10. ALS vs. cervical stenosis 10. Both can cause painless 4-extremity
weakness with lower extremity upper
motor neuron signs. Fasciculations in the
lower extremities would not be seen in
cervical stenosis but could be seen in
ALS
RADICULOPATHY
1. Muscles innervated by L5
1. Gluteus medius and TFL (hip
abduction/inversion)
Tibialis anterior (dorsiflexion)
Tibialis posterior (inversion)
Peroneus longus (eversion)
Note: Peroneal causes mostly weakness of
dorsiflexion and eversion!)
2. Significance of paraspinal denervation
on EMG 2. Lesion is at the level of the ventral root or
anterior horn cell. Not seen in
plexopathy or neuropathy!
11. Cauda equina syndrome clinical 11. Saddle anesthesia, sphincter loss, loss
of ankle reflexes.
12. Sequence of abnormalities on needle EMG 12. <1 week: decreased recruitment
following nerve root compression. 1-3 weeks: fibrillations (first proximally)
Chronic: fasciculations and large polyphasic
motor units
13. What is right below the fourth cervical 13. The second thoracic dermatome
dermatome on the trunk?
14. Varicella Zoster. Where does it live, how does it 14. Lives in DRG. When activated, it
get to the skin to cause symptoms, and what can it utilizes axonal transport machinery to reach
cause besides radiculopathy. the skin. Can also travel centrally to the cord
and cause myelopathy.
PLEXOPATHY 11. Diabetic radiculoplexus neuropathy
7. Describe idiopathic brachial neuritis 7. Young adults M>F, severe pain followed
(Parsonage-Turner Syndrome) by weakness and sensory loss. Immune-
mediated complexes on nerves.
Spontaneous recovery in 6-24 months.
10. What sensory nerve would be affected 10. The medial antebrachial cutaneous
in thoracic outlet syndrome but not nerve (a branch of the medial cord)
ulnar mononeuropathy at the elbow?
11. Acute back/leg pain and leg weakness.
Weakness is unilateral > bilateral,
proximal > distal. Often well controlled
DM2
MONONEUROPATHY Cranial
1. Steroids. No clear evidence for
1. What do the AAN practice parameters antivirals.
recommend for treating Bell Palsy?
MONONEUROPATHY Upper Extremities
1. Carpal tunnel syndrome clinical description 1. Numb digits 1-3, sparing thenar
eminence. Often bilateral and worse in
dominant hand. Weakness may be present in
abductor pollicis brevis (APB)
2. Radial neuropathy at the spiral groove 2 a. Finger drop, wrist drop, weakness of
elbow flexion (from brachioradialis). The
extensor pollicis longus and brevis,
supinator, and abductor pollicis longus are
also involved.
b. Numbness over dorsum of hand between
thumb and index finger.
8. What nerve supplies the first dorsal 8. Ulnar (unless theres an anastomosis as
interosseus of the hand (FDIH)? in #7!)
9. What nerve supplies the teres major? 9. Subscapular, from the upper trunk
13. What is the clinical difference between a 13. Both cause ptosis and miosis. Only the
Horners syndrome from an internal carotid proximal lesion causes anhidrosis,
artery dissection and a Horners syndrome from because the fibers destined for the sweat
a lesion in the proximal sympathetic pathway. glands are carried on the external carotid
artery.
1. Heavy metals that can cause neuropathy 1. Arsenic, lead, mercury, thallium. (Not
aluminum)
2. Toxin that can produce a clinical picture
identical to B12 deficiency 2. Nitrous oxide
6. HNLPP nerve conduction study findings 6. Mild diffuse slowing, conduction block at
entrapment site.
10. Lead neuropathy 10. Motor > sensory, predilection for the
radial nerve
11. Diabetic neuropathy treatment some 11. They mention that amitriptyline is
random thoughts that seem to come up on the effective, SSRIs are not. Narcotics not first
test repeatedly. line. Mexiletine acts locally. Capsaicin
releases substance P, which causes burning
as a side effect.
16. Guillain-Barre syndrome Lab 16. Cell count normal, or at least <50.
Elevated protein. Spinal MRI, nerve
biopsy, electrolytes are often normal
18. Multifocal motor neuropathy vs. ALS on 18. Both have fibrillations, fasciculations,
EMG. How are they similar? Different? normal sensory amplitudes, neurogenic
MUAPs. Only MMN has focal conduction
blocks in motor nerves, usually not at
entrapment sites.
23. Hypoproliferative anemia in autonomic failure 23. Treatment with erythropoietin can
improve orthostatic tolerance.
POLYNEUROPATHY, continued
25. Polyarteritis nodosa: symptoms, biopsy 25. Mononeuritis multiplex, rash, other
systemic symptoms (i.e. renal failure).
Biopsy shows necrotizing arteritis. No
onion bulbs, which would be suggestive of
primary demyelination.
31. Triggers of porphyria attacks 31. Many drugs. In RITE, there is a case of
sulfonamides inducing an attack.
32. The preganglionic cell bodies for the 32. The intermediolateral column of the
sympathetic system are where? spinal cord. These get input from the
posteriolateral hypothalamus.
33. How does gabapentin exert its anti-nociceptive 33. Modulation of pre-synaptic alpha-4-delta
effects? calcium channels.
34. What are the most efficacious treatments for 34. IVIg, plasmapheresis, and steroids
CIDP?
POLYNEUROPATHY, continued
35. Similarities and differences between 35. Both have subacute, non-length
CIDP and POEMS dependent neuropathy, slow nerve
conduction velocities, and high CSF protein.
POEMS will also have endocrinopathy, M
protein, skin changes, and elevated serum
VEGF levels. Also may have papilledema.
9. Antibodies for myasthenia gravis (MG) 9. ACh receptor antibody, MUSK antibody
13. Infantile myasthenia gravis 13. Infants born to mothers with auto-
immune MG can have symptoms from
circulating antibodies. Clear within 3
months. Treatment is supportive. No
relationship to severity of mothers disease.
14. What happens to patients with ocular 14. Ptosis and/or ophthalmoplegia improves
myasthenia when an ice pack is applied to their
face?
15. What is the most common clinical picture in 15. Prominent facial, bulbar, and neck
myasthenia gravis associated with anti-MUSK weakness. A subset has relative sparing of
antibodies. ocular muscles.
16. Contaminated black tar heroin users are 16. Wound botulism
predisposed to what neuromuscular junction
disease?
18. Tick paralysis pathophysiology 18. Blockade of Ach release and sodium
channels. Normal CSF protein, normal nerve
conduction studies.
21. Most sensitive test for myasthenia gravis 19. Single fiber EMG
10. Pompes disease EMG 10. May show electrical myotonia without
clinical myotonia
11. Duchenne and Becker MD - genetics 11. X-linked recessive due to absent
dystrophin (Duchenne) and abnormal size
dystrophin (Becker). 1/3 are new
mutations.
MYOPATHY, continued
16. Myotonia congenita. Clinical and EMG 16. Congenital muscle hypertrophy,
stiffness after rest, Cl- channel defect,
myotonia on EMG.
21. Statin myopathy findings and mechanism 21. Subacute, CK. Attributed to inhibition
of mevalonic acid, a precursor to CoQ10.
24. Inclusion body myositis 24. Distal upper and proximal lower
extremity weakness. Biopsy: Rimmed
vacuoles. Poorly responsive to steroids.
EMG shows fibs and myopathic units.
26. Isaac Syndrome associated antibody 26. Voltage gated K+ antibody seen in
about 40%. Can be autoimmune or
paraneoplastic
29. Proximal weakness with myotonia 29. Proximal myotonic myopathy (DM2)
30. Clinical and genetic features of Miyoshi 30. Distal weakness, onset age 12-30, very
myopathy high CK levels. Caused by a mutation in
dysferlin.
35. Merosin deficient muscular dystrophy 35. Most common congenital dystrophy
(Merosin is the same thing as lamin 2) Moderately high CK
Proximal weakness and contractures
Cerebral white matter changes, often
asymptomatic
37. What organs are affected in different 37. Myophosphorylase def muscle only
glycogen storage diseases? Pompes muscle and liver
38. Critical illness myopathy risk factors 38. Critically ill patient treated with steroids
39. Critical illness myopathy how do you 39. Preservation of deep tendon reflexes,
distinguish it from critical illness neuropathy or normal nerve conduction studies, normal
NMJ disease? repetitive stimulation.
43. Paramyotonia congenita - clinical 43. Stiffness worse with activity and cold,
no weakness.
44. Increased internalized nuclei are a prominent 44. Myotonic dystrophy. DM1 has more ring
feature of which myopathy? fibers and targetoid fibers than DM2.
46. Drugs that increase risk of statin-induced 46. Drugs that interact with CYP3A4,
myopathy. especially Ca-channel blockers.
NERVE AND MUSCLE PHYSIOLOGY
4. What modalities are affected with loss of large 4. Fine discriminative touch, vibration,
fibers? position sense. (Pain/temp on small fibers)
5. What is tetany and what causes it? 5. Hypocalcemia causes altered resting
membrane potential, leading to prolonged
opening of voltage-gated sodium channels.
8. What are the afferent and efferent limbs of the 8. It is monosynaptic from sensory neuron
muscle stretch reflex. (afferent) to the anterior horn cell
(efferent).
