MBB2 Flashcards
(349 cards)
1
Q
what cns immune cells get activated due to multiple sclerosis inflammation
A
microglia majorly (from fibrin entering)
and astrocytes
2
Q
For botulism:
what causes it?
where can it be found?
what is the pathophysiology
what symptoms are present?
what would you see on emg/ncs?
A
- Clostridium Botulinum, gram positive bacilli with spores (A,B,E,F,G)
- canned food, honey (infantile, intestinal form), deep wound
- disrupts snare protein formation –> decreased ach
- flaccid paralysis (proximal>distal, symmetric), subacute onset, hypotonia, dysautonomia, decreased reflexes.
facilitation of motor response, presynaptic dysfunction?
3
Q
what are the three types of seizures where consciousness is most likely impaired
A
- atonic
- focal unaware
- tonic clonic (secondary or primary)
4
Q
what is a pathognomonic finding for neuromyelitis optica
what parts of the cns are affected
A
- NMO IgG against aquaporrin 4 on astrocyte end feet
- optic nerve and spinal cord
5
Q
what is the inheritance of duchenne muscular dystrophy?
what causes it
name clinical manifestations
what are treatments
A
- x-linked recessive
- deletion or mutation in dystrophin gene
- progressive muscle weakness before age 5, toe walking, waddling gait, inability to run, inability to walk properly, compensatory muscle hypertrophy, pseudophypertrophy of calves, gower’s sign, cardiomyopathy
- exondys 51- skips over exon 51 to allow for more dystrophin production
- ataluren- reads through ribosomal nonsense mutations, allowing functional protein to be made
6
Q
describe the side effects of each of these chemo treatments
cytarabine
methotrexate
ifosfamide
A
: seizures coma, ataxia dysarthria, dementia
confusion, ataxia, drowsiness
- decreased glutathione, hallucinations, encephalopathy, ataxia, confusion
7
Q
what is the difference between muscle spasm and spasticity
A
- spasm: sudden involuntary contraction of a muscle due to tissue trauma or strain
- spasticity: increase fflexor or extensor tone due to increase velocity-sensitive stretch reflexes
8
Q
what type of tumor can have calcifications in the white matter
A
oligodendroglioma
9
Q
if anti epileptic drugs do not work, what can you do (4) and for which seizure type
A
- surgical resection of anteromedial temporal lobe or focal neocortical regions (focal unaware)
- stimulation of vagus nerve (prevents synchronous stimulation of neurons necessary for seizure) (any partial)
- responsive neurostimulation (any partial)
- deep brain stimulation of the anterior thalamus
10
Q
HIV dementia invades which two cells types
A
microglia and astrocytes (not neurons )
11
Q
what causes HIV PML
what parts of the brain would be normally involved
what cells are affected
what is the treatment
A
- latent jc virus, also seen with natalizumab treatment (MS and crohns)
- fronto parietal area and occipital lobe
- oligodendrocytes (looks similar on imaging to ms)
- none, will progress to dementia and death in 3-6 months
12
Q
what is thoracic outlet syndrome
what 2 things are compressed
A
- when the vasculature/nerves that go between the collar bone and first rib get compressed
- subclavian artery and lower trunk
13
Q
what does the dorsal column carry
what are the two parts
A
- fine touch and proprioception
- gracilus: medial: legs
- cuneate: lateral : arms
14
Q
what is baclofen used for
- what does it bind to
- what two ways can it be administered
A
- muscle relaxant for multiple sclerosis, cerebral lesions, spinal cord lesions
- binds to GABA- B receptors (2 gpcr’s put together)
- oral or intrathecal
15
Q
name at least 5 causes of floppy baby syndrome
A
- polio
- botulism
- down syndrome
- CNS encephalopathy
- spinal muscular atrophy
- cerebral palsy
- kernicterus
- guillan barre
- myasthenia gravis
- intracranial hemorrhage
- inborn errors of metabolism
16
Q
what is autonomic dysreflexia?
describe symptoms
what is the usual precipitating cause of the dysreflexia
A
- condition that persists with injuries above T6; noxious stimuli below T6 cause bananas autonomic response
- dangerous elevation in bp, flushing above level of injury, bradycardia, goose bumps
- bladder distension (also constipation, fracture, pressure wound)
17
Q
what are two meds that can treat sleep disorders, what do thy treat
A
- ramelteon, binds mt1 and mt2 receptors, treat insomnia
- tasimelteon, bind mt1 and mt2, can treat non 24 hour sleep wake disorders
18
Q
what is the mnemonic for glasgow coma scale
what would a score of 15 mean
what would a score of 3 means
A
- Everyone Must Vote 4 65 years
-Eye movement- 4
Motor- 6
verbal repsonse- 5 - completely alert and conscious
- unconscious completely (lowest possible score)
19
Q
what does mngie stand for
what symptosm does it have
what mutation is present
A
mitochondrial neurogastrointestinal encephalopathy syndrome
skeletal muscle abnormalities and gastrointestinal dysmotility
- thymadine phosphorylase
20
Q
briefly describe the pupillary light reflex
name the three parts
A
- upon exposure to light in one eye, both eyes should constrict
- optic nerve (afferent) –> midbrain (edinger westphal) –> occulomotor nerve (efferent)
21
Q
what age group as craniopharyngiomas common in? where are they located?
what microscopic/imaging findings would their be
A
- children/adolescents, near the pituitary
- imaging: mixed solid/cystic tumor
- microscopic, white keratin, calcifications, basiloid cells lining cysts
22
Q
For Pompe Disease (Type II GSD):
what enzyme is affected
what is the clinical manifestation
A
- acid maltase
- muscle weakness respiratory difficulty
23
Q
what is the treatment for acute muscle spasms (3)
how do they act
can they be used for spasticity or muscle spasms from cerebral palsy/spinal cord injury?
A
- carisoprodol, methocarbamol, cyclobenzaprine
- Clarissa is pro meth >carbs but now she can’t afford her sick benz.
- depression/sedation
- no
24
Q
what is the most dangerous type of status epilipticus: convulsive, absence, or partial
A
convulsive
25
small vessel damage from hypertension can cause _______ or _______
- lacunar infarct or hypertensive hemorrhage
26
what symptoms would an infarct to the right or left anterior cerebral artery stem produce
left: (1) grasp reflex, (2) frontal lobe behavioral abnormalities, (3) right leg weakness and sensory loss ,(4) transcortical aphasia, (5) larger infarcts can cause right hemiplegia
right: (1) grasp reflex, (2) frontal lobe behavioral abnormalities, (3) left leg weakness and sensory loss, (4) left hemineglect, (5) larger infarct causes left hemiplegia
27
what is free running sleep disorder/non24 hr sleep wake disorder
what population is it common in
what is the treatment
- when sleep gets delayed further and further every day (like as if you have no light stimulus)
- blind people
- melatonin/tasimelteon before desired bed time
28
what non movement symptoms can you see in parkinson's disease
- memory problems/hallucinations
- mood problems (depression anxiety)
- dysautonomia (bowel, orthostatic hypotension, inconteninece, erectile)
- sleep problems
- choke on food
29
describe the process of how a migraine occurs (5 steps)
1) initiated deep in the brain
2) signal sent to cortex (posteriorly to anteriorly)
3) changes in neural activity and blood vessel activity causes visual changes, numbness, tingling,
4) presence of substance p, cgrp and nka cause dilation of bv and inflammation
5) inflammation reaches trigeminal nerve and irritates it --> severe pain
30
what are the four old antiepileptic drugs, and what side effects do they have in common
- don't be BBRaSH, Please Please Vaccinate your Children
- bone marrow suppression, birth defects, rash, steven johnson syndrome, hepatic inflammation
- phenytoin, phenobarbitol, valproate, carbamazepine
31
for emery-dreifus muscular dystrophy:
what is the inheritance
what are symptoms
- autosomal recessive, autosomal dominant, or x linked recessive
- contractures, muscle wasting, weakness, cardiomyopathies, cardiac conduction abnormalities
- lmna, emd, fhl1
32
what is the rate limiting step for conversion of serotonin to melatonin
what receptors does melatonin bind to and what kind of receptor is it
serotonin n acetyle transferase
mt1 mt2 g protein coupled receptor
33
what is the most common cranial neuralgia
describe it
what can treat it
- trigeminal neuralgia
- a brief stabbing/sharp facial pain in at least one V region that is triggered by facial movement
- carbamazepine, gabapentin
34
for myxopapillary ependymoma
who is predominantly affected and what is the who grade
where is the tumor usually
what is a common presenting symptoms
describe the gross and microscopic findings
- teenagers and young adults
- WHO I
- conus medullaris/filum terminale region
- back pain
- well circumscribed enhancing lesion
- cuboidal to elongated ependymal cells surrounding a stromal core in a papillary fashion
myxoid material in microcysts
35
what is more likely to present with an aura and forced head/eye deviation: primary generalized tonic clonic seizure or secondary generalized focal to bilateral tonic clonic seizure
focal to bilateral tonic clonic seizure
36
name three things you can detect on an EEG
- encephalopathy (creutzfeld jacob, hsv encephalitis)
- seizures
- coma
37
what are two other names for spinal muscular atrophy
what is the inheritance and the gene/chromosome affected
- what is affected
- how do the different types differ
- autosomal recessive proximal spinal muscular atrophy, 5q spinal muscular atrophy
- autosomal recessive, SMN gene on chromsome 5
- proximal muscles and respiratory muscules progressively wasting
- lower numbers are earlier onset and more severe
- higher numbers are later onset and less severe
38
how do cavernous hemangioma and arteriovenous malformation differ
- cavernous hemangioma is collection of blood vessels with no brain tissue in between them, usually incidental finding on autopsy
- arteriovenous malformation is common in young women, has arteries and blood vessels in close proximity with brain tissue in between them, can ve very dangerous if bleed
39
Infarct of what would cause alexia without agraphia?
what is alexia
- posterior cerebral artery
| - inability to read
40
what is familial hemiplegic migraine
- a type of migraine that is genetically linked; mutation of voltage cated ca channels on chromosome 19
41
what two cancer types originate from neurons
neuroblastoma
| medulloblastoma
42
what grade is medulloblastoma and where is it usually located
name 2 special consequences of being in it's particular location
what symptoms can you get
what would you see on micro
what population is affected
- grade 4 in the cerebellum
- hydrocephalus symptoms since near ventricles (nausea vomiting headache), can get drop mets to the spine through ventricles
- nausea vomiting headache gait ataxia/instability
- small blue cells (can be in lines), homer wright rosettes)
children
43
aura are common with what type of seizures
focal unaware (complex partial)
focal to bilateral tonic clonic (secondary generalized motor, think, this can start as focal unaware)
44
how does htn cause intraparenchymal hemorrhage
- causes a charcot vouchard aneurysm which can rupture
45
in what condition do you see paroxysmal breathing pattern and a bell shaped chest?
