Neuro Flashcards

(648 cards)

1
Q

Multiple Sclerosis HLA association

A

HLA - DR2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Metachromatic Leukodystrophy missing enzyme

A

Arylsulfatase A - buildup of sulfatides leads to impaired degradation of myelin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Adrenoleukodystrophy pathophysiology

A

Impaired addition of CoA to LCFA. LCFA accumulate and destroy the adrenal glands and myelin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Krabbe’s Disease missing enzyme

A

Galactocerebrosidase. Buildup of galactocerebroside destroys myelin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Oligoclonal IgG bands on LP are seen with…

A

MS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Cause of SSPE

A

Slowly progressing, persistent measles infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Cause of Progressive Multifocal Leukoencephalopathy

A

Immunosuppressed patient - JC virus reactivation - infection of oligodendrocytes (it’s a bunch of bullshit)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

<p>Central Pontine Myelinolysis presents as...</p>

A

<p>Locked In Syndrome - patient can only move eyes and blink

| They have acute paralysis, dysarthria, dysphagia, diplopia and loss of consciousness</p>

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Cause of Central Pontine Myelinolysis

A

Rapid IV correction of hyponatremia (usually in alcoholics/malnourished)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

<p>Epsilon 4 allele of Apolipoprotein E</p>

A

<p>Increased risk for sporadic Alzheimer disease - late onset
ApoE4 is found on chromosome 19</p>

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

<p>Epsilon 2 allele of Apolipoprotein E</p>

A

<p>Decreased risk for sporadic Alzheimer disease
protective!
found on chromosome 14</p>

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

<p>Presenilin - 1 and Presenilin-2</p>

A

<p>Mutation results in early onset (familial) Alzheimer disease
note that presenilin-1 is found on chromosome 14</p>

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why does Down Syndrome increase Alzheimer Disease risk?

A

The amyloid precursor protein, which eventually results in production of A-beta-amyloid, is located on chromosome 21, which patients with DS have 3 of.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Components of neuritic (senile) plaque in AD

A

A-beta-amyloid, Entrapped neuritic processes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Cause of cerebral amyloid angiopathy

A

A-beta-amyloid depositing on blood vessels in the brain, weakening wall of vessel, increasing risk for hemorrhage. Seen with AD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Component of neurofibrillary tangles

A

Hyperphosphorylated tau protein (microtubule associated protein)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Second most common cause of dementia

A

Vascular/Multi-infarct dementia (behind AD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Component of Pick bodies

A

ROUND aggregates of tau protein (microtubule associated protein that helps the microtubules arrange properly) in neurons of cortex.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Diffuse atrophy of the frontal/temporal lobes

A

Pick disease (frontotemporal dementia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

MPTP leads to

A

Parkinson disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Composition of Lewy bodies

A

alpha-synuclein. Seen with Parkinson disease and Lewy-Body dementia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

When (chronologically) is dementia seen with Lewy body dementia vs. Parkinson disease?

A

EARLY onset (onset before or concomitant with tremor) with LBD; LATE onset (long after tremor onset) with PD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

CORTICAL Lewy bodies are seen in…

A

Diffuse Lewy body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Efferent neurons leaving the striatum use which NT?

A

GABA (inhibitory effect on cortex)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Expansion of the lateral ventricles due to brain atrophy
Hydrocephalus ex vacuo
26
What is the trinucleotide repeat in Huntington disease?
CAG (HUNT animals in a CAGe).
27
Why does anticipation happen in trinucleotide repeat diseases?
Expansion of the repeat during SPERMATOGENESIS.
28
Cause of symptoms in normal pressure hydrocephalus
Stretching of corona radiata
29
What will a LP do for NPH?
Improve symptoms because the disease is caused by increased CSF
30
Pathophysiology of NPH
Ineffective resorption of CSF into the arachnoid granulations
31
What is the conformation of abnormally folded proteins in spongiform encephalopathies?
Beta-pleated sheets
32
Spike wave complexes on EEG is indicative of...
CJD
33
Where do adult/child CNS tumors usually occur with respect to the tentorium cerebelli?
Kids - below and Adults - above
34
Adult CNS tumor that crosses the corpus callosum
GBM
35
Butterfly glioma
GBM
36
Adult CNS tumor with central necrosis surrounded by living cells
GBM
37
GBM histology
Pseudopalisading appearance (central necrosis with living cells surrounding it) with endothelial proliferation
38
Cells which make up a GBM
Astrocytes
39
Stain for GBM
GFAP (made of astrocytes)
40
Why are meningiomas seen more in females?
Estrogen most likely plays a role in tumor formation
41
Whorled appearance of cells on CNS tumor biopsy
Meningioma
42
CNS tumor showing psammoma bodies
Meningioma
43
S100 Positive CNS tumor
Schwannoma
44
CNS neoplasm associated with NF2
Bilateral acoustic schwannomas
45
Meiningioma histology
Whorled pattern with psammoma bodies
46
Calcified tumor in frontal lobe white matter in adults that presents with seizures
Oligodendroglioma (note: calcified, but NOT psammoma bodies)
47
Fried egg appearance on CNS neoplasm biopsy
Oligodendroglioma
48
Chicken wire capillary pattern on CNS neoplasm biopsy
Oligodendroglioma
49
Histology of oligodendroglioma
Fried egg appearance, may be calcified (NOT psammoma bodies), with "chicken wire" capillary pattern
50
#1 CNS tumor in kids
Pilocytic astrocytoma
51
Classic gross description of a pilocytic astrocytoma seen on imaging
Cystic lesion (piloCYSTic astrocytoma) with a mural nodule connected to it. Usually seen in cerebellum in kids.
52
Rosenthal fibers
Thick, eosinophilic corkscrew fibers seen in pilocytic astrocytoma
53
Staining for pilocytic astrocytoma
GFAP
54
Medulloblastoma is derived from (embryologically)
Neuroectoderm
55
Histology of medulloblastoma
Small, round blue cells with Homer-Wright rosettes.
56
Homer-Wright Rosettes
Medulloblastoma. Small round blue cells wrapping around neuritic processes.
57
CNS tumor showing small round blue cells wrapping around neuritic processes
Medulloblastoma
58
Childhood CNS tumor which can invade the spinal cord ("drop mets")
Medulloblastoma
59
Cheesy mnemonic for medulloblastoma
David Wright blasts a Homer (medulloBLASToma w/ Homer-Wright rosettes) and drops the Mets (Drop Mets). Mets Suck, go Phils
60
Childhood CNS tumors that can compress the 4th ventricle and cause hydrocephalus
Medulloblastoma (spreads via CSF), ependymoma
61
Primary location of ependymoma
4th Ventricle in children
62
Perivascular pseudorosette
Seen in ependymoma - tumor cells line up around blood vessels
63
Childhood CNS tumor in which tumor cells line up around blood vessels
Ependymoma. This is a description of perivascular pseudorosettes.
64
Origin of craniopharyngioma (embryo)
Oral epithelium - Rathke's pouch
65
Classic presentation of craniopharyngioma (visual)
Bitemporal hemianopsia
66
Two types of craniopharyngioma
Adamintomatous - seen in kids, calcifices. "Wet keratin" appearance (Dr. Stevens described it as wetting sidewalk chalk and spreading it around with your hands). When drained, fluid has a "machine oil" like consistency. Papillary -seen in adults (>65), no calcifications.
67
A drained intracranial mass shows "machine oil" consistency...dx?
Adamantinomatous craniopharyngioma
68
A group of progressive hereditary nerve disorders related to defective production of proteins involved in structure and function of peripheral nerves or myelin sheath
Charcot-Marie-Tooth disease
69
3 Hz spikes on EEG is indicative of...
Absence seizures
70
Brief (15 min-3 hr) headaches with excruciating periorbital pain with lacrimation and rhinorrhea.
Cluster headaches
71
Treatment for cluster headaches (abortive and prophylactic)
Abortive: Inhaled O2, sumitriptan Prophylactic: verapamil, lithium, prednisone
72
Pulsating headache with nausea, photophobia, phonophobia. Lasts 4-72 hours, usually unilateral
Migraine
73
Migraine treatments
Triptans (5HT-1 agonists) - abortive for an acute migraine; Propanolol/Topiramate - prophylactic
74

Triad seen with Meniere disease

Vertigo, tinnitus and sensorineural deafness | this is endolymphatic hydrops

75
Cerebellar hemangioblastoma is associated with which genetic disorder?
Von-Hippel-Lindau syndrome.
76
Foamy cells seen on CNS tumor biopsy
Hemangioblastoma
77
CNS tumor producing EPO and causing secondary polycythemia
Hemangioblastoma (associated with VHL disease)
78
CNS tumor in a child with histology showing sheets of cells with many mitotic figures
Medulloblastoma
79
What effect does demyelination (e.g. in MS or GBS) have on the length and time constants of a nerve?
Decreases the length constant (impulses travel shorter distances) and increases time constant
80
What is the first area damaged as a result of global cerebral ischemia?
Hippocampus
81
Which sequelae of Wernicke-Korsakoff syndrome is permanent?
Memory loss
82
Toxicity of ethylene glycol
Nephrotoxicity due to formation of envelope shaped oxalic acid crystals
83
Toxicity of Methanol
Ocular toxicity due to formation of formaldehyde (+ high anion gap acidosis)
84
Inhibitor of alcohol dehydrogenase used in methanol and ethylene glycol toxicities
Fomepizole, (can also use hemodialysis or EtOH)
85
Antidote for methanol and ethylene glycol toxicities
Ethanol (and fomepizole, which inhibits alcohol dehydrogenase)
86
MOA of disulfiram
Inhibits aldehyde dehydrogenase, causing acetaldehyde to build up, resulting in hangover-like effect (METRONIDAZOLE, cefoperazone, cefotetan, chlorpropamide, griseofulvin)
87
Cause of hypoglycemia in chronic alcoholics
Lack of eating + excess NADH due to high EtOH levels inhibiting gluconeogenesis
88

Why are alcoholics commonly thiamine (vitamin B1) deficient?

