Neuromuscular Flashcards
what nerve provides sensation to the first web space of the foot?
deep peroneal nerve (branch of common peronial, which is a branch of the sciatic)
what nerve provides sensation to the upper and lateral part of the lower leg?
common peroneal n. (branch of the sciatic n.)
what nerve provides sensation to the lower lateral part of the lower leg and the doral medial foot?
superficial peroneal n.
what nerve innervates the volar (palmar) surface of digits 1-3
median nerve
what sphincter is under voluntary control for micturition?
external urethral sphincter
what is the innervation of the external urethral sphincter (and form what spinal level does it arise)?
motor neurons from Onu’f nucleus in the anterior horn of S2-S4.
most usefull muscle to test to distinguish muscles of the upper brachial plexus and the lateral chord.
Teres Minor
where is cerebral voluntary control of micturition located?
Frontal micturition center in the paracentral lobule.
What is innervation of the internal urethral sphincter and detrusor muscle?
Sympathetic inputs from T11-L1 cause bladder neck contraction.
What is the innervation of the internal urethral sphincter?
sympathetic.
what are the 4 EMG features of CIDP?
- Conduction block, 2. temporal dispersion, 3. prolonged distal latency, 4. slowed conduction.
Protein involved in LGMD 1B, inheritence, and clinical feature
Lamin A/C, AR, Cardiac arrhythmias
Protein in LGMD 1c, inheritence, and clinical correlate
Caveolin-3, AR, rippling muscle disease
protein in LGMD 2A, inheritence, and clinical presentation
Calpain-3, AD, scapular winging, rectus abdominal weakness, hamstring weakness.
Protein in LGMD 1A, inheritance and clinical presentation
Myotilin, AR, adult onset, distal predominance
protein in LGMD 2B, inheritance, and clinical feature
Dysferlin, AD, posterior calf atrophy
LGMD’s that make up 10% of all LGMD, inheritance, and proteins
LGMD 2C, D and E, AD, sarcoglycans
Protein in LGMD 2G, inheritance, and clinical feature
Telethonin, AD, quadriceps and anterior tibial weakness
Protein in LGMD 2J, inheritance, and clinical feature
Titin, AD, can be distal
What LGMD results in walker warburg disease when severe, and what protein is involved?
LGMD 2K, POMT1
What LGMD results in fukuyama disease when severe, and what protein is involved?
LGMD 2L, Fukutin
what LGMD results in muscle eye brain disease when severe and what protein is involved?
LGMD 2M, POMGnT1
which type of motor neuron causes muscle contraction?
alpha
what type of motor neuron innervates intrafusal muscle fibers? What is its role?
gamma afferents- maintains 1alpha efferent firing during contraction.
what type of muscle fiber afferent (aka sensory n.) prevents muscle over-contraction during muscle firing?
1b afferents. arise from golgi organs in the msucle.
what are the 3 muscles innervated by the musculocutaneous nerve?
1.coracobrachialis, 2. brachialis, 3. biceps
what movements are weak with musculocutaneous n. injury? Fibers from what nerve root travel in this nerve?
- elbow flexion 2. supination with the arm flexed at the elbow but NOT with the arm extended.
C5,6 and 7
where does numbness occur with musculocutaneous nerve injury?
lateral part of fore arm.
what type of injury causes musculocutaneous nerve injury?
anterior shoulder dislocation.
what is the age of onset of oculopharyngeal muscular dystrophy?
30s-60s
What is the trinucleotide repeat, gene and chromosome in oculopharyngeal muscular dystrophy?
GCN (where N can be any amino acid), PABN1, 14q11.1
what is the nucleotide repeat, gene, and inheritance in myotonic dystrophy type I?
CTG, DMPK, AD, 19q13.2
what is the nucleotide repeat, gene, and inheritance in myotonic dystrophy type II?
CCTG, ZNF9, AD Chr 3
what myopathy is depicted?
Nemeline myopathy
what are the 3 EMG features of Myasthenia Gravis?
- greather than 10% decrement in CMAP with repetitive 3 Hz stimulation. 2. Single fiber EMG “jitter” 3. neuromuscular blockade
What disorder causes sensorimotor neuropathy, upper motor neuron signs, gait characterized by walking on insides of feet, and tightly curled hair? What are the pathologic findings?
Giant axonal neuropathy. Large focal axonal swelling that contain tightly packed disorganized neurofilaments.
What are the two late responses in NCV studies?
F-wave and H-response
How is an F-wave obtained?
Supramaximal stimulation of a motor nerve while recording from a muslce. The impulse travels first antidromically (opposite normal direction) and then backfires and travels orthodromically (normal direction).
How is the H-wave obtained and what reflex arc does it represent?
Stimulate tibial nerve at the popiteal fossa while recording at the soleus. Represents S1 reflex arc.
what 3 things affect CMAP amplitude?
- motor axon 2. NMJ 3. muscle fibers
What is the Motor unit potential (MUP) finding in chronic denervating lesions?
Large polyphasic MUPs
In what disorders is insertional activity increased?
Denervation and myotonic disorders
What are the 5 types of spontaneous muslce activity?
- fibrillation potentials
- fasciculation potentials
- myokymia
- myotonic potentials
- complex repetitive discharges
spontaneous rhythmic depolarizations of individual muscle fibers.
fibrillation potentials, seen in myopathies and neuropathies.
spontaneous depolarizations of a single motor unit
Fasciculation potentials. seen in neuropathic disorders.
adjacent, abnormal muscle fibers depolarizing each other. Machine sound on EMG
complex repetitive discharges. Seen in myopathies and acute neuropathies
spontaneous rapid depolarization of an individual msucle fiber
myotonia, seen in myotonic disorders
Brief, rhythmic bursts of electrical activity originating from the motor unit
Myokymia. Seen in demyelinating neuropathies and radiation neuropathies.
what is recruitment in EMG studies?
number of MUPs firing during voluntary muscle contraction
What EMG findings occur in early axon loss lesions?
decreased recruitment.
What EMG findings occur in myopathic processes?
early or rapid recruitment with excessive short, small amplitude MUPs.
Time course of fibrillation potentials after denervating injury.
3 weeks
time course of large, polyphasic MUPs after denervating injury
3-6 months
What are the SNAP findings in myasthenia gravis?
Normal
does myasthenia gravis affect presynaptic or post-synaptic membrane?
post-synaptic
where is the defect in neurotransmission in Lambert Eaton Myashthenia Syndrome?
pre-synaptic
what are the 4 nerve conduction findings in Lambert Eaton Myasthenia syndrome?
- low or borderline CMAP amplitudes 2. increment in CMAP after exercise 3. decrement with slow repetitive stim 4. increment after fast repetitive stim.
which muscle fibers have slow ATPase activity, large oxidative capacity are red, small, and have lots of mitochondria?
Type I
Which muscle fibers have fast ATPase activity, high glycolytic capacity, moderate oxidative capcity, contract quickly, are red and large in diameter?
Type IIa- fast oxidative-glycolitic
Which muscle fibers have fast ATPase activity, high glycolytic capacity, low oxidative capacity, contract quickly, are pale, and large in diameter.
Type IIb-fast glycolitic
how long do symptoms have to exist before CIDP is diagnosed?
8 weeks
time frame after axonal lesion that NCV shows conduction block
7-10 days. After this time frame conduction block cannot be detected.
what are the nerve roots of the brachial plexus?
C5-T1
which two nerves branch directly off of brachial plexus roots, what roots do they arise from, and what muscles do they innevate?
Dorsal scapular nerve (C4 and C5 -rhomboids and levator scapulae), and long thoracic nerve (C5-C7-, serratus anterior).
which brachial plexus roots give rise to the upper trunk?
C5 and C6
Which nerves branch off of the upper brachial plexus trunk, and which muscles do they innervat?
- Suprascapular- supraspinatus and infraspinatus
- nerve to subclavius- subclavius duh
which nerve roots give rise to the middle brachial plexus trunk?
C7
Which nerve roots give rise to the lower brachial plexus trunk?
C8/T1
which brachial plexus trunks give rise to the lateral cord?
upper and middle
What disease manifests of polyneuropathy, autonomaic features, onset in the 3rd or 4th decade, and AD inheritance? what protein is involved?
Familial amyloid polyneuropathy type 1 (FAP type 1).
transtheretin.
what disease manifests as onset of carpal tunel and slowly progressive polyneuropathy manifesting in the 4th or 5th decase with an AD inheritance pattern?
Familial amyloid polyneuropathy type 2 (FAP2).
what nerve does the lateral cord give rise to and what muscle(s) do(es) it inervate?
lateral pectoral nerve–>pec major
what two nerves does the lateral cord end in?
Median and musculocutaneous.
which brachial plexus trunks contribute to the posterior cord?
Upper, middle, and lower.
what 3 nerves arise from the posterior cord, what root fibers do they carry, and what muscles do they innervate?
- upper subscapular n., C7 and C8, subscapularis 2. lower subscapular nerve, C5 and C6, Teres major 3. thoracodorsal n., C6,7,8, latissimus dorsi.
what two nerves does the posterior cord end in?
axillary and radial
what brachial plexus trunk gives rise to the medial cord?
lower
what 3 nerves does the medial cord give rise to, what root fibers do they carry, and what do they innervate?
- medial pectoral nerve, c C8, T1, pec minor 2. medial brachial cutaneous nerve, sensation to medial arm 3. medial antebrachial cutaneous nerve, sensation to medial forearm.
what nerve does the medial cord end in?
Ulnar nerve- note, it contributes fibers to the median nerve before continuing as Ulnar nerve.
what are the demyelinating CMTs?
CMT1 , CMT3 and CMTX
what are the symptoms of CMT1A?
onset in the 1st two decades, progressive weakness, muscle atrophy, mild sensory loss, hammar toes and pes cavus foot.
What is the gene defect in CMT1A
duplication of PMP22 on Chr 17
What are the symptoms of CMT1B?
similar to CMT1A but more severe.
what gene is involved in CMT1B
myelin protien0 gene
What is the inheritance of CMTX?
X-linked so males are more severely affected.
what gene is involved in CMTX?
Connexin 32.
What is the inheritance of CMT1?
AD
What type of CMT is axonal?
CMT2
Which CMT commonly causes otpic atrophy
CMT2A2
What CMT commonly causes foot ulcerations?
CMT2B
What CMTcauses vocal cord paralysis, intercostal and diaphragmatic weakness?
CMT2C
In which CMT is hand weakness common?
CMT2D
what is the inheritence of CMT2?
AD
What CMT has a severe presentation in infancy?
CMT3
What disease is characterized by focal mononeuropathies caused by compression, what is the gene defect, and what is the inheritence?
Hereditary neuropathy with laiability to pressrue palsies, AD, deletion of PMP22.
what is the most common nerve affected in HNPP?
Peronial followed by Ulnar.
what are the 4 main motions of muscles innervated by the median nerve?
- wrist flexion and abduction 2. forearm pronation 3. finger flexion digits 2 and 3. 4. thumb flexion, opposition
what is the sensory innervation of the median nerve?
shown in green
what are the 3 main motions of muscles innervated by the Radial Nerve?
- extension of all joints below the shoulder 2. forearm supination 3. thumb abduction
what is the sensory innervation of the radial nerve?
fibers from which roots are carreid by the radial nerve?
shown in pink
C5-C8
what are the 4 main motions of muscles innervated by the Ulnar nerve
- finger adduction and abduction 2. THumb adduction 3. flextion of digits 4 and 5 4. wrist flexion and adduction
what is the main sensory input of the Ulnar nerve?
what is the main motion of muscles innervated by the axillary nerve? Fibers from what nerve roots are carried by the axillary nerve?
arm abduction beyond 1st 15 degrees. C5 and 6
what is the main sensory innervation of the axillary nerve?
lateral upper arm.