- spinal muscular atrophy 1/werdnig hoffman (floppy baby!)
46
what happens in a central herniation/what arteries are affected
- bilateral temporal lobes get pushed down, displacing brainstem downward, basilar
central pontine arteries get severed (duret hemorrhages) --> usually fatal
47
what is the inheritance of huntingtons disease and which repeat on which gene
what symptoms are common
how would you treat
- autosomal dominant, >40 CAG repeats on huntington gene on chromosome 4
- chorea (jerky movements v similar to dyskinesias), pscyhiatric problems, cognitive decline
- treat the psychiatric conditions more than the motor, anti-dopaminergic drugs liek risperidone, haloperidol atypical antipsychotics,
tetrabenzine (vmat2 inhibitor), but could make psychosis worse, SSRI's to treat depression
48
what is xanthochromia?
what two conditions can you distinguish when you see xanthochromia
discolored csf
- trauma- when you centrifuge, rbc's will go to the bottom and csf will be clear
- sub arachnoid hemorrhage, rbc's have had time to lyse, so xanthochromia will be retained
49
what is a kayser fleischer ring?
what disease is it common in
- dense deposit of copper around the border of the cornea
| - wilson's disease
50
what are the possible responses for vocal response for glasgow coma scale
5- oriented
4- confused
3- inappropriate (can say words, but doesn't make sense)
2- incomprehensible (making sounds, not words)
1) no response
51
what is process s
what is process c
what happens at both of their peaks
- the homeostatic drive for sleep (sleep debt) that accumulates during the day and peaks at night
- the circadian fluctuation in alertness that peaks at night
- peaks are both at the same time, but the sleep debt overwhelms the circadian alertness and we sleep to relieve the debt
52
what are two other names for anterior spinal cord syndrome
what is it
what causes it
name clinical manifestations
- anterior spinal artery syndrome, beck syndrome
- occlusion of anterior spinal artery/infarction of anterior 2/3 of spine
- atherosclerosis, arotic dissection, aortic aneurysm, aortic trauma, abdominal surgery
- loss of motor function at spinal level and below bilaterally
- low of pain and temperature bilaterally below level of spine
- proprioception and fine touch intact bilaterally
53
for Lambert Eaton Myasthenic Syndrome:
what are the type types and demographics effected
what is the pathophysiology
what are symptoms
what would you see on ncs/emg
what meds can you use
- paraneoplastic due to small cell lung cancer, prostate cancer, thymoma, or lymphoproliferative, male, 60s
- primary: bimodal 30 and 60 females >men
- antibodies to voltage gated calcium channels --> decreased ach --> muscle weakness
- proximal >distal muscle weakness (legs > arms), areflexia, autonomic dysfunction (dry mouth, constipation, orthostatic problems, erectile dysfunction, micturation problems)
- decrement of amplitude at low frequencies, amplification at high frequencies
- 3,4 Diaminopyridine (increase Ca influx, not in US)
- prednisone, azathioprine
- IVIG or PLEX in severe cases
- remove cancer
54
what microscopic and gross findings are associated with amyotrophic lateral sclerosis
- anterior nerve root, precentral gyrus atrophy
- degeneration of corticospinal neurons
- astrogliosis
- eosinophilic bunina bodies
55
what demographic is most likely to have an ependymoma
where can it be
what microscopic findings can you find
- children
infratentorial, spine, supratentorial
- perivascular pseudorosettes, true ependymal rosette
56
For Cori Disease (Type III GSD):
what enzyme is affected
what is the clinical manifestation
- glycogen debranching enzyme
| - myopathy
57
describe some changes to the brain when aging (macro and micro)
- brain gets smaller,
- widening of sulci
- narrowing of gyri
- widening of ventricles
- loss of dendritic spines and decrease in complexity of dendritiic tree
- demyelination (mostly in prefrontal cortex/genu and temporal lobe)
- build up of ROS
58
what symptoms outside of the brain can you experience with huntington's disease (mnemonic)
- STOIC Women Hunt
- skeletal muscle wasting
- testicular atrophy
- osteoporosis
- impaired glucose tolerance
- cardiac failure
- weight loss
59
what would emg testing show for focal entrapment/ nerve compressive neuropathy
- focal loss/slowing of tranmission/potentials at the sight of compression
- decreased or absent muscle response (denervation) that are innervated by the compressed nerve
60
what are normal csf values for pressure, wbcs, rbcs, proteins, glucose and appearance
```
Opening pressure : 100 to 180 mm H20
Appearance: clear & colorless
Cells: 0 to few WBCs, 0 to few RBCs
Protein: 15 – 50
Glucose: about 60 (2/3 of serum glucose)
```
61
where does the inferior division of the middle cerebral artery supply?
what symptoms would an infarct to the right or left side produce
- lateral cortex below sylvian fissure (only top half)
- left: (1) fluent aphasia (wernicke), (2) right visual deficit, (3) confusion, (4) may have right arm and face weakness or sensory loss
- right: (1) profound left hemineglect, (2) left visual deficit and sensory loss (3) right gaze preference, (4) motor neglect, (5) some right sided weakness
62
what is an essential tremor; what is the inheritance
what is the pathophysiology
what treatment is very effective in decreasing symptoms?
- a usually hereditary ACTION tremor, thats it nothing else; autosomal dominant
- not sure, but increased blood flow to cerebellar hemispheres and red nucleus, also maybe afferent motor area of thalamus that receives input rom the cerebellum
- deep brain stimulation of the ventral intermediate nucleus of the thalamus
63
for tuberous sclerosis
what is the inheritance and genes involved
what features would you expect
- autosomal dominant, tsc1 and 2
- angiomyolipoma, rhabdomyoma, subependymal giant cell astrocytomas (SEGA) --> astrocytes in sheath under ependymal layer that bulge into ventricle
64
what are the two parts of the corticospinal tract and how do they differ
- lateral corticospinal (90%), decussates at medulla and modules motor and interneurons
- anterior corticospinal (10%)- decussates at the level of the spine it innervates
65
what specific type of neurons are very susceptible to changes associated with aging
why (give at least 3 reasons)
- dopaminergic neurons in sustantia nigra
- dopamine can be converted into superoxides (ROS)
- neuromelanin sequesters iron, which is a catalyst for ROS formation
- dopaminergic neurons in snc have many branches to basal ganglia and requires hella energy
- snc has highest amount of mitochondrial dna deletions
66
what kind of drugs can cause parkinsons and why?
give some examples
- antipsychotics and anti-emetics; they block dopamine
| - risperidone, haloperidol, promethazine
67
for TB meningitis:
how fast of an onset is it
how can it present?
what lab values would have have
what is the treatment
- subacute, 4 weeks or less
- headache, fever, cranial nerve palsies (its' a basilar meningitis), neck pain, confusion, obtunded, seizures vomiting
- mild to severely depressed glucose, mononuclear wbcs, elevated protein; positive afb stain or pcr
- RIPE, rifampin, isoniazid, pyrizinamide, ethambutol
68
when is your core body temperature at it's highest? at it's lowest?
when does sleep start to occur; when does it stop
- highest: during the day
- lowest: when sleeping (2 hours before waking)
as core body temperature starts to decrease; as cbt starts to increase
69
for Lyme disease:
when does it occur
how can it present?
what lab values would have have
what is the treatment
- in summer in the north east
- erythema migrans, polyarthralgia, bilateral facial nerve palsy, headaches, fever, neck
- normal pressure, normal glucose, mononuclear wbc's, elisa testing
- ceftriaxone or penicillin
70
what causes tabes dorsalis
what part of the spinal cord is affected
- tertiary syphillis
| - demyelination of the posterior column
71
_____ accumulates in the _____ (part of the brain) to the overwhelming extent that it makes you sleep
what substance is an antagonist to this receptor
- adenosine, basal forebrain
| - caffeine (adenosine receptor antagonist)
72
what gene and chromosome is affected in neurofibromatosis type 2
inheritance?
what features would you expect
- merlin chromsome 22
autosomal dominant
- bilateral schwannomas at the cerebellarpontine angles, meningiomas, epyndemomas
73
what is the most common cause of cns helminth infection
what are presenting symptoms
what lab values are important
what is the treatment
- cysticercosis due to taenia solium from pork tapeworm larva
- low grade meningitis with hydrocephalus, mass effect, or seizures
- ct/mri absolutely necessar; would show cysts/calcified lesions of varying ages; mononucular cells AND eosinophils
- supportive, steroids, anti helminth drugs (albendazole), anti seizure
74
what medications can be used for essential tremor? what part of the tremor can be minimized?
- propanalol, primidone, topiramate
| - hand tremor
75
what are clinical manifestations of peroneal neuropathy?
what can cause it?
what can it be mistaken for and how do they differ
- weakness in ankle dorsiflexion and eversion --> foot drop
- cross your legs
- l5 radiculopathy (will also have issures with ankle inversion)
76
what is the most common cause of non epidemic fatal encephalitis
how does it spread
what is the initial rpesentation
what is the neurologic presentation
what would you see on imaging
what lobe of the brain is characteristically involved
what would you use to treat
- HSV 1!