Chronic high levels of acetaldehyde are broken down by acetaldehyde dehydrogenase, which uses thiamine (and folate) to do its job

89
Use of Fomepizole
Inhibits alcohol dehydrogenase, used in acute methanol and ethylene glycol toxicity
90
What do you need to give alcoholic patients who are hypoglycemic?
Glucose WITH THIAMINE
91
What can happen if you give a hypoglycemic alcoholic glucose without thiamine?
Wernicke encephalopathy
92
How does alcoholism cause hyperuricemia?
Creates a lactic acidosis. Lactic acid competes with uric acid for excretion.
93
Cancers which alcoholics are at an increased risk for include...
Squamous cell CA of the esophagus, Signet ring cell CA of the stomach, Hepatocellular CA
94
MOA of carbamazepine/phenytoin
Decrease axonal conduction by preventing sodium influx through fast Na+ channels
95
1st Line drug for Tic Douloureux (Trigeminal neuralgia)
Carbamazepine
96
MOA of anti-convulsants lamotrigine/topirimate/felbamate
Block excitatory effects of glutamic acid
97
MOA of ethosuxamide and (maybe) valproic acid
Decrease presynaptic calcium influx through T type calcium channels in thalamus (note: this is just one of multiple proposed mechanisms for valproic acid)
98
DOC for absence seizures
Ethosuxamide (can also use valproate)
99
1st line for acute status epilepticus
Benzos (lorazepam/diazepam)
100
How does fosphenytoin differ from phenytoin?
Can be used parenterally because it is more water soluble (for use in status epilepticus)
101
1st line for prophlaxis against status epilepticus
Phenytoin/Fosphenytoin
102
What is unique about the metabolism of phenytoin (as far as anti-SZ meds are concerned)?
It is eliminated by zero order kinetics (constant amount of drug removed per unit time - also EtOH and aspirin)
103
Which anti-seizure drugs induce P450s?
Phenytoin/Carbamazepine
104
Which anti-seizure drug inhibits P450s?
Valproate
105
Major SFX of phenytoin
Gingival hyperplasia, lymphadenopathy, hirsuitism, megaloblastic anemia (decrease folate), sedation, aplastic anemia, nystagmus/diplopia/ataxia
106
Teratogenicity of phenytoin
Cleft lip/palate
107
DOC (3) for tonic/clonic seizures
Phenytoin/Carbamazepine/Valproate
108
Anti-seizure drugs used for bipolar disorder
Carbamazepine.Valproate (manic phase)
109
What does carbamazepine do to P450s?
INDUCES (just like phenytoin)
110
SFX of carbamazepine
Increase ADH secretion, SJS, CNS depression
111
Teratogenicity of Carbamazepine
Cleft lip/palate (like phenytoin) AND NTD's (like valproate)
112
Patient with history of seizures presents with symptoms consistent with SIADH. What is causing this?
Use of carbamazepine. It increases the release of ADH.
113
MOA of valproate
Inhibits GABA transaminase, which breaks down GABA. Increase GABA = CNS depression. Valproate also blocks T-type calcium channels in the thalamus. This is why it is used for absence seizures.
114
What does valproate do to P450s?
INHIBITS
115
Toxicity of valproate
Hepatotoxicity (measure LFTs); Pancreatitis; Alopecia (opposite of Phenytoin!)
116
Valproate Teratogenicity
Spina Bifida. CI in pregnancy!
117
Teratoginicities of anti-seizure meds
Phenytoin (cleft lip/palate); Valproate (spina bifida); Carbamazepine (cleft lip palate and spina bifida - BOTH)
118
MOA of ethosuxamide and its use
blocks T-type calcium channels in thalamus. DOC for absence seizures
119
What anticonvulsant is considered safest in pregnancy for treatment of pre-existing seizures?
Phenobarbital
120
Toxicity of Felbamate
Hepatotoxicity and aplastic anemia
121
Toxicity of lamotrigine
Stevens Johnson Syndrome (black box warning)
122
First line drug in febrile seizures (kids)
Phenobarbital
123
MOA, Uses, Toxicity of Vigabatrin
MOA: GABA analogue; Uses: infantile spasms, refractory epilepsy; Toxicity: diffuse atrophy of peripheral retinal nerve fiber layer. Patients should have annual visual field testing.
124
Minimal Alveolar Concentration (MAC) definition
Concentration of inhaled anesthetic as a percent of inspired air, at which 50% of patients don't respond to a surgical stimulus (a measure of potency).
125
What does an increase in lipid solubility do to the potency of an inhaled anesthetic?
Increases it - allows it to cross BBB
126
What effect does decreased blood solubility have on the onset time of an inhaled anesthetic?
Decreases it - decreased solubility in blood = rapid onset (gets to the BBB quickly)
127
Lipid/blood characteristics of nitrous oxide
Low solubility in both. Low blood solubility = fast onset. Low lipid solubility = low potency.
128
SFX and antidote for halothane
Malignant hyperthermia. Antidote = dantrolene. Also causes arrhythmias and hepatitis.
129
Barbiturate used for anesthesia induction
Thiopental - rapid onset, short acting
130
BZ used for preoparative sedation with anterograde amnesia
Midazolam (antidote = flumazenil)
131
Important characteristics of propfol
Anti-emetic, rapid induction
132
Ketamine MOA
NMDA-receptor blocker, used for anesthesia induction
133
Propofol MOA
Potentiates GABA at GABA-A receptors
134
SFX of Ketamine
Hallucination, vivid nighmares, CV stimulation, increased ICP
135
What terminates the effect of barbiturates such as thiopental?
Redistribution into tissues such as skeletal muscle and fat.
136
What effects do barbiturates have on cerebral blood flow?
Decrease
137
MOA of local anesthetics
Bind to and block activated sodium channels (most effective in rapidly firing neurons)
138
How many molecules of curare-like molecule (nondepolarizing neuromuscular blocking drugs) does it take to block one NMJ?
Only ONE. Even though there are two binding sites for acetylcholine, post-synaptic activation of the nicotinic receptor requires BOTH alpha subunits to be simultaneously stimulated by Ach.
139
MOA of curare drugs (non-depolarizing NMJ blockers)?
Competitive antagonist at nicotinic receptors (at the NMJ).
140
Antidote for curare drugs (non-depolarizing NMJ blockers)?
Since they are competitive inhibitors, they can be overcome with acetylcholinesterase inhibitors. Choose neostigmine because it doesn't cross BBB like physostigmine.
141
Nondepolarizing NMJ blocker safe in renal/hepatic failure
Atracurium, but it is spontaneously broken down to laudanosine, which can cause seizures
142
MOA of succinylcholine (depolarizing NMJ blocker)
Ach receptor agonist, producing sustained depolarization (phase I) and eventual desensitization (phase II)
143
Two phases of succinylcholine use
Phase I: prolonged depolarization due to succinylcholine's agonist activity. Flaccid paralysis. Potentiated by Ach-esterase inhibitors; Phase II: desensitization of nicotinic receptors. Antidote = Ach-esterase inhibitors.
144
Enzymes that metabolize succinylcholine
Pseudocholinesterase (rapid)
145
Succinylcholine SFX
Hyperkalemia, malignant hyperthermia, hypercalcemia
146
What receptor does baclofen bind?
GABA-B
147
Adult CNS tumor that crosses the midline
GBM
148
What (embryo) forms the nucleus pulposus?
Notochord
149
Frog-like appearance of aborted fetus
Anencephaly
150
Anencephaly results in maternal poly- or oligo- hydramnios, and why?
Polyhyramnios. Fetus has no brain, thus no swallowing centers, and can't swallow amniotic fluid, so it builds up.
151
Congenital failure of development of the cerebellar vermis
Dandy-Walker formation
152
Presentation of Dandy-Walker formation
Massively dilated 4th ventricle with absent cerebellum w/ hydrocephalus (know the image! http://goo.gl/unfcYT)
153
Massively dilated 4th ventricle with absent cerebellum w/ hydrocephalus
Dandy Walker formation (know the image!!)
154
Congenital herniation of cerebellar tonsils through the foramen magnum
Arnold-Chiari Malformation
155

Common presentations of Arnold-Chiari malformation (3)

Hydrocephalus (due to herniated cerebellar tonsils obstructing CSF flow) Syringomyelia thoraco-lumbar Meningomyelocele with paralysis below the defect

