Week 1 (Exam 1) Flashcards
(352 cards)
1
Q
Strong risk factors for taking triptans
A
Ischemic heart dz, Raynaud’s, HTN, hemiplegic or basal migraine, renal or hepatic impairment, MAOIs or other 5-HT1 agonists
2
Q
Bulbar ALS
A
Degeneration of lower brainstem cranial motor nuclei
Occurs early, progresses rapidly
Deglutination and phonation difficulties
3
Q
How do you treat an acetaminophen overdose?
A
Acetylcysteine: substitutes for depleted glutathione
4
Q
What do glucose levels look like in bacterial meningitis?
A
Decreased: below 50
5
Q
Acetaminophen MOA
A
Inhibits PGs in the CNS, but not in the periphery
6
Q
Bat-Wing lateral ventricles
A
Agenesis of the Corpus Callosum
7
Q
Toxicities of Carbamazepine
A
Induces CYP450 (induces auto-induction) Leukopenia / neutropenia / thrombocytopenia
8
Q
What does Tau do?
A
Hyperphosphorylated, Axonal Microtubule-Associated protein that enhaces its assembly
MAP2 and Ubiquitin?
9
Q
What brain regions are primarily affected by CO poisoning?
A
II and V of cerebral Cortex
Sommer’s Sector
Purkinje cells
Bilateral Necrosis of Globus Pallidus
10
Q
What anti-epilleptic drugs cause accumulation of the parent drug (especially when used together)?
A
Valproic Acid and Lamotrigine
They inhibit conjugation of drugs by UGT
11
Q
Most common sources of metz to the brain (highest to lowest)
A
Lung, breast, melanoma
12
Q
Neurofibrillary Tangle Histology
A
Pyramidal Neurons: “Flame”
Round Nucleus: “Globose”, Basket Weave around it
Basophilics: Bielschowsky Stain (silver stain)
Resistance to clearance in vivo: “Ghost” or “tombstone”
13
Q
3 etiologies of Hydrocephalus
A
Increased production (in choroid plexus) Obstruction (Intraventricular foramina, congenital, infections) Decreased absorption
14
Q
Where do people usually get brain tumors?
A
Adults are supratentorial
Kids are infratentorial, cerebellum and brainstem
15
Q
GAD-stimulating drugs (pre-synaptic)
A
Valproic Acid
Gabapentin
Pregabalin
16
Q
Schwannoma
A
Cerebellar Pontine Angle
Acoustic Neuroma: CN VIII, Tinnitus and Hearing loss
NF2: Loss of Merlin
17
Q
Pyogenic Meningitis
A
Supprative exudate covering brainstem and cerebellum
18
Q
Toxicities of Donepezil
A
Altered cardiac conduction
N/V diarrhea
Abrupt discontinuation problems
can worsen peptic ulcer, asthma/COPD, bladder outflow obstruction
19
Q
What neoplastic disease is associated with HIV?
A
Primary CNS Lymphoma
20
Q
What does Leptomeningeal fibrosis cause?
A
Hydrocephalus
21
Q
Source of Embolus involving Patent Foramen Ovale
A
From Venous System, Bypasses Lungs via PFO straight to Brain: Paradoxical
22
Q
Gaba-T inhibiting Drugs (pre-synaptic)
A
Vigabatrin
Valproic Acid
23
Q
Protozoa causing Chronic Granulomatous Meningoencephalitis
A
Acanthamoeba
24
Q
Sites of primary thrombosis
A
Carotid Bifurcation
Origin of MCA
Either end of Basilar A
25
Presentation of NMDA encephalitis
Most commonly in young or middle aged women
| Commonly associated with teratoma
26
Safinamide
Reversible MAO-B inhibitor for Parkinsons
27
What 4 drugs are associated with Osteopenia / Osteoporosis and why?
```
Carbamazepine
Phenytoin
Phenobarbital
Valproic Acid
Induce CYP450-dependent Vitamin D catabolism
```
28
HSV-1 Encephalitis
Children and young adults
Necrotizing and Hemorrhagic
Inferior and Medial Temporal Lobes (cingulate gyrus)
Cowdry Type A intranuclear viral inclusions
29
Donepezil MOA
Reversible, noncompetitive inhibition of centrally active acetylcholinesterase to treat Alzheimer dementia (off label for other types
30
How to treat sialorrhea associated with parkinsons
Botulinum toxin A injection into salivary glands
31
Black box warnings for Dihydroergotamine
All ergot alkaloids are contraindicated with potent inhibitors of CYP3A4
Concominant use associated with increased risk of vasospasm leading to cerebral/extremity ischemia
32
Tolcapone MOA
Selective and reversible inhibitor of COMT (degrades levodopa when decarboxylase is blocked)
33
Memantine MOA
NMDA receptor antagonist: decreases glutamate via Mg site binding
Tx Alzheimers, can combo with Donepezil
34
Turcot Syndrome:
Medulloblastoma or glioblastoma
| Mutations in APC or mismatch repair genes
35
Etiology of Vasogenic Edema
Often follows Ischemic Injury
36
B12 deficiency presentation
Anemia, Numbness, Tingling and slight Ataxia in LE
Rapid progressio to spastic weakness of LE
Becomes Complete paraplegia (point of no return)
37
Tension headache presentation
Mild to moderate (does not prohibit daily activity)
Bifrontal, Bioccipital (neck, shoulders, band-like)
Dull, aching, squeezing, pressure
No prodrome or aura
38
How does Central Chromatolysis come about?
Nissl removed from center of cell to periphery
39
Classic migraine presentation
Aura: 15-30 minutes (visual sx or other neuro)
40
Kernohan's Notch Phenomenon
Transtentorial Herniation presses the cerebellar peduncle against the tentorium cerebelli.
Ipsilateral Hemiparesis or Hemiplegia (Right herniation = left peduncle notch = right motor impairment)
41
Duloxetine MOA
Tricyclic Antidepressant: NE and 5HT reuptake inhibitor
42
Lasmiditan MOA
High affinity and selective for 5HT1F receptor agonist
Decreases stimulation of trigeminal system
Treats migraines without causing vasoconstriction
43
Warm, fresh water brain-eating ameba
Naegleria Fowleri
44
Pathophysiology of CTE
Accumulation of p-Tau as neurofibrillary tangles, b-Amyloid
| Depigmentation of Substantia Nigra
45
Etiology of Primary CNS Lymphoma
Immunosuppressed (think AIDS)
| CD20+ B cell origin (often EBV infected)
46
What does M. Tb do in chronic meningitis?
