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Neurology Flashcards

1
Q

Alar and Basal plate

A

Alar (Dorsal) - Sensory
Basal (Ventral) - Motor

Separated by sulcus limitans

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2
Q

Derivatives of…
Prosencephalon
Mesencephalon
Rhombencephalon

A

Telencephalon (Cerebrum, Lateral ventricles)
Diencephalon (Thalamus/Hypothalamus, 3rd ventricle)

Mesencephalon (Midbrain, Aqueduct)

Metencephalon (Pons/Cerebellum, Upper 4th ventricle)
Myelencephalon (Medulla, Lower 4th ventricle)

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3
Q

Derivatives of…
Neuroectoderm
Neural crest
Mesoderm

A

CNS neurons, Ependymal cells, Oligodendroglia, Astrocytes

PNS neurons, Schwann cells

Microglia

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4
Q

Increased AFP

Increased AChE

A

Meningocele
Meningomyelocele

Note - In spina bifida occulta (tuft of hair, skin dimple) only bony vertebrae fails to fuse so AFP is normal

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5
Q

Increased AFP
Polyhydramnios (swallowing center)

Associated with…
Maternal T1DM
Low folate

A

ANENCEPHALY

Malformation of anterior neural tube - No forebrain and open calvarium

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6
Q

Mutation responsible for holoprosencephaly - May present anywhere from cleft lip/palate to cyclopia

Associated with…
Patau syndrome
Fetal alcohol syndrome

A

Sonic hedgehog signaling pathway - failure of left and right hemispheres to separate

example of developmental field defect (initial embryonic disturbance causes multiple malformations)

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7
Q

Cause of hydrocephalus in lumbosacral meningomyelocele

A

CHIARI II MALFORMATION

Herniation of cerebellar vermis and tonsils through foramen magnum with aqueductal stenosis

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8
Q

Cystic enlargement of 4th ventricle in posterior fossa
Noncommunicating hydrocephalus
Spina bifida

A

DANDY-WALKER SYNDROME

Agenesis of cerebellar vermis

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9
Q

Cape-like loss of pain and temperature
Fine touch preserved
loss of upper extremity pain and temperature sensation

Associated with…
Chiari I malformation (cerebellar tonsillar ectopia –> headache and cerebellar symptoms)
Trauma
Tumors

A

SYRINGOMYELIA

Fibers crossing anterior white commissure - Spinothalamic tract

damage to ventral white commissure and ventral horns

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10
Q

Taste and sensation on anterior, posterior, and base of tongue

A

Anterior (Arches 1/2):
Taste - Chorda tympani (CN VII)
Sensation - Lingual (CN V3)

Posterior (Arches 3/4)
Taste/Sensation - CN IX

Base (Arches 3/4)
Taste/Sensation - Internal Laryngeal (CN X)

Note - All motor function is by CN XII except the palatoglossus (CN X)

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11
Q

Nissl staining does not show…

A

Axon - no RER

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12
Q 
Cell marker for cell involved in...
Physical support/repair
BBB
Excess neurotransmitter removal
K+ metabolism
Glycogen fuel reserve
A

GFAP (Astrocyte)

Responsible for reactive gliosis in response to neural injury

Note - GFAP stains all Glial cells (Astrocytes, Oligodendrocytes, Ependymal cells), while Synaptophysin stains neurons

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13
Q

Fuse to form multinucleated giant cells in CNS of HIV patients - Not discernible by Nissl stain

A

MICROGLIA

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14
Q

Internal acoustic meatus tumor extending to cerebellopontine angle - GFAP+

A

VESTIBULAR (CN VIII) SCHWANNOMAS

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15
Q

Difference between oligodendrocytes and Schwann cells

A

Oligodendrocytes (CNS) can myelinate many axons, while Schwann (PNS) can only myelinate one

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16
Q

Fast adapting receptors responsible for…

Dynamic light touch
Vibration/Pressure

A

Meissner - Glabrous skin
Pacinian - Deep skin, Ligaments, Joints

Note - Both large, myelinated

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17
Q

Slow adapting receptors responsible for…

Pressure, Slippages, Joint angle change
Pressure, Static touch, Position sense

A

Ruffini - Dendritic endings with capsules

Merkel - Large, myelinated

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18
Q

Pain/Temperature receptors in skin and viscera

A

FREE NERVE ENDINGS

C (slow, unmyelinated) and Ad (fast, myelinated) fibers

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19
Q

Layer of peripheral nerve with inflammatory infiltrate in GBS

A

Endoneurium - invests single nerve fiber

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20
Q

Layer of peripheral nerve that must be attached in microsurgery

A

Perineurium - surrounds a fascicle of nerve fibers

Note - All fascicles and blood vessels surrounded by epineurium

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21
Q 
Location of synthesis of...
Acetylcholine
Dopamine
GABA
Norepinephrine
Serotonin
A

Basal Nucleus of Meynert

Ventral Tegmentum/SN pars compacta

Nucleus Accumbens

Locus Ceruleus (posterior rostral pons)