156
Loss of pain and touch position sense in the upper extremity
Syringomyelia (SPARES position sense and fine touch - DCML)
157
Big congenital malformation to associate with syringomyelia
Arnold Chiari malformation
158
Triad of Horner syndrome
Ptosis, anhidrosis, miosis
159
Expansion of a syringomyelia can result in...
Compression of the anterior horns (LMN paralysis); Compression of the lateral horn, which includes the hypothalamospinal tracts (Horner syndrome)
160
Inherited degeneration of anterior motor horn
Werdnig-Hoffman disease (autosomal recessive) - "floppy baby"
161
CNS lesion with random upper and lower motor neuron signs
ALS
162
What distinguishes ALS from syringomyelia?
NO sensory impairment with ALS
163
Mutation in familial ALS
Zinc copper superoxide dismutase (removes free radicals)
164
GAA trinucleotide repeat
Friedrich Ataxia - in frataxin gene
165
SSX of Friedrich ataxia
Ataxia with loss of vibratory sense and proprioception + muscle weakness. Due to cerebellar damage + damage to multiple spinal cord tracts.
166
Frataxin gene function
Mitochondrial iron regulation.
167
Gene Mutated in Friedrich Ataxia
Frataxin (GAA trinucleotide repeat)
168
#1 cause of death in Friedrich Ataxia
Hypertrophic Cardiomyopathy
169
Most common causes (3) of meningitis in neonates
Group B strep; E.coli; Listeria
170
Most common cause of meningitis in children/teens
Neisseria Meningitidis
171
Most common cause of meningitis in adults/elderly
Strep pneumo
172
Most common cause of meningitis in unvaccinated kids
H. flu
173
Most common cause of meningitis in immunocompromised
Cryptococcus neoformans
174
How does N. meningitis get from the environment to the meninges?
Inhaled -> nasopharynx -> blood -> choroid plexus -> meninges
175
Most common viral cause of meningitis
Coxsackievirus
176
Where should you stick the needle on an LP and why?
L4-L5 vertebral levels. Cord ends at L2, subarachnoid space with CSF ends at S2.
177
Which meningeal layer is not pierced during an LP?
Pia
178
What are the general CSF characteristics with bacterial meningitis?
High neutrophils with low glucose (bacteria consume glucose) + positive gram stain/culture
179
What are the general CSF characteristics with viral meningitis?
High lymphocytes with normal CSF glucose (viruses don't consume glucose)
180
What are the general CSF characteristics with fungal meningitis?
High lymphoctes with low CSF glucose (fungi consume glucose)
181
Which infectious cause of meningitis shows the highest rate of complications?
Bacterial (pus, exudate increases ICP)
182
What neuroendocrine tumor can cause global cerebral ischemia?
Insulinoma due to repeated episodes of hypoglycemia
183
Where do infarcts most commonly occur with moderate global ischemia?
Watershed regions
184
What happens in cortical laminar necrosis?
Global cerebral ischemia necroses certain layers of more vulnerable cerebral cells but not others (which contains 6 layers). This creates bands of necrosis with non-necrosed cells in-between.
185
Cerebral lines of necrosis with non-necrosed cells in-between describes...
Cortical laminar necrosis - caused by moderate global ischemia.
186
Why does a thrombotic stroke form a pale infarct?
Thrombotic strokes are due to atherosclerosis, which ruptures and forms a thrombus. The body lyses the thrombus, but it keeps forming over and over again because of the exposed subendothelium, so blood never returns to the area.
187
Why does an embolic stroke form a hemorrhagic ("pink") infarct?
Embolic strokes are due to emboli coming from other areas of the body. They lodge in cerebral arteries for long enough to cause neuronal death, then the body lyses them. Since they don't recur like thrombotic strokes (because there is no exposed subendothelium), blood rushes back to the area of necrosis, causing hemorrhage.
188
Where do lacunar strokes most commonly occur?
Lenticulostriate arteries in the deep structures of the brain
189
What underlying vascular pathology leads to lacunar strokes?
Hyaline arteriolosclerosis (secondary to HTN/DM)
190
What is the earliest change seen following CNS ischemia?
Red neurons (12-24 hours)
191
Describe the microscopic changes seen following an ischemic CNS stroke.
Red neurons (12-24 hours); Neutrophils (3-5 days); Microglia (5-7 days); Granulation-like tissue; Gliosis (>1 week, final change)
192
What is the final histologic stage seen with CNS ischemia?
Gliosis
193
What is the main risk factor for Charcot-Bouchard aneurysms?
HTN
194
What does the LP show with a subarachnoid hemorrhage?
Blood or Xanthochromia (yellow tinge to CSF due to bilirubin breakdown)
195
What is the main cause of subarachnoid hemorrhage?
Rupture of berry aneurysms
196
What is the #1 site for berry aneurysms?
The branch point of the ACOM in the anterior circle of Willis
197
Why (histologically) are berry aneurysms weak?
VASCULAR MEDIA LAYER FAILS TO DEVELOP AT BRANCH POINT of aneurysm, causing a sacular outpouching of the blood vessel, increasing rupture risk.
198
List two syndromes that increase risk for development of berry aneurysms.
Marfan syndrome and ADPKD
199
Treatment for wet macular degeneration
VEGF inhibitors (bevacizumab)
200
Presentation of wet macular degeneration
Acute vision loss over a period of months to weeks
201
Physical findings of wet macular degeneration
Subretinal fluid/hemorrhage, gray subretinal membrane, or neovascularization
202
Vessel that ruptures in an epidural hematoma
Middle meningeal a.
203

Lens (biconcave) shaped lesion on head CT

Epidural hematoma

204
Head trauma with lucid interval that precedes neurologic signs
Epidural hematoma
205
Vessels that rupture in a subdural hematoma
Bridging cerebral veins
206
Crescent shaped lesion on head CT
Subdural hematoma
207
Structures (3) compressed with an uncal herniation and sequelae
CN III (eye down and out); brainstem (coma); Paramedian artery on brainstem (brainstem hemorrhages)
208
Which cells are destroyed in MS?
Oligodendrocytes
209
What geographical association is seen with MS?
More commonly seen in regions away from the equator.
210
What is significant about the speech of a person with MS?
They commonly exhibit "scanning speech", which is similar to drunken speech.
211
Explain INO seen with MS
Damage of the MLF is commonly seen with MS. When patient is asked to look laterally, the eye that is adducting (via CN III) receives a signal from the abducting eye (via CN VI) to adduct. If the MLF is damaged, this signal doesn't make it, and the eye fails to adduct.
212
25 year old female is asked to look left. Her left eye abducts, but the right eye does't adduct. What's the problem?
MS - intranuclear ophthalmoplegia secondary to damage to the RIGHT MLF. Note: The MLF is named for eye it GOES TO, (i.e. the adducting eye - CN III nucleus) not where it originates (the abducting eye -CN VI nucleus). See First Aid p 442 for a picture.
213
Treatment for MS acute attack
High dose steroids
214
Long term MS treatment, shown to slow progression of disease
IFN - Beta
215
Mu, Kappa, and Delta receptors use which type of signaling?
Gi (lowers cAMP) - found on interneurons, serves to dampen neurotransmission
216
What receptor does morphine bind to?
Mu opioid receptors
217
Why should you not give pure oxygen to a patient in opioid-induced respiratory depression (unless they are mechanically ventilated)?
CO2 respiratory centers causing increase in breathing via HIGH CO2 are shut down by opioids because they have mu receptors. Thus, the only drive for respiration the patient has at this point is peripheral oxygen sensors, which stimulate breathing with LOW O2. If you shut these receptors off with high O2, there is no drive for the patient to breathe on their own.
218
Antidotes (IV and oral) for opioid OD
IV - naloxone; Oral - naltrexone (has a T in its name, passes by your Teeth)
219
SFX of opioids
Constipation, miosis, respiratory depression, urinary retention, addiction
220
What is significant about the metabolism of morphine?
It is metabolized by glucuronidation, and its metabolite is MORE POTENT than the parent compound - can cause nephrotoxicity
221
Long-t1/2 opioid agonist used opioid addiction tapering
Methadone
222
MOA & use of pentazocine, and a situation where it should not be given.
MOA : weak opioid (mu) agonist; Use: analgesia with limited addiction potential; Should not be used in opioid addiction because it can precipitate an acute withdrawal crisis (because it is only a weak agonist, and acts like an antagonist in the presence of a complete agonist like morphine)
223
Which opioid antagonist can be used to reduce alcohol craving?
PO Naltrexone
224
What effect do opioid analgesics have on synaptic ion channels?
Open K+ channels, increase potassium influx; Close Ca2+ channels, decrease calcium influx; Overall: decreases synaptic transmission
225
Which side effects aren't reduced by opioid tolerance? (2)
Miosis, constipation
226
Explain the pharmacodynamic tolerance of opioids.
Opioids act through Gi, decreasing cAMP. Unlike most tolerance mechanisms, tolerance to opioids is not because of down-regulation of receptors. Opioid tolerance happens because other GPCR pathways in cells increase cAMP, requiring a bigger stimulus to produce significant inhibition.
227
Explain why clonidine is used to manage opioid withdrawal.
Long term opioid use inhibits norepinephrine release, causing upregulation of alpha-1 and beta-1 receptors. When opioids are withdrawn, NE levels go back to normal, causing the excess sympathetic symptoms secondary to higher number of receptors (agitation, tachycardia, sweating, HTN). Clonidine is given because it is an alpha-2 agonist that decreases the release of NE, thus calming the sympathetic effects.
228
Opioid agonist used for diarrhea
Loperamide
229
Opioid agonist used for cough suppression
Dextromethorphan (abuse potential - makes you trip balls)
230
Treatment for a neonate born to a heroin-addicted mother showing signs of opioid withdrawal?
Tincture of opium
231
Neurotransmitter changes seen in Parkinson disease (2)
Loss of dopaminergic neurons in nigrostriatal pathway; Excess cholinergic activity (leads to muscle rigidity and resting tremor)
232

MOA of carbidopa

Noncompetitive PERIPHERAL (NOT CENTRAL) inhibitor of aromatic amino acid decarboxylase (AAAD). Prevents peripheral conversion of L-dopa to dopamine, so L-dopa can cross BBB. It increases the T1/2 of Levidopa and decreases the plasma dopamine levels