- respiratory droplets and saliva
- asymptopmatic, gingivastomatitis, if immunocompromised can spread quickly and cause death
- headache and fever, seeizures and coma later on, if fronto temporal lobe involved, behavioral changes
- loss of gray white matter interface, edema mass effect
temporal lobe
- acyclovir
77
name acquired causes of cerebral palsy
- meningitis, encephalitis, head injuries trauma falls, motor vehicle accidents
78
where in the brain do metastatic tumors tend to be and what do they look like
- at the gray white junction, tend to be well circumscribed and enhanced and microscopically look like tumor of origin
79
for polymyositis:
who is affected
what are the symptoms
what infiltrate is responsible
women age 20+ >men
- proximal symmetric weakness and bulbar symptoms, myocarditis, ild, malignancy
- CD8
80
what are two components of consciousness/normal mental status
what two parts of the brain are very important
- arousal/wakefulness and content/awareness
- ascending reticular activating system (projects to hypothalamus and cerebral cortex)
- cerebral cortex
81
what type of seizure is a tonic clonic seizure
what is it characterized by
what would an eeg show
- primary generalized motor
- loss of consciousness and post ictal lethargy/confusion; 30-120 seconds, first part is tonic --> contraction, fall to the ground with ictal cry, cyanosis, incontinence, tongue biting, overall stiffness
second part is clonic symmetric jerks
- generalized polyspike waves or polyspike
82
what chromosomal deletions confer a better prognosis for oligodendroglioma
1p 19q
83
what medications would you use for strep pneumo and gram positive meningitis
what about listeria
ceftriaxone and vancomycin
- ampicillin
84
what type of aphasia has inability to repeat, fluent, but no comprehension
- wernicke's aphasia
85
what causes prion diseases
how does the disease usually progress
what would eeg show
what would imaging show
what symptoms could yo uhave
- accumulation of abnormal folded PRP sc protein
- progression in 12 months to akinetic mute and coma
- typical triphasic waves
- atrophy and enhancement of basal ganglia and cortical areas
- dysphagia, apraxia, cerebellar problems, myoclonus, aphasia
86
what are the most common populations affected by fungal meningitis
what types of fungi are common
what are symptoms
what labs/imaging are important
- chronic steroid use, hiv, immunosuppressed, iv drug user
- cryptococcus, cocciodes, aspergillus, candida, nocardia, histoplasma, blastomycoses, actinomyses
- meningitis symptoms, maybe mass effect
- need to get ct/mri, glucose slightly decreased, proteins increased
87
what are the main symptoms of parkinson's disease
does the disease usually affect both sides of the body symmetrically or asymetrically
what are a common side effect of Parkinson disease medication; what is another possible side effect
bradykinesia
resting tremor
rigidity
festinating gait (and stooped posture)
- asymmetrically
- dyskinesias, hallucinations
88
what are symptoms of myasthenia gravis in order of most common to least common?
does weakness get better or worse with activity?
how are reflexes?
1) occular: ptosis and binocular double vision
2) oropharyngeal: swallowing, chewing
3) limb muscles proximal >distal
4) head drop an sob from respiratory muscle weakness
gets worse with activity (fatiguable weakness)
- reflexes completely intact (sensory and motor fine)
89
what is the classic tetrad for bacterial meningitis (mnemonic)
MEN are a HANdFull
- headache
- altered mental status
- neck stiffness
- fever
90
what is advances sleep phase disorder
what can you do to treat it
- rhythm is phase advanced, meaning you are sleeping earlier
| - light therapy at niht before the cbt min or melatonin in the morning
91
what does sdh stain for
what does COX stain for
- nuclear dna
| - mitochondrial dna
92
what characterizes dystonia?
what treatment is effective at helping decrease symptoms
- contraction of antagonistic muscles --> twisting/contorting movements
- deep brain stimulation of the globus pallidus
93
how does chronic inflammatory demyelinating polyneuropathy differ from aidp
- peaks at 8 weeks vs 4 weeks
- due usually to MGUS (can become multiple myeloma)
- requires longer immunotherapy
94
for dermatomyositis,
what is the cause
what are symptoms
what demographic is affected
- its a microangiopathy that affects the skin and muscles, complement deposition --> ischemia, cd4 and inflammatory infiltrates
proximal>distal weakness and legs> arms, heiotrope (aroudn the eyes) rash, gotran papules, myocarditis, vasculitis, malignancy
women>men
95
describe what happens in hepatic encephalopathy
- increased glutamate --> swelling of astrocytes --> edema
| - increased ammonia
96
what is an apnea test for, and describe it
what is a positive result?
- tests for brain death, remove the ventilator and supply oxygen via small catheter, in normal person, the increase of PCO2 would cause subsequent hyperventilation to remove PCO2, in brain dead person, not capable of doing this so co2 would accumulate
- pco2 >60 or >20 from baseline
97
name congenital causes of cerebral palsy
- periventricular leukomalacia- damage to white matter (sensitive from weeks 26-34)
- cerebral dysgenesis (abnormal formation, can be due to genes, infection, fever)
- intracranial hemorrhage- stroke, maternal hypertension, maternal infection
- hypoxic ischemic encephalopathy (lack of oxygen to the brain), maternal htn, placental detachment, uterus ruptura, stress delivery can cause stroke
98
describe creutzfeld jacob disese
- rapidly progressive dementia with degeneration of the cerebral cortex, basal ganglia, brainstem, and spinal cord
- genetic, sporadic (most common), or transmissable from surgery, transplant, or eating beef
99
what are melanopsin retinal ganglion cells and what do they do
what is the main neurotransmitter they transmit
- retinal ganglion cells that respond to cell but send non-visual photic info to the suprachiasmatic nucleus in the hypothalamus
- glutamate and pituitary adenylyl cyclase activating peptide (PACAP)
100
what is a less severe form of duchenne muscular dystrophy
what causes it
how does it differe
- becker muscular dystrophy
- out of frame mutation
- less severe phenotype, can ambulate past 15, less likely to get cardiomyopathy
101
for charcot marie tooth 1,
what is the cause/gene/chromosome?
what are the symptoms (3)
what is the common presenting symptoms
- familial, usually PMP22 gene duplication on chromosome 17
- champagne neck legs (due to muscle atrophy, pes cavus (high arch foot), hammer toes, sensory numbness, distal>proximal weakness
- foot drop
102
what is the only nerve that comes off of a trunk of the brachial plexus
suprascapular nerve
103
the anterior spinal artery supplies most of the spinal cord except for what
- the dorsal column
104
how does age differ for those that suffer from primary generalized tonic clonic seizure to those who suffer focal to bilaterall tonic clonic seizure (secondary generalized)
- primary: childhood or adolescence
| - secondary: any age but if >30, almost always secondary
105
what pathology microscopic findings can you find for alzheimers
- tangles- hair shaped in the hhippocampus or globose in the cortex
- neuritic (dystrophic neurites around amyloid core) and diffuse plaques (no neurites)
- cerebral amyloid angiopathy (CAA)- donut shaped hyalinaosis of leptomingeal or intrapareynchemal arteries that can rupture and cause lobar infarct
- hirano body- glassy eosinophilic bodies made of actin
- granulovacuolar degeneration- clear cytoplasmic inclusions with basophilic granules
106
give acute reasons to do a lumbar puncture
give chronic/subacute reasons to do a lumbar puncture
- subarachnoid hemorhage, infection (meningitis, encephalitis), demyelinating disease (guillain barre)
- infection, multiple sclerosis, idiopathic intercranial hemorrhage, neurosarcoid, carcinomatous meningitis
107
what are the two types of surgery you can do for parkinsons?
pallidotomy (put a hole in the globus pallidus internus)
- deep brain stimulation of globus pallidus internus > subthalamic nuclei
108
Name three indications for diagnostic lumbar puncture
name 2 indications for therapeutic lumbar puncture
- measure csf pressure
- extract csf for lab testing
- to inject radioactive tracer or contrast dye
- inject intrathecal anesthetic or chemo
- to remove csf to reduce pressure
109
what are two causes of osmotic demyelination syndrome
- chronic alcoholism
| - correcting hyponatremia too quickly or over correcting
110
what is the difference between spasticity and rigidity
- spasticity is velocity dependent and affects certain muscles preferentially
upper extremities flexors and adductors >extensors
lower extremities: extensors >flexors
- rigidity has resistance t passive movement regardless of velocity or muscle direction
111
what is the kindling model
- you can keep giving repetitive subthreshold stimuli for seizures and overtime, you will have an abnormal enough neuronal pattern that you will get a seizure from the subthreshold stimuli
112
what type of aphasia has impaired repetition, fluent, and impaired comprehension
transcortical sensory aphasia
113
for subdural hemorrhage,
what is usually the cause
are veins or arteries the cause?
what is the shape of the
- trauma
- cerebral bridging veins goign from cortex to sinuses (NOT DURAL SINUS LACERATION)
- crescent (follows dura mater)
114
describe what happens in a myasthenia crisis
what can treat it
- internal intercostal, accesory muscle, and diaphragm failure --> decrease pulmonary function
- external intercostal muscles weak --> decreased ability to cough --> aspiration
- weakened oropharyngeal muscles -- > can't swallow and can't clear secretions --> aspiration
- IVIG or PLEX (plasma exchange)
115
what is ocrelizumab
what disease can it help treat
- antibody against cd20 that will help prevent disease progression due to b cells in cns meningeal lymphoid follcles
multiple sclerosis
116
what is Musk? what does it do?
- muscle specific tyrosine kinase
| - clusters ach at the post synaptic membrane
117
what are causes of secondary headache (mnemonic)
headcahes are a BIIH
- brain tumor
- infection
- intercranial pressure
- hemorrhage
118
what two meningitis can have normal glucose levels
lyme disease and viral meningitis
119
what is a zeitgeber?
name 5 examples
which zeitgeber has the most influence
- an environmental or social cue that can reset or entrain a circadian rhythm
- BLUE LIGHT (most influence), noise, social interactions, food, temperature
120
how does acute confusional state differ from dementia (4 things)
- acute: has altered state of consciousness, acute/subacute onset, reversible, autonomic hyperactivity
- dementia: no altered state of consciousness, chronic, irreversible, not autonomic hyperactivity
121
what drugs are hepatic enzyme inducers (4)
phenytoin, carbamazepine, topiramate, and barbituates
122
does remyelination occur in multiple sclerosis?
does axonal injury occur
yes but it's incomplete and not as effective
yes, can see on t1 mri (hypodense)
123
what is a great treatment for focal dystonia (and local muscle spasms)
what medical treatments can you try for generalized dystonia
BOTULINUM TOXIN
- trihexaphenydil, muscles relaxers (baclofen,flexiril), carbi/levodopa, clonazepam (benzos)
124
what is pseudo dementia
- severe depression that presents like dementia
125
how do symptoms vary for large fiber neuropathy and small fiber neuropathy
large: muscle weakness, gait instability, loss of sensation/numbness
small: burning, prickling, sharp pain, autonomic dysfunction
126
name at least 5 things that could be mistaken as a seizure
- panic attack
- GERD
- migraine
- psychogenic seizures
- TIA
- tic
- sleep disorder
- movement disorder
- breath holding spell
127
dementia is a disease of what part of the brain
where does it start and spread to over time
CORTEX
medial temporal lobe --> temporal and parietal lobe --> frontal lobe --> occipital lobe (final, v severe dementia)
128
what two pathways can glioblastoma form from
- glial precursor cells (de novo)
| - from astrocytomas (secondary pathway)
129
what is a "sensory trick" that can be associated with dystonia
you can touch a certain part of the body and that will momentarily stop the dystonia
130
what gene and chromosome is affected in von hippel lindau disease
inheritance?