MISCELLANEOUS
1. What are the clinical manifestations of Stiff 1. Adult onset proximal stiffness, wosrse
person Syndrome? What is the treatment? with motion or emotion, lordosis. May be
paraneoplastic or associated with diabetes.
Treatment is diazepam.
1. What is conduction block and what conditions 1. A large drop (usually >50%) between
are associated with it? distal and proximal sites. Seen in acquired
demyelinating neuropathies (CIDP, MMN,
or focal demyelinating of a single nerve
from compression.) Not seen hereditary
neuropathies like CMT1A.
2. Where are the cell bodies of sensory nerves 2. In the dorsal root ganglion (DRG). Any
and how does this affect nerve conduction lesion distal to the DRG (i.e. plexopathy,
studies? neuropathy) will cause Wallerian
degeneration of distal sensory nerve and
absent sensory nerve action potential.
(SNAP). Any lesion proximal to the dorsal
root ganglion (i.e. radiculopathy) may cause
numbness, but there will be no degeneration
of the distal nerve, since it is still connected
to its cell body. Thus, the SNAP will be
normal. (NOTE: This concept has been on
every RITE exam at least twice.)
3. What are fasciculations and when are they 3. Irritation of the alpha motor neuron. Seen
seen? in ALS (as a later finding), radiculopathy,
cramp-fasciculation syndrome , or in
normal muscle.
5. What does the compound motor action potential 5. The number of functional axons in the
(CMAP) correlate with? nerve.
6. What is the most sensitive study to diagnose 6. Median palmar sensory studies show
carpal tunnel syndrome? prolonged latency.
7. What is the affect of cold skin on nerve 7. It artificially prolongs the latency and
conduction studies? increases the amplitude.
12. Decremental response to 2-3 Hz? 12. Classically, this is myasthenia gravis.
However, this can also be seen in LEMS,
botulism, or even severe denervation.
13. What are the limbs of the blink reflex? 13. Afferent is carried by the CN V ipsi to
the stimulus. Efferent is CN VII bilaterally.
14. What would you see on a blink reflex in a 14. Stim Left absent R1 and R2
patient with a CN VII palsy on the left? Stim Right absent R2
15. What are the blink reflex findings in a left 15. Stim Left prolonged ipsi R1, bil R2
trigeminal lesion? Stim Right normal
16. What causes myotonic potentials? 16. Repetitive discharge of the muscle
membrane.
17. What causes complex repetitive discharges 17. Ephaptic transmission of impulses
(CRDs)? from muscle fiber to muscle fiber. Can be
neurogenic or myopathic.
18. In what conditions are CRDs seen? 18. Same as fibrillations. Chronic
neuropathic process and some myopathies
(especially inflammatory myopathies.)
19. What would you see on EMG in a patient 19. A decreased number of very large motor
who had had polio as a child? units firing rapidly.
20. Abnormal jitter and blocking on single fiber 20. Seen in myasthenia gravis, but not
EMG? specific. Would not be seen in metabolic or
steroid-induced myopathies.
21. What is seen on EMG in an upper motor neuron 21. Decreased activation, or a decreased
process? number of normal motor units, firing slowly.
22. What are the nerve conduction findings in 22. First: Decreased recruitment and
CIDP? prolonged F-waves. Later: Slow motor
conduction velocities and conduction block.
EMG/NERVE CONDUCTION STUDIES cont
24. What part of the motor unit is the source of each 24.
of these discharges?
a. Myotonia a. Muscle fiber
b. Fasciculations b. Motor neuron
c. Neuromytonia c. Motor neuron
d. End-plate spikes d. Muscle end-plate
e. Myokymia e. Motor neuron
27. What is the affect of sedation on nerve 27. Loss of late responses (F-waves and H-
conduction studies? reflexes)
28. EMG in neonate with congenital myotonic 28. Myopathic motor units and low
dystrophy amplitude motor responses. (CMAPs)
Myotonic potentials dont show up until
later
29. What will a facial motor response look like in 29. It will be normal, because the site of
a purely demyelinating Bells Palsy. demyelination is proximal to the part of
the nerve that is studied.
30. Nerve conduction studies in patients with spinal 30. Low CMAPs with normal velocities..
muscular atrophy. Normal sensory responses.
31. Significance of a peroneal motor response 31. Accessory peroneal nerve. (Normal
with a higher amplitude proximally than distally. variation.)
34. What causes increased fiber density on single- 34. Denervation and reinnervation. The most
fiber EMG? muscle fibers in a given motor unit, the
greater the likelihood that two or more fibers
will be within the recording radius of the
single fiber EMG electrode.
1. QSART
9. What are the end organs for sensing vibration and 9. Pacinian corpuscles, in the subcutaneous
where are they? connective tissues and periosteum.