233
Why is carbidopa given with L-dopa?
It blocks PERIPHERAL conversion of L-dopa to dopamine (NOT CENTRAL - AAAD is needed for conversion of L-dopa to dopamine!). Dopamine can't cross BBB, but L-dopa can. Thus, carbidopa increases the amount of L-dopa available to cross the BBB and decreases peripheral side effects of dopamine.
234
SFX of levodopa
Dyskinesia (on-off effects), psychosis, hypotension, vomiting
235
Drug which inhibits aromatic amino acid decarboxylase (AAAD)
Carbidopa
236
Explain the on-off effects of levodopa
Dyskinesia when on the medicine, bradykinesia when off the medicine
237
COMT inhibitors used in PD
Entacapone, tolcapone
238
MOA of entacapone/tolcapone
Inhibit COMT, decreasing metabolism of L-dopa in the periphery, enhancing L-dopa uptake across BBB
239
MOA of selegiline
MAO-B inhibitor used in PD. Decrease metabolism of dopamine.
240
MAO-B inhibitor used in PD
Selegiline
241
Selegiline is a selective MAO-B inhibitor, what is significant about this with respect to side effects?
There is no interaction with tyramines (compounds found in wine and cheese). MAO-A inhibitors (phenelzine) block GI metabolism of tyramines, allowing them to enter the circulation and precipitate a hypertensive crisis. MAO-B inhibitors do not! Note: according to UWorld, this is the most commonly tested side effect on the USMLE.
242
Benztropine MOA and use
MOA: muscarinic receptor blocker; Use : calms down excess Ach activity in PD (decreases tremor/rigidity, but not bradykinesia)
243
What is the significance of aromatic amino acid decarboxylase in PD?
Metabolizes L-dopa to dopamine
244
DA agonists (4) used in Parkinson disease.
Bromocriptine, pergolide, Pramiprexole, Ropinerole. (the latter 2 are preferred)
245
Trihexyphenidyl MOA and use
MOA: muscarinic receptor blocker; Use : calms down excess Ach activity in PD (decreases tremor/rigidity, but not bradykinesia)
246
Antimuscarinic drugs (2) used in PD
Benztropine, Trihexyphenidyl
247
MOA of pramiprexole
Dopamine agonist used in PD
248
Antiviral drug used in PD
Amantadine (increases dopamine release)
249
Why is amantadine used in PD?
Increases dopamine release
250
SFX of amantadine
Antimuscarinic ; Livedo reticularis (UNIQUE!) - skin looks pale and blood vessels dilate to form a purple mesh
251
Cocaine MOA
Blocks dopamine, NE, and 5HT reuptake
252
Only drug approved for ALS
Riluzole (decreases glutamate release)
253
MOA of amphetamine
Block reuptake of NE/DA, also causes the NE/DA reuptake transporters to work in reverse, releasing more NT into the cleft.
254
Memantine MOA and use
MOA: NMDA receptor antagonist; Use: Alzheimer Disease
255
Donepezil MOA and use
MOA: Acetylcholinesterase inhibitor; Use: Alzheimer Disease
256
Acetylcholinesterase inhibitors used in Alzheimer disease
Donepezil, galantamine, rivastigmine
257
Antioxidant that may confer some protection to excitotoxicity in Alzheimer disease
Vitamin E
258
Components of the BBB (3)
Endothelial cells (non-fenestrated); Basement membrane; Astrocytic end feet
259
Proximal symmetric weakness with no sensory loss
Myopathies
260
Distal asymmetric weakness with atrophy, fasciculations, sensory loss and pain
Peripheral neuropathy
261
Most common causes of peripheral neuropathy (2)
DM and EtOHism
262
Signs of a polyneuropathy?
Stocking/glove sensory loss.
263
#1 motor neuropathy
GBS
264

SSX of Guillan-Barre syndrome

Ascending symmetric muscle weakness, areflexia, and paresthesias in hands/feet usually following a viral or GI illness or flu shot (don't forget campylobacter!) NO SENSORY LOSS!

265
CSF findings in GBS
Elevated protein without elevated WBC
266
Most common motor neuron disease
ALS
267
SSX of MS
Weakness, spasticity, sensory loss, incontinence, vision loss that resolve and RETURN PROGRESSIVELY WORSE.
268
Classic MRI finding with MS
White matter lesions (plaques)
269
Signs seen with UMN lesions
spastic paralysis, hyperreflexia, Babinski sign, Hoffman sign, clonus
270
Signs seen with LMN lesions
flaccid paralysis, hyporeflexia, negative babinski sign, fasciculations, atrophy
271
Congenital damage to corticospinal tracts and/or basal nuclei resulting in spasticity, flaccid paralysis, dystonia
Cerebral palsy
272
Reflexes seen (immediately) with sudden spinal cord trauma
Temporary flaccid paralysis and hyporeflexia (spinal shock) - eventually progresses to UMN characteristics
273
Pt presents with acute onset of weakness and sensory loss in LE, radicular pain, saddle anesthesia, and urinary/bowel incontinence
Cauda equina syndrome - surgical emergency
274
Treatment for cauda equina syndrome
Immediate surgical repair
275
SSX of anterior spinal artery syndrome
BL loss of pain/temperature (spinothalamic tracts); BL weakness with hyperreflexia (corticospinal tract); Only thing spared is DCML
276
Artery occluded in anterior spinal artery syndrome
The artery of Adamkiewicz
277
Scrape tip of 3rd digit, thumb and 2nd digit flex in UM neuron lesion
Hoffman sign
278

SSX of Sequard syndrome (3 major)

CL loss of pain/temp below the lesion (spinothalamic tract); IL loss of position/vibratory below lesion (DCML); IL flaccid paralysis at the level of the lesion (anterior horn)

279
SSX/cause of tabes dorsalis
Loss of discriminative touch/proprioception due to loss of DCML. Cause: tertiary syphilis
280
SSX/cause of subacute combined degeneration
Peripheral neuropathy, loss of proprioception/balance (loss of DCML), loss of pain/temp (loss of spinothalamic tracts), ataxic gait. Cause: chronic B12 deficiency.
281
Weber/Rinne results with sensorineuronal hearing loss
Sound localizes to good ear (Weber); Air conduction > bone conduction (Rinne)
282
Weber/Rinne results with conductive hearing loss
Sound localizes to bad ear (Weber); Bone conduction > air conduction (Rinne)
283
What is the term for unequal pupils?
Anisocoria
284
Vessel affected in lateral medullary syndrome
PICA
285
Lesions to what two nerves can cause anisocoria?
CN III or sympathetics
286
Temporal lobe tumor/lesion causes what visual defect?
Pie in the sky
287
What is a Marcus-Gunn pupil and what causes it?
Pupil appears to dilate with direct stimulation to light. Indicates CN II lesion.
288
What is an Argyll-Robertson pupil and what causes it?
Eyes that accommodate, but don't react to light. Indicates tertiary syphilis.
289
Describe the position of the limbs in decorticate posturing.
UL: flexed (held to core); LL: extended
290
What causes flexion of UL in decorticate posturing?
Rubrospinal tract
291
Describe the position of the limbs in decerebrate posturing.
All 4 limbs are extended.
292
How is nystagmus named?
For the fast phase (quick jerk back to midline).
293
Explain the results of caloric testing
Cold: nystagmus opposite to the stimulated side; Warm: nystagmus to the same side as is stimulated; COWS
294
Structure(s) degenerated in Huntington disease.
Caudate (mainly) and putamen
295
Lesion to sub thalamic nucleus produces
Contralateral hemiballismus.
296
Wild unilateral flinging of an arm/leg
hemiballismus
297
Slow, writhing movements
Athetosis
298
Sudden, brief, uncontrolled muscle contraction
Myoclonus (hiccups!)
299
Sustained, involuntary muscle contractions
Dystonia
300
Slow, zigzag motion when pointing or extending toward an object
Intention tremor. Indicative of cerebellar disease
301
Action tremor exacerbated by holding limb position
Essential tremor
302
With what do patients typically self-medicate to reduce essential tremor?
EtOH.
303

Function of VPL thalamus

Receives input for body pain/temperature, touch, vibration, proprioception VPL = very painful legs

304

Function of VPM thalamus

```

Receives pain/temperature from face also taste (trigeminal and gustatory pathways) VPM = very painful mouth

```
305
Function of LGB of thalamus
Vision
306

Function of MGB of thalamus

Hearing (from the superior olive and inferior colliculus of tectum) Medial = Musical

307

Function of VL lobe of thalamus

Receives input from basal nuclei - fine tunes motor output from cortex before sending it back

308
Function of lateral area of hypothalamus
hunger
309
Function of ventromedial area of hypothalamus
satiety
310
Function of anterior hypothalamus
Cooling
311
Function of posterior hypothalamus
heating
312
Function of suprachiasmatic nucleus of hypothalamus
Circadian rhythm
313
Romberg test shows that a patient sways with eyes open...dx?
Cerebellar ataxia
314
Romberg test shows that a patient sways with eyes closed...dx?
Sensory ataxia (DCML)
315
SSX of blockage of anterior choroidal artery
CL hemiparesis (CST); CL homonymous hemianopsia (optic tract)
316
SSX of blockage of lenticulostriate arteries
CL hemiparesis (posterior limb of internal capsule - pure motor stroke)
317
SSX of MCA blockage
Motor/sensory symptoms involving the CL face/upper limb/trunk. May produce Broca aphasia.
318
SSX of ACA blockage
Motor/sensory symptoms involving the CL lower limb.
319
Blockage of this artery can cause Broca aphasia
Superior division of MCA
320
Blockage of this artery can cause Wernicke aphasia
Inferior division of MCA
321
SSX of PCA blockage
Visual symptoms - CL hemianopsia - can have macular sparing!
322
Impaired comprehension, repetition, naming and speech output
Broca Aphasia
323
Fluent speech full of nonsense words and phrases
Wernicke Aphasia
324
Aphasia characterized by poor repetition
Conductive aphasia
325
What structure is damaged in conduction aphasia?
Arcuate fasciculus
326
SSX of anterior watershed strokes?
Broca aphasia and lower limb effects.
327
SSX of posterior watershed stroke?
Wernicke aphasia and visual loss.
328
Define apraxia.
Loss of ability to perform a learned/familiar task
329
Define agnosia
Inability to recognize a familiar object regardless of intact sense
330
SSX of Gerstmann syndrome
Acalculia (can't do math), agraphia (can't write), finger agnosia (can't distinguish one finger from another) and left-right confusion
331
Cause of Gerstmann syndrome
Damage to the angular gyrus in the dominant hemisphere
332

SSX of Kluver-Bucy syndrome

Docility, placidity, hypersexuality, hyperorality and hypermetamorphosis. "hyperorality, hypersexualty and disinhibited behavior"

333
Cause of Kluver-Bucy syndrome
BL amygdala lesions
334

Signs and Symptoms of diffuse Lewy Body Dementia

Memory Loss; Parkinsonian-Like Movement disorder (onset at the SAME time as memory loss); Visual hallucinations (pretty specific for DLBD as far as degenerative diseases are concerned); Autonomic dysfunction (urinary incontinence); Sleep disturbances

