Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Eugenie Notes > Notes 3A > Flashcards

Notes 3A Flashcards

(117 cards)

Start Studying
1
Q

F wave?

A

obtained after super maximal stimulation of a motor nerve
electrical impulse travels antidromically (conduction along the axon opposite to the normal direction of the impulses) along the motor axons toward the motor neuron, backfiring and then orthodromically (conduction along the motor axon in the normal direction) down the nerve to be recorded at the muscle.

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

H reflex?

A

electrophysiologic equivalent of the ankle reflex (S1 reflex arc) and is obtained by stimulating the tibial nerve at the popliteal fossa while recording at the soleus.

The electrical impulse travels orthodromically through a sensory afferent, enters the spinal cord, and synapses with the anterior horn cell, traveling down the motor nerve to be recorded at the muscle

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

Siponimod? MOA and SE?

A

sphingosine 1 -P inhibitor
Need to check CYP2A9 prior to initiating treatment

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

Ocrelizumab MOA? only DMT approved for?

A

Anti-CD20
Only one approved for PPMS

*can have infusion reactions, tx with tylenol and solumedrol*

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

Large polyphasic motor unit potentials are seen in?

A

chronic re-innervation

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

Types of spontaneous activity?

A

fibrillation potentials
fasciculation potentials
myokymia
myotonic potentials

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

What is recruitment?

A

measure of the number of MUPs firing during inc force of voluntary muscle contractionm

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

myopathic MUAPs are

A

small and short in duration

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

neurogenic MUAPs are

A

large and prolonged

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

Fibrillation potentials

A

occur when an individual muscle is denervated. Fibrillation potentials are small (<500 µV), short (<5 ms in duration), biphasic or triphasic, and fire regularly or sometimes irregularly.

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

Positive sharp waves

A

emanate from denervated muscle fibers and have the same clinical significance as fibrillations. They have a sharp initial positive deflection followed by a longer, lower amplitude negative phase than fibrillation potentials.

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

Myokymia

A

Myokymia consists of bursts of MUAPs, usually 2 to 10, firing at rates of 20 to 150 Hz. The bursts consist of regular or irregular doublets, triplets, or multiplets. Between bursts there is electrical silence.

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

Reduced recruitment is when there is? seen in

A

less than expected MUPs firing more rapidly than expected

axonal loss, conduction block, end stage myopathy

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

Early or rapid recruitment is when there is?

A

myopathic processes with lots of muscle fibers in which an excessive number of short-duration and small amplitude MUPs fire during contraction

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

With poor effort or with CNS disorders causing weakness, recruitment is?

A

reduced, with normal MUPs firing at slow or moderate rates

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

In neuropathic disorders with denervation and re-innervation, MUPs are

A

inc duration and amplitude, and may be polyphasic

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

In a radiculopathy, SNAPs are

A

normal (SNAPs are recorded distally to the lesion, in the postganglionic projections from the DRG)

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

EMG findings in radiculopathy?

A

An axon loss radiculopathy will also injure motor fibers in the intraspinal canal region affecting the respective myotome.

This leads to denervation, with fibrillation potentials seen 3 weeks after the onset of motor axon loss, decreased recruitment, and 3 to 6 months later, large and polyphasic motor unit potentials (MUPs). The presence of these large and polyphasic MUPs is dependent on reinnervation and collateral innervation, typically occurring in a proximal to distal fashion, with proximal muscles more successfully reinnervated as compared to distal muscles.

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

Mg dx with EMG/NCS

A

NCS is normal EMG with slow repetitive nerve stimulation showing more than 10% decremental response in CMAP amplitude

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

Lambert Eaton Dx with EMG/NCS

A

rapid stimulation produces an incremental increase in CMAP amplitude (>50% inc)

  • this occurs with rapid stimulation (20 to 50 Hz) because the frequency of stimulation is faster than the time it takes for calcium to leave the presynaptic terminal (100 to 200 ms), leading to higher levels of calcium influx and larger end-plate potentials
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Skeletal muscle Type 1

A

slow-oxidative

  • slow ATPase activity and large oxidative capacity
  • Large num of mitochondria
  • Red in color, small diameter
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Skeletal muscle type 2A

A

fast oxidative glycolytic fibers

  • Fast ATPase
  • High glycolytic capacity
  • Moderate oxidative capacity
  • Fast and resistant to fatigue
  • Red and large
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Skeletal muscle type 2B

A

fast oxidative glycolytic fibers

  • Fast ATPase
  • High glycolytic capacity
  • Low oxidative capacity
  • Fast and fatigable
  • Pale and large
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

CIDP

features

timeline

EMG/NCS shows

Biopsy?

