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0
Q

What are the primary vesicles?

A

1) Prosencephalon (forebrain)
2) Mesencephalon (midbrain)
3) Rhombencephalon (hindbrain)

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1
Q

How are the alar & basal plates organized?

A

In closed spinal cord, alar plate (sensory) is dorsal & basal plate (motor) is ventral.

In the medulla & pons, the basal plate is medial & the alar plate is lateral. Within these, the visceral nuclei are generally closest to the sulcus limitans.

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2
Q

What are the secondary vesicles?

A

1) Telencephalon
2) Diencephalon
3) Mesencephalon
4) Metencephalon
5) Myelencephalon

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3
Q

What does the telencephalon give rise to?

A

Cerebral hemispheres & lateral ventricles

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4
Q

What does the diencephalon give rise to?

A

Thalamus
Hypothalamus
Retina
Third ventricle

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5
Q

What does the mesencephalon give rise to?

A

Midbrain & cerebral aqueduct

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6
Q

What does the metencephalon give rise to?

A

Pons
Cerebellum
Top half of 4th ventricle

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7
Q

What does the myelencephalon give rise to?

A

Medulla

Bottom half of fourth ventricle

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8
Q

When does the neural tube develop?

A

Day 18-day 21

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9
Q

What is seen in the amniotic fluid with a neural tube defect?

A

^AFP

^AChE

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10
Q

What are the caudal neural tube defects?

A

Spina bifida occulta –> bony canal not closed, tuft of hair

Meningocele –> meninges herniate through spinal canal defect

Meningomyelocele –> meninges & spinal cord

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11
Q

What is seen with anencephaly

A

^AFP
Polyhydramnios (no swallowing center in brain)
No forebrain, open calvarium
“Frog-like appearance”

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12
Q

What causes anencephaly?

A

Maternal T1DM
Lack of folate

Anterior neural tube does not close

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13
Q

What causes holoprosencephaly?

A

Failure of R & L hemispheres to separate

May be due to sonic the hedgehog defect

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14
Q

What is seen with holoprosencephaly?

A

Cleft lip/palate

Severe form –> cyclopia

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15
Q

What is seen with congenital cerebral aqueduct stenosis?

A

Enlargement of lateral & third ventricles

Enlarging head circumference (sutures not yet fused)

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16
Q

What is seen with Arnold-Chiari malformation?

A

Type I - modest herniation, usually clinically silent

Type II:
Tonsillar herniation
Hydrocephalus
Meningomyelocele
Syringomelia
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17
Q

What is seen with Dandy-Walker malformation?

A

Agenesis of cerebellar vermis –> cystically enlarged 4th ventricle
The cerebellum is basically missing.
Hydrocephalus & spina bifida can be seen

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18
Q

Where is the most common site of a syringomyelia?

A

C8-T1

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19
Q

What is seen with a syringomyelia?

A

Bilateral loss of pain & temperature sensation in upper extremities
“Cape-like”
Later stages –> LMN signs of arms; Horner’s syndrome
Can extend downward
Fine touch & proprioception are spared

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20
Q

What causes syringomyelia?

A

Trauma
Chiari I malformation

Scoliosis is a hint that syringomyelia may be present

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21
Q

What is the sensory innervation of the tongue?

A

Anterior 2/3:
Sensation - V3
Taste - VII

Posteior 1/3:
Sensation & taste - IX or X (extreme posterior is X)

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22
Q

What neural cells arise from the neuroectoderm?
Neural crest?
Mesoderm?

A 
Neuroectoderm:
CNS neurons
Ependymal cells
Oligodendrocytes
Astrocytes

Neural crest:
PNS neurons
Schwann cells

Mesoderm:
Microglia

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23
Q

How can one stain for the cell bodies of neurons?

A

Stain for Nissl substance (RER). Only present in the cell body.