335
What is the first sign of magnesium toxicity?
decreased deep tendon reflexes
336
Patient has a CN III palsy. Where is the most likely aneurysm?
Ipsilateral posterior communicating artery (PCOM)
337
SSX of a CN III palsy?
Down and out eye; Blown pupil; droopy eyelid (loss of levator palpebrae)
338
What forms from the metencephalon (2) ?
Cerebellum and pons
339
What forms from the myelencephalon?
Medulla
340
What forms from the mesencephalon?
Midbrain
341
What forms from the diencephalon?
Anything that has "thalamus" in it: thalamus, hypothalamus, subthalamus, epithalamus
342
What forms from the telencephalon (2) ?
Cerebrum and basal nuclei
343
Which nerve gives general sensation to the anterior tongue?
CN V3 (lingual branch)
344
CGG triplet repeat
Fragile X syndrome
345
CTG triplet repeat
Myotonic dystrophy
346
CN (2) providing input to the nucleus ambiguus
CN IX and CN X
347
Function of the nucleus ambiguus
Provides motor innervation to muscles of the pharynx and larynx via CN IX and X.
348
Functions of the principal (chief) trigeminal nucleus
Transmits discriminative touch/pressure to the face, mucous membranes, and other head structures via CN V (all branches).
349
Function of the spinal trigeminal nucleus
Transmits pain, temperature, and crude touch to the face via CN V.
350
Function of the spinal mesencephalic nucleus
Proprioception for muscles of mastication
351
Things seen with fetal dilantin (hydantoin/phenytoin) syndrome
Skull/face abnormalities; Hypoplasia of fingernails/toenails
352
The longest stage of sleep (spend the most time in)
2
353
Stage of sleep with sleep spindles and K complexes on EEG
2
354
Stage of sleep with delta waves on EEG
3
355
Stage of sleep with beta waves on EEG
REM (also awake with eyes open)
356
Stage of sleep with theta waves on EEG
1
357
Meds (2) for seizure prophylaxis in preeclampsia
Magnesium sulfate (more commonly used), Labetalol
358
Meds to lower BP in pregnancy
Labetalol; Hydralazine
359
Most important test to perform when assessing toxicity for magnesium sulfate
Deep tendon reflexes
360
Magnesium sulfate is primarily used for
Seizure prophylaxis in pre-eclampsia
361
What is rameleton?
Melatonin agonist used for insomnia
362
Sleepwalking occurs in what stage of sleep?
3
363
Night terrors occur in what stage of sleep?
3
364
What is the primary neutotransmitter during REM sleep?
Ach
365
What neurotransmitter is essential for initiating sleep?
5-HT
366
What do EtOH/Benzos/Barbiturates do to sleep?
Decrease REM
367
In what stage of sleep do nightmares occur?
REM
368
At what age does the Babinski sign disappear?
12-15 months
369
At what age should a child be able to sit alone, crawl, transfer toys from hand to hand?
7-9 months
370

What makes up APGAR score?

Appearance, pulse, grimace, activity, respirators; Taken at 1 and 5 minutes

371
In the embryo, what induces the overlying ectoderm to differentiate into the neuroectoderm and form the neural plate?
Notochord (within mesoderm)
372
What does the neural plate give rise to?
Neural tube and neural crest cells
373
What does the notochord become?
Nucleus pulposus of the intervertebral disc in adults
374
What does the alar and basal plates become?
``` Alar = dorsal = sensory Basal = ventral = motor ```
375
Describe the three primary vesicles
Forebrain (prosencephalon) Midbrain (mesencephalon) Hind brain (rhombencephalon)
376
What does the forebrain (prosencephalon) develop into?
Telencephalon --> cerebral hemispheres, basal ganglia, hippocampus, amygdala, and lateral ventricles Diencephalon --> thalamus and hypothalamus and optic tract and nerve, third ventricle
377
What does the midbrain (mesencephalon) develop into?
Mesencephalon --> Midbrain, Aqueduct
378
What does the hindbrain (rhombencephalon) develop ?into
Metencephalon --> Pons and cerebellum and upper part of the 4th ventricle Myelencephalon --> medulla and lower part of 4th ventricle
379
how do neural tube defects occur?
Neuropores fail to fuse during week 4 leading to persistent connection between the amniotic cavity and the spinal canal
380
What can act as a confirmatory test for neural tube defects?
Increased acetylcholinesterase in amniotic fluid is helpful confirmatory test (fetal AChE in CSF transudates across defect into the amniotic fluid) This is after you find an elevated alpha fetoprotein
381
Is anencephaly due to failure of the brain to develop?
NO! There is a malformation of the anterior neural tube resulting in no forebrain and an open calvarium. The brain is exposed to the acidic amniotic fluid which eats away at the brain.
382
What is anencephaly associated with?
Maternal diabetes type I | maternal folate supplementation also decreases risk
383
What is Holoprosencephaly?
Failure of the left and right hemispheres to separate, usually occurs during weeks 5-6 moderate form has cleft palate/lip, and most severe form results in cyclopia
384
What is the mutation in Holoprosencephaly?
Sonic hedgehog signaling pathway mutation Can also be seen in fetal alcohol syndrome and Patau syndrome (trisomy 13)
385
Describe Chiari II malformation
Significant cerebellar tonsillar and vermian herniation through the foramen magnum with aqueductal stenosis and hydrocephalus Often presents with throaco-lumbar myelomenigocele and paralysis below the defect
386
Describe Dandy Walker
Agenesis of cerebellar vermis with cystic enlargement of the 4th ventricle that fills the posterior fossa. Associated with hydrocephalus and spina bifida
387
What will Dandy walker lead to?
Truncal ataxia, head tremor and truncal instability
388
Cystic enlargement of the central canal of the spinal cord
Syringomyelia
389
Which tracts are typically damaged first with a syringomyelia?
Crossing fibers of spinothalamic tract - results in a "cape like" bilateral loss of pain and temp in upper extremities Fine touch sensation is PRESERVERD!
390
What is the most common level of a syringomyelia?
C8-T1
391
What controls the taste of the tongue?
anterior 2/3 CN 7 | posterior 1/3 CN 9 and 10 (way back)
392
What controls the sensation on the tongue?
CNV3 controls the anterior 2/3 | CN IX controls the posterior 1/3
393
What controls the motor innervation of the tongue?
CNXII
394
What are the muscles of the tongue derived from?
Occipital myotomes
395
Describe the tongue development in terms of branchial arches
1st branchial arch forms the anterior 2/3 - this is why sensation is via CN V3 and taste from CN 7 3rd and 4th branchial arches form the posterior 1/3 - this is why sensation and taste mainly come from CN9 and the extreme back is CN10
396
What comes from Neuroectoderm?
CNS neurons, ependymal cells (inner lining of ventricles, make CSF), oligodendroglia, astrocytes
397
What comes from the neural crest?
PNS neurons, Schwann cells
398
What comes from mesoderm?
Microglia (macrophages of CNS)
399
What does Nissl substance stain?
rough endoplasmic reticulum of neurons - note that rER is not found in axons
400
What do astrocytes to?
Physical support, repair, potassium metabolism, removal of XS neurotransmitter, maintenance of BBB Reactive gliosis occurs in response to injury
401
What is the astrocyte marker?
GFAP
402
What are microglia?
CNS phagocytes - have small irregular nuclei and relatively little cytoplasm. Respond to tissue damage by differentiating into large phagocytic cells can be seen with silver stain
403
What happens to microglia cells in HIV infected pts?
Fuse to form multinucleated giant cells in the CNS
404
What makes myelin in the CNS? PNS?
CNS - oligodendrocytes | PNS - Schwann cells
405
Describe oligodendrocytes
White matter, myelinate many axons in the CNS; have a fried egg appearance note that oligodendrocytes are destroyed in MS
406
Describe Schwann cells
Schwann cells myelinate ONE axon in the PNS, they promote axonal regeneration note that schwann cells are damaged in Guillain Barre
407
What is the predominant glial cell in white matter?
Oligodendrocyte
408
What is an acoustic neuroma?
Type of schwannoma - typically located in the internal acoustic meatus (CN VIII), bilateral in neurofibromatosis type 2
409
What receptor type is responsible for pain and temperature?
Free nerve endings
410
What is the location of free nerve endings?
All skin, epidermis and some viscera
411
Describe free nerve endings
C fibers - slow, unmyelinated fibers - burning dull pain, itch Adelta - fast myelinated fibers - pricking pain
412
What receptor type is responsible for dynamic, fine/light touch and position sense?
Meissner's corpuscles
413
Where are meissner's corpuscles located?
Glabrous (hairless) skin like the fingertips eyelids and lips or palsm soles and digits
414
Describe meissern's corpuscles
Large myelinated fibers that adapt quickly
415
Describe Pacinian corpuscles?
Large myelinated fibers | resembles an onion
416
Where are pacinian corpuscles located?
Deep skin layers, ligaments and joints
417
Which receptor type is responsible for vibration and pressure and increased tension?
Pacinican corpuscles
418
Describe Merkel's discs
Large, myelinated fibers that adapt slowly | look like melanocytes
419
Where are merkel's discs located?
Hair follicles, fingertips and oral and anal mucosa
420
Which receptors are responsible for pressure, deep static touch like with shapes and edges and position sense?
Merkel's discs
421
List the layers of a peripheral nerve
Endoneurium - inner, single nerve fibers , inflammatory infiltrate in guillain barre Perineurium - around Epineurium - outer
422
What must be rejoined in microsurgery for limb reattachment?
Perineurium
423
Describe the Epineurium
Dense connective tissue that surrounds entire nerve including the fascicles and blood vessels
424
Describe Norepinephrine in disease states
Increased in anxiety | decreased in depression
425
Where is norepinephrine synthesized?
Locus ceruleus (pons)
426
Describe Dopamine in disease states
Increased in schizophrenia | Decreased in Parkinson's and depression
427
Describe serotonin and disease states
Decreased in anxiety and depression
428
Describe Ach in disease states
Decreased in Alzheimer's and Huntington's and increased in REM sleep, also increased in Parkinson's disease
429
Describe GABA in disease states
Decreased in anxiety and Huntington's
430
Where is Dopamine synthesized?
Ventral tegmentum and SNc (midbrain)
431
Where is serotonin synthesized?
Raphe nucleus in the pons
432
Where is ACh synthesized?
Basal nucleus of meynert
433
Where is GABA synthesized?
Nucleus accumbens
434
What is the Locus ceruleus associated with?
Stress and panic
435
What is the nucleus accumbens and septal nucleus associated with?
reward center, pleasure, addiction, fear
436
What can cross rapidly across the BBB?
Nonpolar/lipid-soluble substances cross rapidly via diffusion
437
What can occur if infarction or neoplasm destroys the endothelial cell tight junctions of the BBB?
Vasogenic edema
438
What are the two inhibitory neurotransmitters?
GABA and glycine
439
Is glutamate inhibitory or excitatory neurotransmitter?
Excitatory
440
What happens to serotonin in the pineal gland?
Converted to melatonin
441
What disease is associated with degeneration of the basal nucleus of Meynert and less CNS ACh?
Alzheimer's disease
442
What is GABA?
Main inhibitory neurotransmitter of the CNS - made from glutamate When you block GABA you get an excitatory response note that Benzodiazepines and barbiturates increased the effect of GABA function (anti-anxiety)
443
What is Glycine?
Inhibitory neurotransmitted of the spinal cord
444
What is the main excitatory neurotransmitter of the CNS?
Glutamate
445
What happens when you block the Mesocortical dopaminergic pathway?
Increased negative symptoms of schizophrenia like social withdrawal and depression
446
What happens when you block the mesolimbic dopaminergic pathway?
Relieves the positive symptoms of schizophrenia
447
What happens when you block the nigrostriatal dopaminergic pathway? What if you stimulate it?
Block = Parkinson's disease Stimulate = extrapyramidal side effects
448
What happens if you block the tuberoinfundibular dopaminergic pathway?
Increased release of prolactin from the anterior pituitary
449
Describe the lateral area of the hypothalamus
Hunger Destruction leads to anorexia, failure to thrive inhibited by leptin "if you zap your lateral nucleus you shrink laterally"
450
Describe the ventromedial area of the hypothalamus
Satiety Destruction (craniopharyngioma) leads to hyperphagia stimulated by leptin
451
What does the anterior hypothalamus do?
Cooling (AC) Parasympathetics damage leads to hyperthermia
452
What does the posterior hypothalamus do?
Heating, sympathetic damage leads to hypothermia
453
What does the suprachiasmatic nucleus do?
Circadian rhythm | receives input from retina
454
what is the posterior pituitary derived from?
neuroectoderm
455
Which hypothalamic nuclei make ADH and oxytocin?
ADH - supraoptic (water balance) | Oxytocin - paraventricular
456
Which hypothalamic nucleus secretes GnRH?
Preoptic nucleus
457
What does the Arcuate nucleus of the hypothalamus secrete?
Dopamine and GHRH
458
What does the Mamillary body do?
Receives input from the hippocampus | hemorrhagic lesion leads to Wernicke's encephalopathy
459
What is the limbic system?
Responsible for the 5 F's: Feeding, Fleeing, Fighting, Feeling and F-ing collection of neural structures involved in emotion, long term memory, olfaction, behavior modulation and autonomic nervous system function
460
How do we not let the limbic system rule our lives?
The perfrontal cortex can inhibit the limbic system so that we don't act on instinct alone - prefrontal cortex is for decision making and allows for delayed gratification to exist
461
List the 5 structures that make of the limbic system
Hippocampus, amygdala, fornix, mammillary bodies, cingulate gyrus
462
What is the most common cause of damage to the flocculonodualr lobe of the cerebellum?
Medulloblastoma in childhood
463
What are the deep nuclei of the cerebellum?
From lateral to medial: Don't Eat Greasy Foods Dentate, Emboliform, Globose, Fastigial Interposed nuclei = Emboliform + Globose
464
What is the difference between a medial or lateral injury to the cerebellum?
Medial - Balance and truncal coordination Lateral - voluntary movement of extremities, so when injured you have propensity to fall toward the injured side (ipsilateral side - due to a double CL)
465
What is the major output of the cerebellum?
Brachium conjunctivum (Superior cerebellar peduncle) --> CL ventrolateral nucleus of the thalamus) --> motor cortex
466
What is the importance of the basal ganglia?
Important in voluntary movements and making postural adjustments
467
What makes up the striatum of the basal ganglia? The Lentiform of the basal ganglia?
``` Striatum = caudate (cognitive) + putamen (motor) Lentiform = putamen + globus pallidus ```
468
What occurs with lesion to the subthalamic nucleus?
Hemiballismus (increased movement) | note that the Hemiballismus matches the CL subthalamic nucleus
469
What is Pramipexole?
Dopamine agonist
470
Degenerative disorder of CNS associated with Lewy bodies and loss of dopaminergic neurons
Parkinson's disease
471