A

symmetric demyelination with proximal and distal weakness with or without sensory loss and hypo/areflexia.

progressive, needs to be for 8 weeks for dx

demyelinating polyneuropathy with prolongation of distal motor latencies, reduction of motor conduction velocities, prolongation of F-wave latencies/absence of F-wave, partial motor conduction block, abnormal temporal dispersion

onion-bulb formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
normal latency should be? conduction velocity?
\<4 ms \>50
26
The presence of a conduction block suggests?
demyelination
27
Wallerian degeneration is completed in ___ days from the injury
7-10
28
Fibrillation potentials (sign of spontaneous muscle activity) appear in ___ days from injury
3 weeks( 21 days)
29
Carpal tunnel where is sensation spared?
thenar eminence because the palmar sensory branch that innervates the thenar eminence travels outside the carpal tunnel
30
Sign of severe CTS is
thenar muscle atrophy
31
GBS: which variant has worse prog
axonal variant
32
NIF of less than ___ or vital capacity less than ___ supports elective intubation in GBS
-30 15-20
33
Which nerves branch off the roots of the brachial plexus
dorsal scapular nerve (innervates the rhomboids and elevator scapulae) long thoracic nerve (innervates the serratus anterior)
34
Trunks of the brachial plexus
Upper: C5-6 Middle C7 Lower C8-T1
35
cords of the brachial plexus: Lateral
named in reference to the axillary artery Lateral (C5-7): gives rise to lateral pectoral nerve, median nerve, musculocutaneous
36
cords of the brachial plexus: Posterior
Posterior C5-T1: Gives rise to upper subscapular nerve, lower subscapular nerve, thoracodorsal nerve, axillary nerve, radial nerve
37
cords of the brachial plexus: Medial
C8-T1 medial pectoral nerve, medial brachial cutaneous nerve, medial antebrachial cutaneous + median, ulnar nerve
38
Familial amyloid polyneuropathy mutation, inheritance Type 1 vs Type 2
AD transythretin mutation Type 1: Polyneuropathy + autonomic features Type 2: carpal tunnel syndrome, mild sensory polyneuropathy + absence of prominent autonomic features
39
CMT features nerve biopsy shows
heriditary sensorimotor polyneuropathy , high arched foot onion bulb appearance
40
Demyelinating types of CMT
CMT1, CMTx, CMT4
41
CMT1 inheritance mutation features CSF may show
AD (most common type) DUPLICATION OF PMP22 on chromosome 17 hammertoes, high arched feet, palpably enlarged nerves and pes cavus can see elevated protein in CSF
42
CMTx inheritance mutation
X linked Connexion-32 gene
43
CMT2 inheritance features
AD axonal neuropathy (not demyelinating) sx appear later in life, more UE than LE can see optic atrophy (CMT2A), foot ulcerations, vocal cord paralysis, no peripheral nerve hypertrophy
44
CMT3 (Dejerine Sottas Syndrome) features
most severe form, AD and AR presents in infancy with proximal weakness, absent DTRs, hypertrophy of the peripheral nerves
45
HNPP (hereditary neuropathy with pressure palsy) inheritance mutation features biopsy
AD deletion in PMP22 focal mononeuropathies (most commonly peroneal nerve, followed by ulnar nerve) biopsy shows tomacula (myelin thickening)
46
Lesion in deep peroneal nerve presents with?
inability to dorsiflex (eversion is intact) Superficial = eversion Common = dorsiflexion and eversion
47
Foot drop + eversion impaired + inversion impaired ?
L5 radiculopathy
48
Foot drop + eversion impaired + intact inversion?
common peroneal neuropathy
49
Tangier's disease inheritance features unique PE finding
AR defect with deposition of triglycerides causes sensory neuropathy with disassociated sensory loss (loss of pain and temp with preservation of the posterior column) Deposition of TG can cause yellow/orange tonsils
50
Anti-Hu is seen in some sensory neuropathies and is assoc with?
Small cell lung ca