Raphe Nucleus

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22
Q

Mechanism of glucose and amino acid transfer through BBB

A

Carrier-mediated transport

Note - Nonpolar/lipid-soluble substances can cross rapidly by diffusion

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23
Q

Areas with BBB containing fenestrated capillary endothelium

A

Area postrema
OVLT (osmotic sensing)
Neurohypophysis

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24
Q

Mechanism of vasogenic edema

A

Neoplasms and infarction destroy BBB endothelial tight junctions

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25
Function of locations of hypothalamus... ``` Lateral Ventromedial Anterior Posterior Arcuate Paraventricular Supraoptic Suprachiasmatic Tuberomammillary ```
``` Hunger (Ghrelin) Satiety (Leptin) Cooling (Parasympathetic) Heating (Sympathetic) Dopamine, GnRH, GH ADH, Oxytocin, CRH, TRH ADH, Oxytocin Circadian rhythm Arousal ```
26
Mechanism of circadian rhythm control by light
SCN releases NE NE acts on the pineal gland Pineal gland releases Melatonin Note - Circadian rhythm controls nocturnal release of ACTH and Prolactin as well
27
Controls extraocular movements during REM sleep
PPRF (Paramedian pontine reticular formation/conjugate gaze center) Note - Increased ACh in REM
28
Substances associated with decreased REM and delta wave sleep
Alcohol Benzodiazepines Barbiturates Norepinephrine
29
``` Prominent EEG waveform during... Awake with eyes open Awake with eyes closed Stage N1 Non-REM (light sleep) Stage N2 Non-REM (deep sleep) Stage N3 Non-REM (deepest sleep) REM ``` Note - REM occurs every 90 minutes and increases in duration throughout the night
Beta (highest frequency, lowest amplitude) Alpha Theta Sleep spindles, K complexes Delta (lowest frequency, highest amplitude) Beta
30
``` Stage of sleep where the following occurs... Bruxism Sleepwalking Night terrors Bedwetting Loss of motor tone Variable pulse and blood pressure Dreaming/Nightmares Penile/clitral erection Memory processing ```
``` N2 N3 N3 N3 REM REM REM REM REM ```
31
Input to VPLN of thalamus
Spinothalamic Dorsal column Medial lemniscus Note - To primary somatosensory cortex (postcentral gyrus)
32
Input to VPMN of thalamus
Trigeminal (face sensation) Gustatory (taste) Note - To primary somatosensory cortex (postcentral gyrus)
33
Input to LGN of thalamus
CN II (vision) Note - To calcarine sulcus
34
Input to MGN of thalamus
Superior Olive/Inferior Colliculus of Tectum (hearing) Note - To temporal lobe
35
Input to VLN of thalamus
Basal ganglia/Cerebellum Note - To motor cortex (precentral gyrus)
36
``` Function of the limbic system - includes... Hippocampus Amygdala Fornix Mamillary bodies Cingulate gyrus ``` ("5 Fs")
``` Feeling Feeding Fleeing Fighting Sex ```
37
``` Mechanism of Schizophrenia/Antipsychotic effects... Mesocortical Mesolimbic Nigrostriatal Tuberoinfundibular ```
Decreased in schizophrenia - Negative symptoms Increased in schizophrenia - Positive symptoms Decreased by antipsychotics - EPS Decreased by antipsychotics - Hyperprolactinemia
38
Input and output of the cerebellum
Input: Contralateral cortex via middle cerebellar peduncle Ipsilateral proprioceptive information via inferior cerebellar peduncle from spinal cord Output: Contralateral cortex via superior cerebellar peduncle
39
Deep nuclei (lateral to medial) | "Don't Eat Greasy Foods"
Dentate Emboliform Globose Fastigial Note - Deep nuclei are the connection between the Purkinje cells and the Superior cerebellar peduncle
40
Wide-based (truncal) cerebellar gait Dysarthria Head tilting
CENTRAL VERMIS (FASTIGIAL) LESION May also develop Vertigo/Nystagmus from involvement of the Flocculonodular lobe medial lesion affects the medial structures bilaterally
41
Basal ganglia direct pathway
Cortical inputs stimulate Striatum (Caudate, Putamen) Striatum releases GABA inhibiting GPi GPi stops releasing GABA disinhibitng the Thalamus Increased movement Dopamine from SNc binds D1 stimulating this pathway Note - Motor cortex also directly modulates movement via the pedunculo-pontine nucleus
42
Basal ganglia indirect pathway
Cortical inputs stimulate the Striatum (Caudate, Putamen) Striatum releases GABA inhibiting GPe GPe stops releasing GABA disinhibiting the STN STN stimulates GPi to inhibit Thalamus Decreased movement Dopamine from SNc binds D2 inhibiting this pathway
43
Treatment of... High-frequency tremor of sustained posture Worse with movement
ESSENTIAL TREMOR Autosomal dominant Treat with... Propranolol Primidone
44
Lesion resulting in hemiballismus
Contralateral subthalamic nucleus (STN) Note - Usually from a lacunar stroke
45
Intracellular eosinophilic inclusions - Composed of a-synuclein
LEWY BODY
46
Mechanism of neuronal damage in Huntington's Presents as caudate atrophy and hydrocephalus ex vacuo - choreoathetosis - dementia - death
Autosomal dominant CAG repeat Loss of Ach and GABA in caudate leads to neuronal death via NMDA-R binding and glutamate excitotoxicity
47
Location of Broca's area
Left posterior inferior frontal gyrus Broca's aphasia - language output deficit. Slow effortful speech that is agrammatic and telegraphic. Comprehension is largely preserved
48
Location of Wernicke's area
Left posterior superior temporal gyrus Wernicke aphasia- speech is preserved but language content is incorrect. Also have impaired comprehension and experience "word salad", an inability to repeat, normal fluency, and are unaware of their deficits Can be due to stroke affecting the middle cerebral artery
49
Transcortical motor aphasia
Broca's aphasia with intact repetition
50
Transcortical sensory aphasia
Wernicke's aphasia with intact repetition
51
Transcortical mixed aphasia
Global aphasia with intact repetition