what features would you expect
- vhl gene on chromosome 3
- autosomal dominant
- hemangioblastoma (vascular tumor with vaculoated stroma), pheochromocytoma, rcc
131
what is the definition of status epilipticus
what are the usual causes
what is the first line treatment
- failure of mechanisms that terminate seizures or abnormal processes that keep seizures prolonged to at least 5 minutes; by 30 minutes the yare at risk for serious neuron injury and death/mortality (for convulsive SE)
- epilepsy, fever (children), stroke
- IV lorazepam (benzo)
132
what is the relationship between mitochondria and ros production and aging
- mitochondria make a lot of ros during aerobic respiration, as you age, you accrue more and more mitochondrial mutations that cause dysfunction and makes more ros, the more ros there are, the more damage to mitochondria, which leads to more ros production
133
where are three common places for pressure injuries? why are they dangerous
- sacrum (most common), ileum, heels
| - can get infected and die, can take forever to heel
134
what type of seizure is a myoclonic seizure
what is it common in
what is it characterized by
what would an eeg show
- generalized motor
- epilepsy syndromes, progressive myoclonic epilepsy
- brief <1 second bilateral synchronous jerks, difficult to assess consciousness since brief
- 4-6 Hz polyspike wave
135
for carbon monoxide poisoning,
what symptoms can you get
what delayed sequelae can you get
what part of the brain can have discoloration
what is the treatment
- headache, dizziness, visual problems, nausea, vomitng, loss of conscious, coma, vegetative state
- seizures, cognitive deficit, tic, headache, blindness, delirium, dementia, parkinsonism
- globus pallidus (even putamen)
hyperbaric oxygen
136
what is delayed sleep phase disorder?
what can you do to treat it
- rhythm is phase delayed, aka you are falling asleep later
| - give melatonin before the dim light melatonin onset or give bright light in the morning after the cbt min
137
what condition is associated with intrathecal synthesis of igg or oligoclonal bands
multiple sclerosis
138
what is the cellular mechanism for the hyperexcitability in seizures
- too much excitability (ie too much inward Na or Ca) or too little inhibition (ie too little inward cl or too little outward K)
- sprouting of excitatory axons and loss of inhibitory interneurons
139
what is kennedy's disease
what is the inheritance, repeat and gene
when does it typically present
- what are the symptoms
- bulbar and spinal muscular atrophy
- x linked recessive, CAG on androgen receptor gene
- can present at any time, but typically middle adulthoo
```
- BULGER
BUlbar symptoms
Lower motor neuron symptoms
Gynecomastia
Erectile dysfunction
Reduced fertility
```
140
what is a focal aware seizure/simple partial seizure characterized by
- can vary significantly by the part of the brain is affected, BUT YOU ARE CONSCIOUS
- can have flushing on one side of the face (autonomic)
- can have contralateral limb tingling or tonic clonic seizure
- can have visual scotomas, blurred, flashing lights, auditory ringing or hissing
- turning of the head/grimace
141
what is a possible pathophysiology for tension type headaches
- increaseed sensitivity of nocicepters and neurons in trigeminal caudalis
142
an ischemic stroke to the lenticulostriate arteries affects what part of the brain?
what main artery do the lenticulostriate arteries come off of?
- basal ganglia
| - middle cerebral artery
143
most dementias are a _____ mater injury, but vascular dementia is a ____ mater injury
describe the progression of vascular dementia
grey
white
infarct/stroke aka decline --> level out --> another infarct with decline --> level out (constantly getting holes in the brain as aftermath of stroke)
144
what happens macroscopically months to years post stroke
what about microscopically?
- hole in the brain (macrophages eat the dead tissue but no new scar/fibrous tissue is laid down)- gliosis around hole, macrophages stay a while
- cystic cavity
145
what is Pick's disease
describe what you would see on gross and micro pathology
- disease of frontal lobe and temporal lobe degeneration with sparing of the occipital lobe --> personality, behavioral, and language problems
- gross: knife edge, severe atrophy of temporal and frontal lobe
- micro: pick bodies (accumulation of tau 3r), pick cells (swollen neurons that stain for ab crystallin), microvacuolization of the cortex/status spongiosus
146
for the sensory motor lacunar syndrome
what is the defect
where is the infarct
what arteries are involved
- unilateral weakness and sensory loss of face, arm and leg
- thalamus, adjacent internal capsule, pons
- perforating branches of PCA and small branches of MCA
147
what is a focal unaware/impaired seizure/complex partial seizure characterized by
- impaired consciousness and post drowsiness/slowness, < 2 minutes
- can vary, can start with aura, then get auditory hallucinations, deja vu or jame vu
then automatisms: chewing, licking lips, blinking, picking at clothes, walking
148
# define seizures
define epilepsy
what is the new definition of epilepsy
- the abnormal hyperexcitation and synchroniztion of a population of cortical neurons
- multiple unprovoked seizures without a systemic or specific insult
- 2 unprovoked seizures >24 hr apart
or 1 unprovoked seizure with a higher risk of getting a second seizure within 10 years, OR having an epilepsy syndrome
149
how do mt1 and mt2 receptors differ
binding of melatonin to mt1 results in the hypnotic effect of inhibiting suprachiasmatic nucleus firing --> sleep
binding of melatonin to mt2 regulates phase shifting effects of melatonin
150
describes the different types of astrocytomas and what they look like on imaging and microscopically
- diffuse astrocytoma (II)- doesn't enhance on imaging, has hypercellularity and nuclear atypia
- anaplastic astrocytoma (III)- may have speckled enhancement, has hypercellularity plus atypia PLUS mitoses
- Glioblastoma multiforme (IV)- ring enhancing on imaging, nuclear atypia, hypercellularity, mitoses PLUS endothelial proliferation AND/OR pseudopalisating necrosis
151
giving melatonin at night causes phase ____
giving it in the morning causes phase ___
- advance
| - delay
152
what condition are bunina bodies associated with
what makes up those bunina bodies
amyotrophic lateral sclerosis
tdp43
153
what is another name for a focal to bilateral tonic clonic seizure
what is it characterized by
- secondary generalized seizure
- start out with a focal seizure (simple or partial) with or without symptoms that then becomes generalized
- sometimes the focal seizure may not be noticeable or too fast
- variable symmetry and look to seizure
- will have post ictal confusion/ somnolence
1-3 min
154
what is the difference between muscle power and tone?
muscle power: ability to move muscle against resistance (think weakness)
tone: amount of resistance during passive movement (think of rigidity and flaccidity)
155
for primary cns lymphoma, what two populations are most commonly affected?
what virus and cell type is most common
- what would imaging and micro show
- immunocompromised (hiv, transplant, immunosupressed) and elderly
- ebv, large b cell
- imaging: diffuse enhanced multiple lesions
- micro- abnormal lymphoid cells perivascularly
156
where is the first place the lymphocytic infilatration occurs in multiple sclerosis and why
- peri vascular space in post capillary venule
- infiltrate is coming from periphery and is able to cross bbb due to inflammation making bbb leaky. and perivascular space is an anatomic space intentially for infiltrate
157
what does natalizumab do?
what condition is it good for
- prevents leukocytes from entering the cns via the blood by blocking a4B1 integrin from interacting with VCAM chemokine
multiple sclerosis
158
for upper trunk brachial plexopathy
what is another name for this/how does it occur
what part of the brachial plexus is involved?
what muscles are involved
what are the symptoms?
- erb palsy (lateral neck flexion injury)
- C5,C6
- proximal muscles, infraspinatus, deltoids, biceps , supinator
- waiter's tip (arm straight and internally rotated, hand flexed, loss of sensation across lateral arm (C5,c6 dermatome)
159
what 3 tests can you do to test for wilson's disease? What symptoms do you get
what part of the brain can you see gross changes and what changes can yo usee
- ceruloplasmin levels
- urine and liver copper levels
- genetic testing
- hepatic cirrhosis and failrue in childhood
- neuropsychiatric problems: parkinsonism, dementia, dysarthria, dystonia, dysphagia
- basal ganglia, can see atrophy and cavitation
160
how do viral meningitis and bacterial differ in glucose levels and wbc levels
- viral: increase mononuclear wbcs, normal or slightly decreased glucose
- bacterial: increased PMNs, severely decreased glucose
161
what nerve is affected in carpal tunnel syndrome
Name three symptoms of carpal tunnel syndrome
- median nerve
- sensory loss/damage of palmar 3.5 fingers and posterir 3.5 finger tips
- thenar atrophy
- claw hand with inability to flex index and third finger (median nerve innervates 1 and 2 lumbricals)
162
for intracranial hemorrhage, what is it at danger of expanding into
will anticoagulants help?
- a hematoma
| - NO, will make it worse, TAKE IV VITAMIN K
163
how does alpha synuclein accumulation differ in multiple system atrophy vs parkinsons and lewy body dementia
in MSA, alpha synnuclein is in glial cells
whereas parkinsons and lewy body dementia is in neurons
164
when staining, what do polymyositis, dermatomyositis, and inclusion body myositis have in common?
how do they differ?