Obliterative Endarteritis in Subarachnoid Space (gelatinous or fibrinous exudate)
Obliterates Cisterns and encases CNs (CN sx)
Tuberculoma (inactive lesions can Calcify)
Stains Acid Fast
47
GAT-1 inhibiting Drug (pre-synaptic)
Tiagabine
48
Felbamate MOA
NMDA receptor antagonist (for epilepsy)
49
Histology of AT/RT
+EMA and Vimentin
Divergent differentiation
Rhabdoid cells (eosinophilic cytoplasm with sharp borders and eccentric nucleus)
50
What cells are responsible for demyelination seen in MS and how?
TH1: secrete IFN-y which activates Macrophages (PAS+)
TH17: recruited Leukocytes
CD4
51
Ependymoma presentation
First two decades of life
4th ventricle is decrete, exophytic, enhancing
Supratentorial (cystic and paraventricular)
Ependymal rosettes (true) more dx than perivascular
52
Paretic Neurosyphilis
Insidious, associated with mood alterations, delusions of grandeur, ends with dementia
Associated with Granular Ependymitis (proliferation of subependymal glia under damaged ependymal lining, hydrocephalus)
53
What causes PML?
JC Polyomavirus
54
Subfalcine (cingulate) Hernia
Cingulate Gyrus displaced under the falx
55
How does aspirin become toxic?
Salicylates uncouple ox phos
Respiratory center thinks there's no ATP
Hyperventilation, Resp Alkalosis
Depleted Bicarb, Metabolic Acidosis
56
Ziconotide black box warnings
Severe psychiatric sx and neurological impairment
| Hightened risk of harming patient when used in error
57
Choroid plexus papilloma
Most common in children, lateral ventricles
| Hydrocephalus: obstruction or CP carcinoma (more CSF)
58
Hirano Bodies
Elongated Glassy Eosinophilic Bodies, Lots of Actin
| Abundant in Alzheimers
59
What drug is both a Na channel blocker and an AMPA receptor blocker?
Topiramate
(the other is perampanel, but just does AMPA blocking)
This treats epilepsy
60
What complication of Meningococcemia is deadly?
Adrenal Hemorrhage
| This is Waterhouse-Friderichsen Syndrome
61
What parts of the spine are involved in Tabes Dorsalis?
Gracile Fasciculus: Ipsilateral Leg
| Cuneate Fasciculus: Ipsilateral Arm
62
How does capsaicin treat pain?
Stimulates TRPV1 to deplete substance P
Same as Camphor
Menthol does TRPM8
63
Treatment for trigeminal neuralgia
Carbamazepine or Oxcarbamazepine
64
Indications for Tramadol
Management of severe pain
| Off label for RLS and premature ejaculation
65
Meningioma WHO 1 presentation
Compresess brain, penetrates bone
Radiation induced
Usually happens after your 30s
66
Acetaminophen benefits
does not combat inflammation: No GI ulcers, No platelet suppression, No renal impairment
67
Testing for MS
MRI of the head, CT, Spine: Ovoid lesions
| Lumbar puncture: Oligoclonal bands and/or high IgG
68
Red Stroke
Hemorrhagic, Intra-cerebral or sub-arachnoid
Emboli associated, Venous
Secondary to Reperfusion
69
Primidone
Treats non-PD tremor
| Barbiturate and anticonvulsant
70
Histology of Leigh Syndrome
Spongiform periventricular brain tissue with vascular proliferation
71
Anterior Cerebral Arterydeficits
```
Alien Hand
Contralateral Leg (more than arm or face)
```
72
Where does CMV localize?
Paraventricular Subependymal Regions
| Causes Radiculoneuritis in lower spinal cord and roots
73
Apomorphine
DA agonist, sublingual film or sub Q injection for episodes (Parkinson's)
74
Drug classes that enhance Post-Synaptic GABA-ergic neuronal transmission
1. Barbiturates (phenobarbital / Primidone)
2. Benzos
3. Topiramate
75
Carbidopa MOA
Immediate precursor to DA, but can cross BBB
Peripheral DOPA decarboxylase inhibitor
Can combo as Carbidopa + Levodopa + Entacapone
76
SSPE
Widespread Gliosis and Myelin Degeneration
| Viral inclusions, NF tangles
77
Findings in Pick Disease
Asymmetric atrophy of Frontal and Temporal Lobes with Sparing of Posterior 2/3 of superior temporal gyrus
KNIFE-EDGE thin GYRI
78
What shape is Neisseria meningitidis?
G- Diplococci
79
White Stroke
Bloodless, Thrombus associated w/ atherosclerotic lesions
| Arterial
80
What does PML preferentially affect?
Oligodendroglial cells: demyelination of subcortical area
81
Treat Parkinsons patientorthostatic HPTN
a-Agonists and mineralocorticoids
82
Toxicities of CArbidopa
GI effects, Postural HPTN, Arrhythmias, etc
| Wearing-off and On-Off Phenomena
83
Fronto-Temporal Dementias WITHOUT Tau pathology
Vascular Dementia
84
Genetics of Friedreich Ataxia
AR GAA trinucleotide repeat on Chr 9q13
| FRATAXIN protein
85
Presentation of TSC
Hamartomas Renal Angiomyolipomas, cardiac rhabdomyomas, cutaneous lesions, Candle guttering (drop-=like masses bulge into ventricles)
86
Presentation of Parkinson Dz
Diminished Facial Expression
Festinating Gait (short, fast steps)
Pill-Rolling Tremor
Lewy bodies
87
Phenytoin adverse effects
Induces CYP450
Gingival Hyperplasia
Hypocalcemia / Vit D/ Osteoporosis
Zero-Order: Saturable pharmacokinetics
88
Celecoxib MOA
Inhibits COX2 only
89
When treating restless leg, how do you choose which therapy to give which patients?