52
Hyperphagia Hypersexuality Hyperorality
KLUVER-BUCY SYNDROME Bilateral Amygdala lesions - Associated with HSV-1 encephalitis which involves the temporal lobe and limbic system
53
Agraphia Acalculia Finger agnosia L/R disorientation
GERSTMANN SYNDROME Dominant (L) parietal cortex lesion
54
Lesion resulting in... Intention tremor Loss of balance Falling towards towards ipsilateral side
Cerebellar hemisphere
55
Lesion resulting in... | Anterograde amnesia
Bilateral hippocampal
56
Lesion resulting in... | Eyes looking away from the lesion
Paramedian pontine reticular formation (PPRF)
57
Lesion resulting in... | Eyes looking towards the lesion
Frontal eye fields Note - Above Broca's area
58
Equation for CPP
MAP - ICP
59
Regulation of CPP by CO2 and O2
PCO2 directly related to CPP until > 90 O2 indirectly related to CPP only when < 50 Note - Therapeutic hyperventilation (decreased PCO2) results in vasoconstriction/decreased blood flow and thus decreased ICP
60
Lesion resulting in... | Coma
Reticular activating system (Midbrain)
61
Upper leg weakness Upper arm weakness Defects in higher order visual processing
WATERSHED STROKE Often due to severe hypotension
62
Contralateral face/body paralysis Contralateral face/body sensory loss Absence of cortical signs (neglect, aphasia, visual field loss) Note - Embolic phenomenon most often occur in the cortex
LENTICULO-STRIATE LACUNAR STROKE Arises from ICA/MCA and supplies Striatum/Internal capsule Note - Lacunae are liquefactive necrosis from Lipohyalinosis/Microadenomas (HTN, DM)
63
Contralateral U/L limb paralysis Contralateral proprioception deficits Ipsilateral hypoglossal deviation
MEDIAL MEDULLARY SYNDROME Stroke in paramedian branches of anterior spinal artery Note - Anterior spinal artery arises from between both vertebral arteries
64
``` Ipsilateral face pain and temperature loss Contralateral body pain and temperature loss Vomiting Vertigo Nystagmus Dysphagia Decreased gag reflex Hoarseness Ipsilateral Horner's Ataxia Dysmetria ```
LATERAL MEDULLARY (WALLENBERG) SYNDROME Posterior inferior cerebellar artery stroke - Nucleus ambiguus (speech/swallow) deficits specific to this syndrome Note - PICA arises from vertebral
65
Ipsilateral upper and lower facial paralysis Ipsilateral face pain and temperature loss Contralateral body pain and temperature loss Vomiting Vertigo Nystagmus Decreased lacrimation Decreased salivation Decreased taste (anterior tongue) Ataxia Dysmetria
LATERAL PONTINE SYNDROME Anterior inferior cerebellar artery stroke - Upper and lower facial paralysis is specific to this syndrome Note - AICA arises from beginning of basilar artery
66
Most common site of Saccular (berry) aneurysm Note - Associated with ADPKD and Ehlers-Danlos
Junction of ACA with anterior communicating artery
67
Microaneurysm of lenticulostriate arteries resulting in internal capsule intraparenchymal hemorrhage Note - Not seen on angiogram
CHARCOT-BOUCHARD ANEURYSM Chronic hypertension
68
Mydriasis Ptosis "Down and out" eye
POSTERIOR COMMUNICATING ARTERY ANEURYSM Compression results in CN III palsy
69
Bitemporal hemianopia
ANTERIOR COMMUNICATING ARTERY ANEURYSM Compression of optic chiasm - May also be seen with Meningiomas, Pituitary adenomas, and Craniopharyngiomas
70
Lobar (not basal ganglia, internal capsule) intraparenchymal hemorrhage in elderly patient
Amyloid angiopathy
71
Bloody or yellow (xanthochromic) spinal tap
SUBARACHNOID HEMORRHAGE Nimodipine to prevent subsequent vasospasm and ischemic infarct May also lead to communicating or obstructive hydrocephalus
72
Regions most vulnerable to ischemia Note - NCHCT may be normal for first 6-24 hours, but DWI will detect within first hour
Hippocampus (Pyramidal) Neocortex Cerebellum (Purkinje) Watershed zones
73
``` Histologic features of ischemic stroke at... 12 - 48 hours 1 - 3 days 3 - 5 days 1 - 2 weeks > 2 weeks ```
``` Red neurons Necrosis + Neutrophils Macrophages (Microglia) Reactive gliosis + Angiogenesis Glial scar + Myelin remnants ``` Note - Slow microglia recruitment due to BBB allows for myelin persistence in CNS decreasing neuronal regeneration
74
Headache Seizures Focal deficits Associated with... Pregnancy OCP Factor V Leiden
VENOUS SINUS THROMBOSIS May progress to venous hemorrhage
75
Anatomy of dural venous sinuses Note - Superior saggital sinus is the main location of CSF return via arachnoid granulations
Vein of Galen and Inferior saggital sinus form Straight sinus Straight sinus, Superior saggital sinus, and Occipital sinus drains to Confluence of sinuses Confluence of sinuses drains to Transverse sinuses Transverse sinuses drain to Sigmoid sinuses and then internal Jugular
76
Anatomy of ventricular system Note - Ependymal cells of choroid plexus make CSF, which is then drained to arachnoid granulations
Lateral ventricle drains to 3rd ventricle via interventricular foramina of Monro 3rd ventricle drains to 4th ventricle via cerebral (Sylvian) aqueduct 4th ventricle drains to subarachnoid space via foramina of Luschka (lateral) and foramen of Magendie (medial)
77
``` Normal mental status Headache worse in morning Diplopia (CN VI palsy) Loss of vision based on head position, sneezing Papilledema Elevated opening pressure ``` Note - Imaging may be normal or show slit ventricles and empty sella
IDIOPATHIC INTRACRANIAL HYPERTENSION Associated with... Vitamin A excess Danazol Tetracycline ``` Treat with... Weight loss Acetazolamide Topiramate LPs, Shunt, Optic nerve fenestration ```
78
Mechanism of communicating hydrocephalus - leads to increased ICP, papilledema, and herniation
Decreased CSF absorption by arachnoid granulation - may be due to scarring post-meningitis
79
Mechanism of NPH - communicating hydrocephalus
Expansion of ventricles distort fibers of corona radiata Note -- Does not result in increased subarachnoid space volume
80
Mechanism of noncommunicating hydrocephalus
``` Structural blockage of CSF flow... Foramen of Monro colloid cyst Sylvian aqueduct stenosis 4th ventricle obstruction via Chiari II Foramina of Luschka/Magendie via Dandy-Walker ```
81
Ventricular enlargement on imaging without triad of communicating hydrocephalus - ICP is normal
EX VACUO VENTRICULOMEGALY
82
Vertebral level for spinal tap
L3-L5 Spinal cord ends at L1/2 and subarachnoid space ends at S2 - needle should go below spinal cord but above end of subarachnoid space
83
Tract anatomy responsible for... Fine touch Pressure/Vibration Proprioception
DORSAL COLUMN 1st order neuron (DRG) ascends ipsilaterally Synapse in Medulla at Nucleus Cuneatus/Gracilis 2nd order neuron decussates in Medulla and ascends contralaterally via Medial Lemniscus Synapses in VPL of Thalamus Note - Gracilis (LE) inside, Cuneatus (UE) outside
84
Tract anatomy responsible for... Lateral - Pain, Temperature Anterior - Crude touch, Pressure
SPINOTHALAMIC TRACT 1st order neuron (DRG) Synapse at ipsilateral gray matter of spinal cord 2nd order neuron decussates at Anterior White Commissure and ascends contralaterally Synapse in VPL of Thalamus Note - Cervical (inside) to Sacral (outside)
85
Tract anatomy responsible for... | Voluntary movement
LATERAL CORTICOSPINAL TRACT UMN descents ipsilaterally through internal capsule and decussate at pyramids (Medulla) to decent contralaterally Synapse with cell body at Anterior Horn of spinal cord LMN leaves spinal cord Synapse at NMJ Note - Cervical (inside) to Sacral (outside)
86
Degeneration of anterior horns of spinal cord - LMN lesions only
Poliomyelitis (asymmetric) | Werdnig-Hoffmann (symmetric)
87
Mutation in ALS Note - No bowel/bladder or sensory deficits
Superoxide dismutase 1
88
Loss of motor function below vertebral level Loss of pain/temperature below vertebral level Retained fine touch, proprioception, vibration
ANTERIOR CORD SYNDROME Watershed at T8 - Adamkiewicz supplies ASA below this
89
Ataxic gait Paresthesia Impaired proprioception/vibration Note - Positive Romberg unlike Cerebellar ataxia
SUBACUTE COMBINED DEGENERATION Spinocerebellar tracts Lateral Corticospinal Dorsal Associated with... Vit B12 deficiency Pernicious anemia Vit E deficiency
90
``` Begin with gait ataxia Progress to dysarthria and dysphagia Spastic weakness Staggering gait/Falls Kyphosis Hammer toes Pes Cavus Diabetes Hypertrophic cardiomyopathy ```
FRIEDREICH ATAXIA Autosomal recessive GAA repeat on Chromosome 9 - Deficient Frataxin (iron binding protein) leads to impaired mitochondrial function due to iron overload and oxidative stress Note - Spinal cord pattern similar to Vit B12/E deficiency - peripheral nerves - loss of tactile discrimination and of position and vibration sensation (dorsal columns) - spastic paresis (corticospinal tracts) - limb dystaxia (spinocerebellar tracts)
91
Ipsilateral hemiparesis Ipsilateral loss of position/vibration and fine touch Contralateral loss of pain/temperature Note - Loss of pain/temperature occurs two levels below lesion.
BROWN-SEQUARD SYNDROME
92
``` Vertebral levels for dermatomes... Neck Thumbs Nipples Umbilicus Inguinal ligament ```
``` C3/4 C6 T4 T10 L1 ```
93
``` Nerve roots for reflexes... Biceps Triceps Patellar Achilles Anal wink ```
``` C5/6 C7/8 L3/4 S1/2 S3/4 ```
94
Paralysis of conjugate vertical gaze Impaired convergence Absent pupillary light reflex nausea and vomiting and headache due to obstruction of cerebral aqueduct causing hydrocephalus
PARINAUD SYNDROME Pinealoma (germinoma) above superior colliculus (dorsal midbrain) Note - Inferior colliculus is auditory Note- pineal gland is between thalamic bodies and modulates circadian rhythm by secreting melatonin in response to darkness or sympathetic stimulation
95
``` Location of cranial nerve nuclei... Midbrain Pons Medulla Spinal cord ``` Note - Lateral nuclei are sensory (alar plate) while medial nuclei are motor (basal plate)
CN III, IV CN V, VI, VII, VIII CN IX, X, XII CN XI
96
``` Cranial nerve exits... Cribriform plate Optic canal Superior orbital fissure Foramen Rotundum Foramen Ovale Foramen Spinosum Internal auditory meatus Jugular foramen Hypoglossal canal Foramen magnum ```
``` CN I CN II CN III, IV, V1, VI CN V2 CN V3 Middle Meningeal Artery CN VII, VIII CN IX, X, XI CN XII Brainstem ```
97
Only cranial nerve without thalamic relay to the cortex
Olfactory (CN I)
98
CN responsible for salivation from... Submandibular Sublingual Parotid
CN VII CN VII CN IX
99
Vagal nuclei responsible for visceral sensory information
NUCLEUS SOLITARIUS CN VII, IX, X
100
Vagal nuclei responsible for motor innervation of swallowing
NUCLEUS AMBIGUUS CN IX, X, XI
101
Vagal nuclei responsible for parasythmatiecs to the thorax
DORSAL MOTOR NUCLEUS CN X
102
Parasympathetic innervation of the pupil
Preganglionic from Edinger-Westphal nucleus Synapse in ciliary ganglion Postganglionic short ciliary nerves to sphincter pupillae Note - Sympathetics from Superior Cervical Ganglion
103
Corneal reflex afferent and efferent
Afferent - Nasociliary (V1) Efferent - VII Note - Lacrimation reflex by same fibers
104
Gag reflex afferent and efferent
Afferent - IX | Efferent - X
105
``` Physical exam presentation of... CN V lesion CN X lesion CN XI lesion CN XII lesion ```
Jaw deviates towards lesion Uvula deviates away from lesion Inability to turn head away from lesion (SCM), raise ipsilateral shoulder (trapezius) Tongue deviates towards lesion
106
Muscles of mastication responsible for... Jaw opening Jaw closing
Opening - Lateral pterygoid Closing - Masseter, Temporalis, Medial pterygoid
107
Contents of cavernous sinus
CN III, IV, V1, VI Postganglionic sympathetic pupillary fibers Internal carotid artery Syndrome may result from... Pituitary adenoma Carotid-cavernous fistula (ICA dissection) Cavernous sinus thrombosis (S. aureus infection) Note - CN VI most susceptible to injury
108
Tonotopy of cochlea...
Apex (wide, flexible) - Low frequency | Base (thin, rigid) - High frequency
109
Weber test localizaiton
Conductive - Localizes to affected ear | Sensorineural - Localizes to unaffected ear
110
Structures contained in... Optic disc Macula
Retinal vessels, optic cup Fovea Note - Retinal vein appears darker than retinal artery
111
Drugs decreasing aqueous humor production by nonpigmented epithelium of ciliary body Note - Ciliary body innervates zonule fibers for lens accommodation
b-blockers (Timolol, Betaxolol, Carteolol) a2-agonists (Brimonidine) Carbonic anhydrase inhibitors (Acetazolamide) ``` Brimonidine may cause... Blurry vision Hyperemia Foreign body sensation Allergy ```
112
Aqueous humor pathways (2)
Trabecular outflow via canal of Schlemm to episcleral vasculature Uveoscleral outflow Note - Majority is via trabecular meshwork
113
Refractive errors in... Hyperopia Myopia Presbyopia
Short eyes focuses light behind retina - farsighted Long eyes focuses light in front of retina - nearsighted Age-related inelasticity of lens decreases accommodation - farsighted
114
Mechanism of open-angle glaucoma Note - Painless
May be primary (idiopathic) May be secondary to blocked trabecular meshwork... WBCs (uveitis) RBCs (vitreous hemorrhage) Retinal elements (retinal detachment)
115
Mechanism of primary narrow-angle glaucoma
Forward movement of lens against central iris Obstruction of normal aqueous flow through pupil Fluid builds up behind iris Peripheral iris pushed against cornea Flow impeded through trabecular meshwork Note - Secondary is due to hypoxia (e.g. retinal disease) inducing vaso proliferation in iris that contracts the angle
116
``` Painful, red eye Sudden vision loss with halos Poorly reactive mid-dilated pupil Rock-hard eye (IOP) Frontal headache Increased cupping Optic disc atrophy ```
ACUTE NARROW ANGLE GLAUCOMA Ophthalmic emergency - Do not give epinephrine because of mydriatic effect Note - Chronic is asymptomatic with gradual loss of peripheral vision
117
Hypopyon - accumulation of pus in anterior chamber
UVEITIS Uvea is between sclera and retina - Iris, Ciliary body, Choroid
118
Gradual distortion and eventual loss of central vision (scotomas) Yellow extracellular deposits (Drusen) in retinal pigment epithelium
DRY MACULAR DEGENERATION Prevent progression with... Antioxidants Multivitamins
119
Rapid loss of vision due to bleeding secondary to choroidal neovascularization Distortion of straight lines Grayish-green subretinal discoloration Adjacent fluid/hemorrhage
WET MACULAR DEGENERATION Often acute exacerbation of dry macular degeneration Treat with... Anti-VEGF (Ranibizumab)
120
``` Retinal hemorrhages Macular edema Cotton wool spots Lipid deposition (granular) Vascular proliferation ```
DIABETIC RETINOPATHY
121
Retinal hemorrhage Vessel engorgement Edema
RETINAL VEIN OCCLUSION Compression from nearby arterial atherosclerosis
122
Crinkling of retinal tissue Flashes and floaters Monocular curtain-like vision loss
RETINAL DETACHMENT Separation photoreceptor layer from pigmented epithelium
123
Layers of retina
``` Light Ganglion cell layer Inner plexiform layer Inner nuclear layer (bipolar, amacrine) Outer plexiform layer Outer nuclear layer Photoreceptors Pigment epithelium/Bruch's membrane ```
124
Acute, painless monocular vision loss Pale retina Cherry-red spot at fovea
CENTRAL RETINAL ARTERY OCCLUSION Evaluate for carotid artery atherosclerosis or other embolic source
125
Painless, progressive vision loss | Night-blindness (rods) initially
INHERITED RETINITIS PIGMENTOSA Spicule shaped deposits around macula
126
CMV, HSV, VZV infection followed by... Retinal edema Necrosis Note - Optic disc hard to discern
RETINITIS
127
Enlarged blind spot Bilateral optic disc swelling Elevated optic disc with blurred margins
PAPILLEDEMA Note - Impaired axoplasmic flow
128
Mechanism of pupillary light reflex
CN II to pretectal nucleus Bilateral Edinger-Westphal activation CN III to ciliary ganglion Bilateral pupillary contraction
129
Pathway for mydriasis
1st neuron in hypothalamus to center of Budge (C8-T2) 2nd neuron travels with cervical sympathetic ganglion near lung apices/subclavian to superior cervical ganglion 3rd neuron travels in ICA plexus and through cavernous sinus to enter the orbit as the long ciliary nerve Note - Lesions above T1 may result in Horner's
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Muscles and nerves responsible for ptosis
Superior tarsal muscle (superior cervical ganglion) | Levator palpebrae superioris (CN III)
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MARCUS GUNN PUPIL
Afferent pupillary defect - relative dilation when light is shone in affected eye
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Ptosis Miosis No anhidrosis
ICA DISSECTION Sympathetics to sweat glands go from the superior cervical ganglion to ECA (not ICA)
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Function of IO and SO muscles
IO - Adduction/Elevation, Extortion SO - Adduction/Depression, Intorsion Note - When isolated from other muscles, the SO abducts the eye instead
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Mechanism of CN III palsy in ischemic and compressive lesions
Ischemic - Damage central motor fibers first resulting in ptosis and down/out gaze first Compressive - Damage peripheral parasympathetics first resulting in blown pupil first
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Eye moves upward with contralateral gaze Problems going down stairs Head tilt away from affected eye