- all will have rounded atrophic fibers and lymphotic infiltration/inflammation
- dermatomyositis will have perifascicular atrophy (around the muscle fiber bundle)
- inclusion body myositis will have purple cytoplasmic inclusions
165
how would staining for duchenne muscular dystrophy vs becker muscular dystrophy differ
- when staining for dystrophin, duchenne would not stain at all, becker would have some dystrophin stained, but still reduced
166
describe what happens in an uncal herniation; what arteries get occluded
- uncus dispalces the midbrain laterally, causing compression of the contralateral cerebral peduncle --> ipsilateral paralysis
- brainstem gets displaced caudally --> hemorrhaging
- ipsilateral occulomotor nucleus compressed --> fixed and dilated pupil looking down and out
posterior cerebral artery
167
if you eat pufferfish, what toxin are you at risk for? how does it work
what side effects would you see
- tetrodotoxin
blocks na channels
- initial tingling aroudn mouth, eventually tingling in legs and arms and paralysis, respiratory distress, cardiac arrhythmias and death
168
describe which artery supplies the following
midbrain
medial pons
lateral pons
medial medulla
lateral medulla
- posterior cerebral artery
- basilar artery
- superior cerebellar artery or aica
- vertebral artery
- vertebral artery/pica
169
how does carbidopa and levodopa work together?
what condition is this for?
what side effects are present
- levodopa is pro drug of dopamine that crosses the BBB and and gets converted into dopamine via acid decarboxylase
- carbidopa peripherally inhibits acid decarboxylase from converted dopa to dopamine
- parkinsons
- nausea, dyskinesias, hallucinations, orthostasis
170
describe what happens in a cingulate/subfalcine herniation; what arteries get occluded
- edema on one side pushes cingulate gyrus underneath the falx; can occlude pericollossal arteries, not well defined symptoms
171
what are the four P's of what you should observe for tone/muscle strength
- Position at rest
- Posture (activity)
- Palpation
- Passive stretch
172
what are causes of interparenchymal hemorrhage
- htn (most common), arteriovenous malformation, amyloid, mass
173
spasticity is _____ dependent
velocity
174
what is myoclonus
what are the causes
what is the first line medication
what are second line medications
- sudden rhythm jerking
- epipleptic, opioids, gapapentin, metabolic (liver, hyponatremia, hypoglycemia, hashimoto's encephalopathy, biotin deficiency, hyperthyroid, hyperglycemia
- clonazepam
- valproic acid and leviteracetam
175
where is the most common place in the cns to find lesions in multiple sclerosis and why
where are other places you can find lesions
peri ventricular because many post capillary venule confluences are in periventricular region (start there and then spread out)
- juxta cortical
- infratentoral
- spinal
- essentially anywehre in the cns
176
a lesion in the brain that has peripheral enhancememnts, esp crossing the corpus callosum, is most likely what
what two other things could it be
high grade glioma/brain tumor (GBM most likely)
CNS lymphoma or rlly bad MS
177
what causes cerebral palsy
- are the changes permanent? do they get worse over time
describe clinical manifestations
- damage to the motor area of the brain either due to malformation or injury before, during or after birth
- no and no, stay constant (static encephalopathy)
- coordination problems (ataxia)
- scissored gait, walks on toes, crouched gait (hypertonia)
- stiff muscles (spasticity)
- too floppy or too stiff
- one arm or leg can be weak
- tremor or involuntary movements
178
what condition is gower's sign associated with?
describe a positive gower's sign
duchenne muscular dystrophy
- cannot get up from sitting on the floor without using your hands to push you up
179
what vitamin deficiency can cause dementia?
what symptoms would you expect
what would you expect grossy/microscopically
- thiamine (b1) deficiency, wernicke korsakoff encephalopathy
- ataxia and acute gaze palsy --> confusion/delirium --> confabulation, amnesia and psychosis
- necrosis, demylination and hemorrhaging of mamillary bodies
180
what type of aphasia has inability to repeat, non fluent, but intact comprehension
- broca's aphasia
181
what is multiple sclerosis characterized by (2)
- inflammation and demyelination
182
For McArdle's Disease (Type V GSD):
what enzyme is affected
what is the clinical manifestation
- myophosphorylase
| - exercise intolerance, rhabdomyolysis
183
describe what happens in a cerebellar tonsillar herniation
what are presenting symptoms
- cerebellar tonsil displaced downward, displacing medulla, affecting respiratory and cardiac centers
- headache, neck stiffness, head tilt
184
what type of aphasia has inability to repeat, fluent, and impaired comprehension
conduction aphasia
185
what are two things on autopsy that you would find in someone with parkinsons
- intraneuronal alpha synuclein positive lewy bodies
| - loss of substantia nigra pars compacta (less melanin)
186
what group of viruses can cause meningitis and encephalitis
which are more common in meningitis
which are more common in encephalitis
herpes viruses (hsv2, ebv, vzv)
- hiv, mumps enterviruses (coxsackie)
arboviruses (western equine, st louis, california, zika)
187
what are the two places of whatershed in the brain? what happens if you get an infarct there
- MCA-ACA watershed
- MCA- PCA watershed (can lose higher cortical visual processing)
"man in a barrel syndrome"- proximal arms and proximal leg paralysis, look like a man in a barrel (BARRELS HOLD WATER)
- if in dominant hemisphere, can have transcortical aphasia
188
what broader disease group does werdnig hoffman belong to
describe pathologic findings
describe the demographic and prognosis
- spinal muscular atrophy
- skeletal muscle and anterior root atrophy
- loss of lower motor neurons in the spinal cord and brain stem, with the remaining motor neurons being ballooned
- atrophic muscle fibers with a few scattered normal or hypertrophic fibers
- comes spontaneously as a baby and can result in death in 2 years if no mechanical ventilations
189
describe the genes that can be implicated in alzheimers disease and what chromosome they are on
what do they put you at increased risk for
- Amyloid Precursor Protein (APP)- chromosome 21
- presenilin 1 - chromosome 14
- presinilin 2- chromosome 1
- Apo Lipoprotein E (e4 allele)- chromosome 19
associated with increase in AB plaque formation
190
what does flair MRI and T1 with gadolinium contrast show in multiple sclerosis
how can you tell the difference between an active and inactive lesion
the presence of inactive and active lesions is called what
- flair- demyelination
- t1 with gad: perivascular inflammation
- active: both gad and flair will show it
- inactive: flair will show, gad will not
- dissemination in space and time (characteristic of ms)
191
what protein is often implicated in parkinson's disease
- alpha synuclein
192
what happens macroscopically 1 week post stroke
what about microscopically?
- necrotic tissue
| - macrophages infiltrate, some petechial hemorrhage
193
how does pilocytic astrocytoma differ from the other astrocytomas (5)
- in children, usually infratentorial
- grade 1
- if removed, usually never comes back
- has cystic components with an enhancing mural nodule
- has rosenthal fibers microscopically
194
what staining finding would indicate a mitochondrial myopathy
- ragged red fibers
195
what are clinical manifestations of radial nerve neuropathy
- sensory deficit to the posterior 2.5 fingers (not the finger tips though)
- wrist and finger drop (loss of extension)
196
where does the superior division of the middle cerebral artery supply?
what symptoms would an infarct to the right or left side produce
- lateral cortex above the sylvian fissure
- left: (1) right arm and face motor weaknes +or - sensory loss, (2) nonfluent aphasia (broca's)
- right: (1) left arm and face motor weakness + or - sensory loss, (2) left hemi neglect
197
what is kearne sayres syndrome?
- mitochondrial myopathy characterized by external opthalmoplegia and pigmentary degeneration of the retina, also has cardiomyopathies
198
how does duration and post ictal state differ for generalized non motor seizure (absence) vs. focal unaware (partial complex)
absence: seconds, no post ictal confusion (right back to normal)
focal unaware: minutes, post ictal lethargy
199
what is one way to measure demyelination in multiple sclerosis
what findings would you expect
- visual evoked potentials, measure action potential speed in visual pathways
- delay in action potential response, means slowed conduction
200
describe the differences between type 1 and type 2 muscles fibers in regards to what type of movement they do, how they stain, and what metabolism they do
type 1: slow twitch, weight bearing, stain light (atpase 9.4), mitochondrial metabolism
type 2: fast twitch, fast movement, stain dark (atpase 9.4), glycogen
201
how does history differ for generalized non motor seizure (absence) vs. focal unaware (partial complex)
what about age
absence: no history; childhood, rarely in adulthood
partial complex: history of febrile seizures, encephalitis, trauma stroke; any age
202
what happens macroscopically 12 hours post stroke
what about microscopically?
- nothing
| - some neurons become pyknotic (aka dying off)
203
verrucay bodies are associated with what type of tumor?
schwannomas
204
for type 1 myotonic muscular dystrophy:
describe the inheritance and gene affected
describe the symptoms
- autosomal dominant ctg repeat on chromosome 19 (dmpk gene)
- distal muscle wasting, frontal balding, cataracts, first degree heart block, third degree heart block, sudden cardiac death, endocrine abnormalities, respiratory insufficiency (diaphragm ad intercostal muscle weakness), intellectual disability
205
what are the three types of brain cancers that you cannot observe?
high grade gliomas (3 and 4)
low grade gliomas (2)
cerebral lymphoma
206
where does the deep branches of the middle cerebral artery supply?
what symptoms would an infarct to the right or left side produce
- globus pallidus and putamen
- left: (1) right motor hemiparesis (2) larger infarct can have cortical deficits
- right (1) left motor hemiparesis, (2) larger infarct can have cortical deficits
207
for the ataxia hemiparesis lacunar syndrome
what is the defect
where is the infarct
what arteries are involved
- unilateral weakness of face, arms and legs and dysarthria PLUS ataxia
- basis pontis, posterior limb of the internal capsule, corona radiata
- anterior choroidal artery, perforating branches of the pca, small branches of the mca
208
what type of aphasia has impaired repetition, impaired fluency, but intact comprehension
transcortical motor aphasia
209
what is perampanel? what is it used for
- ampa receptor antagonist
seizures
210
the longer your _______, the worse your prognosis for traumatic brain injury
what are common symptoms of traumatic brain injury
what are common longterm effects of brain injury
post traumatic amnesia (anterograde)
- headache, dizziness, psychological (depression, anxiety irritability), dyssomnia
- SEIZURE
211
what is the diagnostic criteria for neuromyelitis optica
- need 2 of 3
- positive MNO IgG against aquaporin 4
- mri doesn't show signs of ms (medulla and hypothalamus involved, aka vomiting,nausea, yawning, hiccups, and autonomic dysfunction)
- spinal cord lesion contiguously spanning 3 vertebral segments
212
frontotemporal dementia with tdp 43 pathology is associated with which 3 gene mutations
what would you see grossly and micro
- valosin containing protein, tdp43, progranulin
- grossly, atrophy of frontal temporal lobe, basal ganglia and pallor of substantia nigra
- will see early on astrocytic gliosis and death of neurons and then eventually microvacuolization/status spongiosis
- will see td43 in the cytoplasm; ODD FINDING BECAUSE USUALLY IN NUCLEUS
213
what is locked in syndrome
what part of the brain is affected
- syndrome in which you are conscious and alert, but all you can do is blink and vertical gaze, everything else is lose (have no motor or sensory response), initially may need ventilation but can remove it later
- upper pons
214
what is the difference between coup and contracoup injury
- coup: injury is at site of impact; usually due to injury at rest
- contracoup: injury is contralateral to sight of injury; usually due to acceleration/decceleration injury
215
describe a cluster headache
are men or woman more likely to get it
what is a unique side effect that can occur
- a sudden headache that unilaterally lasts up to 40 minutes multiples times a day and up to 3 months at a time, can wake you out of sleep, genetic component
- men
- autonomic symptoms, tears from one eye, rinorrhea or congestion from one nare, ptosis, miosis,
216
what is central core myopathy
- associated with malignant hyperthermia assoicated with generalized anesthesia (RYR1) receptor
217
what are clinical signs of viral encephalitis
- increased intracranial pressure, altered consciousness, focal neurological deficits, seizures, bladder and bowel problems (spine), siadh (hypothalamus), accentuated reflexes, tremors, flaccid paralysis (spine), hypothermia, diabetes insipidus (hypothalamus)
218
for periodic paralysis:
what is it
what are the two types and their triggers
what is the inheritance
what genes are involved
what is the treatment
- channelopathy that results in periods of paralysis
- hypokalemic: RASH (rest after exercise, alcohol, stress, high carb meal)
- hyperkalemic: FRAC (fasting, rest after exercise, anesthesia, and cold)
- autosomal dominant
- hypokalemic: scn4a, CACNA1s
- hyperkalemic: scn4a
- hypokalemic: potassium supplements, k sparing diuretics, carbonic anhydrase inhibitors
- hyperkalemic: thiazide diuretics, carbonic anhydrae inhibitors, diet control
219
briefly describe the oculocephalic reflex
name the three parts
what is another reflex that utilizes the same pathway; how does it work
- when you turn your head in one direction, your eyes should move in the opposite direction
- vestibular nerve VIII (afferent) --> vestibular nucleus and medial longitudinal fasciculus --> nerves III, VI and IV
- caloric reflex (COWS)- put cold water in ear, causes decreases firing on that side and the perception that the head is moving away from the cold ear; eyes will drift towards the cold ear and then nystagmus to the opposite ear
if warm, perception will be that head is turning towards warm ear, eyes will drift to contralateral side and nystagmus towards the warm ear
220
what can you give for cluster headache treatment for acute pain?
what about preventative?