Comorbid depression / obesity: DA agonist (ropinirole)
No comorbidies, pain, anxiety, insomnia, addiction problems: a-2-delta Ca channel ligand (gabapentin)
Intermittnent disabling: DA agonist, C/Levodopa, benzos
Pregnant: Fe supp/non-pharm, Clonazapam, C/Levodopa
90
Toxicities of Memantine
Confusion, Dizziness, Headache
Skin Hypersensitivity rxn
Avoid abrupt discontinuation
91
Neuromyelitis Optica
Synchronous Bilateral Optic Neuritis and Spinal Cord Demyelination
Ab to Aquaporins (maintain astrocyte food processes, blood brain barrier)
Try to Tx by decreasing Abs w/ Plasmapharesis
92
Marantic Emboli
Proteinaceous
| From Non-Bacterial Thrombotic Endocarditis, Hypercoaguable state (like advanced malignancy)
93
Signaling groups of Medulloblastoma (IV/IV)
WNT (older kids, Chr 6, b-Catenin, 90% survival)
SHH (MYCN amp, intermediate between WNT and 3/4)
Grp 3: MYC amp and 17(i17Q), infants and children, worst
Grp4: I17Q, no MYC, +/- MYCN, intermediate
94
Histology of Wericke Encephalopathy
Foci of hemorrhage and necrosis in mammillary bodies and walls of 3rd and 4th ventricles
95
Tumor grading
I: low proliferative potential, try resecting it
II: infiltrative, cytological atypic
III: Anaplasia and Mitoses (Radiation / chemo)
IV: Microsvascular proliferation / necrosis (fatal)
96
How do you treat Status epileptics?
1. IV Lorazepam or Diazepam, 3-5 minutes add more
2. IV Fosphenytoin + Valproic Acid + Levetiracetam + Phenytoin
(no IV access? Midazolam)
97
Pilocytic Astroma appearance
```
Well-circumscribed, Cystic with mural nodule
Hair-like with long bipolar processes
Rosenthal Fibers (long standing gliosis)
Eosinophilic Granular Bodies
GFAP positive
Bi-phasic: Loose Glial, Dense cellular
```
98
Indicators of diffuse axonal injury
Silver stain or amyloid precursor protein and a-Synuclein immunostains
Axonal Spheroids
99
What are neutrophil levels like in Bacterial Meningitis?
Over 100
100
Presentation of Friedreich Ataxia
Begins in first decade of life with Gait ataxia
Cardiac Arrhythmias, DM in 10%, Wheelchair 5 years in
Intercurrent Pulmonary Infections & Cardiac Dz
101
Why are barbiturates more lethal than benzos?
High doses of Barbiturates are GABA independent (benzodiazepines are GABA Dependent)
102
Receptors expressed on mengiomas
70% express Progesterone, 31% express estrogen receptors
| this means that many have relief after delivering a baby
103
Selegiline MOA
Irreversible MAO inhibitor (B>A)
104
Genetics of pilocystic astrocytoma
First 2 decades of life, cerebral hemispheres or cerebellum
| NF1 predisposes, loss of Neurofibromin in tumor
105
MOA of Topiramate
1. GABAa Agonist (increases frequency)
2. Fast inactivation of Na channels
3. AMPA receptor Antagonist
106
Drug that has a side effect of Permanent, progressive, bilateral, concentric vision loss?
Vigabatrin: prescribable only via REMS program
107
HSV-2 Encephalitis
50% of neonates born by vaginal delivery to women with active primary HSV develop this
108
Fronto-Temporal Dementias WITH Tau pathology
Pick Disease
| Progressive Supranuclear Palsy
109
How to treat psychosis associated with parkinsons
Quetiapine and clozapine, pimavanserin (5-HT2a inverse agonist)
110
7 responses to the increased CSF in hydrocephalus
PAPILLEDEMA
Absorption in transventricular and nerve root sleeves
Dilation of frontal and temporal horns
Elevation of Corpus Callosum
Thinning of cerebral mantle
Stretching, perforating Septum Pellucidum
Enlargement of Third Ventricle Downward
111
Tramadol MOA
Codeine analog: Partial u opioid agonist
| NE and 5HT reuptake inhibitor (primary MOA)
112
Brain tumor with Rosenthal Fibers and teeth. It looks like machine oil when you drain it. What is this a reaction to?
Reaction to craniopharyngioma (this is astrogliosis)
113
What does Pneumococcal Meningitis cause?
Chronic Adhesive Arachnoiditis
114
Genetics of ALS
SOD1 dysfunction on Chr 21
| Also TDP-43 is implicated on the chart
115
Signaling components of Limbic Encephalitis
ANNA-1 Ab: Anti Hu, Small cell lung CA
NMDA receptor: Ovarian Teratomas
VGKC-complex Ab: Peripheral neuropathy
116
Which Leukodystrophy is X-Linked?
Most are AR, but Adrenoleukodystrophy is X-Linked
117
Hygroma
Separation of arachnoid from dura from contraction of underlying brain parenchyma s/p infarct
118
Clinical applications of benztropine
Parkinsons: tremor and dystonia in younger people, without cognitive impairment
Reduces excessive saliva, drooling
119
6 agents that prolong Fast-Inactivation state of Na channels (for epilepsy)
```
Carbamazapine (and other azepines)
Lamotrigine
Phenytoin
Topiramate*
Valproic Acid
Lacosamide (and other amides)*
```
120
Source of Embolus causing Dissection of Carotid or Vertebral
Atherosclerosis of Aortic Arch
121
Ketamine MOA
Non-competitive NMDA receptor antagonist (blocks glutamate)
122
Toxicities of Celecoxib
Same as Ibuprofen
123
Genetics of Secondary Glioblastoma
Preceded by lower grade lesion (TP53)
| IDH1 (R132H mut has way better px) and IDH2
124
MERRF presentation
Myoclonus, Seizure Disorder, Myopathy
Ataxia (neuron loss from cerebellar system)
Ragged Red Fibers
125
"The scream" on histology
Ependymoma
126
Neurofibromatosis 1 genetics and function
AD 17q11 NF1 mut for neurofibromin gene
| Usually regulates RAS signaling
127
Black box warnings for Ibuprofen
Fetal Renal Dysfxn, oligohydramnios ~20 wks
Closure of Ductus Arteriosus at 30+ weeks
Risk of MI, Stroke, contraindicated in CABG
Increased GI Bleeding
128
AT genetics
AR Chr 11q22-q23 making ATM gene
| Fails to remove cells with DS DNA breaks
129
Pramipexole
D2 agonist like ropinirole
| Decreased HPTN and somnolence but more hallucinations
130
Syndromes that comes with Thiamine (B1) deficiency
Wernicke: Psychotic, ophthalmoplegia, reversible
Korsakoff: Confabulation, irreversible
Beriberi: cardiac failure
131
Tapentadol
Stronger opioid, works at mu and blocks NE reuptake
132
What is the main thing affected in hypertensive cerebrovascular disease?