51
Anti-Yo is seen in some cerebellar degenerations and is assoc with?
ovarian ca
52
Sciatic nerve gives rise to
tibial and common peroneal nerve
53
Multifocal motor neuropathy (MMN) features AB assoc treatment
purely motor demyelinating neuropathy, presents with asymmetric weakness from involvement of individual peripheral nerves + hypo/areflexia. **no sensory deficits** anti-GM1 IVIG, no response to steroids or plasmapharesis
54
Hypokalemic periodic paralysis Inheritance Types presents with triggered by tx
AD Type 1: Ca channel mutation (CACN), Type 2: Na channel (SCN4A) presents with episodes of weakness without myotonia, during attack K is low. exercise, carb high meals, alcohol, emotional stress, cold diet + acetazolamide and K sparing diuretics
55
Hyperkalemic periodic paralysis inheritance mutation presents with tx
AD Sodium channel SCN4A episodes of weakness triggered by exercise and fasting can give glucose during attacks tx with thiazides
56
Anderson-Tawil Syndrome mutation features
K channel mutation in gene KCNJ2 periodic paralysis, ventricular arrhthymia, dystrophic features
57
Paramyotonia inheritance mutation features
AD SCNA4 exercise induced myotonia most appreciated in the eyelids (in contrast to myotonia congenita)
58
in steroid induced myopathy, there is atrophy of? EMG? CK?
Type 2 fibers EMG and CK nonspecific
59
3 types of Anti-ACH Abs in MG
binding blocking modulating
60
most common ACH Ab assoc with MG
binding (causing a complement mediated destruction of the receptor
61
MOA of tensilon test
ACHesterase inhibitor = causes transient improvement of weakness
62
Dystrophic myotonia Type 2 (aka proximal myotonic myopathy) Inheritance, mutation features
AD CCTG repeat expansion in intron of zinc finger protein 9 gene on chromosome 3 myotonia of proximal muscles (type 1 is distal)
63
Dystrophic myotonia Type 1 inheritance, mutation features
AD CTG repeat expansion in myotonic dystrophy protein kinase gene on chrom 1 myotonia of the distal muscles: ptosis, facial weakness, frontal balding, atrophy of the masters and temporals, weakness and atrophy of small muscles of the hands and extensor of the forearm and peroneal muscles, testicular atrophy, infertility, intellectual delay
64
Facioscapulohumeral muscular dystrophy (FSHD) inheritance, mut features
AD D4Z4 mutation on chromosome 4 * Predominantly affects the **face and shoulder** * Presents with difficulty lifting their arms above their head with relative sparing of deltoid muscles + weakness of lower abdominal muscles (**Beevor sign)** * Forearm more atrophic than the arm—\> P**opeye** * Weakness of foot dorsiflexion with preservation of plantar flexion is characteristic
65
Symptoms that distinguish cholinergic crisis (pyridostigmine overdose) from myasthenic crisis
In pyridostigmine overdose: nausea, vomiting, diaphoresis sialorrhea, excessive bronchial secretions, mitosis, bradycardia and diarrhea
66
Central core myopathy inheritance, mut features pathology
AD RYR1 (ryanodine receptor gene) on chrom 19 * Can print with weakness and hypotonia after soon after birth and delay in motor development * **Pelvic girdle more affected than shoulder girdle** * Pathology: loss of oxidative activity of NADH within the center of muscle fibers (pale areas)
67
Autonomic ganglionopathy caused by features Ab Tx
dysfunction in the parasympathetic and sympathetic nervous system * orthostatic hypoT, absent HR variability, hypo or anhidrosis, dry mouth and eyes, pupillary abnormalities, sexual dysfunctions, early satiety, constipation, and diarrhea due ti abnormal gastric and intestinal motility may have Ab against ganglionic nicotinic ACH rec PLEX and or IVIG
68
In 50% of patients with clinic MG and Ab-ACH negative, what is pos
Anti-MUSK (muscle specific tyrosine kinase)
69
Significance of Anti-MUSK (+) MG?