- high flow oxygen
- DHE im/iv/nasal spray (ergot)
- vagus stimulation
- samutriptan
- lithium, verapamil,
steroids
221
what cells in the brain are most susceptible to anoxic injury and what syndromes can happen as a result
what is another condition that can present very similarly
thalamus, cortex, purkinje cells in the cerebellum, hippocampus, layers 3 4 5 of the cortex ---> man in barrel syndrome, parkinsonism, myoclonus
- hypoglycemia (but can be reverse)
222
what type of seizure is absence epilepsy
what age group is this common in
what is it characterized by
what would you see on eeg
what would yo usee on mri
- generalized non-motor
- school age children (3-12)
- 3-20 seconds of abrupt arrest in activity with staring and blinking; no post ictal state; can happen many times a day; spontaneous remission
- generalized 3 Hz spike waves
- normal mri
223
what are clinical manifestations of ulnar nerve neuropathy
what can it cause it?
- sensory deficits for half of the fourth finger and all of the pinky on both sides
- interossei atrophy
- ulnar claw hand, 4th and 5th finger extension at MCP (due to loss of lumbricals) and flexion at the IP
- leaning on elbow af
224
phase shifting circadian rhythm forwards is called phase_____
phase shifting circadian rhythm backwards is call phase _____
delay
advance
225
how does type 2 myotonic muscular dystrophy differ from type 1
- less severe
- cctg repeat on znf9 on chromosome 3
- cataracts, myotonia and proximal muscle weakness only
226
mnemonic for warning signs of secondary headache
```
SSNOOP
Systemic symptoms (weight loss)
Secondary risk factors (HIV, Cancer)
Neurologic (altered conscious)
Onset (quick abrupt)
Oolder age
P previous headache history (is it newo r
Posture (better or worse laying down or sitting up)
```
227
what is parsonage turner syndrome?
what are usually symptoms
- idiopathic/autoimmune brachial plexus neuritis that can affect any part of the brachial plexus
- severe shoulder/arm pain that is followed by weakness
228
for the pure motor hemiparesis/dysarthria hemiparesis lacunar syndrome
what is the defect
where is the infarct
what arteries are involved
- unilateral face, arm and leg weakness with dysarthria
- basis pontis, posterior limb of the internal capsule, corona radiata
- anterior choroidal artery, perforating branches of the pca, small ranches of the mca
229
after one unprovoked seizure, what is the likelihood of getting a second? a third? how long after the second or third seizure is another seizure likely to happen
33%
73%
1 yr
230
during the first and second part of the day, describe circadian alertness and homeostatic drive for sleep
first: circadian increases and sleep is low
second: circadian and sleep are high
231
how does microglia contribute to the process of aging in the brain
- when younger, in two states: ramified (surveillance), and activated (released inflammatory cytokines like tnf a and il1 b); you cycle between these two states
- as you age, you become less able to completely go back to ramified state, so you are in an intermediate state where you have more mhc II and ilb released, like a chronic inflammatory state, and also it's easier to go into full activated state --> more inflammation and damage
232
what does melas stand for
mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes
233
what are the two most common metastases to the brain
name others
- lung then breast (although in women breast is more than lung)
- renal cell carcinoma, choriocarcinoma
234
what type of brain tumor usually has a necrotic core
gbm
235
what symptoms would an infarct to the right or left middle cerebral artery stem produce
- left: (1) right hemiplegia, (2) right hemianesthesia, (3) right homonymous heminopsia, (4) left gaze preference, (5) global aphasia
- right (1) left hemiplegia, (2) left hemianesthesia, (3) left homonymous hemianopsia, (4) profound left hemineglect (5) right gaze preference
236
what are the two types of taupathies
- progressive supranuclear palsy
| - corticobasal degeneration
237
what is apomorphine
what can it be used to treat
what are side effects
- subcutaneous d1/d2 receptor agonist
- parkinsons
- severe nausea, vomiting, hallucinations, dyskinesias (don't use odansetron 5h3 antagonists for anti emetric due to risk of hypotension)
238
for fascioscapulohumeral muscular dystrophy:
what is the inheritance
what is affected
what is the gene mutation
when is the onset
- autosomal dominant
- face, neck and shoulders, sometimes peroneal involvement
- DUX4 on chromosome 4q (you can't DUCK your HEAD)
- adolescence
239
what is the difference between parkinson's disease and parkinson's plus diseases
- the symptoms of the parkinson's plus diseases occur much earlier than they do in parkinsons (more rapid and debilitating)
- parkinson's plus can kill you but parkinson's disease can't
- parkinson's plus is not responsive to dopa treatments
240
how does history differ for those that suffer from primary generalized tonic clonic seizure to those who suffer focal to bilaterall tonic clonic seizure (secondary generalized)
- primary: no history of anything, normal
| - secondary: history of tumor, trauma, encephalitis or simple or complex partial seizures
241
for the clumsy hand/dysarthria lacunar syndrome
what is the defect
where is the infarct
what arteries are involved
- dysarthria plus clumsiness of hand
- basal ganglia, anterior limb of internal capsule, basis pontis
- anterior choroidal artery, perforating branches of the pca, small branches of the mca
242
exposure to blue light at night would cause phase_____
exposure to blue light in the morning would cause phase _____
how many hours within what zeitgeber should blue light be given to produce the greatest effect
- delay
- advance
- 3-4 hours before or after the core body temperature minimum
243
what are the three types of brain cancers you cannot do stereotaxic radiosurgery on
high grade gliomas (3 and 4)
low grade gliomas (2)
cerebral lymphoma
244
describe features that must be present in order to be considered brain dead
- no cardio or pulmonary function
- evidence of no brain activity whatsoever (including brainstem)
- no reflexes
- no response to stimuli
- eyes closed
- positive apnea test
245
for myotonia congenita:
what is the inheritances and the names of the diseases associated with them
what are the symptoms
what is the gene mutation
- autosomal dominant- thomsen disease
- autosomal recessive- becker disease
- myotonia without muscle weakness, stiff due to cold or inactivity, improves with exercise
- CCLN1
246
what are the causes of epilepsy in children, adults, and elderly
children: congenital, fever, or metabolic
adult: tumor or trauma
elderly: tumor, stroke, degenerative disease
247
what is seasonal affective disorder?
what is the pathology?
what is the treatment
- happens in late fall and winter when there is less bright light, have depression, increased eating and increased daytime sleeping (hypersomnia)
- physiologic, psychologic, and genetic factors, phase delay
- antidepressants, psychotherapy, light therapy in the morning
248
For Tauri Disease (Type VI GSD):
what enzyme is affected
what is the clinical manifestation
- phosphofructokinase
| - exercise intolerance, muscle cramping
249
what is wilson's disease characterized by; what is the inheritance
what two things can you use to treat and what are the side effects
- copper in the cns; autosomal recessive
- need to chelate copper to increase renal excretion
- penicillamine: nausea vomiting, bone marrow suppression, cross allergenicity with penicillin
- trienterine: rash, irondeficiency, muscle spasms
250
what do the spinothalamic tracts carry
where do they decussate
anterior: crude touch
lateral: temperature and pain
- anterior white commissure at the level of the spine
251
name common causes of bacterial meningitis
- strep pneumo
- neisseria meningitidis
- listeria (children, elderly, immunocompromised)
- ecoli, children
252
what is sundowning?
what is the pathology?
what is the treatment
- condition common in institutionalized dementia; causes late evening, afternoon, causes agitation, anxiety, hallucinations, pacing, confusion
- degeneration of the SCN
- blue light in the evening
- naps in the afternoon
- antipsychotics
- activities in the afternoon
253
what is clasp knife spasticity
- resistance to a movement until abrupt relaxation of the muscle that allows quick finish of the movement
254
for lower trunk brachial plexopathy
what is another name for this/what causes it
what part of the brachial plexus is involved?
what muscles are involved
what are the symptoms?
- klumpke palsy (upper arm extension injury)
- C8, T1 (radius and ulna)
- distal arm muscles (hand muscles)
- sensory deficit over medial arm (c8,t1 dermatome), inability to use hand muscles
255
what is the pathophysiology of myasthenia gravis (3)
1) IgG antibodies to ach receptor binding and blocking ach binding
2) internalization of ach receptor at nmj
3) complement mediated destruction of post synaptic membrane --> loss of post synaptic membrane involutions/surface area
256
name four cns infections that can have eosinophilia
- tb, neurosyphillis, coccidiodes, taenia solum
257
what is the only brain cancer type that surgical resection is not recommended
cerebral lymphoma
258
what two conditions have anterior root/horn atrophy
- amyotrophic lateral sclerosis
| - spinal muscular atrophy/werdnig hoffman
259
a consequence of brain edema is ______
herniation
260
what are the four genes involved in circadian rhythm and describe
- clock, bmal1, per, and cry
- during the day, clock and bmal1 proteins are made, form heterodimers and bind to promoter region for per and cry genes
- this causes creation of per and cry, which is phosphorylated by creatine kinase epsilon, which causes degradation
- by the evening, the amount of per and cry present overwhelms creatine kinase epsilon, and they form a heterodimer that enters the nucleus and inhibits bmal1 and clock (negative feedback inhibition)
261
Alzheimers is associated with a loss in ______ neurons
what medication can you give subsequently
cholinergic
- cholinesterase inhibitors and nmda antagonists (donepazil, mimatine)
262
what are the most common causes of viral meningitis?