Lenticulostriate arteries: Lacunar Infarcts (Pure motor hemiparesis)
Thalamic Lacunae: Contralateral Sensory Deficits
Basal Ganglia Lacune: Hemiballismus
133
Indications for Sumatriptan
Moderate to severe migraine
134
Histology of PML
Irregular white matter destruction:
Subcortical area of demyelination with lipid-laden macrophages in the center with fewer axons
Enlarged oligodendrocyte nuclei (filled with virus parts)
Reactive Astrocytes
135
Presentation of Leukodystrophies
Diffuse involvement of White matter
| Deterioration of motor skills, spasticity, hypotonia or ataxia
136
Tonsillar Hernia
Cerebellar tonsils displaced through Foramen Magnum
| Life Threatening
137
VHL gene, lesion, features
VHL on chronic 3p25.3, decreases HIF-1, more VEGF
| RCC, Hemangioblastoma, Pancreatic Endocrine Neoplasm, Polycythemia
138
Clonidine
a2-adrenoreceptor agonist used for HTN and as epidural pain relief
139
Antoni B Schwannoma
Hypocellular, Myxoid extracellular matrix
140
Vasogenic vs Cytotoxic Edema Fluid Dynamics
Vasogenic Increases Extracellular Fluid
| Cytotoxic increases Intracellular Fluid
141
How does rabies get to the brain?
Binds at Ach receptors and ascends via motor nerves
142
What drug is only used for absence (petit mal) seizures?
Ethosuximide
| Only inhibits T-Type Ca Channels
143
Where do most AVM's occur?
Subarachnoid s[ace involving MCA and Posterior Branches
144
CSF has High WBC, High protein, and normal glucose
Brain Abscess
145
Antoni A Schwannoma
Spindle Cells, Verocay bodies (palisading nuclei around nuclear free zones)
146
How to identify medulloblastoma (IV / IV)
Cerebellum, midline, occludes CSF flow
| Sheets of anapestic cells, abundant mitoses, homer wright rosettes
147
Soap Bubbles in brain
Cryptococcus
148
What slows and reverses Cerebral Edema?
Corticosteroids
149
Subdural Emphema
Infection of skull bones or sinuses, spreads to subdural space. Thrombophlebitis of bridging vessels can lead to infarct
150
Periventricular Leukomalacia
Infarcts in Supratentorial White Matter
| Pre-mature infants, chalky yellow plaques = necrosis and calcification
151
Glioblastoma WHO grade
IV / IV
152
All Anti-Epileptic drug warnings
Abrupt withdrawal can cause status epileptics
| Suicidal behavior and ideation
153
What does Borrelia Burgdorferi (lyme dz) do in chronic meningitis?
Rash, progresses to neuro sx in ~4 weeks
CN palsies and peripheral neuropathies
CSF: Abs, can cross rxn w/ mono, RA, SLE, etc
154
What is the significance of an X chromosome Filamin A and Double Cortin Protein mutation?
Males: Lissencephaly
Females: Subcortical band heterotopias
155
How does Astrogliosis present on histology?
Gemistocytes and Dead Red Neurons, Rosenthal Fibers
156
What regions are affected by methanol poisoning
Selective bilateral putamen necrosis
157
PCA-1 Ab
Subacute Cerebellar Degeneration
| Anti-Yo, women with ovarian, uterine, breast CA
158
Primidone MOA
Barbiturate
159
How to slow cognitive decline associated with Parkinsons
Rivastigmine or donepezil (cholinesterase inhibitors)
| Memantiine (NMDA antagonist)
160
Atypical II/IV vs Anaplastic (malignant) II/IV Meningioma WHO1
Atypical: worse, clear and chordoid
Anaplastic: Papillary/Rhaboid subtypes, more mitoses
161
Amitriptyline MOA
Tricyclic Anti-depressant
| Inhibits NE and 5HT reuptake (off label for neuropathic pain)
162
Macrophages are often in the CNS upon demyelination. What are these markers?
CD68 and CR3
163
Huntington's Brain
Decreased body of corpus callosum
Decreased head of caudate nucleus (putamen later)
Decreased Globus Pallidus
164
Ubrogepant MOA
Calcitonin gene related peptide receptor antagonist
Inhibits pathologic dilation of intracranial arteries without unwanted vasoconstriction
Treats migraines without aura in adults
165
Presentation of ALS
```
50's
Early asymmetric weakness of hands - Dropping stuff
Fasciculations
Progressive Muscular Atrophy
Progressive Bulbar Palsy
```
166
If non-aspirin NSAIDs are reversible, why should you use as little as possible?
They suppress platelet aggregation, but increase risk of MI and stroke
167
Toxicities of Amitriptyline
Black box warning for suicidal thinking and behavior in young people (all tricyclics)
168
Dihydroergotamine MOA
Binds serotonin, adrenergic, and DA receptors
| Constricts blood vessels for migraines
169
Tabes Dorsalis
Sensory nerve dmg in dorsal roots
Locomotor Ataxia, Loss of Pain Sensation, Charcot (damaged) Joints, Lightning Pains, Absence of DTRs
Form of Neurosyphilis
170
Arnold Chiari Malformations
I: Silent, may impair CSF flow
II: Vermis Pushed through Foramen Magnum
Usually associated with Myelomeningocele
171
MAO-A vs B
A preferentially metabolizes Norepi and 5HT
| B preferentially does Phenylethlyamine and Benzylamine
172
What cells are dominant in meningitis?
Earl on, PMNs. Late, Lymphs
173
Pinecytoma vs Pineblastoma
Pinecytoma: well differentiated, LG usually adults
Pineblastoma: High grade, necrosis, mitoses, HG, usually kids, RB gene, spreads throughout CNS
174
What is the major difference in targets between leukodystrophies and mitochondrial encephalomyopathies?