What are Lewy bodies composed of?
Intracellular inclusions of alpha synuclein
472
What are the symptoms of Parkinson's disease?
``` Your body becomes a TRAP Tremor at rest (pill rolling) Rigidity Akinesia (or bradykinesia) Postural instabilidy ```
473
What are the symptoms seen in Huntington's disease?
Chorea, aggression, depression and dementia
474
What happens in Huntinton's disease?
AD trinucleotide repeat neuronal death via NMDA-R binding and glutamate toxicity Atrophy of striatal nuclei
475
Mutation on Chromosome 4, CAF repeat and Caudate loses ACh and GABA describes...
Huntington's disease
476
what are the side effects of olanzapine?
Increased prolactin, orthostatic hypertension, anticholinergic effects, weight gain and somnolence
477
What are some drugs you can use to treat the chorea seen in Huntington's disease?
Tetrabenazine - 1st line treatment | Olanzapine
478
What can cause hemballismus?
CL subthalamic destruction due to lacunar stroke
479
What is athetosis and when is it seen?
Slow writhing movements especially in the fingers | Seen in Huntington's disease due to Basal ganglia lesion
480
What is myoclonus?
Sudden, brief uncontrolled muscle contraction like jerks, or hiccups Common in metabolic abnormalities such as renal and liver failure
481
What is dystonia?
Sustained involuntary muscle contractions | Blepharospasm (sustained eyelid twitch) or writer's cramp
482
What is an essential tremor?
Postural tremor, Action tremor, exacerbated by holding posture/limb position - seen at rest and with movement Genetic predisposition
483
How can you teat an essential tremor?
Beta blockers of primidone | Patients often self-medicate with EtOH which decrease tremor amplitude
484
What is a resting tremor and when is it seen?
Uncontrolled movement of distal appendages - most noticeable in the hands tremor is alleviated by intentional movement seen in Parkinson's disease - pill rolling tremor (at rest)
485
What is an intention tremor?
Appears with voluntary movement Slow, zigzag motion when pointing/extending toward a target Occurs with Cerebellar dysfunction
486
What is the Arcuate fasciculus?
Connects Broca's and Wernicke's areas
487
What artery can injure the principal visual cortex?
PCA
488
What deficits are seen with injury to the principal visual cortex?
deficits are seen in the CL visual field as homonymous hemianopsia with macular sparing
489
What is the precentral gyrus?
Primary motor cortex
490
Artery of the lateral aspect of the brain
MCA
491
Artery of the medial aspect of the brain
ACA
492
Artery involved with lower extremity deficit in sensation or movement?
ACA
493
Artery involved with face deficit in sensation or movement?
MCA
494
What is Kluver Bucy syndrome associated with?
HSV-1 infection
495
A patient can no longer complete complex tasks like balancing a check book
Frontal lobe lesion
496
What are the consequences of lesion to the frontal lobe?
Disinhibition and deficits in concentration, orientation and judgment, may have reemergence of primitive reflexes
497
What are the consequences of a right parietal lobe lesion?
This is the non-dominant lobe | Spatial neglect syndrome (agnosia of the CL side of the world)
498
What are the consequences of a dominant parietal lobe lesion?
Gerstmann Syndrome Agraphia, Acalculia, Finger agnosia, L and R disorientation - at angular gyrus
499
What 3 things make up the reticular activating system?
Reticular formation, locus ceruleus, Raphe nuclei
500
What are the consequences of a lesion at the reticular activating system in the midbrain?
Reduced levels of arousal and wakefulness - COMA
501
What are the consequences of a lesion at the cerebellar hemispheres?
Intention tremor, limb ataxia, loss of balance | Damage to the cerebellum results in Ipsi deficits - fall toward the side of the lesion
502
What are the consequences of a lesion at the cerebellar vermis?
truncal ataxia and dysarthria
503
What are the consequences of a lesion at the hippocampus?
Anterograde amnesia - inability to make new memories
504
What are the consequences of a lesion at the paramedian pontine reticular formation?
Eyes look away from the side of lesion
505
What are the consequences of a lesion at the frontal eye fields?
Eyes look toward the lesion
506
Nonfluent aphasia with intact comprehension
Broca's aphasia - Broken Boca | understands what you're saying but can't form words
507
What makes up Broca's area?
Inferior frontal gyrus of the frontal lobe
508
Fluent aphasia with impaired comprehension
Wernicke's aphasia - Wordy but makes no sense | impaired content
509
What makes of Wernicke's area?
Superior temporal gyrus of the temporal lobe
510
Nonfluent aphasia with impaired comprehension
Global aphasia
511
Poor repetition but fluent speech and intact comprehension
Conduction aphasia can't say no ifs ands or buts damage and arcuate fasciculus
512
when do watershed zones get damaged?
severe hypotension
513
What drives cerebral perfusion?
PCO2 levels | PO2 can modulate perfusion in severe hypoxia
514
Why would you use therapeutic hyperventilation?
Therapeutic hyperventilation (decreased PCO2) helps to decreased intracranial pressure in cases of acute cerebral edema (stroke, trauma) via decreasing cerebral perfusion
515
What is damaged in Weber syndrome?
Posterior communicating artery
516
What is damaged in medial inferior pontine syndrome?
Basilar artery
517
What is injured in medial medullary syndrome?
Anterior spinal artery
518
What is injured in Wallenberg syndrome (lateral medullary syndrome)?
Posterior inferior cerebellar artery
519
What is injured in lateral inferior pontine syndrome?
Anterior inferior cerebellar artery
520
What is injured in lateral superior pontine syndrome?
Anterior inferior cerebellar artery
521
What occurs in Medial medullary syndrome?
Injured : Paramedian branch of Anterior spinal artery. - CL spastic hemiparesis - CL tactile and kinesthetic sensory loss - Tongue deviates toward the side of the lesion (hypoglossal nucleus)
522
What occurs in Wallenberg syndrome (AKA Lateral medullary)?
Injured: PICA - CL loss of pain and temp on body - Ipsi loss of pain and temp on face - hoarseness, difficulty swallowing and loss of gag reflex - ipsi horner syndrome - vertigo nystagmus N/V - Ipsi cerebellar deficits (inferior cerebellar peduncle injury) NO loss of vibratory or position sense
523
What occurs in Weber syndrome?
Injured - Paramedian branch of PCA) - CL hemiparesis (cerebral peduncle lesion) - Ipsi ptosis, pupil dilation and eye down and out (CNIII damage)
524
What occurs in Medial inferior pontine syndrome?
Injured: Paramedian branch of Basilar A - CL spastic hemiparesis - CL loss of light touch vibratory sense - Paralysis of gaze tot he side of the lesion - Ipsi loss of lateral rectus m.
525
What occurs in Lateral inferior pontine syndrome?
Injured: AICA - Ipsi facial n paralysis - Ipsi loss of tase from ant 2/3 of tongue - Ipsi deafness and tinnitus - nystagmus, vertigo, N/V - Ipsi limb and gait ataxia - Ipsi loss of pain and temp on face - CL loss of pain and temp on body - Ipsi Horner syndrome
526
What occurs in lateral superior pontine syndrome?
Injured: AICA - Ipsi loss of taste in Ant 2/3 tongue - ipsi limb and gait ataxia - ipsi loss of pain and temp on face - ipsi loss of light touch and vibration on face - ipsi jaw weakness and deviation of jaw toward the lesion - CL loss of pain and temp on body - ipsi horner syndrome
527
What occurs if you have an MCA problem on the left hemisphere? right hemisphere?
Left is usually dominant = aphasia | right = hemineglect (nondominant side)
528
What is the injury for someone with hemiparesis and hemiplegia?
CL lateral striate artery problem | affects striatum and internal capsule
529
what effects are specific to PICA lesions?
Nucleus ambiguous injury like that seen in lateral medullary (Wallenberg) syndrome "Don't pick a (PICA) horse (hoarseness) that can't eat (dysphagia)"
530
What is specific to facial nucleus effects?
AICA lesions like seen in lateral pontine syndrome | "Facial droop means AICA's pooped"
531
What is commonly seen with a saccular aneurysm of the posterior communicating artery?
CN III palsy - eye down and out with ptosis and blown pupil
532
What is commonly seen with a saccular aneurysm of the Anterior communicating artery?
visual field defects
533
A patient is denying the Left half of their world what is the lesions?
MCA Right parietal lobe
534
What is associated with berry aneruysms?
ADPKD, Ehlers Danlos syndrome, Marfan's syndrome
535
What is the most common site of a berry aneurysm?
Bifurcation of the anterior communicating artery
536
What is the most common complication of berry aneurysm? second complication?
Most common - rupture leading to subarachnoid hemorrhage or hemorrhagic stroke can also lead to bitemporal hemianopsia if it compresses the optic chiasm
537
What are Charcot - Bouchard microaneurysms?
Asociated with chronic hypertension affects the small vessels like in basal ganglia or hypothalamus due to lenticulostriate arteries causes hematomas in the brain parenchyma
538
What is the middle meningeal artery a branch of?
Maxillary artery
539
What can an epidural hematoma lead to?
transtentorial herniation, CN III palsy
540
Hyperdense blood collection that doesn't cross suture lines
Epidural hematoma Can cross the falx and tentorium
541
Crescent shaped hemorrhage that can cross suture lines
Subdural hematoma can lead to midline shift cannot cross the falx or tentorium
542
Where does the bleeding occur in a subdural hematoma?
Between the arachnoid and dura
543
Where does bleeding occur in a subarachnoid hemorrhage?
Between the arachnoid and pia
544
What are two risks seen with subarachnoid hemorrhage?
Risk of vasopasm due to blood breakdown (not visible on CT) - TX with nimodipine (dihydropyridine CCB - dilates blood vessels) Risk of rebleed - visible on CT
545
List the layers for lumbar puncture
``` Skin Superficial fascia Ligaments - supraspinatus - interspinous - ligamentum flacum Epidural space (anesthetics) Dura mater Subdural space arachnoid membrane subaracnoid space - CSF - more potent if you put anesthetics here ```
546
When does damage start with hypoxia in the brain?
Irreversible damage begins after 5 minutes of hypoxia
547
What ares of the brain are most vulnerable to ischemic damage?
Hippocampus, neocortex, cerebellum and watershed areas
548
What is seen on MRI and CT with different types of strokes?
Bright areas on noncontrast CT indicate hemorrhage Brign on diffusion weighted MRI in 3-30 mins and remains bright for 10 days = ischemic stroke dark on noncontrast in 24 hrs = ischemic stroke
549
How does tPA work?
degradation of fibrin through conversion of plasminogen to plasmin
550
When can you give tPA?
Ischemic stroke Within 4.5 hrs of the stroke as long as the patient presents within 3 hrs of onset and there is no major risk of hemorrhage
551
Describe a RIA?
Brief, irreversible episode of focal neurologic dysfunction typically lasting <24 hrs without acute infarction
552
What causes a communicating hydrocephalus?
Decreased CSF absorption
553
What causes a normal pressure hydrocephalus?
increased subarachnoid space volume but no increased in CSF pressure - expansion of ventricles distorts the fibers of the corona radiate and leads to the wet, wobbly and wacky (form of communicating hydrocephalus)
554
What is a non-communicating hydrocephalus?
Caused by a structural blockage of CSF circulation within the ventricular system
555
How many spinal nerves are there?
31 total | 8 cervical, 12 thoracic, 5 lumbar, 5 sacral and 1 coccygeal
556
what is a vertebral disc herniation
Nucleus pulposus herniates through the annulus fibrosus
557
Saddle anesthesia
Cauda equine syndrome
558
A patient presents with urinary incontinence, paresthesias of medial thigh and she reports shes unable to feel toilet paper on her perineum when wiping after urination. The patient reports mild back pain with the initial onset of her symptoms.
Cauda Equina Syndrome
559
What is the Romberg test for?
``` The Romberg does NOT test the cerebellum@ This was developed to check for Tabes DOrsalis (dorsal columns) in tertiary syphilis Need 2/3 to work 1. Proprioception in legs 2. vision 3. vestibular apparatus ```
560
Describe the findings in Poliomyelitis and Werdnig Hoffman disease?
LMN lesions only, due to destruction of anterior horns - flaccid paralysis Werdnig Hoffman is AR
561
Describe the findings in multiple sclerosis
mostly white matter of cervical region, random and asymmetric lesions due to demyelination Charcot's triad: scanning speech, intention tremor and nystagmus
562
Describe the findings in Amyotrophic lateral sclerosis
Combined UMN and LMN deficits with NO sensory, cognitive or oculomotor deficits. (demyelination of motor neurons of anterior horn and lateral corticospinal tract)
563
What can cause ALS?
Defect in superoide dismutase I
564
What drug can be used in ALS?
Riluzole treatment modestly increases survival by decreasing presynaptic glutamate release
565
What is seen with complete occlusion of anterior spinal artery?
Bilateral loss of paint and temp, motor command and UMN and LMN signs slow below the lesion you have complete motor loss and loss of pain and temp and areflexia - will have intact pinpoint and vibratory discrimination
566
What is sparred in an injury to the anterior spinal artery?
Dorsal columns and Lissauer's tract
567
What is a water shed area when thinking of an anterior spinal artery lesion?
Upper thoracic ASA territory is a watershed area, as artery of Adamkiewicz supplies ASA below T8
568
What are the findings in a patient with Tabes dorsalis?
Degeneration of dorsal columns and roots leading to impaired sensation and proprioception and progressive sensory ataxia (inability to sense or feel the legs) will have absence of DTRs and positive Romberg
569
What can be seen in a Vitamin B12 or vitamin E deficiency?
Subacute combined degeneration leading to demyelination of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts patients will have ataxia gait, paresthesia, impaired position and vibration sense
570
What are the lab findings in a patient with Poliomyelitis?
CSF with increased WBCs with slight elevation of protein with no change in CSF glucose. Virus can be recovered from stool or throat
571
What is the defect in Friedrich's ataxia?
Impairment in mitochondrial functioning
572
How does Friedrich's ataxia present?
In childhood with kyphoscoliosis
573
In a patient with Horner's syndrome, what causes the Ptosis?
Loss of the superior tarsal muscle
574
What is the dermatome at the inguinal ligament?
L1
575
what is the dermatome at the knee caps?
L4
576
What is the function of the superior colliculi?
Conjugate vertical gaze center
577
WHat is the function of the inferior colliculi?
Auditory
578
What is Parinaud syndrome?
Paralysis of conjugate vertical gaze due to lesion in the superior colliculi
579
where are the cranial nerve nuclei located?
``` In tegmentum portion of brain stem Midbrain - 3 and 4 pons - 5-8 medulla - 9, 10, 12 Spinal cord - 11 ```
580
What is the nucleus solitaries?
Visceral sensory info for CN 7, 9, 10 | taste, baroreceptors, gut distension
581
What is the nucleus ambiguous?
Motor innervation of pharynx, larynx, and upper esophagus CN 9 and 10 (swallowing, palate elevation)
582
What is the Doral motor nucleus?
Sends autonomic (PS) fibers to the heart lungs and upper GI for CN 10
583
what are the branches of the Facial nerve?
``` Ten Zebras Bit My Chin Temporalis Zygomatic Buccal Marginal mandibular Cervical ```
584
What nerves run through the cavernous sinus?
3, 4, V1 and V2, and 6
585
Cavernous sinus syndrome
Ophthalmoplegia and decreased corneal and maxillary sensation with normal vision
586
CNV motor lesion
Jaw deviates toward the side of lesion due to unopposed force from the opposite pterygoid muscle
587
CN V lesion
Uvula deviates away from the side of lesion. Weak side collapses and uvula points away
588
CN XI lesion
Weakness turning head to CL side of lesions (SCM) Shoulder droop on side of lesion (trapezius)
589
CN XII lesion
Tongue deviates toward the side of lesion due to weakened tongue muscles on the affected side
590
How do aminoglycosides cause hearing loss?
By damaging the outer hair cells
591
What can cause Facial nerve palsy?
Complication in AIDS, Lyme disease, Herpes simplex and (less common) herpes zoster, sarcoidosis, tumors and diabetes
592
Which mastication muscle opens the jaw? what opens the jaw?
Closes: Lateral pterygoid Opens: Masseter temporalis, medial pterygoid all by cnV3
593
What can cause retinitis?
Commonly viral - CMV, HSV, HZV, or immunosuppresion
594
Acute painless monocular vision loss with retina whitening with cherry-red spot on macula
Central retinal artery occlusion
595
Painless acute onset of blurred vision in one eye with Blood and thunder fundus - dilated and tortuous retinal veins
Retinal vein occlusion
596
Acute painless onset of monocular vision loss in background of atherosclerosis leading to retinal ischemia that comes and goes, commonly from ischemic originating from atherosclerotic carotid A disease
Amaurosis fugax
597
Remembering what nerve innervate the eye muscles...
LR6SO4R3 Lateral rectus - CN 6 Superior oblique - CN 4 The rest - CN 3
598
what should you not give a patient with acute closure closed angle glaucoma?
Epinephrine - has mydriatic effect (dilation of pupil)
599
Painless often bilateral opacification of the lens leading to decreased vision
Cataracts
600
Enlarged blind spot and elevated optic disc with blurred margins seen on fundoscopic exam
Papilledema due to increased intracranial pressure causing optic disc swelling
601
What is seen with CNIV damage?
Eye moves upward, particularly with CL gaze and ipsilateral head tilt
602
What is a Marcus Gunn pupil?
Afferent pupillary defect due to optic nerve damage or retinal detachment have decreased bilateral pupillary constriction when light is shone in the affected eye relative to the unaffected eye
603
A patient has a posterior communicating artery aneurysm - what effect is seen with CN III?
PS output is affected first (periphery of the nerve) signs include a diminished or absent pupillary light reflex and a "blown pupil" also seen with an uncal herniation
604
A patient has diabetes - what effect is seen with CNIII?
effects the inner portion of the CN III leading to los of motor components - ptosis and down and out eye
605
a patient complains of floaters and flashes in their vision followed by monocular loss of vision like a curtain drawn down
Retinal detachment fundoscopy will show a wrinkled retina
606
What exactly is retinal detachment
Separation of the neurosensory layer of the retina (photoreceptor layer with rods and cones) from the outermost pigmented epithelium
607
What can cause multi infarct dementia?
Syphilis, HIV, Vitamins B1, B3, or B12 deficiency, Wilson's disease and Normal pressure hydrocephalus
608
What is the gold standard for looking at MS?
MRI
609
List 3 treatments for MS?
Interferon beta, immunosuppression and natalizumab
610
what is another name for Guillain-Barre?
Acute inflammatory demyelinating polyradiculopathy
611
What is Guillain Barre syndrome?
Autoimmune condition that destroys Schwann cels leading to inflammation and demyelination of peripheral nerves and motor fibers
612
Results in symmetric ascending muscle weakness/paralysis beginning in lower extremities
Guillain Barre
613
What can cause Guillain barre?
Campylobacter jejuni or CMV infection leading to molecular mimicry causing autoimmune attack of peripheral myelin
614
What are the lab findings on someone with Guillain Barre?
Increased CSF protein with normal cell count (albuminocytologic dissociation). Increased protein leads to papilledema
615
Progressive multifocal leukoenceophalopathy
Demyelination of CNS due to destruction of oligodendrocytes Seen in 2-4% of AIDS patients due to reactivation of latent viral infection (JC) rapidly progressive and usually fatal - virally infected oligodendrocytes
616
Multifocal perivenular inflammation and demyelination after infection (commonly measles or VSV) or certain vaccines (rabies or smallpox)
Acute disseminated (postinfectious) encephalomyelitis
617
AR lysosomal storage disease most commonly due to arylsulfatase A deficiency. Causes build up of sulfatides leading to impaired production of myelin sheath
Metachromatic leukodystrophy
618
How much of the brain to partial seizures involve?
Affect I focal area of the brain - most commonly originates in medial temporal lobe
619
What are causes of seizures in children? adults? elderly?
Children - genetics, infection (febrile), trauma, congenital, metabolic adults - tumors, trauma, stroke, infection Elderly - stroke, tumor, trauma, metabolic and infection
620
Name 5 types of Diffuse generalized seizures
``` Absence (petit mal) - 3 Hz no postictal confusion, will have blank stare Myoclonic Tonic-clonic (grand mal) Tonic Atonic - can be mistaken for fainting ```
621
Name two types of Partial (focal) seizures?
Simple partial - consciousness intact, can be motor, sensory, autonomic, psychic Complex partial - impaired consciousness
622
what can be triggers for cluster headaches?
Alcohol or vasodilators
623
what are migraine patients at increased risk for?
Stroke
624
Congenital disorder with port wine stains (nevus flammeus), typically in V1 distribution, ipsilatereal leptomeningeal angiomas, pheochromocytomas.
Sturge Weber syndrome can cause glaucoma, seizures, hemiparesis, and mental retardation Occurs sporadically
625
Describe Tuberous Sclerosis
``` AS HAMARTOMAS: Hamartomas in cns and skin Adenomya sebaceum (cutaneous angiofibromas) Mitral regurgitation Ash leaf spots cardiac Rhabdomyoma Tuberous sclerosis autosomal dOminant Mental retardation renal Angiomyolipomas Seizures ```
626
Café au lait spots, Lisch nodules (pigmented iris hamartomas) neurofibromas in skin, optic gliomas, pheochromocytomas,
Neurofibromatosis type I (von Recklinghausen disease) AS mutated NF1 gene on Chrom 17 (tumor suppressor)
627
Describe von hippel-landau disease
Cavernous hemangiomas in skin, mucosa, organs; bilateral renal cell carcinoma hemangioblastoma in retina, brain stem, cerebellum, pheochromocytomas AD mutated tumor suppressor VHL gene on chrom 3
628
What is the mnemonic for the 4 most common adult tumors?
``` MGM Studios Metastasis Glioblastoma Meningioma Schwannoma ```
629
Brain tumor that arises from arachnoid cells, are external to brain parenchyma and may have a dural attachment ("tail")
Meningioma | often asymptomatic may present with seizures or focal signs
630
Which brain tumor has spindle cells concentrically arranged in a whorled pattern and can often have psammoma bodies?
Meningioma
631
how do you treat a schwannoma?
Resectable or treated with stereotactic radiosurgery
632
Brain tumor commonly found at the cerebellopontine angle
Schwannoma S-100 positive
633
Where is Oligodendroglioma brain tumor most commonly found?
Frontal lobes
634
Brain tumor with a chicken-wire capillary pattern with fried egg cells
Oligodendroglioma
635
What are the three most common childhood brain tumors?
Animal kingdom, Magic kingdom, Epcot Astrocytoma Medulloblastoma Ependymoma
636
Where is a pilocytic astrocytoma most commonly found?
Posterior fossa (cerebellum)
637
Which brain tumor shows Rosenthal fibers?
Pilocytic astrocytoma
638
Where is medulloblastoma most commonly found?
Cerebellar vermis, highly malignant
639
Which brain tumor shows Homer Wright rosettes and is radiosensitive?
Medulloblastoma
640
What can occur with a Medulloblastoma?
Can send drop metastasis to spinal cord
641
Which brain tumor has rod shaped belpharoplasts (basal ciliary bodies) found near nuclei?
Ependymoma
642
What can Hemagnioblastomas produce?
EPO leading to secondary polycythemia
643
Which brain tumor has foamy cells ad high vascularity?
Hemangioblastoma
644
What is the most common childhood supratentorial brain tumor?
Craniopharyngioma
645
Brain tumor that's epithelial lined mass with cystic degeneration and calcifications
Craniopharyngioma
646
Which hernia can result in compression of the anterior cerebral artery?
Cingulate (subfalcine) herniation under the falx cerebri - can lead to lower leg weakness with out sensory loss, paralysis of CL foot, re occurrence of primitive reflexes (frontal lobe)
647
List 4 things that can occur with an uncal (transtentorial) herniation
- can compress CN III leading to a blown pupil with down and out eye - can have CL homonymous hemianopsia - Ipsilateral paresis - Compression in paramedian A
648
What is a transtentorial herniation?
When medial part of temporal lobe herniates over the free edge of tentorium - can compress ipsilateral posterior cerebral artery, occulomotor nerve and cerebral peduncle