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24
What do Meissner's corpuscles sense?
Dynamic touch Fine touch Position sense Found on hairless (glabrous) skin
25
What do Pacinian corpuscles sense?
Vibration | Pressure
26
What do Merkel's discs sense?
Pressure | Deep static touch
27
What are the layers surrounding a PNS nerve?
Epineurium surrounds whole nerve Perineurium surrounds fascicle of nerve fibers Endoneurium surrounds individual fibers
28
Where is NE synthesized?
Locus ceruleus | pons
29
Where is dopamine produced in the brain?
``` Ventral tegmentum (VTA) Substantia nigra (pars compacta) ```
30
Where is serotonin produced in the brain?
Raphe nucleus (pons)
31
Where is ACh produced in the brain?
Basal nucleus of Meynert
32
Where is GABA produced in the brain?
Nucleus accumbens
33
What is the BBB composed of?
1) Tight junctions between endothelial cells 2) Basement membrane 3) Astrocyte foot processes
34
What are the circumventricular organs?
Area postrema - chemo induced vomiting OVLT - osmolarity sensing Neurohypophysis Median eminence of hypothalamus They have fenestrated capillaries to circumvent the BBB
35
Where is ADH produced?
Supraoptic nucleus | Hypothalamus
36
Where is Oxytocin produced?
Paraventricular nucleus | Hypothalamus
37
Where does Leptin act in the brain?
``` Acts at the hypothalamus: Lateral area (hunger) --> inhibited by leptin --> satiety Ventromedial area (satiety) --> stimulated by leptin --> satiety ``` Destruction of the Lateral area --> anorexia Destruction of the Ventromedial area --> hyperphagia
38
What parts of the hypothalamus control body temperature?
Anterior hypothalamus (parasympathetic) --> cooling Posterior hypothalamus (sympathetic) --> heating
39
What in the CNS controls sleep/wake cycles?
Suprachiasmatic nucleus | hypothalamus
40
What is secreted by the neurohypophysis?
Oxytocin & Vasopressin
41
``` What are the inputs & outputs of these Thalamic nuclei? VPL VPM LGN MGN VL ```
VPL - STT & DCP from body --> sensory cortex VPM - Trigeminal & gustatory pathway --> sensory cortex LGN - CNII (SLO AIM) --> calcarine sulcus MGN - Hearing (SLO AIM) --> Auditory cortex VL - Motor input from basal ganglia --> motor cortex
42
What structures make up the limbic system?
``` Hippocampus Amygdala Fornix Mamillary bodies Cingulate gyrus ```
43
What does the cerebellum control?
Ipsilateral coordination of movement. It generally makes sure that the movement you get is the one that you want.
44
What are the inputs & outputs of the cerebellum?
Inputs: Contralateral cortex via middle cerebellar peduncle Ipsilateral proprioceptive input via inferior cerebellar peduncle Outputs: Purkinje fibers --> deep nuclei of cerebellum --> contralateral cerebral cortex via superior cerebellar peduncle
45
What are the deep nuclei of the cerebellum?
Medial-->Lateral "Fat Guys Eat Donuts" Fastigial Globose Emboliform Dentate
46
What is the homunculus of the cerebellum like?
Medial - balance & truncal coordination Lateral - voluntary movement of extremities (ipsilateral) Floculus/Nodulus - Truncal balance & vestibulo-ocular reflexes
47
What are climbing & mossy fibers?
They are inputs to the cerebellum. Climbing fibers originate in the inferior olivary nucleus, while mossy fibers carry everything else.
48
What dopamine receptors correspond to the direct & indirect pathway of the basal ganglia? What are the effects of each pathway?
``` D1 = D1rect (D2 = indirect) ``` ``` INdirect = INhibitory (Direct = excitatory) ``` Dopamine normally stimulates the excitatory pathway & inhibits the inhibitory pathway --> ^movement
49
What is the direct basal ganglia pathway?
SN activate Putamen (D1 receptor) --> Putamen inhibits Globus Pallidus interna --> releases thalamus from GPi inhibition --> ^movement
50
What is seen histologically with Parkinson's disease?
``` Lewy bodies (intracellular, round, pink; alpha-synuclein) Loss of Substantia Nigra pars compacta ```
51
What is seen clinically with Parkinson's disease?
``` Your body becomes a TRAP: Tremor (resting; pill-rolling tremor) Rigidity (cogwheel) Akinesia (slowed voluntary movement; expressionless face) Postural instability --> shuffling gait ``` Dementia seen late in disease process.
52
What is the striatum composed of?
Putamen + Caudate
53
What are the lentiform nuclei?
Putamen + Globus pallidus
54
What causes Huntington's disease?
Autosomal dominant expansion (during spermatogenesis) of CAG repeats on chrom. 4 "Hunting 4 CAGs" Caudate loses ACh & GABA (CAG) --> Caudate atrophy (Neuronal death occurs via glutamate toxicity) (Direct basal ganglia pathway predominates)
55
What is seen clinically with Huntington's disease?
Choreoathetosis Aggression Depression (suicide) Dementia
56
What causes Hemiballismus?
Contralateral subthalamic nuclei lesion (no indirect basal ganglia pathway) Either flailing or rotatory movement of 1 arm & 1 leg (both same side)
57
What is myoclonus? | What is it seen in?
Jerks; hiccups Often seen in matabolic diseases (liver failure, renal failure, etc.)
58
What is seen with essential tremor? | What is the treatment?
Active tremor Exacerbated by holding a position Genetic predisposition Pts often self medicate with EtOH. Treatment is propranolol.
59
What causes intention tremor?
Intention tremor is a slow zigzag movement when extending arm toward a target. Seen in cerebellar dysfunction.
60
What is the organization of the homunculus?
Feet are medial | Head is lateral
61
What causes Kluver-Bucy syndrome? | What is seen?
Bilateral amygdala destruction. Can be associated with HSV-1. Symptoms: Hyperorality Hypersexuality Disinhibition
62
What is seen with frontal lobe lesions?
Disinhibition Concentration deficits Poor judgement Reemergence of primitive reflexes
63
What is seen with lesions in the right parietal lobe?
Contralateral hemineglect
64
What is seen with a lesion to the reticular activating system?
Reduced levels of arousal (coma) It is located in the midbrain.
65
What side do cerebellar defects cause a fall towards?
Patients fall toward the side of their lesion (ipsilateral defects)
66
What is seen with a lesion to the paramedian pontine reticular formation?
PPRF lesion --> eyes look away from side of lesion
67
What is seen with a lesion in the frontal eye fields?
Eyes look toward lesion
68
Where is Broca's area? | Wernicke's area?
Broca's - inferior frontal gyrus Wernicke's - Superior temporal gyrus
69
What is seen with conduction aphasia? | What causes it?
Fluent speech & intact comprehension but impaired repetition Caused by a lesion to the arcuate fasciculus (connects Broca's & Wernicke's)
70
What is the brain autoregulatory range for CO2? | O2?
CO2 --> ^ in perfusion up to 90 mmHg of CO2 O2 --> ^ in perfusion if PO2 < 50 mmHg Thus normally CO2 drives cerebral autoregulation. Hyperventilation can decrease ICP.
71
What is seen with a MCA infarction?
Contralateral paralysis & loss of sensation of upper limb & face Aphasia if in left (dominant) hemisphere Hemineglect if in right (nondominant) hemisphere
72
What is seen with an ACA infarction?
Contralateral paralysis & loss of sensation in leg
73
What can be caused by lacunar strokes? | What arteries are involved?
Lenticulostriate arteries are typically involved --> Striatum, Thalamus, or Internal capsule (deep structures) Internal capsule --> pure motor stroke Thalamus -- pure sensory stroke
74
What causes lacunar infarcts?
Unmanaged HTN --> hyaline arteriolosclerosis (of lenticulostriate vessels) --> lacunar infarcts (little hollow "lakes")
75
What can cause Medial Medullary Syndrome? | What is affected?
Anterior spinal artery occlusion or paramedian branches Affected: Lateral CST --> contralateral hemiparesis Medial lemniscus --> contralateral proprioceptive defect Hypoglossal nerve --> Lick your wounds
76
What can cause Lateral Medullary Syndrome? | What is affected?
Lateral Medullary = Wallenberg = PICA syndrome Affects: Nucleus ambiguus --> dysphagia & hoarseness (PICA specific) Vestibular nuclei --> vertigo & vomiting STT & Trigeminal nuc. --> no pain/temp to limbs/face Sympathetic fibers --> ipsilateral Horner's Inferior cerebellar peduncle --> ataxia
77
What is seen with AICA occlusion?
Paralysis of face is specific to AICA "Facial droop means AICA's pooped"
78
What can PCA occlusion cause?
Contralateral hemianopsia with macular sparing. If bilateral --> cortical blindness (pupillary light reflex intact)
79
What can be seen clinically with Saccular aneurysms?
Berry aneurysms: Rupture --> subarachnoid hemorrhage Bitemporal hemianopia (compression of optic chiasm)
80
What are the risk factors for saccular aneurysms?
Berry aneurysms: ADPKD Ehlers-Danlos Marfan's Age HTN Smoking Blacks
81
Where is the most common site of a berry aneurysm?