does not respond to pyridostigmine
70
Features of congenital muscular dystrophies at birth?
* hypotonic and weak, rest failure, bulbar dysfunction, scoliosis, developmental delay and anomaly of cerebral cortex causing seizures
71
Pure autonomic failure (Bradbury-Eggleston Syndrome) caused by features
loss of intermediolateral cell column neurons, results in deposition of alpha-synuclein in autonomic nervous system (Lewy Bodies) autonomic sx that are worse in the morning, after meals, with exertion and heat exposure
72
What is seen in dermatomyositis and not seen in polymyositis
perifasicular atrophy
73
Inclusion body myositis features pathology
myopathy affecting those \>50yo asymmetric weakness and atrophy of the wrist and finger flexors, quads, anterior tibial muscle → painless weakness that is distal leading to th**umb flexing weakness with relative preservation of finger abductors (dorsal interossei)** **Pathology:** endomysial inflammation, intracytoplapmic vacuoles with granular material known as rimmed vacuoles
74
Limb Girdle Muscular Dystrophy features assoc with
proximal weakness with involvement of shoulder or pelvic girdle with sparing of facial muscles assoc with cardiomyopathy and conduction defects → need cardiac screening
75
Difference between LEMS / MG
LEMS is less likely to have ocular or bulbar sx than MG LEMS also more likely to have depressed DTRs
76
Tx of LEMS? autoimmune LEMS
3,4 DAP (diaminopyridine) steroids, IVIG, PLEX
77
Nemaline myopathy features path
* Proximal weakness, cardiomyopathy and prominent compromise of rest muscles Fibers have rod like structures (nemaline rods)below the sarcolemma
78
Pompe Disease (glycogen storage disease Type 2) is a deficiency of
Acid Maltase
79
McArdles Disease (glycogen storage disease type 5) is a def of
myophosphorylase
80
Cori's Disease (glycogen storage disease Type 3) is a def of
glycogen debranching enzyme
81
Congenital MG caused by features difference in Tx from regs MG
* Due to acetylcholine receptor deficiency * Most commonly present a birth (\> males) * Typical presentation: ophthalmoparesis in infancy with facial diparesis * Not immune mediated, so not treated like MG
82
Emery-Dreifuss muscular dystrophy gene, inheritance presents with assoc with
* AD, LMNA * Presents with contractures in elbows, ankles and neck. * Muscles weakness tends to affect the UE and shoulder girdle first and layer the pelvic girdle and distal legs * Cardiac involvement
83
Difference between nerve structures (pre and post ganglion) in parasympathetic vs sympathetic
PARA: (ganglia are close to the target site) long pre and short post SYMP: short pre and long post
84
The neurotransmitter for all sympathetic and parasympathetic preganglionic fibers is _____ (acts on ____ receptors). Postgang in parasym release ______ while postgan in sympathetic release \_\_\_\_\_\_\_\_.
The neurotransmitter for all sympathetic and parasympathetic preganglionic fibers is acetylcholine (acts on nicotinic receptors. Postgang in parasym release acetylcholine while postgan in sympathetic release norepinephrine
85
Autonomic system in the spine: Para vs symp
Parasympathetic - Onuf's nucleus S2-S4 (GU and distal colon: controls the urethral and anal sphincters) Sympathetic: intermediolateral cell column from T1-L2
86
With lesion of the cauda equina, a flaccid bladder results from?
Loss of detrusor tone, sensation of bladder fullness is lost, voluntary control over urination is lost → overflow incontinence
87
with lesions affecting the paracentral lobule (such as hydrocephalus or tumors), what is affected
voluntary control over the external urethral sphincter is lost
88
* Lesion above the conus medullaris lead initially to
* a flaccid bladder leading to urinary retention with or without overflow incontinence.
89
