum. Stains positive for GFAP. Pseudopalisading, pleomorphic tumors cells: tumor cells border areas of necrosis and hemorrhage

151
Q

Meningioma presentation and pathology

A

Presentation: Seizures or focal neurologic signs due to compression of brain tissue

Pathology: spindle cells in whorled paterns; psammoma bodies

152
Q

Hemangioblastoma

A

Associated with VHL syndrome. Usually cerebellar. Can make EPO leading to polycythemia.

153
Q

Schwannoma: location and tumor marker

A

Found at cerebellopontine angle. S-100 positive.

154
Q

Oligodendroglioma

A

Slow growing, usually in frontal lobes. Fried egg cell appearance. Often calcified

155
Q

Pilocytic astrocytoma

A

Well circumscribed posterior fossa tumor of childhood. GFAP +. Benign with good prognosis. Rosenthal fibers

156
Q

Medulloblastoma histology

A

Homer-Wright rosettes. Solid small blue cells

157
Q

Ependymoma

A

Ependymal cell tumor found in 4th ventcile. Can cause hydrocephalus. Poor pronosis. Perivascular rosettes on histology

158
Q

Craniopharyngioma

A

Benign tumor of childhood in sella turcica. Presents with bitemporal hemianopia. Derived from Rathke pouch remnants. Commonly calcified

159
Q

Cingulate herniation

A

Cingulate gyrus herniates under falx cerebri. Can compress ACA

160
Q

Transtentorial herniation

A

Can displace brain stem leading to duret hemorrhages; usually fatal

161
Q

Uncal hernation

A

Uncus herniates downward and compresses CNIII and PCA

162
Q

Cerebellar tonsilar herniation

A

Herniates into foramen magnum. Can compress brainstem resulting in coma and death

163
Q

MOA of opioids

A

Agonize opioid recetpors. Open K+ channels and close Ca++ channels to reduce synaptic transmission. Inhibit release of ACh, NE, 5-HT, glutamate, substance P

164
Q

Butorphanol

A

MOA: kappa opioid agonist, mu opioid partial agonist
Less resp depression than full opioid agonist
Can cause withdrawal in someone also taking full agonist due to competition at mu receptor

165
Q

Tramadol

A

MOA: weak opioid agonist; inhibits 5-HT and NE reuptake
Toxicity: decreased seizure threshold, serotonin syndrome

166
Q

Ethosuximide toxicity

A

Itching, stevens johnson syndrome

167
Q

Phenytoin: MOA, pharmacokinetics, side effects, use

A

MOA: Increases Na+ channel inactivation
Kinetics: 0 order kinetics
Side effects: gingival hyperplasia, P450 induction, stevens johnson
Use: First line for tonic-clonic seizures and prophylaxis of status epilepticus; can be used for everything except absence

168
Q

Carbamazepine: MOA, side effects, use

A

MOA: Increases Na+ channel inactivation
Side effects: agranulocytosis and aplastic anemia, teratogenic, P450 induction, SJS
Use: First line for partial seizures and tonic-clonic seizures, first line for trigeminal neuralgia

169
Q

Valproic acid: MOA, side effects, use

A

MOA: Increases Na+ channel inactivation, increases GABA
Side effects: hepatotoxicity, neural tube defects
Use: First line for tonic clonic seizures, effective against all seizure types. Myoclonic seizures, bipolar

170
Q

Gabapentin: MOA, side effects, use

A

MOA: inbhits VG Ca++ channels
Side effects: sedation and ataxia
Use: Partial seizures, peripheral neruopaty

171
Q

Phenobarbitol for seizures: MOA, side effects, use

A

MOA: Increases GABAa action
Side effects: sedation, tolerance and dependence, resp depression, P450 induction
Use: First line in neonatal seizures

172
Q

Topiramate: MOA, side effects, use

A

MOA: blocks Na+ channels, increases GABA
Side effects: Sedation, mental dulling
Use: Partial and tonic-clonic seizures. Migraine prophylaxis

173
Q

Lamotrigine

A

MOA: blocks VG Na+ channels
Side effects: SJS
Use: All seizure types, including absence

174
Q

Levetiracetam use

A

Partial and tonic-clonic seizures

175
Q

Tiagabine MOA and use

A

Inhibits GABA reuptake. Used for partial seizures

176
Q

Vigabatrin

A

Increases GABA. Used for partial seizures

USMLE (17 decks)

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