Anterior communicating artery branch points
82
Where are Charcot-Bouchard microaneurysms found?
The lenticulostriate arteries They are due to HTN and can cause intracerebral hemorrhage.
83
What arteries arise from the vertebral arteries?
Anterior spinal artery Posterior spinal arteries PICA
84
What is the shape of an epidural hematoma? | Subdural hematoma?
Epidural - biconvex (lens) shape, crosses falx/tent but not sutures Subdural - crescent shape, crosses sutures but not falx/tent
85
What is seen in epidural hematoma?
``` Temporal bone fracture --> ruptured middle meningeal artery Lucid interval CNIII palsy (transtentorial herniation) ```
86
Who is at risk for subdural hematoma?
Neonates (shaken-baby) Elderly (brain atrophy) Often caused by whiplash-like injury
87
What is seen with subarachnoid hemorrhage?
WHOML Xanthochromatic (yellow) spinal tap Risk of vasospasm 2 days afterward (Tx: Nimodipine)
88
What is the timeline of histology following a stroke?
``` 12h - Red neurons appear 24h - PMN's 3 days - Macs 1 week - Gliosis 2 weeks+ - Glial scar ```
89
What is the treatment for an ischemic stroke?
tPA within 4.5h if no risk of hemorrhage
90
What is a TIA?
Focal neurologic dysfunction lasting < 24h | No acute infarction seen on MRI
91
What is the path of CSF?
Ependymal cells of lateral ventricles --> Interventricular foramina of Monroe --> 3rd ventricle --> Cerebral aqueduct --> 4th ventricle --> Foramina of Luschka & Foramen of Magendie --> subarachnoid space --> Arachnoid granulations
92
What is seen with normal pressure hydrocephalus?
``` Urinary incontinence (wet) Ataxia (wobbly) Cognitive dysfunction (wacky) ``` Results from overproduction of CSF. Treatment is a VP shunt.
93
What can cause hydrocephalus ex vacuo?
Alzheimer's Advanced HIV Pick's disease Any condition with brain atrophy.
94
How many spinal nerves are there & from what divisions?
``` 8 cervical 12 thoracic 5 lumbar 5 sacral 1 coccygeal ``` 31 total
95
Where do spinal nerves exit?
C1-C7 exit above the corresponding vertebrae | All others exit below the corresponding vertebrae
96
Where does disc herniation occur most commonly? | What is breached?
Occurs most commonly at L4-L5 or L5-S1 Nucleus pulposus herniates through anulus fibrosis
97
Where does the spinal cord end? The Subarachnoid space? Where should LP be performed?
Cord ends at L2 Subarachnoid ends at S2 LP between L3-L4 or L4-L5 "L3-L5 keeps the spine alive"
98
What comprises the DCP in the spinal cord?
Fasciculus gracilis (legs; medial) Fasciculus cuneatus (arms; lateral)
99
Describe the dorsal column pathway
1st order: Cell body in DRG, enters cord & ascends in ipsilateral dorsal column, synapse in nucleus cuneatus/gracilus (medulla) 2nd order: Decussates in medulla, ascends in contralateral medial lemniscus, synapses on VPL (thalamus) 3rd order: Goes from VPL to sensory cortex
100
Describe the spinothalamic tract
1st order: Sensory nerve ending has cell body in DRG, enters cord & synapses on ipsilateral nucleus proprius 2nd order: Decussates at anterior commissure (at level where it entered) & ascends contralaterally. Synapses on VPL 3rd order: VPL --> sensory cortex
101
What do the anterior & lateral spinothalamic tracts do?
Lateral - pain & temperature Anterior - crude touch & pressure
102
Describe the lateral corticospinal tract
1st order: UMN cell body in primary motor cortex. Descends ipsilateral through internal capsule & decussates at pyramidal decussation (caudal medulla). Then descends contralaterally & synapses on cell body of anterior horn. 2nd order: LMN leaves spinal cord & synapses on neuromuscular junction
103
What are the classical deficits in MS?
SIIIN ``` Scanning speech Intention tremor Internuclear ophthalmoplegia Incontinence Nystagmus ```
104
What tracts are affected by ALS?
Lateral CST --> UMN signs Anterior horns --> LMN signs Both are seen, with no sensory, cognitive, or oculomotor deficits.
105
What causes familial ALS?
Mutation in superoxide dismutase 1 --> free radical injury of neurons
106
What is seen with ALS?
40-70y males Atrophy & hand weakness is often early sign (motor only) Mixed UMN & LMN signs No sensory, cognitive, oculomotor defects (Stephen Hawking)
107
What is the treatment for ALS?
Riluzole --> inhibits presynaptic glutamate release | "Give Lou Riluzole"
108
Where is the watershed area for the anterior spinal artery?
Upper thoracic area Below T8, artery of Adamkiewicz supplies ASA
109
What is seen with Anterior Spinal Artery syndrome?
The most common result of spinal ischemia. Back pain at the level of the lesion Loss of sensory motor function (LMN shock then eventual UMN) Preservation of proprioception & vibratory sense
110
What tracts are affected by Tabes dorsalis? | What is seen?
Demyelination of dorsal roots & DCP ``` Symptoms: Impaired sensation & proprioception Progressive sensory ataxia Charcot's joints (injuries go unnoticed --> ulcers) Radicular pain Argyll-Robertson pupils (accommodation but no light reflex) Absent DTR's Positive Romberg ```
111
What tracts are affected by B12 deficiency?
Subacute combined degeneration = demyelination of: Dorsal columns Laeral CST Spinocerebellar tracts Combined = ascending & descending tracts affected
112
What is seen in subacute combined degeneration?
``` Poor proprioception (DCP) Spastic paresis (CST) Gait ataxia (spinocerebellar) ```
113
How is Werdnig-Hoffman disease inherited? | What is seen?
Autosomal recessive Congenital degeneration of anterior horn cells Hypotonia (floppy baby) Tongue fasciculations Prognosis = 7mo
114
What causes Friedreich's Ataxia?
Autosomal recessive expansion of GAA in frataxin gene --> impaired mitochondrial iron regulation --> free radical damage
115
What is seen in Friedreich's Ataxia?
``` Degeneration of cerebellum & multiple cord tracts Death by hypertrophic cardiomyopathy Childhood kyphoscoliosis (both coronal & saggital plane) Ataxia Frequent falls Nystagmus Dysarthria Pes cavus Hammer toes ``` "Friedreich is fratastic, slurring & falling, but he's got a big heart.
116
What causes Brown-Sequard syndrome? | What is seen?
Hemisection of the spinal cord At level of lesion - Ipsilateral LMN signs & loss of all sensation Below lesion: Ipsilateral UMN signs (CST) & loss of proprioception & vibration sense (DCP) Contralateral loss of pain & temperature (STT)
117
What level must a spinal cord lesion be to cause Horner's syndrome?
Above T1
118
``` What do are the dermatomes of these structures? Kneecaps Penis Umbilicus Nipples Arms Posterior head ```
``` Kneecaps - L4 Penis - S2,3,4 keeps the penis off the floow Umbilicus - T10 at the belly butTEN Nipples - T4 (teat pore) Arms - C5,6,7,8,T1 Posterior head - C2 ```
119
What are the clinical DTR's & their nerve roots?
S1,2 - achilles L3,4 - patella C5,6 - biceps C7,8 - triceps
120
What are the primitive reflexes?
Moro reflex - flail out limbs when scared (hold on for dear life) Rooting - turn when stroke cheek Sucking - suckling when roof of mouth is touched Palmar - fingers curl when palm is touched Plantar - Babinsky sign (not considered as such when infant) Galant - lying face down, stroke one side of spine, flexes toward
121
What is Parinaud syndrome? | What causes it?
Parinaud syndrome is paralysis of conjugate upward vertical gaze due to a lesion in the superior colliculi (often pinealoma, MS, stroke). Downward gaze is normally preserved.
122
What nucleus controls the pupillary sphincter & ciliary muscle?
Edinger-Westphal nucleus
123
What is mediated by the trigeminal nerve?
Muscles of mastication | Sensation of the face & anterior 2/3 of tongue
124
What is mediated by the facial nerve (CNVII)?
``` Facial movement Taste from anterior 2/3 of tongue Lacrimation Salivation (sublingual & submandibular) Stapedius muscle in ear Orbicularis oculi ```
125
What is mediated by the glossopharyngeal nerve (CNIX)?
``` Taste & sensation from posterior 1/3 of tongue Swallowing Salivation (parotid) Carotid body & sinus Stylopharyngeus ```
126
Where are the CN nuclei located?
Midbrain = 3-4 Pons = 5-8 Medulla = 9,10,12 Spinal cord = 11
127
What cranial nerves facilitate the gag reflex?
In on IX | Out on X
128
Describe the path of the carotid sinus & aortic arch baroreceptors
Carotid sinus --> Hering's nerve --> CNIX --> Nucleus solitarius Aortic arch baroreceptors --> CNX --> Nucleus solitarious
129
What is nucleus solitarius for? | What are its cranial nerves?
Visceral sensory information | CNVII, CNIX, CNX
130
What is nucleus ambiguus for? | What are its cranial nerves?
Motor innervation of pharynx, larynx, upper esophagus | CNIX, CNX
131
What is the dorsal motor nucleus for? | What are its cranial nerves?
Parasympathetics to heart, lungs, upper GI | CNX
132
What passes through the cavernous sinus?
``` EOM nerves (III, IV, VI) and V1, V2 Portion of internal carotid ```
133
What is seen in cavernous sinus syndrome? | What causes it?
Ophthalmoplegia (CNIII,IV,VI run through it) Impaired corneal (V1) & maxillary sensation (V2) Normal vision It can be caused by: Neoplasm Internal carotid aneurysm or fistula
134
Which way will the jaw deviate from a CNV lesion?
Toward the defect, due to unopposed pterygoids
135
Which way does the uvula deviate with a CNX lesion?
Away from the side of the lesion
136
Which way will there be weakness in head turning with a CNXI lesion?
Contralateral head turn will be weak (SCM aids in opposite head turn). Trapezius will droop on same side of lesion.
137
What is damaged in noise-induced hearing loss?
Stereociliated cells within the organ of corti are damaged. High frequencies are lost first.
138
What is seen with an UMN facial lesion? | LMN facial lesion?
UMN (cortex-facial nucleus) --> contralateral lower face paralysis LMN (facial nucleus-muscle) --> ipsilateral whole face paralysis
139
What is Bell's Palsy seen in?
``` AIDS Lyme disease HSV Sarcoisosis Tumors Diabetes ```
140
What muscles open the mouth? | Close it?
Open - Lateral pterygoid ("Lateral lowers") Close - Masseter, temporalis, medial pterygoid
141
What is the clinical term for nearsighted? | Farsighted?
Nearsighted = you can see near only = myopia Farsighted = you can only see far = hyperopia
142
What occurs within the eyeball during accomodation?
Ciliary muscles contract --> zonular fibers relax --> lens becomes fatter --> you can see close
143
What is uveitis? | What conditions is it seen with?
Inflammation of iris, ciliary body, & choroid (all anterior chamber) Associated with chronic inflammatory conditions: Sarcoidosis RA HLA-B27 conditions
144
What causes retinitis?
Usually viral (CMV, HSV, HZV) Associated with immunosuppression
145
What is the pathway of aqueous humor in the eye?
Produced by ciliary epithelium (ciliary body) --> around lens --> through pupil into anterior chamber --> trabecular meshwork --> Canal of Schlemm
146
What type of receptor dilates the pupil? | Constricts?
A1 dilates | M3 constricts
147
What are the causes of open angle glaucoma? | What are the risk factors?
Caused by blockage of trabecular meshwork: WBC's (uveitis) RBC's (hemorrhage/trauma) Retinopathy Risk factors: Age, Black race, family history
148
What are the symptoms of chronic glaucoma?
Painless Loss of peripheral vision Cupping on optic exam
149
What causes closed angle glaucoma? | What is seen?
Lens moves forward & blocks pupil --> aqueous humor builds up behind iris --> pushes iris forward which blocks trabecular meshwork ``` Symptoms: Ophthalmic emergency Pain Rock hard eye Sudden vision loss Halos Frontal headach ```
150
What type of drugs are used to treat glaucoma?
Cholinomimetics You want to contract the pupil (closed angle) & contract the ciliary muscle (open angle).
151
What are the risk factors for cataracts?
``` Age Galactokinase deficiency or classic galactosemia Diabetes Corticosteroid use Smoking EtOH Excessive sunlight Trauma Infection ```
152
What is seen with CNIII damage?
Eye looks down & out Ptosis Dilated pupil
153
What is seen with CNIV damage?
Eye moves upward (especially w/ contralateral gaze) | Problems while walking down the stairs
154
What is the pathway to cause mydriasis?
1) Hypothalamus --> Ciliospinal center of Budge (C8-T1) 2) Exit at T1 --> Superior cervical ganglion 3) Plexus along internal carotid --> through cavernous sinus (with V1) --> long ciliary nerve in the orbit --> pupillary dilator muscles
155
What is the pathway to cause miosis?
Edinger-Westphal nucleus --> Ciliary ganglion (via CNIII) --> Short ciliary nerves --> pupillary sphincter muscles
156
What is the pathway for the pupillary light reflex?
CNII --> Pretectal nucleus --> Bilateral Edinger-Westphal nuclei --> CNII --> constriction
157
What is a Marcus Gunn pupil?
Relative Afferent Pupillary Defect By using the swinging light test, you will see that when the light is moved to the impaired eye, it will dilate some rather than re-constrict as is seen in a normal eye. Caused by damage to the retina or early optic tract.
158
What is seen with vascular vs compression damage to CNIII?
Vascular impairment --> O2 can't diffuse to inner motor components --> Ptosis, down & out gaze Compression --> peripheral parasympathetic fibers affected first --> blown pupil
159
What is seen with retinal detachment? | What are the risk factors?
Retina detaches from choroid --> retinal ischemia --> sudden monocular loss of vision ("like a curtain drawn down") Risk factors: Diabetes Myopia Inflammation
160
What causes age-related macular degeneration?
Dry (nonexudative; 85%) - deposition of yellow material beneath pigmented epithelium --> gradual loss in central vision (scotoma) Wet (exudative; 15%) - choroidal neovascularization --> bleeding --> rapid loss of vision
161
How is age-related macular degeneration treated?
Nonexudative - multivitamin & antioxidants Exudative - anti-VEGF injections or laser
162
What genes are associated with Alzheimer's?
Early onset: Amyloid precursor protein (APP; chrom 21) Presenilin-1, Presenilin-2 Late onset: ApoE4 Protective: ApoE2
163
What is seen histologically with Alzheimer's? | Grossly?
Senile/Neuritic plaques - Extracellular beta-amyloid core with surrounding neuritic processes Neurofibrillary tangles - Intracellular aggregations of hyperphosphorylated Tau protein (MT-associated protein) Amyloid angiopathy may be present Gross: Widespread cortical atrophy (narrow gyri, wide sulci) Hydrocephalus ex vacuo
164
What is seen clinically in Pick's disease?
Change in personality Aphasia Parkinsonian aspects Dementia
165
What is seen histologically with Pick's disease? | Grossly?
Intracellular Pick bodies - ROUND aggregates of tau protein Grossly: Frontotemporal atrophy Spares parietal lobe & posterior 2/3 of superior temporal gyrus
166
What causes Lewy body dementia? | What is seen clinically with Lewy body dementia?
Defect in alpha-synuclein --> Lewy body deposition Parkinsonism Dementia Hallucinations
167
What causes Creutzfeld-Jakob disease?
Sporadic, inherited, or transmitted misfolding of PrPc --> PrPsc Assumes a beta-pleated sheet form --> converts more copies of itself to altered form --> vacuole formation
168
What is seen clinically with CJD?
Creutzfeldt-Jakob disease ``` Rapidly progressive dementia Startle myoclonus Ataxia Spike wave complexes on EEG Death in <1y ```
169
What is seen with familial fatal insomnia?
A type of inherited spongiform encephalopathy Insomnia Startle myoclonus
170
Neck flexion --> shocklike sensation down spine & into extremities What is it called? What can cause it?
Lhermitte's sign Caused by MS
171
What are the findings on labs with MS?
Oligoclonal IgG bands in the CSF | Periventricular plaques on MRI (gold standard)
172
Who is affected by MS?
White women in their 20's-30's with HLA-DR2 far from the equator
173
What is the treatment for MS?
Long term: IFN-beta Natalizumab (prevents immune cells from penetrating the CNS) Acute --> Corticosteroids
174
What infections are associated with Guillan-Barre syndrome?
Campylobacter jejuni CMV Leads to autoimmune attack on Schwann cells due to molecular mimicry.
175
What is seen in Guillan-Barre syndrome?
Ascending paralysis Facial paralysis in 50% Autonomic dysfunction
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What is the treatment for Guillan-Barre?
Respiratory support Plasmapheresis IVIg
177
What is the gold standard in diagnosing Guillan-Barre?
Increased CSF protein with normal cell count | albuminocytologic dissociation
178
What causes Progressive Multifocal Leukoencephalopathy?
AIDS --> Reactivation of JC virus in oligodendrocytes --> central demyelination --> rapid progression & death
179
What is the cause of Charcot-Marie-Tooth disease? | What is seen?
aka Hereditary Motor & Sensory Neuropathy Various inherited etiologies affecting peripheral nerves or Schwann cells Symptoms: Leg weakness/atrophy High arches & hammertoes
180
What is seen with partial temporal seizures?
Most common point of origination: Funny feeling in stomach rising up Weird smells
181
What is seen with frontal partial seizures?
They occur at night
182
What is the difference between simple partial & complex partial seizures?
Complex = loss of consciousness
183
What is seen with juvenile myoclonic epilepsy?
Myoclonic seizures shortly after arising in the morning Seen in teens Precipitates by lack of sleep & EtOH (college lyfeee)
184
What is seen with absence seizures? | What is the treatment?
EEG = 3 Hz Ages 4-10 No postictal state Tx: Ethosuximide
185
What are the types of generalized seizures?
Absence (petit mal) Myclonic - quick, repetitive jerks Tonic-clonic (grand mal) - alternating stiffening & movement Tonic - stiffening Atonic - "drop seizures" often mistaken for fainting
186
What is the treatment for myoclonic seizures?
Valproic acid
187
What is status epilepticus?
Continuous seizure for >30 mins or recurrent seizures for >30 mins without regaining consciousness
188
What is seen with a patient's eyes in a seizure?
Patients look away from a seizure & into a stroke
189
What causes peripheral vertigo?
Inner ear pathology: Semicircular canal debris (BPPV) Vestibular nerve infection Ménière's disease Positional testing --> delayed horizontal nystagmus
190
What causes central vertigo? | What is seen?
Brainstem or cerebellar lesion --> central vertigo Findings: Position testing --> immediate nystagmus in any direction
191
What is seen in Tuberous Sclerosis?
``` HAMARTOMAS: Hamartomas in CNS & skin Adenoma sebaceum Mitral regurgitation Ash-leaf spots Rhabdomyoma in heart Tuberous sclerosis aut. dOminant Mental retardation Angiomyolipoma in kidney Seizures ```
192
What is seen in Neurofibromatosis Type I?
``` Cafe-au-lait spots Lisch nodules Neurofibromas in skin Optic gliomas Pheochromocytomas ```
193
What is seen in Neurofibromatosis Type 2?
Bilateral Schwannomas | Meningiomas
194
What is seen in von Hippel-Lindau disease?
Bilateral renal cell carcinoma Cavernous hemangiomas in skin & organs Hemangioblastoma Pheochromocytomas Autosomal dominant mutation of VHL on Chromosome 3
195
What is seen grossly with glioblastoma? Histologically? What should be stained for?
"Butterfly glioma" with necrosis Histology: Pseudopalisading cells surrounding necrotic area Stain for GFAP
196
What is seen on histology in meningioma?
Psammomas and whorls in middle-aged girls | often calcified, estrogen receptor positive
197
What stain should be used for Schwannoma?
S-100
198
What is seen histologically with oligodendroglioma?
OligoHENdroglioma: Chicken wire capillary pattern Fried egg cells Often calcified
199
What is seen grossly with Pilocytic astrocytoma? Histologically? What stain should be used?
Grossly - cystic portion & solid portion (piloCYSTic astrocytoma) Histology - Rosenthal fibers (eosinophilic corkscrews) GFAP+
200
What is seen on histology of medulloblastoma?
Small blue cells (primitive neuroectodermal) | Homer-Wright rosettes
201
What are drop metastases? | What are they seen in?
Medulloblastoma can send drop metastases down to the spinal cord through CSF.
202
What is seen histologically with an ependymoma?
Perivascular rosettes | Rod-shaped blepharoplasts found near the nucleus
203
Where is the most common site for an Ependymoma?
4th ventricle. Can cause hydrocephalus
204
What are the tumors found in adults?
GBM = Metastasis > Meningioma > Schwannoma > Pituitary adenoma > Oligodendroglioma
205
What are the primary CNS tumors found in children?
Pilocytic astrocytoma = Medulloblastoma > Craniopharyngioma = Ependymoma = Hemangioblastoma
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What is seen with Hemangioblastoma?
Arise during middle age Associated with von Hippel-Lindau syndrome Can produce EPO --> polycythemia
207
What toxicities are seen with opioid analgesics?
``` Addiction Respiratory depression Constipation Biliary colic (sphincter of Oddi constriction) Miosis Additive CNS depression ```
208
What changes are seen on CBC following glucocorticoid administration?
Increased serum neutrophil count All other WBC's are diminished This is due to demargination of PMN's that were previously attached to a vessel wall (in the lung).
209
Why does aspirin use decrease the incidence of CRC?
COX-2 activity is seen to be high in som adenomatous polyps.
210
What chemical exposure is a cause of Parkinson's disease?
MPTP MPPP is a synthetic opioid used in a prior time. One of the byproducts of its production was MPTP. In the body, MPTP is converted to MPP+ by MAO. This substance is toxic to dopaminergic neurons.
211
What composition of bile is pro-gallstone?
High cholesterol Low phosphatidylcholine Low bile salts
212
What causes pituitary apoplexy?
Hemorrhage into a preexisting pituitary adenoma. Presents much like a subarachnoid hemorrhage but has a history of pituitary adenoma symptoms. Cardiovascular collapse can occur secondary to ACTH deficiency --> acute adrenal insufficiency.

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