BUZZWORDS: acute onset dysautonomia in a smoker with a lung mass
Paraneoplastic autonomic ganglionopathy Ab against ganglionic nicotinic ACH receptors
90
BUZZWORDS: Duchenne muscular dystrophy (protein, inheritance)
dystrophin X linked rec
91
BUZZWORDS: tongue, ear, throat pain assoc with syncope
glossopharyngeal neuralgia
92
BUZZWORDS: tilt table test showing inc in HR \>30 from baseline, or more than 120 within 10 mins of head up tilt, **without** significant changes in BP but with sx of orthostasis
POTS most common form of dysautonomia
93
BUZZWORDS: Emery Dreifuss gene and inheritance
LMNA AD (could also be X linked and emerin
94
BUZZWORDS: central core myopathy gene, path, assoc clinically
RYR1 central pare cores in NADH stains from absence of mitochondria assoc with malignant hyperthermia
95
BUZZWORDS: Tarul disease (enzyme)
phosphofructokinase def
96
BUZZWORDS: cause of Hirschsprungs Disease
maldevelopment and absence of the myenteric plexus, sometimes due to RET proto-oncogene mutation
97
BUZZWORDS: Becker's muscular dystrophy protein, inheritance
abnormal or reduced dystrophin XLR
98
BUZZWORDS: syncope with hypotension and bradycardia after putting on a tight neck tie
carotid sinus hypersensitivity
99
BUZZWORDS: Ab assoc with LEMS
presynaptic P/Q type voltage gated calcium channel antibodies
100
BUZZWORDS: oculopharyngeal dystrophy (gene affected)
CTG repeat on PABP2 gene
101
BUZZWORDS: pathologic finding in critical illness myopathy
myosin loss
102
BUZZWORDS: Anderson's disease (enzyme)
Branching enzyme deficiency
103
ALS affects what parts of the brain
anterior horn cells, motor cortex, brainstem
104
Split hand phenomenon in ALS?
weakness and atrophy of the lateral hand (thenar and FDI muscles) with relative sparing of the medial hand (hypothenar)
105
Tx for psuedobulbar affect in ALS
dextromethorphan-quinidine
106
What cognitive impairment is assoc with ALS?
FTD - have TDP-43(+) tau inclusion
107
Familial ALS-FTD is assoc with what mutation?
C9ORF72 on chromosome 9
108
Familial ALS mutation?
copper/zinc superoxide dismutase (SOD1) on chromosome 21
109
Things usually spared in ALS?
bladder/sphincter issues - Onuf's nucleus is spared sensation autonomic sx
110
PLS (primary lateral sclerosis) features
UMN signs at least 3 years from symptom onset wihtout evidence of LMN dysfunction. Presents in the 60s with progressive spastic tetraparesis and later cranial nerve involvement
111
Most common cause of spinal cord involvement in patients with HIV
HIV-related myelopathy: vaculoar myelopathy in which there is lateral and posterior column demyelination with microvacuolar changes and axonal preservation (upper extremities typically spared)
112
HTLV-1: tropical spastic paraparesis features MRI labs Tx
chronic progressive myelopathy endemic to equatorial and south Africa as well as parts of Asia, south america and caribbean. Transmitted via blood, sex, breast feeding. p/w slow spastic paraparesis, LE paresthesias, painful sensory neuropathy and bladder dysfunction MRI with inc T2 signal and atrophy of the thoracic cord Dx with serum and CSF PCR no tx at the moment
113
Hereditary Spastic Paraparesis inheritance, mut features
AD SPAST gene encoding spastin protein Progressively worsening spasticity of the LE with variable weakness and difficulty walking
114
What spinal levels are most susceptible to watershed injury?
T4-T8: blood supply is scarce
115
Artery of Adamkiewicz: supplies and affected in
T8-L3 levels can get infected in aortic arch dissection
116
oculopharyngeal dystrophy: mutation
GCG repeat, PABP2 gene
117
Duchenne MD inheritance mutation features
X linked dystrophin gene mutation proximal muscles weaken with sparing of ocular, facial, bulbar All should get cardiac checkup

Eugenie Notes (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions