Clinical Neuro - Beary Flashcards
(177 cards)
1
Q
In a case that appears to be a myopathy, what initial lab value will I use in my work up?
A
CPK
2
Q
CPK will be markedly increased in…
A
Muscle dystrophies Inflammatory myopathies Hyperthyroid myopathy Rhabdomyolysis
3
Q
CPK will be normal or decreased in…
A
Dermatomyositis Hypothyroidism Steroid Induced Myopathy
4
Q
Steroid induced myopathy will show what type of muscle fiber atrophy?
A
type II
5
Q
A big risk factor for statin myopathy would be…
A
combo statins with fibrates or CCBs
6
Q
Taking antipsychotics could cause what form of myopathy?
A
neuroleptic malignant syndrome - rhabdomyolysis
7
Q
What mutation causes increased Ca release from the SR and can cause malignant hyperthermia?
A
Autosomal dominant ryanodine receptor mutation
8
Q
How do you tx malignant hyperthermia?
A
dantroline
9
Q
Critical illness myopathy might appear in ICU pt exposed to prolonged disuse and neuromuscular blocking agents. A biopsy would show…
A
type I fiber atrophy
10
Q
With dermatomyositis I am going to first check for …. and I am also going to look for… if there are anti-Jo-1 abs present,
A
malignancies interstitial lung disease
11
Q
A biopsy showing perifascicular atrophy and vasculatic changes will make consider the diagnosis of…
A
dermatomyositis
12
Q
A muscle biopsy showing CD8+ mononuclear inflammation/MHC-1 antigen + between muscle fibers will make me consider dx of …
A
polymyositis
13
Q
A chronic, progressive, asymmetric weakness of finger/wrist extensors, knee extensors, and ankle dorsiflexors along with a biopsy showing ringed vacuoles will make me want to dx…
A
inclusion body myositis
14
Q
The MC muscle dystrophy is… with what pattern of inheritence?
A
Duchenne (DMD) X linked recessive (Xp21)- dystrophin error
15
Q
If a pt who was initially thought to have DMD is still ambulatory by 16 yo, he will be dx’d with …
A
Becker MD
16
Q
What drug should be offered to all pts dx’d with DMD?
A
corticosteroids
17
Q
What is a main sign of Becker MD?
A
calf hypertrophy
18
Q
If a pt has adult onset idiopathic dilated cardiomyopathy, what else needs to be in my ddx?
A
Becker MD
19
Q
Chromosome 4 D4Z4 tandem repeat truncated to 10 or less causing DUX4 gene transcript is found in what disease?
A
Fasicoscapulohumeral MD
20
Q
In fascioscapulohumeral MD, what will be one of the first ssx? What muscle does this MD spare? What is this called?
A
eye or lip weakness spares deltoids - ‘pop eye arms’
21
Q
What is the MC muscular dystrophy in adults?
A
myotonic dystrophy
22
Q
What is associated with Type 1, autosomal dominant myotonic dystrophy?
A
Chrom 19, CTG repeat (anticipation) distal weakness - facial, pharyngeal, finger flexor, foot flexor Hatchet face, cataracts, myotonia death by other organ involvement in their 50s
23
Q
What is associated with Type 2, autosomal dominant myotonic dystrophy?
A
Chrom 3, CCTG repeat (no anticipation) proximal weakness in leg an hand live a normal life span
24
Q
What does anticipation (increased length of trinucleotide repeat sequence) mean for clinical appearance?
A
earlier onset with each generation
25
Pompe disease (GSD Type II) is considered static, and is a mutation in... What are ssx in infants? What are ssx in adults?
acid maltase on chromosome 17 infants - hypotonia, enlarged tongue, cardiomegaly, hepatomegaly (death 1-2 yo) adults - pelvic weakness\> shoulder weakness, abdominal and diaphragm weakness
26
Which glycogen storage disease will have a normal forearm exercise test?
Pompe disease
27
What is a normal forearm exercise test?
\>2 fold increase in lactate with normal or increased ammonia
28
McArdle Disease (GSD Type V) is considered dynamic and is a deficiency in... what does this mean for the pt?
myophosphorylase on chromosome 11 they cannot break down glycogen in muscles - with high intensity exercise there will be cramps, myalgias with second wind phenomenon, myoglobinuria and muscle contracture, CK elevated to 1k for about 1-2 months
29
Which GSD has an abnormal forearm exercise test?
McArdle Disease (GSD Type V)
30
In carnitine palmitoyltransferase II deficiency, the defect causes ...
impaired beta oxidation
31
What is the MCC of recurrent myoglobinuria in adults?
CPT II deficiency
32
What are features of CPT II deficiency that directly contrast McArdle GSD?
no second wind phenomenon normal CK between bouts of exercise
33
What should CPT II deficient people avoid?
valproic acid
34
If a pt presents as a child with multiple organs apparently involved in an issue, I should be thinking about...
mitochondrial myopathies
35
Hypokalemic periodic paralysis uses what ion channels?
Cav or ligand gated Na channels
36
What are triggers for hyopkalemic periodic paralysis?
cold, rest after exercise, high carb intake, etoh, stress
37
What are triggers for hyperkalemic peirodic paralysis?
cold, fasting, rest after exercise, K+ rich foods
38
What channel is used in hyperkalemic periodic paralysis?
Nav SCN4A
39
Should someone with hyperkalemic periodic paralysis eat carbs?
YES
40
In which periodic paralysis syndrome does paralysis last longer?
hypokalemic
41
Which periodic paralysis syndrome has an EMG that shows increased CMAP amplitude with electrical myotonia?
hyperkalemic
42
AD Myotonia Congenita, aka Thomsen's Dz is mostly in male infants and is a mutation in... causing...
calcium channels painless muscle stiffness without periodic paralysis inability to re-open eyes after face is washed
43
Myotonia is worse in what climate?
cold
44
It is not uncommon to find what kind of disorders associated with periodic paralysis?
thyroid
45
In a normal pt, with repeated nerve stimulation, n (quanta (ACh) ready for release), m (number of quanta released), and EPP all go (up/down). What is still generated and why?
down MFAP - muscle fiber action potential
still generated because EPP is always above threshold
46
Describe Myasthenia Gravis features and ssx
Post-synaptic autoimmune disease abs to ACh receptors,
causes inhibition of excitatory effects of nACh receptors
ocular type involves muscles only, rare pupils involved
fatiguable weakness, good in the morning and worse as the day goes on
47
What is the MC presenting symptom of Myasthenia Gravis? What tumor is a common association?
diplopia and ptosis thymoma
48
What are some tests you could do to dx MG? Which is the most sensitive test?
Tensilon test with Edrophonium - prolongs ACh life in cleft and relieves ssx briefly
ice pack test - 2 min of ice to eyes causes at least a 2 mm improvement
Single fiber EMG is most sensitive test -
49
What happens with repetitive nerve stimulation to someone with MG?
decremental response will be present
m (# quanta released) will be normal
EPP is initially low and decreases because less ACh R available
EPP decreases threshold, MFAP is not generated, CMAP is decreased
50
All pts dx'd with MG and \< 60 yo should get a ... What is another reason not to get one?
thymectomy anti-MuSK abs present
51
How do you tx MG with rx?
steroids/prednisone to decrease ocular version conversion to general
thymectomy
AChE inhibitor/pyridostigmine for ssx releif (not modifying)
52
What medications might trigger a Myasthenia Crisis?
aminoglycosides, beta blockers, CCB, Mg Sulfate
53
How do you tx Myasthenia Crisis?
IVIG or plasma exchange
avoid NM blockers, use etomidate
54
Compare and Contrast Myasthenia Crisis with Cholinergic Crisis
* Myasthenia Crisis
* under rx'd
* resp depression
* can't count to 10
* pupils nl or mydriasis
* improvement w/ Tensilon test
* tx IVIG or plasma exchange
* Cholinergic Crisis
* over rx'd
* N/V/D
* generalized muscle weakness
* pupils have miosis
* Tensilon test worsens paralysis
* tx with Atropine
55
What malignancy is associated with Lambert Eaton Syndrome?
small cell lung cancer
56
What is the pathophys behind Lambert Eaton Disease?
abs to P/Q type voltage gated Ca channels on
1. presynaptic motor nerve terminals
2. autonomic nerve terminals
3. cerebellar purkinje cells
57
What are the ssx of Lambert Eaton and how would you diagnose it?
* proximal weakness that improves throughout the day (increased d/t increased Ca entering nerve terminals)
* dx probs with repeated nerve stimulation
* initial EPP generally is normal for m (#quanta released), but initial n (quanta) is decreased bc presynaptic Ca concentration is low
* initial EPP is below threshold
* no MFAP generated
58
In lambert eaton, what is the 'ramping up effect' a/w 50 hz Repeated Stimulation?
incremental increased response in CMAP because rate stimulation is greater than that of calcium clearing from the presynaptic nerve terminus
59
How do you tx Lambert Eaton?
3,4 DAP
inhibits presynaptic Kv channel, increasing Cav channel opening, increasing ACh release
removing cancer in lung also improves ssx
60
Compare and Contrast Myasthenia Gravis with Lamber Eaton
* MG
* normal reflexes
* no ANS involved
* worse with use
* abs to AChR
* responds to edrophonium
* 10% have thymoma
* LE
* decreased or no reflexes
* ANS ssx
* repair phenomenon, throughout day
* abs to P/Q Cav channel
* no response to edrophonium
* a/w small cell lung cancer
61
Within 3 days of a MG mom giving birth, the child is having transient feeding difficulties, weak cry, breathing difficulties, and floppiness. What are we suspecting and what can we do about it?
Transient Neonatal MG
transplacental passage of AChR abs
Tx - vent support, neostigmine, recovery is usually within 3 weeks
62
What ssx of botulism is found in all forms of botulism?
dilated, poorly reactive pupils
63
What are the 5 cognitive domains within mental status?
1. social function/behavior
2. executive function
3. memory
4. language
5. visual/perceptual/spatial - attention
64
Where do executive function and social cognition and behavior reside in the brain?
frontal, temporal, subcortical
65
The center for language is usually found in what area of the brain, description wise?
dominant side (L usually) frontotemporoparietal area
66
Visuospatial function comes from what area of the brain?
non-dominant parietal
67
The L hemisphere usually dominates praxis which is... What else does the L brain do?
mechanical language expression/reception as well as complex motor programming (praxis) and sequential analysis
68
R side of the brain is usually thought of as artistic, what does it do?
dominant for visual-spatial orientation, appreciation for art/musical color, and expression/reception of language tone (prosody)
69
If I have dyspohonia or dysarthria, what area of the CNS would have damage, most likely?
brainstem
70
If I am unable to express or comprehend the emotional part of speech (melody, emphasis, inflection, gestures) I may have a lesion where?
non-dominant hemisphere
71
What are some possible deficits that I could find if there was damage to the R side of the brain?
extinction, inattention, spatial neglect, emotional indifference, euphoria
72
The frontal lobe has a few different functions depending on area..
What is each responsible for and what would be deficits if there was a lesion in that area?
dorsolateral
medial frontal
orbital frontal
* dorsolateral
* judgement, abstract, problem solving
* deficits of impaired planning, perseveration, retrival
* medial frontal
* motivation, initiation
* deficit with mutism, emotional/cognitive motor apathy, urinary incontinence
* orbital frontal
* emotional/social/sexual restraint
* deficit shows disinhibition, witzelsucht emotional lability, echopraxia, impulsiveness
73
hypermetabolism in what area would cause OCD?
orbital frontal area of frontal lobe
74
When I am clinically assessing the frontal lobe, I might check for frontal release signs. What is an example of this?
Glabellar tap - continue tapping forehead and a positive test is the person will continue to be surprised every time
75
What is the circuit of Papez?
* hippocampus
* mammillary bodies
* antero/dorsomedial thalamus
* cingulate gyrus
* enterorhinal cortex
76
What kind of memory is circuit of papez involved with? What does this kind of memory do?
long term memory - declarative
conscious aquisition, retention and retrieval of knowledge; it can be episodic or semantic
77
What is an example of non-declarative long term memory?
procedural memory - skills or habits relatively impervious to decay
78
What is in charge of encoding episodic information?
What is essential for episodic recollection?
hippocampus encodes
frontal lobes recollect
79
What is it called when old memories are more strongly consolidated than recent ones and can therefore be more easily recalled?
Ribot's rule
80
Where is semantic information stored in the brain?
lateral temporal lobe (L\>R)
81
Where is Wernicke's area located?
posterior superior temporal gyrus
82
What happens in Wernicke's area?
where sounds are married with meaning
holds all the memory of what the sounds of words mean
83
A lesion in Wernicke's area might produce...
sensory aphasia - fluent speech but meaningless
84
Damage to what area might cause dyslexia?
angular gyrus
85
What is the pathway of sound being interpreted and speech created?
1. primary auditory cortex
2. Wernicke's area interprets
3. goes via arcuate fasciculus to
4. Broca's area
86
Where is Broca's area? What does this area do? Damage here would cause what?
inferior frontal gyrus
word programming needed for production
damage here - motor aphasia - can't get words out
87
A pt's speech is sparse and agrammatic, they are not able to repeat you. Where is the lesion?
Broca's
88
A pt's speech is paraphasic and a little bit of a word salad, but they have fully formed words. Where is this lesion? Are they able to comprehend you?
Wernicke's
no comprehension
89
What lobe of the brain is responsible for spatial attention and awareness?
parietal lobe (R\>L)
90
A deficit in the R parietal lobe might cause...
What are some tests that can be administered to test this?
neglect of sensory or motor intention
tests: draw clock face, bisect line, test for sensory extinction
91
If a pt has a conduction apraxia, what are they unable to do?
cannot imitate gestures
92
a pt with ideomotor apraxia cannot...
perform a task when cued
93
Gerstman Syndrome occurs in the L inferior parietal lobe and consists of 4 main elements...
1. agraphia
2. acalculia
3. L-R distortion
4. Finger agnosia (can't distinguish)
94
What are some tests you might use to see the function of the primary somatonsensory cortex?
2 point discrimination
stereogenesis
graphesthesia
touch
vibration
proprioception
95
The secondary somatosensory cortex will be very good with a sense of what two things?
pain and temp
96
What pathway does the "where" analysis of motion and space?
occipital/parietal dorsal pathway
97
What pathway does the "what" analysis of form/color/face/letters?
occipital/temporal ventral pathway
98
Bilateral lesions in the occipital/parietal dorsal pathway would cause what syndrome? What kind of lesion is this usually from?
Balint syndrome
optic ataxia, oculomotor apraxia, simultanagnosia (Navon figure)
usually from MCA-PCA watershed stroke
99
If someone has a lesion in the occipital/temporal ventral pathway, and cannot recognize people by face, but can recognize them by their voice, they have...
prosopagnosia
100
Alice in Wonderland Syndrome is caused by a lesion where?
occipital/temporal ventral pathway
101
Alexia without agraphia is caused by a lesion where? What kind of visual field defect might go with this?
L PCA stroke
R homonymous hemianopia
102
A pt is having parosmias. What is going on?
perversion of smell
103
What area of the cortical brain has an area for smell? What happens if there are lesions here?
uncus of temporal lobe
olfactory hallucinations
deja vu
104
If a pt says they are having deja vu with olfactory hallucinations, what should be done and why?
get MRI
medial temporal lobe lesion is probably
105
What syndrome is associated with an orbital frontal tumor that compresses the olfactory nerve causing ipsilateral anosmia and optic atrophy?
What else could be happening?
Foster Kennedy Syndrome
CL papilledema due to increased ICP from tumor - atrophic disc cannot swell
106
What is seen in pre-chiasmal disease, classicly due to retinal ischemia?
altitudinal scotoma
107
What might occur if there is a temporal lobe lesion compressing the optic nerve?
Would this be pre or post chiasmal?
"pie in the sky" - CL homonymous superior quadratopia
post-chiasm
108
What might occur if there is a parietal lobe lesion involving the optic nerve?
"pie on the floor" - CL inferior homonymous quadranatopia
post-chiasmal
109
If a pituitary tumor is influencing the optic nerve, what might the pt be seeing? Where would this lesion be?
bitemporal hemianopia
binocular chiasmal
110
If the doc doing a funduscopic exam sees a swollen disc, but the pt claims to be seeing normally, this would be...
caused by...
papilledema
increased ICP, impedes retinal venous return
111
75 yo female with sudden painful vision loss, HA, jaw claudication, anorexia. PMHx of temporal arteritis and polymyalgia rheumatica.
What will I see on PE?
Dx? Tx?
fundus is always abnormal
Arteritic Ischemic Optic Neuropathy
tx with emergent steroids
112
Pt describes a 'shade curtain' falling over eye and has transient, painless blindness for \<10 minutes.
Dx? Sign of? Concerns?
PE?
Amaurosis Fugax
sign of retinal vascular insufficiency, consider retinal TIA
most often from Internal carotid artery
funduscopic pallor causing cherry red spot inside fovea
113
If I pinch my pt's neck and activate the sympathetic chain, resulting in pupillary dilation, this would be called..
ciliospinal reflex
114
In optic neuritis, a common presentation of MS, the fundus exam will be... and the pt will feel...
normal
pain with EOM
115
Near Light Dissociation is what?
pupils constrict more to accommodation than they do to light
116
This pt appears to have hypertropia, the visual axis in one eye is higher than the other. This could be due to..
compression of the trochler nerve
117
A bilateral palsy of the abducens nerve must mean there is...
increased ICP
118
What does a complete palsy of CN VI appear as?
eye adducted and cannot move laterally beyond midline
119
With an abducens palsy, a pt might have diplopia. Based on diplopia, how do you know which eye is affected?
Diplopia is always worse looking toward the side with paralyzed muscle
120
Cavernous Sinus Syndrome impacts what nerves?
III, IV, VI, V1
121
In cavernous carotid aneurysm/fistuals, what nerve is often involved first, and why?
CN VI due to its close proximity to ICA
122
Orbital Apex Syndrome includes which CN?
II, III, IV, V1, V2
123
in poliomyelitis, inflammation is confined to...
grey matter
124
in pachymeningitis, inflammatory process is limited to...
spinal dura
125
cervicomedullary lesion presents in what symptomatic fashion?
'around the clock' pattern of weakness
possible neck pain
CSF +/- obstruction (ICP)
downbeat nystagmus, papilledema
126
At what cervical level will you start to see 'onion skin' pattern of facial numbness if there is a lesion there?
C4
127
With a spinal cord lesion, what happens to reflexes at, above, and below the lesion?
at the lesion is decreased
below the lesion is increased
above the lesion is normal
128
conus medullaris will present with what?
bilateral saddle sensory loss
129
cauda equina will present with what?
radicular pain, asymmetric sensory loss
130
Complete cord transection injury will have spinal sensory loss where?
1-2 levels below the lesion
131
How do you know if you have spinal shock or neurogenic shock?
* spinal shock
* acute onset
* decreased or no reflexes
* no sensation
* flaccid paralysis
* is less than 48 hours
* neurogenic shock
* injury to T6 or above
* delayed onset
* hypotension, bradycardia, hypothermia
* lasts 1-3 weeks
132
A person with autonomic dysreflexia is at risk for...
malignant HTN
133
Hemicord severed/ Brown Sequard syndrome presents with what ssx?
* ipsilateral vibration and position loss below the lesion
* ipsilateral UMN weakness below the lesion
* CL loss of pain and temp 1-2 segments below the lesion
134
a small central cord syndrome would cause what?
disruption of bilateral crossing of spinothalamic tract fibers - loss of pain and temp bilaterally, typically suspended cape or vest
135
What causes ascending CL sensory loss? GBS would be in the ddx.
Extramedullary compression
spondyltic myelopathy is MCC of myelopathy in older pts
136
Most intradural, intramedullary tumors are what?
ependymomas
137
Intradural, extramedullary lesions are most likely...
meningiomas, schwannomas, neurofibromas
138
sensory ataxia and stomping gate will be seen in what spinal cord lesion?
Posterior Cord Syndrome
139
What are Argyll Robertson pupils?
Pupils that accommodate but do not react. Are found in neurosyphilis often
140
How could a B12 or Copper deficiency present in a spinal cord lesion?
Posterolateral syndrome - decreased vibration and proprioception, UMN is weak, temp and pain is spared
ataxic gait, increased or decreased reflexes
141
What spinal cord lesion will present with split hand (atrophy medial to thumb), no ptosis or EOM issues, and painless distal asymmetric weakness of hand or limb?
combo anterior horn and corticospinal tract
142
Anterior cord syndrome often happens as secondary to a spinal cord stroke in what regions?
anterior spinal artery around sensory level T10
great radicular artery of Adamkiewicz
143
burning pain caused by nerve trauma
causalgia
144
Large myelinated fibers in charge of muscle control are what type?
A alpha fibers
145
Fairly large nerve fibers for touch, vibration, position, perception are what kind of fibers? Are they myelinated?
A alpha beta
myelinated
146
both cold perception and warm perception and pain are on smaller neurons that are what fibers?
A delta for cold
C fibers for warm
147
Autonomic neurons use what fibers?
C fibers
148
carpal tunnel syndrome can have a ddx of what spinal level radiculopathy?
C6
149
Proximal median nerve weakness will include what muscles?
flexor pollicis Longus
flexor digitorum I and II
pronator teres
150
extension of MCP and flexion of IP joints of digits 2 and 3 would fall under what neuropathy?
proximal median neuropathy
151
distal to pronator teres is the largest motor branch of the median nerve. an issue here is called what? What are its ssx?
anterior interosseous nerve syndrome
cutaneous sensation intact
weak FPL and FDP in digits 2 and 3 (flat finger in OKsign)
152
finger abduction with 4 and 5 claw is seen in what posture and neuropathy?
benediction posture
ulnar neuropathy at elbow
153
What is froment sign?
cannot adduct thumb
154
weak 3rd palmar interossei is ...
Wartenberg sign
155
triceps and anconeus are innervated above or below the spiral groove?
above
156
deltoid and latissimus weakness would suggest
posterior cord brachial plexus injury
157
if inferior and superior gluteal nerves are involved in a 'sciatic' issue, consider...
higher lumbosacral plexus issue
158
deep peroneal will have weak
dorsiflexion
159
common peroneal n will have weak
dorsiflexion and eversion
160
sciatic nerve will have normal...
glutes
161
iliacus weakness/flexion of thigh is weak
femoral neuropathy
162
posterior tibial nerve entrapment and pain worse with weight bearing will be...
tarsal tunnel syndrome
163
hereditary sensory and motor neuropathy with spectrum of disorders; may have pes cavus and hammer toes a/w high arched foot, atrophy of distal leg in a champagne bottle sign
charcot-marie-tooth disease
164
ascending weakness out of proportion to sensory deficit
Guillian barre syndrome
165
Erbs palsy (waiters tip) is an upper trunk plexopathy that will cause weakness in what muscles?
deltoid, biceps, infraspinatus, teres minor
166
hyperabduction of the arm at birth might cause...
Klumpke's palsy
167
immune mediated brachial plexus neuropathy is also known as...
a/w acute onset nocturnal shoulder pain to progressive weakness
parsonage-turner syndrome
168
What are the 3 main mechanisms for degenerative nerve root disease?
disc herniation
uncovertebral joint degeneration
facet joint degeneration
169
weakness in 2 muscles in 1 extremity supplied by different peripheral nerves I am thinking...
nerve root disorder
170
deltoid and biceps weakness might suggest
C5 nerve root disease
171
What is the MC herniated cervical level? What would this affect?
C7 - affects triceps and pronator teres
172
C8 nerve root disorder could affect..
entire hand weakness
173
spinal or radicular shocklike paresthesia with neck flexion is called...
Lhermitte Phenomenon
174
decreased patellar reflex, quad weakness, and decreased medial sensation on foot suggests
T4 radiculopathy
175
L5 radiculopathy would have...
gluteal involvement and patellar reflex would be normal, decreased sensation to the top of the foot
176
decreased achilles reflex makes you think..
S1 radiculopathy
177
If a pt has a radiculopathy and is getting sensory nerve conduction studies, what will the result be?
normal sensory studies in radiculopathies bc nerver root is proximal to DRG