Leukodystrophies involve White Matter
| Mitochondrial Encephalomyopathies involve Gray Matter and Skeletal Muscle
175
Ziconotide MOA
selectively binds N-type voltage sensitive Ca channels on nociceptive afferent nerves of dorsal horn
176
What is associated with chronic / malignant HTN?
Charcot-Bouchard Microaneurisms in basal ganglia
177
Pilocytic Astrocytoma WHO grade
I / IV
178
Most common Pineal Tumor
Germinoma
179
Tay Sachs Presentation
Child born fine until 1 year old, degenerates mentally and physically until death at 2-3 years old
Cherry Red Spots in Maculae
180
Acute Necrotizing Hemorrhagic Encephalomyelitis (ANHE)
Demyelination in young adults and children with recent upper respiratory infection
Fatal in most, deficits in survivors
181
Neurofibromatosis 1 gene, lesion, features
NF1, Pheochomocytoma lesion
| Neurofibromatosis, Cafe-au-lait spots, optic nerve glioma, iris nodules (lische)
182
Genetics of AT/RT
Over 90% have Chr 22, HSNF5/INI1
| Under 5 years old is highly aggressive, fatal within a year
183
What do protein levels look like in bacterial meningitis?
50 - 1000 (increased)
184
Internal Carotid Occlusion / Stenosis
ACA-MCA Watershed Infarct - Cortical border zone
Proximal arm and leg weakness
Transcortical aphasia (lots of starts and stops)
185
Source of Emolus causing A Fib
Left Atrial Appendage
186
Posterior cerebral artery deficits
Contralateral homonymous hemianopia
187
Indications for Amantadine
Tx Parkinson's drug-induced extrapyramidal sx
Adjunctive for dyskinesias w/ levodopa
Monotherapy for mild motor sx
188
Genetics of Oligodendroglioma
IDH1 and 2 isocitrate dehydrogenase (most common and favorable px)
1P19Q loss, favorable px
189
Clinically isolated syndrome
Monofocal or Multifocal (acute disseminated encephalomyelitis / ADEM)
190
Riluzole
Only drug to impact ALS survival (months)
Unknown mechanism, inhibits glutamate release, blocks postsynaptic NMDA and Kainite-type glutamate receptors, inhibits voltage- dependent Na channels
191
Heubner Arteritis
Obliterative Endarteritis associated with Meningovascular Neurosyphilis (causes communicating hydrocephalus)
192
What kind of cells do you see in chronic Aseptic Meningitis caused by HIV?
Microglial Nodules with Multinucleated Giant Cells
193
Bunina Bodies
PAS+ Cytoplasmic inclusions
Remnants of Autophagic Vacuoles
Found in ALS
194
Diastatic Fracture
Crosses a Stuture
| Later fracture lines don't extend across previous fracture lines
195
MOA of Ropinirole
Non-ergot
High affinity for D2 nd D3 DA receptors (
D3 relevance unknown, D2 increases DA
196
Histology of Oligodendroglioma
Perineuronal Satellitosis, Perivascular Aggregation, Subpial accumulation of tumor cells
Perinuclear Halos (fried eggs, artifact)
Delicate Anastamosing Capillaries (chicken wire)
197
Bilateral Schwannomas (CN VIII)
NF2
198
Where do the venous and arterial systems bleed into in the brain?
Arterial into the epidural space (usually skull fracture)
| Venous into Subdural space (usually slower)
199
Aspirin MOA
Irreversible COX 1 and 2 inhibitor
| Decreases PG's and TxA2
200
Source of Emolus causing Myocardial Infarction
Hypokinetic or Akinetic Areas
201
Cerebral Malaria
Caused by Plasmodium Falciparum
| Causes long-term cognitive defects in survivors
202
Histology of Kearn-Sayre Syndrome
Spongiform change in Gray and White Matter
| Neuronal Loss most evident in Cerebellum
203
What type of Brian tumors do people usually get
Adults get glioblastoma (new onset seizure)
| Children usually get medulloblastoma
204
Clinical manifestations of N Meningitidis
Petechia, Purpura, septicemia
| Hemorrhagic infarction of adrenal glands (Waterhouse Friderichsen Syndrome)
205
Histology of TSC lesions
```
Shagreen patches (localized thickenings)
Ash-Leaf patches (hypopigmented areas)
```
206
Pregabalin Indications
Neuropathic pain (diabetic, SC injury)
Post-herpetic neuralgia
Adjunct for seizures
Fibromyalgia
207
Where would you expect different bacteria to colonize different areas of the brain?
Pneumococcal Meningitis over convexities near saggital sinus, H Influenza usually basal
208
Amantadine MOA
unknown mechanism, but we do know its a weak, noncompetitive NMDA antagonist
(this is for parkinsons)
209
Bergman Gliosis
Proliferation of Astrocytes found in advanced ethanol abuse.
| Happens between depleted granular cell layer and molecular layer
210
Intraparenchymal Hemorrhage
In germinal matrix of premature infants
Junction between thalamus and caudate nucleus
goes into ventricles, then becomes sub-arachnoid
211
Where would you target for Device aided therapies for parkinsons
Subthalamic nucleus or globus pallidus interna
212
Colloid Cyst of 3rd ventricle
Young adults
Attached to roof of 3rd ventricle
Obstructs foramen of monro = non-comm. hydrocephalus
can be rapidly fatal, can be positional
213
What are Ependymal Cells?
Ciliated Columnar cells, line the ventricles
| Disruption causes granulations, damaged in CMV
214
Acute Poliomyelitis
Anterior Horn Motor Neuron Nuronophagia
| Causes Flaccid Paralysis
215
Pregabalin MOA
Binds a-2-d on voltage gated Ca channels (modulates Ca influx at nerve terminals)
Does not bind to GABA
216
Presentation of AT
Telangiectasias, Death in early 20s, Immunodeficiency
| Many develop Lymphoid neoplasms, gliomas, carcinomas
217
In practice, we often see elements of both vasogenic and cytotoxic edema. How does this present?
Flattened Gyri, Sulci Narrowed, Ventricles Compressed, can lead to HERNIATION
218
Imaging of Kuru Plaque
Congo red and PAS+
| Found in the cerebellum of vCJD cause by bovine spongiform encephalopathy
219
Neurofibromatosis 2 genetics and function
AD 22q12 NF2 mut for Merlin gene
| Usually integrates cytoskeletal signaling and acoustic neuromas
220
Rasagiline
MAO-B selective irreversible inhibitor with greater potency and similar adverse effects of Selegiline
(parkinsons)
221
Black box warning for Acetaminophen
Medication errors and overdose: Hepatotoxic, Death
222
DHE (Raskin) protocol for headaches
Metoclopromide / prochlorperazine IV 60 sec
wait 5 min
DHE IV 60 sec, wait 3-5 min. Repeat if needed 8 hrs
223
problems with CNS Ach muscarinic agonists given to tx parkinsons
Dementia (that's why you don't give it to old people)
224
Problems with CNS D2 receptors given to tx parkinsons
Psychosis
| N/V
225
Presentation of VHL
Dizziness, headache
Visual Loss
Unilateral Ataxia
226
Histology of HSV
Ground Glass Multi-Nuclei
Microglial Nodules
Intranuclear Inclusions
Margination of Chromatin
227
Cystic (medulloblastoma is solid), biphasic architecture, lower cellularity
Pilocytic astrocytoma
228
Identify chronic meningitis
Elevated protein, lymphocytes, low glucose for at least 4 weeks
M. Tuberculosis, Neuroborreliosis, Neurosyphilis
229
Botulinm Toxin A
Tx non-PD tremor
| Binds cholinergic terminals, decreases ACh
230
Clinical applications for dexmedetomidine
Sedation of mechanically ventilated patients in ICU
231
Source of artery to artery embolus
Stenosed ICA or Vertebral A
232
Propranolol motor indication
Non-PD tremor
233
CSF Findings in MS
Mildly elevated protein
Moderate Pleocytosis in 1/3
Increased IgG
Oligoclonal IgG bands (gamma region)
234
Black box warning for Tolcapone
Risk for fatal acute fulminant liver failure
| Only use in parkinsons w/ L-Dopa/carbidopa who have fluctuations and are not responding well to other stuff
235
Perinatal Ischemic lesions I the depths of the sulci, causing thinned-out glycolic gyri
Ulegyria
236
Identify a basilar skull fracture
Skull deformity, Raccoon eyes, Fluid drainage, Battle's Sign (bruised rear neck)
237
Cluster Headache presentation
```
severe, excruciating
F:M 1:6
Associated with obstructive sleep apnea
100% unilateral, usually orbitotemporal
Frenetic, pacing, rocking
Ipsilateral ptosis, mitosis, conjunctivitis, lacrimation, runny or stuffed nose
```
238
What is the K+ channel opener used to treat epilepsy?
Ezogabine
239
Presentation of MELAS
Recurrent episodes of acute neurons dysfunction, cognitive changes
Muscle involvement with weakness and lactic acidosis
Stroke-Like episodes: reversible, not corresponding to specific vascular territories
240
What cells do you see in hepatic encephalopathy?
Alzheimer's Type II
241
Thickened leptomeninges
Causes Obstructive Hydrocephalus
242
Diagnostic sx of Rabies
Paresthesias around wound
| also has negri bodies in purkinje cells
243
Histology of Prion Disease
Spongiform Change: intracellular vacuoles in neurons and glia
244
The only drug available for Primary Progressive M.S.
Ocrevus
245
Multiple System Atrophy system involvement
```
Striatonigral Circuit (results in Parkinsonism)
Olivopontocerebellar circuit (results in ataxia)
Autonomic NS (dsfxn like orthostatic HPTN)
```
246
Why does CADASIL cause recurrent strokes and dementia?
Thickening of the media and adventitia, loss of SM cells, Basophilic PAS+ Deposits
247
Adverse effects of amatadine
```
CNS depression
Impulse control disorders
Psychosis
Suicidal Ideation / depression
Lived reticular (discoloration, usually legs)
```
248
How to treat Parkinsons associated orthostatic HPTN
Midodrine (a-adrenergic agonist), Doperidone (peripheral D2 antagonist), Fludrocortisone (Mineralocorticoid)
249
Adverse effects of aspirin
Ulcer, Bleeding, Drug interactions
Reye Syndrome in children
Increased Serum Creatinine
250
Dysphagia, Dysarthria, Diplopia, Loss of Consciousness
Central Pontine Myelinolysis
Overly rapid correction of hyponatremia, severe electrolyte imbalance
Demyelinates Basis Points and Pontine Tegmentum symmetrically
251
Wet, Wacky, and Wobbly
Normal Pressure Hydrocephalus
252
NMDA encephalitis has at least one of these
```
Abnormal EEG (extreme delta brush, epileptic, focal or diffuse slow or disorganized activity)
CSF with pleocytosis or oligoclonal bands and/or NMDA abs
```
253
What kind of brain necrosis is associated with hypoglycemia?
Pseudolaminar Necrosis. Also selective for the large pyramidal neurons of cerebral cortex
254
Transtentorial (uncinate, uncle) Hernia
Medial Temporal Lobe pressed against Tentorium
| CN3: Dilated Pupil and Impaired Eye Movement
255
What causes Chronic Meningitis?
Tuberculosis, Spirochetes (neurosyphilis and neuroborreliosis) or Cryptococcus
256
MELAS genetics
Mitochondrial inheritance usually tRNA mutation
257
Necrotic zone of toxoplasmosis
Free Tachyzoites and Encysted Bradyzoites at periphery
258
Other reversible AChE inhibitors for dementia (besides doneepzil)
Rivastigmine: transdermal option
| Galantamine
259
Genetics of MS
DR2 links susceptibility
| IL-2 and IL-7 receptor genes
260
Trigeminal Autonomic Cephalgias
Unilateral trigeminal distribution of headaches
| Cluster Headaches, SUNCT syndrome (short, unilateral, neuralgiform, conjunctivitis, tearing), Paroxysmal hemicrania
261
Where do primary germ cell tumors occur?
Along Midline: Pineal (mostly men) and suprasellar
262
Benzo vs barbiturate MOA
Benzos open Cl- channel with greater frequency, barbiturates open Cl- channel with greater duration
263
CADASIL Genetics
AD NOTCH 3 Mutation (preferentially expressed in vascular smooth muscle)
264
Intranuclear Neuronal Inclusions
Herpes Cowdry Bodies, CMV (both intranuclear and cytoplasmic)
265
Histology Hallmarks of Glioblastoma
1. Necrosis (serpentine)
2. Pseudo-Palisading of cells around necrosis
3. Vascular / Endothelial proliferation
266
Intracytoplasmic neuronal inclusions
Lipofuscin
| Rabies Negri Bodies, Alzheimers NF Tangles, Parkinson Lewey Bodies, CJD
267
Gabapentin MOA
GABA analog that doesn't bind GABA receptors
| a2-d of voltage gated Ca
268
(frequent) initial presentation of MS
Unilateral visual impairment (10-50% develop MS)
269
Tay Sachs Genetics
HEXA gene ecoding Hexosaminidase A on Chr 15
| Never goes away: GM2 Gangliosides
270
How do you minimize the anti-platelet actions of Aspirin?
Take it 2 hours before other NSAIDs
| The other NSAIDS counteract the platelet effects
271
Expanded Roofless 4th Ventricle
Dandy Walker Malformation
Cerebellar Vermis absent or rudimentary, replaced with cyst that has ependymal lining contiguous with leptomeninges on outer surface
272
What drug both prolongs the fast-inactivation and enhances the slow-inactivation state of Na channels
Lacosamide (for epilepsy)
273
Duloxetine Indications
Off label for chemo-induced neuropathic pain, stress urinary incontinence
274
Brain abscesses in Toxoplasmosis
near gray white junction and deep gray nuclei
275
Rotigotine
D2 agonist like ropinirole
| 24 hour patch
276
Medications for acute exacerbation in MS
High dose Corticosteroids
| ACTH (actor gel) or IVIg who don't tolerate traditional steroids
277
Bromocriptine
Early DA agonist, ergot alkaloid derivative for parkinsons
278
Ziconotide indications
only for chronic severe pain patients where intrathecal administration is warranted and when refractory to other treatments
279
MERRF genetics
Mitochondrial inheritance
| Most tRNA mutations (can overlap with MELAS)
280
CSF in Chronic meningitis caused by cryptococcus Neoformans
Inda Ink Prep
Cryptococcal Polysaccharide Ag
Cellular immune dysfunction predisposes it
281
Identify Primary CNS LYmphoma
Multifocal, Periventricular spread
Accumulate around vessels
Hooping: cells separated by reticulin
282
Neurological deficits separated in time and separated in (white matter) space
MS. Gradual recovery, frequency decreases during course
283
Pathophysiology of ALS
Loss of LMNs in spinal cord and brainstem
Loss of UMNs that project into corticospinal tract
Loss of anterior horn neurons, thin anterior roots
284
What disorders do oligodendrocytes respond to?
PML: Intranuclear inclusions
| Multiple System Atrophy (MSA): Cytoplasmic inclusions / a-Synuclein
285
Genetics of Parkinsons
AD: a-Synuclein on Chr 4q21 causes CAG repeats
| Juvenile AR: PARKIN
286
What causes Subacute Sclerosing Panencephalitis (SSPE)
Paramyxovirus (measles [rubeola])
287
Sommer's sector
CA1 and CA2 regions of hippocampus
| Most vulnerable to hypoxia
288
Unfavorable form of oligodendroglioma (similar to glioblastoma)
Anapestic (III/IV): Vascular hypertrophy and necrosis
| Increase grade with increased N:C, Mitoses, cellularity
289
Gorlin Sydrome
Medulloblastoma
| Mutations in PTCH gene: up regulates SHH
290
NMDA encephalitis hits quick and has at least 4 out of these 6 sx:
Abnormal psych behavior or cognitive fxn
Speech dysfxn
Seizures
Movement disorder, dyskinesias, rigidity/ posture shit
Decreased level of consciousness
Autonomic dysfunction or central hypoventilation
291
What is CMV opportunistic of?
AIDS (fetuses and immunocompromised)
292
Genetics of Kearn-Sayre Syndrome
Sporadic Large Mitochondrial DNA deletion/rearrangement
293
Alien Hand Syndrome
Semiautomatic movements of contralateral arm not under voluntary control
Associated with ACA deficits
294
Common migraine presentation
```
Moderate to severe
Women 3:1
Unilateral
Throbbing / sharp / pressure
Retreatment to dark, quiet room
No Aura
```
295
Adverse effects of Ropinirole
```
Dyskinesias
Impulse control disorders
Increased melanoma risk
Orthostatic HPTN
Psychotic effects
Somnolence
```
296
Molar Tooth brain
Joubert Syndrome: Cerebellar Vermis Hypoplasia, Deepened Interpeduncular Fossa, Elongated Superior Cerebellar Peduncles
297
Presentation of Kearn-Sayre Syndrome
Cerebellar Ataxia, Pigmentary Retinopathy, Cardiac Conduction Defects
Progressive inability to move eyes and eyebrows
298
What Anti-epilleptic drugs induce conjugation by UGT and what does this mean?
Phenytoin, carbamazepine, phenobarbital
| Cause reduction of parent drug
299
Contrast Ring Enhancing Lesion with Hypodense Central Necrosis
Glioblastoma (IV/IV)
300
Dexmedetomidine MOA
Selective a2-adrenoreceptor agonist
Inhibits norepinephrine release
vasoconstriction
301
Most common cause of fungal meningitis
Cryptococcus
302
Leigh Syndrome Presentation
Early Childhood Lactic Acidemia, Seizures, Extra-Ocular Palsies and Weakness with Hypotonia
303
Sumatriptan MOA
Selective 5-HT1B and 5-HT1D agonist
| Activates receptors on intracranial bv and sensory nerves of trigeminal system
304
What drugs block the a2d subunit of P/Q type Ca channels?
Gabapentin and Pregabalin
305
Treatment for Paroxysmal Hemicrania
Indomethacin
306
Most important histopathologic indicator of CNS injury
Gliosis: Astrocyte Hypertrophy, Hyperplasia
307
Black box warning for Selegiline
Antidepressants increased risk of suicidal thoughts and behaviors
308
Aggregates recognized histologically as inclusions for three different degenerative CNS diseases
Huntingtoins: Polyglutamine Repeats
Alzheimers: B-Amyloid
Parkinsons: a-Synuclein
309
Progressive Supranuclear Palsy
Truncal Rigidity with other CNS findings
2x in men, 50-70 y.o., Fatal within 5-7 years
Widespread Neuronal Loss, 4R Tau Straight filaments making Globose Neurofibrillary Tangles
310
Post-Polio Syndrome
25 - 35 years after resolution of polio
| Progressive weakness with decreased muscle mass and pain
311
Li-Fraumeni Syndrome
Medulloblastomas, mutations in TP53
312
Where does Meningioma WHO 1 happen?
Dura based: meningothelial origin of arachnoid (EMA+)
| Intraventricular: from stroll arachnoid cells of choroid (Left Lateral ventricle favored
313
Dilated ventricles, shrinking brain substance, normal pressure
Hydrocephalus Ex Vacuo
314
Most common cause of bacterial meningitis
Strep Pneumoniae
315
Valproic Acid MOA
1. Na Channel Blocker
2. Pre-synaptic GAD stimulator
3. Pre-synaptic GABA-T inhbitor
316
Copper-reducing agents (for Wilsons disease)
Penicillamine: copper chelator
| K Disulfide: reduces intestinal absorption
317
Histology of Viral Encephalitis
Perivascular Lymphocytic Cuffs
| Microglial Nodules
318
What is the blood supply of the Internal Border Zone
Between LCA and MCA (watershed)
319
Middle Cerebral Artery deficits
Gaze Preference Toward Side of Lesion
Often have lacunas: small deep infarcts of penetrating branches
Aphasia, face-arm or other sensorimotor loss
320
Indications for Carbidopa
Parkinsonian Syndrome
| Off label for restless leg, other Parkinson-like conditions
321
Morphology of ADEM
Myelin loss with relative axon preservation
Early is PMN, Later is Mononuclear
Accumulation of lipid-laden macrophages (from myelin breakdown)
322
Devic's Disease (Neuromyelitis Optica)
Inflammation and demyelination of optic nerves and SC
Test for Aquaphorin Abs in Blood and CSF
Steroids / plasma exchange, then immunosuppression
323
Benztropine MOA
Cholinergic antagonist at muscarinic receptors like atropine (activates GABA)
Also blocks histamine receptors
324
Chronic Migraine presentation
15+ days/mo
4 hours+
At least 3 mos
325
4 cardinal features of Parkinsons
Bradykinesia
Muscular Rigidity
Resting Tremor
Impairment of postural balance: falls and bad gait
326
Genetics of Meningoma WHO 1
NF2 (22q12): most common
| TRAF7: second most common, lower grade, more stable
327
Black box warnings for Tramadol
```
Medication errors / OD
Addiction, abuse, misuse
Interactions with CYP450
Withdrawl
Respiratory depression (w/ benzos or other CNS stuff)
```
328
What happens with CMV in Utero?
Periventricular Necrosis, leading to Microcephaly and Periventricular Calcification
329
Areas associated with movement disorders in degenerative diseases
```
Basal Ganglia (esp Nigrostriatal Pathway)
Brainstem
```
330
What does CSF look like with a viral infection?
Clear and colorless
| Cloudy or turbid in bacterial meningitis
331
Large cells with paranuclear filamentous inclusions, problem with large cell Medulloblastoma
Atypical teratoid/Rhabdoid tumor
332
Appearance of Oligodendroglioma
Primarily cerebral hemispheres
| Calcification usually restricted to the cortex w/ curvilinear or pyriform distribution
333
What deficiency is strongly associated with alcoholism?
B1 / Thiamine
334
What are the two major responses of macrophages in the CNS?
Formation of Microglial Nodules
| Neuronophagia
335
Histology of B12 deficiency
Swelling of Myelin Layers, becoming vacuoles
Degeneration of Posterior Column Ascending Tracts and Descending Pyramidal Tracts = Subacute combined degeneration of SPC
336
Syringomyelia (Syrinx)
Fluid filled cleft-like cavity in inner portion of cord
| Loss of Pain and Temp sensation in upper body
337
Histology of Meningioma WHO 1
Calcified Psammoma Bodies
En Plaque: sheet like spread of tumor along a surface
Whoreld Clusters of Monotonous Cells
Secretory subtype has PAS+ droplets in cytoplasm
338
Perivascular Pseudorosettes, less cellular and more fibrillar than medulloblastoma
Ependymoma
339
What are extradural abscesses usually associated with?
Osteomyelitis
| Can cause cord compression (neuro emergency)
340
Cowden genetics and presentation
AD 10q23 for PTEN gene / protein (IK3/AKT signaling)
Lipid phosphatase/benign follicular appendage tumors
Internal adenocarcinoma (often breast or endometrial)
341
Ibuprofen MOA
Reversibly blocks COX 1 and 2
342
Indications for Selegiline
Adjunct for Parkinsons with Levo-/Carbidopa
| Off label for early parkinsons
343
What drugs are used to block synaptic Vesicle 2A protein to treat epilepsy?
Levetiracetam
| Brivarecetam
344
Acute Disseminated Encephalomyelitis (ADEM)
Perivenous and Monophasic, follows Viral infection (or rarely viral immunization)
20% die, Lethargy, Coma. Survivors recover completely
345
Glioma grading
Parameters: Nuclear Atypia, Mitoses, Necrosis, Microvascular proliferation
Grading: II (one parameter) III (anapestic, 2 parameters), IV (3-4 parameters)
346
Presentation of Lgl1 encephalitis
More commonly in men
Sleep disturbance half the time
Temporal lobe (esp hippocampal) abnormality
Faciobrachial dystonic seizures (unilateral)
347
What drug blocks T-Type Calcium channels in seizures and also blocks Na channels?
Zonisamidem
348
Entacapone
Nitrocatechol COMT inhibitor with 0.8 hour half life
| Cannot cross BBB: treats "off" sx
349
Trihexyphenidyl
Anticholinergic for parkinsons, like benztropine
350
Tuberous sclerosis genetics and function
AD 9q34 TSC1 gene for Hamartin mut
AD 16p13 TSC2 gene for Tuberin mut
Complex together to regulate mTOR/angiofibromas/mental retardation
351
MCA-PCA Watershed Occlusion
Cortical Border Zone
| Higher order visual processing deficits
352
What co-morbitity skyrockets your chances of having early onset alzheimers
Down Syndrome