- enteroviruses, mumps, hsv 2, hiv (HHEM)
263
describe the pathway from melanopsin retinal ganglion cells (MERGS) to the pineal gland in the absence and presence of light
in the presence of light: melanopsin retinal ganglion cells send glutamate to suprachiastmatic nucleus --> sends GABA to paraventricular nucleus --> PATHWAY INHIBITED, NO MELATONIN MADE
- in absence of light: MERG's don't send glutamate to scn --> disinhibition of paraventricular nucleus --> send glutamate to intermediolateral cell column --> send ach to superior cervical ganglion --> send nor epi to pineal gland --> makes melatonin
264
what tends to be cystic: medulloblastoma or piilocytic astrocytoma
pilocytic astrocytoma
265
what type of cancer is rosenthal fibers common in
pilocytic astrocytoma
266
what type of seizure is an atonic seizure
what is it characterized by
what would an eeg show
- generalized motor
- brief (seconds) lose of tone
- mild: head nod or jaw drop
- severe: fall
- consciousness is usually impaired
- sudden attentuation or generalized polyspike wave
267
what is dantrolene
what can it be used for
what are side effects
- skeletal muscle specific ryonidine receptor antagonist, messes with excitation contraction coupling
- muscle spasticity or cerebral origina, multiple sclerosis, spinal cord injury
- muscle weakness, sedation, hepatitis, hepatoxicity
268
what is amyotrophic lateral sclerosis characterized by
what sensory problems are present
what genetic mutations are associated
what two treatments are available
- loss of motor neurons in the brain stem and spinal cord
- none!
- Sod1, tdp43, c9orf72
- rilutek, blocks glutamateric activation
- radicava, free radical scavenger
269
in multiple sclerosis what inflammatory cells would you expect perivascularly, in the parenchyma, and in the meninges
- peri vascular: t cells, b cells, macrophages
- parenchyma: CD8 >CD4
- meninges: b cells. dendritic cells, plasma cells (meningeal lymphoid follicle)
270
what kind of polyneuropathy is guillain barre? what is another name for it?
what causes it
what are symptoms
what lab finding can yo uhave
what can you do to treat it
- demyelinating, acute inflammatory demylinating polyneuropathy
- campylobacter or cmv, subacute, peaks 4 weeks after gi or upper respiratory illness
- ascending sensory symptoms, proximal and distal muscle weakness, motor weakness, reduced relfexes
- cyto albumin dissociation (high protein count) in the CSF
PLEX or IVIG
271
how do trihexaphenidyl and benztropine work
for what condition?
what are side effects
- anti cholinergics
- parkinsons
- dry mouth, constipation, urinary retention, confusion
272
for limb girdle muscular dystrophies:
what is the inheritance
what is affected
when is the onset
- autosomal recessive >autosomal dominant
- shoulder and pelvic girdle (shoulder, upper arms, hips and thigh weakness)
late childhoold- early adulthood
273
for meningioma, what population is common; what is another population that can be involved
- what microscopic findings are possible
what can you see on imaging
- older females
- young children if NF2 (22q)
- whorling patterns and pseudorosettes
- dural tail (let's you know it's in the meninges), hyperostosis (bone growing over it)
274
what are the possible responses for motor response for glasgow coma scale
```
6- follows command
5- localizes
4- withdraws from pain
3- decorticate
2- decerebrate
1- unresponsive
```
275
name treatments for myasthenia gravis
- pyridostigmine --> acetylcholinesterse inhibitor (SE: N/V/D, bronchial secretions, bradycardia)
- targeted therapy (decrease antibody production)
- prednisone (diabetes, weight gain, swelling)
- mycophenylate (myelosuppression)
- azathioprine (nephrotox, GI, skin rash, leukopenia, malignancy)
- cyclosporine (htn, nephrotox)
- IVIG or PLEX (to remove antibodies)
- thymectomy
- eculizumab ( prevents mac attack complex)
276
when is dim light melatonin onset
when is melatonin at it's highest, when is it at its lowest? what does it directly contrast to
- 2-3 hours before bedtime
| - at night (at cbt min), during the day, exact opposite of core body temperature
277
what are the possible responses for eye movement for glasgow coma scale
4- open spontaneously
3- open upon verbal command
2- open upon stimuli
1- closed
278
what is a disease that has the same parts of the spinal cord affected as friedrich's ataxia
what is the cause
- subacute combined degneration or lichtheim's disease (posterior and lateral columns affected)
- b12, vitamin e or copper deficiency (can be irreversible )
279
what kind of polyneuropathy is diabetic neuropathy? what are clinical manifestations
axonal
- loss of sensation/numbness to feet, gait instability, positive romberg, wide based gait, loss of ankle reflexes
280
what is the difference between lead pipe and cogwheel rigidity
in what disease is cogwheel rigidity common
in what condition can you get leadpipe rigidity
- lead pipe is constant resistant along full range of motion
- cog wheel is jerking resistance along full range of motion
parkinsons
malignant hyperthermia
281
what type of brain hemorrhage has contributions from htn but is not the cause
what two types of brain damage ARE due to hypertension
- subarrachnoid
| - hypertensive hemorrhage and lacunar infarct (--> arteriolosclerosis)
282
name potential inheritances for parkinsons and what genes/age groups are associated
- autosomal dominant (LRRK2 50-60, SNCA (35-45)
| - autosomal recessive (PARKIN <35, PINK1, DJ1 <35)
283
what are branches in the brain off of the internal carotid artery and what do they supply
- anterior cerebral artery (frontal lobe and caudate and internal capsule anterior limb)
- posterior cerebral artery (occipital lobe and thalamus and inferior temporal lobe)
- posterior communicating artery
- opthalmic artery
- anterior choroidal artery (internal capsule posterior limb)
284
name clinical presentations of multiple sclerosis
- optic neuritis (pain and blurred vision
- cerebellitis- ataxia
- myelitis- weakness/numbness and urinary continence
- hemispheric syndrome- contralateral weakness, paralysis, visual field problems,
- brainstem problems- dipllopia, dysphagia, vertigo, incoordination, numbness
285
briefly describe the gag reflex
what are the three parts
- stick something in the back of the mouth near the uvula should cause gagging
- glossopharyngeal IX (afferent) --> medulla --> glossopharyngeal IX and vagus (X) (efferent)
286
what type of drug is tizanidine?
what can it be used for
what is the side effects
what should you keep in mind about it's metabolism?
- alpha 2 agonist
- spasticity, don't know why
- HADD: hypotension, asthenia (muscle weakness), drowsiness, dry mouth
- metabolized by CYP1a2, drug interactions with fluoroquinolone, also antibiotics inhibit cyp1a2 so they would increase levels of tizanidine
287
what are causes of chronic meningitis (mnemonic)
Chronic Live music SSTOPed CDs
- Lyme
- Syphillis
- sinusitis
- TB
- Otitis
- CSF leak
- Dental infection
288
what part of the brachial plexus does the long thoracic nerve come off of
C5,c6,c7 root
289
what is multiple system atrophy characterized by?
what age group is this common in?
what pathology is present (2)
what can you do to treat
- parkinsonism and/or ataxia AND severe early dysautonomia (erectile dysfunction, orthostatic hypotension, urinary problems, gastric paresis)
- age 60
- glial cell inclusions, hot cross bun sign (in pons) on mri
- anything that treats the autonomic problems (prognosis is 7-9 years)
290
what anti epileptic drugs are renally excreted (3)
gabapentin and levetiracetam (and topiramate)
291
for neurosyphillis/syphillis meningitis
what is the cause
when does it occur
what are symptoms
what labs are present
what is the treatment
- treponema pallidum
- can happen 2 years after infection
- can be asymptomatic, tabes dorsalis, cranial nerve palsies (esp VII), cognitive slowing, papilledema, argyl robertson pupils
- protein slightly high, glucose slightly low, fluorescent treponema antibody diagnostic
- 2 weeks of penicillin g or ceftriaxone
292
what are contraindications for lumbar puncture
```
PATCH
(increased intracranial) pressure due to mass lesion or edema
Abcess (spinal)
Thrombocytopenia
Coagulopathy
Hydrocephalus (obstructive)
```
293
for parkinsons disease, you want to ______ acetylcholine and ______ dopamine
whereas for huntington's disease, you want to _____ acetylcholine and _____ dopamine
- decrease, increase
| - increase, decrase
294
define concussion
a mild traumatic brain injury with loss of consciousness up to 30 min and loss of memory (anterograde amnesia most common)
295
what are common presenting symptosm for a brain tumor and why
- headaches (pressure against dura innervated by trigeminal, or do to hydrocephalus
- seizures (if mass is in cortex/have edema)
- vomiting (due to hydrocephalus)
- hemiparesis
- hemianopsia
blurred vision
296
what is progressive supranuclear palsy (PSP) characterized by
what age group is it common in?
what pathology would you expect
- parkinsonism + gait instability/falls + vertical gaze limitations + frontal cognitive signs
- >40, mean is 65
- humming bird sign on imaging, atrophy of the substantia nigra and midbrain, tau protiein in glia, tufted astrocytes, neurofibrillary tangles in diencephalon, brainstem and basal ganglia, neuronal loss
297
describe lewy body dementia
what pathology is it characterized by?
what can you use to treat (3)
- parkinsons + early dementia + hallucinations
- lewy body lmao
- dementia symptoms happen before parkinson's motor symptoms or within a year (parkinsons dementia happens much later on)
- clozapine, quetiapine, primavanserin (antipsychotics that won't affect dopamine levels)
298
what lymphocytes would you see perivascularly for neuromyelitis optica
- t cells b cells macrophages and eosinophils
299
what is fingolimod
what can it be used to treat
- sphingosine-1 phosphate receptor modulator, s1p is necessary chemotactic gradient necessary to allow for leukocytes to leave peripheral lymph nodes and migrate
- multiple sclerosis (won't migrate to cns)
300
what is another name for brown sequard syndrome
what is it
- what causes it
- what are the clinical manifestations
- hemiparaplegic syndrome
- damage to lateral 1/2 of spine
- penetrating trauma, neoplasm, ischemia, inflammation
- loss of ipsilateral motor function and fine touch/proprioception below level of spine
- loss of contralateral pain and touch below level of spine
301
what 3 conditions can deep brain stimulation be used for
parkinson's disease
essential tremor
dystonia
302
angulated atrophic fibers indicate a _______ process
round atrophic fibers indicate a _______ myopathic process
neurogenic
myopathic
303
why would you do lumbar puncture multiple sclerosis
what is the name of the finding
- want to test csf for intrathecal synthesis of igg (tells you b cells have taken residence in brain and are launching an immune response)
- oligoclonal bands
304
how does light exposure affect per levels
- it increases per levels
- light causes MERGs to send glutamate to scn, and will bind to NMDA receptors, Ca will flow in and with cAMP will activate protein kinase, which activates CREB, which binds upstream to clock and bmal1 and increase per production
305
what are features of ataxia
what part of the brain is messed up? due to what causes
- nystagmus, dysarthria (speech), unstable wide gait, limb coordination problems
- cerebellum
- MIINT
- multiple system atrophy, infectious, inflammatory, neoplastic/paraneoplastic, toxic/metabolic
306
what is the difference between persistent vegetative state and minimally conscious state
what part of the brain is involved in persistent vegetative state
- pvs: no response to stimuli at all, but has intact sleep-wake cycles, so can open eyes after sleep, maintain breathing and respiration
- minimally conscious state: one step above pvs, able to intermittently follow commands (like open eyes), rest of the time unresponsive
- everything except the brainstem
307
expression of what mutant enzyme confers a better prognosis in gliomas
IDH 1
308
for subarachnoid hemorrhage:
what are 2 common causes
where does the blood go
- aneurysm rupture (anterior communicating or posterior communicating most common) or trauma
- follows the contours of the brain and into ventricle (subarachnoid space is continuous with ventricles)
309
what happens macroscopically 48-72 hours post stroke
what about microscopically?
- dark discoloration
| - neutrophils infiltrate
310
what are the two cancer types that can't have needle biopsy
- mets
| - pituitary adenoma
311
for Musk Ab+ myasthenia gravis:
what demographic is effected
is the thymus affected
is complement involved
what medication respond well and which don't
women aged 36-38
no
no
good- PLEX
bad- IVIG
312
what are increased risk factors for subarachnoid hemorrhage
- female
- older age
- smoking
- hypertension
- heavy alcohol use
313
For Friedrich's ataxia
what is the inheritance, repeat, gene, and chromosome
- what is affected
- what two other conditions are implicated
- autosomal recessive, GAA frataxin gene on chromosome 9
- psoterior column, lateral corticospinal tract and spinocerebellar tract --> coordination problems and muscle weakness
- diabetes and cardiomyopathy
314
name the four diagnostic tests for myasthenia gravis and how they work
1) ice pack test: use if you have ptosis, ice decreases acetylcholinesterase activity transiently and should improve symptoms
2) pharmacological testing: can give tensilon (edrophonium chloride) which is a short acting acetylcholinesterase inhibitor, should transiently improve symptoms
3) serologic testings for antibodies for acetylcholine receptor (most common) or muscle specific tyrosine kinase
4) nerve conduction testing- decrement in response, proximal nerves more sensitive than distal nerves; can also test two muscle fibers to see how closely connected they fire, in mg will be very far apart (called INCREASED JITTER)
315
what four tyeps of brain tumors can you not use chemo for?
pilocytic astrocytoma (grade 1)
meningioma
pituitary adenoma
mets
316
what neuromuscular effect does organophosphate poisoning do?
- inhibits acetylcholinesterase
317
which has a faster course generally: axonal or demyelinating polyneuropathy
demyelinating (ex: guillain bare)
318
what is nemaline myopathy
what other condition can also have this
a congenital myopathy associated with rod shaped bodies in muscles fibers
- can be seen in aids relatd myopathy as well
319
what is tau
what chromosome and gene is it on
which two isoforms are most impliciated
- microtubule associated protein that stabilizes microtubules; many in axon
- 17 MAPT
- 3R and 4R
320
what lobes of the brain are likely to be impacted due to impact brain injuries?
frontal loe, occipital love, temporal lobe tips
321
Electroencephalograms have good _____ resolution but poor _____ resolution
what do eeg's measure
what signs on an eeg would signify a seizure (3)
temporal
spatial
the extracellular dipole formed from similarly oriented post-synaptic excitatory pyramidal neurons in layer 5
- spike, sharp wave, or spike wave complex
322
what condition is hydrocephalus ex vacuo common?
alzheimers
323
hyperdense MCA and insular ribbon sign are indicative of what
an infarct
324
during the first and second part of the night, describe circadian alertness and homeostatic drive for sleep
- first part: circadian is low and homeostatic drive for sleep is high
- second part: circadian is low and homeostatic drive for sleep is lower
325
for the pure sensory lacunar syndrome
what is the defect
where is the infarct
what arteries are involved
- unilateral sensory loss of face, arm and leg
- VPL of thalamus
- perforating branhces of PCA
326
what gene and chromosome is affected in neurofibromatosis type 1
inheritance?
what features would you expect
- neurofibromin gene 17 (17 letters in neurofibromatosis
autosomal dominant
- plexiform neurofibroma (looks like bag of worms, shredded carrots in myxoid background), skin lesions, cafe au lait spot)
327
what symptoms would an infarct to the right or left posterior cerebral artery stem produce
- left (1) right homonymous hemianopsia, (2) alexia without agraphia, (3) if larger infarct includes thalamus and internal capsule --> aphasia, right hemiplegia and hemi sensory loss
- right: (1) left homonymous hemianopsia, (2) if larger infarct includes thalamus and internal capsule --> left hemiplegia and hemisensory loss
328
does MGMT gene methylation confer a better or worse prognosis for glioma? why
better prognosis; mgmt normally can repair damage caused by chemo --> chemo resistant tumor
when methylated, mgmt is defective and therefore can't repair --> chemo sensitive tumor
329
What are pramipexole and ropinorole?
what are they used to treat
what are the side effects
- dopamine receptor agonists
- parkinsons (somewhat effective monotherapy)
- compulsive behavior, hypersexuality, sleep attacks (knock out)
330
briefly describe the corneal reflex
name the three parts
- upon touch of cornea, eyes should reflexively close
| - V1 trigeminal (afferent) --> pons --> VII facial nerve(efferent)
331
for inclusion body myositis
who is affected
what symptoms
how does it differ from dermatomyositis and polymyositis?
what would you see on biopsy
- men aged >50 >women
- bulbar symptoms, asymmetric finger flexor ankle dorsiflexor and quadriceps atrophy
- no myocarditis, ild, or malignancy associated
- rimmed vacuoles
332
in normal pressure hydrocephalus, what triad of symptoms is common (mnemonic)
- dementia, incontinence, gait problems
wet , whacky and wobbly
333
what is paratonia?
what is usually the cause
- inability to relax a muscle
| - frontal lobe lesions/frontal lobe dementia
334
what is central cord syndrome?
- damage to central gray matter
- usually due to cervical/neck injuries
- upper limbs more motor deficits than lower because upper limb nerves more centrally located
- variable sensory loss
335
what can cause a provoked seizure
would you need to treat this long term
alcohol withdrawal
fever
nah
336
what are causes of acute confusional states
what about infectious dementia
- bacterial meningitis, subarachnoid hemorrhage, traumatic intercranial hemorrhage, hypoglycemia
- hiv, meningitis, creutzfeld jakob, syphillis
337
what is corticobasil degeneration
what is the main age affected
what is the pathology
- parkinsonism + profound asymmetry + (limb apraxia, alien limb, resting and action tremor, myoclonus, frontal cortex deficits, language problems)
- 65
- taupathy, astrocytic plaques and dense glial threads
338
Which benzodiazepine can be used to treat spasticity and muscle spasms?
Why can it treat both
name some conditions it can treat
what side effect can it produce
- diazepam
- acts centrally AND in the spinal cord
- muscle spasms due to trauma or tetanus, multiple sclerosis, cerebral palsy spasm, spinal cord spasm
- if used synergistically with other cns depressants --> respiratory depression
339
what is the criteria for migraine headaches
is it more common in women or men
what is a classic symptom that may or may not occur
what are 2 good acute treatments
what are good preventative treatments
- at least 2 of the following: unilateral, pulsing, moderate to severe pain, aggravated by normal activity
- at least one of the following: nausea/vomiting, photophobia/phonophobia
women
aura
- sumatriptan (5ht agonist), CGRP inhibitors (fremanezumab, erenumab)
- anti convulsants, beta blockers, calcium channel blockers, anti depressant
340
parkinson's disease is due to a loss of _____ from the ______
dopaminergic neurons
substantia nigra pars compacta
341
what demographic is affected in myasthenia gravis (mnemonic)
what organ is commonly implicated in myasthenia gravis, and what finding would you have?
Young Women and Old Men Make Great couples
- young women 20-24
- old men 70-74
Make Great- myasthenia gravis
- thymus, thymus hyperplasia or thymus tumor
342
what is a tic
how does it differ from tourettes
what meds can you give to treat it
- a repetitive stereotyped movement or sound that abruptly interrupts normal activity; there is an urge to do the tic that builds until the act is done
- two or more tics plus a vocal tic that happens before the age of 18 and is present for a year
- SAAV
- SSRIs
- Alpha 2 agonist (clonidine)
- anti pschotics (risperidone)
- VMAT2 inhibitor (tetrabenzine)
343
epidural hematoma is usually due to what cause?
what blood vessel is almost always the cause
is it rapidly occuring or slowly growing?
what is the shape?
- trauma/skull fracture
- middle meningeal artery
- rapidly
- lens
344
what is a major microscopic complication of traumatic brain injury?
where is it most likely to happen?
diffuse axonal injury (spheroids)
mid line structures (esp corpus callosum, and also brainstem and white gray matter junction)
345
what is sclerosis en plaque
what condition is it comon in
- gross dark focal discoloration that is firm (sclerosis)
| - multiple sclerosis
346
in general, what are spinal muscular atrophies characterized by
what is the prototype and the prototype of the prototype
- degeneration of lower motor neurons
| - spinal muscular atrophy and werdnig hoffman syndrome (SMN1)
347
what type of seizure is a tonic seizure
what is it characterized by
what would an eeg show
- generalized motor
- 2-20 second symmetrically tonic contraction, with characteristic flexion of hips and neck (usually fall to floor)
- sudden attentuation with low voltage fast activity (most common) or generalized polyspike waves
348
what is myotonia
what is usually the cuase
delay in relaxing muscle after its been contracted
- chloride channel problem
349
melatonin has its greatest phase shifting affect ___ hours before the ____ or ___ hours after the ____
4 hours before dim light melatonin onset
3 hours after core body temp min