CN IV PALSY
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Left homonymous hemianopia
Right optic tract lesion Note - Right side of retina (Left visual field) goes to the right lobe
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Left upper quadrantic anopia
Right Meyer Loop (temporal) lesion Receives inferior retina - upper visual field Note - MCA territory
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Left lower quadrantic anopia
Right Optic Radiation (parietal) lesion Receives superior retina - lower visual field Note - MCA territory
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Anatomy of INO
Frontal eye field to contralateral PPRF PPRF to ipsilateral CN VI nuclei CN VI nuclei connected to contralateral MLF Contralateral MLF connected to CN III Note - Often dorsal Pons lesion
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Altered proteins associated with... Decreased sporadic Alzheimer's Increased sporadic Alzheimer's Early onset familial Alzheimer's
ApoE2 ApoE4 APP, Presenilin-1/2 Note - Elevated APP in Trisomy 21
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Extracellular b-amyloid depositions - Dark on silver stain Note - Formed by cleavage of APP
SENILE PLAQUES Increased risk of amyloid angiopathy - lobar ICH
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Intracellular hyperphosphorylated tau protein - Insoluble cytoskeletal elements
NEUROFIBRILLARY TANGLES Number correlates with degree of dementia
143
Histology associated with... | Early changes in personality (behavioral) or aphasia (primary progressive aphasia)
FRONTOTEMPORAL DEMENTIA/PICKS disease Inclusions of hyperphosphorylated tau (Pick bodies) Ubiquitinated TDP-43 Note - Disinhibition is right frontal lobe, apathy and depression is left frontal lobe
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``` Acute paralysis (Corticospinal) Neck weakness, Dysarthria, Dysphagia (Pseudobulbar) Loss of consciousness Pons hyperintense on MRI ``` Note - Pseudobulbar because nuclei are intact
OSMOTIC DEMYELINATION SYNDROME (CENTRAL PONTINE MYELINOLYSIS) Sudden correction of hyponatremia (compared to cerebral edema in HYPERnatremia correction)
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CSF with... Increased IgG Myelin basic protein Oligoclonal bands
MULTIPLE SCLEROSIS Treat with... IFN-b Glatiramer Natalizumab (a4-integrin inhibitor) Note - Axons preserved in acute lesions
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``` Ascending paralysis Autonomic dysfunction Mild sensory abnormalities Albuminocytologic dissociation in CSF Lipid laden macrophages ```
ACUTE INFLAMMATORY DEMYELINATING POLYRADICULOPATHY (GUILLAIN-BARRE) Treat with IVIG and Plasmapheresis - Avoid steroids Note - Associated with Campylobacter jejuni
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Viral infection or vaccination followed by... Altered mental status Rapidly progressive multifocal deficits
ACUTE DISSEMINATED ENCEPHALOMYELITIS Multifocal periventricular inflammation and demyelination - treat with steroids Note - No oligoclonal bands
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Pes cavus LE weakness LE sensory deficits
CHARCOT-MARIE-TOOTH Autosomal dominant mutation results in defective myelin formation
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Globoid cells Peripheral neuropathy Developmental delay Optic atrophy
KRABBE DISEASE Lysosomal storage disease - Deficiency of Galactocerebrosidase causes build up of Galactocerebroside
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Central and peripheral demyelination Ataxia Dementia
METACHROMATIC LEUKODYSTROPHY Lysosomal storage disease - Deficiency in Arylsulfatase A causes buildup of Sulfatides
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Drugs associated with PML
Natalizumab (a4-integrin) | Rituximab (CD-20)
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``` Male with... Progressive neurologic dysfunction Adrenal crisis Coma Death ```
ADRENOLEUKODYSTROPHY X-linked - Disrupts metabolism of very-long-chain fatty acids leading to excessive build up in CNS, adrenals, and testes
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Treatment of cluster headaches
Acute - O2, Sumatriptan | Prophylaxis - Verapamil
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Treatment of migraines
Acute - NSAIDs, Triptans, Dihydroergotamine | Prophylaxis - BBs, CCB, Topiramate, Valproate, TCA
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Sudden onset severe vertigo Affected by position N/V Tinnitus Horizontal unidirectional nystagmus - relieved by gaze fixation.
PERIPHERAL VERTIGO
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Intellectual disability Port-wine stain Seizures Episcleral hemangioma/Glaucoma Leptomeningeal angiomas Unilateral tram-track calcifications Note - Non-inherited
STURGE-WEBER Sporadic activating mutation in GNAQ leading to abnormal neural crest derivatives
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``` Intellectual disability Ash-leaf spots Hamartomas in CNS and skin Angiofibromas Mitral regurgitation Cardiac rhabdomyomas Renal angiomyolipomas ``` Note - Increased risk for subependymal astrocytomas
TUBEROUS SCLEROSIS Autosomal dominant mutations in TSC1/2 - Hamartin/Tuberin tumor suppressor genes
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``` Cafe-au-lait spots Lisch nodules (pigmented iris hamartomas) Cutaneous neurofibromas (GFAP+) Optic gliomas Pheochromocytoma Pseudoarthrosis ``` Note - Neurofibromas as neural crest derivatives
NEUROFIBROMATOSIS TYPE 1 (VON RECKLINGHAUSEN DISEASE) Mutation in NF1 tumor suppressor gene on Chromosome 17 - Normally inhibits RAS AD problem in neurofibromin
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Hemangioblastomas in CNS Bilateral RCC Pheochromocytoma Angiomatosis (mucosal cavernous hemangiomas)
VON HIPPEL-LINDAU VHL gene on Chromosome 3
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Pseudopalisading tumor cells Central area of necrosis and hemorrhage GFAP+
GLIOBLASTOMA (ASTROCYTOMA IV)
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Spindle cells in whorled pattern | Psammoma bodies
MENINGIOMA Arachnoid cell origin
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Numerous capillaries Minimal brain parenchyma Elevated EPO (secondary polycythemia)
HEMANGIOBLASTOMA Typically occurs in cerebellum
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``` Cafe au lait spots Cataracts Neurofibromas Multiple meningiomas Spinal ependymomas Bilateral acoustic neuromas ```
NEUROFIBROMATOSIS TYPE 2 Note - Schwannomas, Neurofibromas, and Melanomas are S-100+ (neural crest) AD problem in merlin protein
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Calcified frontal lobe tumor "Chicken-wire" capillaries "Fried egg" cells GFAP+
OLIGODENDROGLIOMA
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Cystic, solid posterior fossa mass in child Well circumscribed GAP+ Eosinophilic corkscrews (Rosenthal fibers)
PILOCYTIC (LOW GRADE) ASTROCYTOMA
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Solid cerebellar mass in child Non-communicating hydrocephalus Homer-Wright rosettes Small blue cells
MEDULLOBLASTOMA Malignant neuroectodermal tumor - Metastasizes to spine (drop metastasis)
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Responsible for detection of tension and stretch in muscle tendons
Tension - Golgi (Ib) in series | Length - Intrafusal (Ia, II) in parallel
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4th ventricle mass in a child Hydrocephalus Perivascular rosettes GFAP+
EPENDYMOMA
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Calcified mass in child | Cholesterol crystals in "motor oil" like fluid
CRANIOPHARYNGIOMA
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Parinaud syndrome Obstructive hydrocephalus Precocious puberty/Elevated b-hCG
PINEALOMA Obstructs Sylvian aqueduct
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Herniation resulting in ACA deficits
SUBFALCINE (CINGULATE) HERNIATION
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Herniation causing Duret hemorrhages - rupture of paramedian basilar artery branches
TRANSTENTORIAL (CENTRAL) HERNIATION Caudal displacement of the brainstem
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Structures responsible for... Blow, down-and-out pupil Ipsilateral paresis Contralateral homonymous hemianopia with macular sparing
UNCAL HERNIATION Ipsilateral CN III Contralateral crus cerebri (Kernohan notch) Ipsilateral PCA Note - Eventually damage to midbrain results in mid-positioned fixed pupils (pons is pinpoint)
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Herniation resulting in paralysis, coma, and death
CEREBELLAR TONSILLAR HERNIATION Compression of brainstem Note - Paralysis is initially flaccid (spinal shock) and becomes spastic days later
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Mechanism of cholinomimetics (M3) in treating glaucoma (Pilocarpine, Carbachol)
Increased outflow via contraction of ciliary muscles and opening of meshwork Note - Pilocarpine is fast acting and good for emergencies (e.g. acute narrow angle glaucoma)
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Mechanism of prostaglandins (PGF2a) in treating glaucoma
Bimatoprost, Latanoprost - Increases uveoscleral outflow of aqueous humor May cause... Browning of iris Eyelash growth
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First line for simple partial or simple complex seizure Note - Complex is partial with LOC and post-ictal
Carbamazepine
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First line for generalized seizure (tonic-clonic, myoclonic) Note - Juvenile myoclonic is worst with sleep deprivation and has no loss of consciousness or post-ictal but is still generalized
``` Valproic acid Phenytoin Topiramate Lamotrigine Levetiracetam ```
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First line for acute and prophylaxis of status
Acute - Diazepam, Lorazepam, Midazolam | Prophylaxis - Phenytoin
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First line for seizures in neonates
Phenobarbital Note - Induces Cytochrome P-450
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First line for trigeminal neuralgia
Carbamazepine
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Antiepileptics safest in pregnancy
Gabapentin Lamotrigine Levetiracetam
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Treatment of malignant hyperthermia
Dantrolene - Ryanodine receptor antagonist
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Mechanism of Tetrabenazine/Reserpine in treating Huntington's
Inhibit VMAT - decreased dopamine packaging and release
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Mechanism of Riluzole in treating ALS
Decreasing Glutamate excitotoxicity - modest survival increase
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Mechanism of Sumatriptan in treating migraine and cluster headaches
5-HT1B/1D agonist... Inhibits trigeminal nerve Prevents vasoactive peptide release Induces vasoconstriction May cause... Coronary vasospasms (CAD, Prinzmetal angina) Mild paresthesia
187
Changes in spinal cord shape moving rostrally
Less gray matter More ovoid shape Cuneate fasciculus appear at the thoracic level Note - Low cervical cord (C7) and Lumbosacral have more ventral gray matter to innervate the limbs Note - Thoracic cord contains lateral horn (preganglionic sympathetics)
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Inhibits REM-sleep related phenomenon and implicated in Narcolepsy
OREXIN (HYPOCRETIN) Note - Treat with Modafinil
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Level of brainstem differentiating extensor and flexor posturing
Decerebrate (extensor) - Below red nucleus (midbrain tegmentum, pons) Decorticate (flexor) - Above red nucleus (cerebrum, internal capsule) Note - Red nucleus' rubrospinal tract responsible for flexion
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Lower back pain Saddle anesthesia Flaccid paralysis of bladder and rectum Impotence
CONUS MEDULLARIS SYNDROME Compression at L1/L2
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``` Radiating lower back pain Saddle anesthesia Loss of anal wink Hyporeflexia Weakness ```
CAUDA EQUINA SYNDROME Disc herniation at L4/L5 or S1/S2
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Differentiation of LEMS from Myasthenia Gravis
In LEMS... Autonomic symptoms Hyporeflexia Incremental response Note - Associated with SCLC
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Hyperreflexia Spasticity Atrophy Fasciculations Loss of neurons in anterior horn Atrophy of lateral corticospinal tract and precentral gyrus
ALS Treat with... Riluzole MAOi (decreased glutamate)
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Cause of right nasal hemianopia
RIGHT PERI-CHIASMAL REGION ICA calcification or aneurysm
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``` Location of lacunar infarcts causing... Pure motor hemiparesis Pure sensory loss Dysarthria-Clumsy hand Ataxia-Hemiplegia ```
Posterior limb of internal capsule, Basal pons VPL/VML thalamus Genu of internal capsule, Basal pons Posterior limb of internal capsule, Basal pons
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Optic tract fibers project to ... lateral geniculate nucleus superior colliculus pretectal area suprachiasmatic nucleus
- most fibers to LGN - reflex gaze - light reflex - circadian rhythms
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berry aneurysms are associated with what congenital anomaly
coarcation of the aorta
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First place that is damage in the brain with thiamine deficiency
mammillary bodies for emotion and memory
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Unilateral tremor in UE at rest and improves during purposeful movements resting tremor rigidity bradykinesia postural instability (late finding)
Parkinsons disease - degeneration of nigrostriatal dopamine neurons resulting in decreased levels of dopamine in the striatum. This causes an imbalance of dopamine and acetylcholine (DA is too low and ACh is too high) tx involves increasing dopaminergic tone and decreasing cholinergic tone
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mechanism of cough syncope
increased intrathoracic pressure during a couching episode decreases venous return to the heart thereby transiently decreasing CO and cerebral perfusion
201
The most common cause of neontatal meningitis is
Group B strep (GBS) prevent by treating mother with IV ampicillin during labor also: E. Coli
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Caused by direct trauma to the lateral aspect of the skull Soon after the trauma, patient has a brief period of loss of consciousness which is followed by a lucid asymptomatic phase before further neurologic deterioration
EPIDURAL HEMATOMA resulting in the laceration of the middle meningeal artery classic biconvex hematoma due to cranial suture lines
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Trauma in vicinity of the petrous bone or along the sphenoid bone
Basilar skull fracture - hemotympanum (blood visible behind the tympanic membrane) - Battle sign (delayed ecchymosis over the mastoid process) - Raccoon sign (periorbital ecchymosis)
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Due to HTN, anticoagulant use, cerebral amyloid angiopathy, cocaine, and or methamphetamine abuse CT shows intraparenchymal brightness Focal neurologic signs
Intracerebral hemorrhage
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-Rupture of bridging veins between the meningeal dura and the superficial cerebral veins
Blood accumulates below the dura, producing symptoms of increased intracranial pressure classically present as a cresent shaped hematoma because it can cross suture lines
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Neurological complication of middle ear infection (otitis media)
cerebellar and temporal lobe abscesses CT shows a multilocular mass with ring-enhancing borders
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Isolated arcuate fasciculus lesion note: arises in Wernicke area and travels to broca area
conduction aphasia characterized by a striking inability to repeat language comprehension nd output are largely preserved
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Tricyclic antidepressant overdose
3 C's: cardiotoxicity (arrhythmias), convulsions, and coma
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Preterm infant with respiratory distress syndrome given oxygen therapy. Now has white pupillary reflex (leukocoria) other conditions with leukocoria? retinoblastoma and cataracts in rubella
Retinopathy of prematurity - occurs almost exclusively in preterm infants - strong association with high partial pressure oxygen therapy - Oxygen causes accelerated neovascularization in the retina because high oxyen results in vasoconstriction of retinal vessels. This resulting ischemia stimulates vascular endothelial growth factor (VEGF) production to compensate

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