RITE Exam Neuromuscular Review
Last updated by Zach London 1/27/16
BOLD = question has appeared on RITE more than once
Highlighting =- On the 2016 exam
MOTOR NEURON DISEASE
1. Clinical description of ALS
2. Riluzole
3. Spinal Muscular Atrophy clinical
4. Spinal Muscular Atrophy genetics
5. Infectious motor neuron disease
6. Kennedys disease clinical
7. Kennedys disease genetic
8. ALS genetic
9. ALS spasticity treatment
10. ALS vs. cervical stenosis
1. Progressive atrophy and upper motor
neuron signs. May start asymmetrically.
Usually patients 50-60s.
2. Glutamate release inhibitor. Class A
evidence for prolonging life in ALS. 8%
benefit of survival at 12 months and 9%
at 18 months.
3. Weakness, hyporeflexia, sparing
extraocular muscles and intrinsic hand
muscles. SMA1 = infants. SMA3= may
survive into adulthood
4. Survival motor neuron gene on
chromosome 5
5. West Nile virus, polio
6. Spinobulbar muscular atrophy, LMN
only primarily affecting cranial
musculature, testicular atrophy and
gynecomastia.
7. X-linked CAG repeat disease,
involvement of androgen receptor
8. 5-10% of cases. Superoxide dismutase
(SOD) gene.
9. The authors of the RITE exam prefer
baclofen to tizanidine
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
2. Significance of paraspinal denervation
on EMG
3. C8-T1 radiculopathy clinical
4. C8-T1 EMG and nerve conduction
findings
5. Spinal stenosis clinical
6. Diaphragm innervation
7. What nerve roots supply brachioradialis?
8. Muscles involved in C5 radiculopathy
9. C6 radiculopathy indications for surgery
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. Lesion is at the level of the ventral root or
anterior horn cell. Not seen in
plexopathy or neuropathy!
3. Weakness of all intrinsic hand muscles
(Abductor pollicis brevis, interossei, etc)
and flexor carpi ulnaris. Note: If
adductor digiti minimi is involved but
not abductor pollicis brevis, think ulnar!
4. Denervation of:
a. abductor pollicis brevis (median)
b. Interossei, adductor digiti minimi
(ulnar)
d. Extensor indicis (radial)
e. Low cervical paraspinal muscles
NORMAL sensory nerve action potential
(because lesion proximal to DRG)
5. Neurogenic claudication, worse with
leaning backwards, better with rest or
leaning forwards (i.e. biking)
6. Phrenic nerve from C3-5
7. C5-6
8. Supraspinatus, infraspinatus, deltoid,
rhomboid, and to a lesser degree biceps,
brachioradialis, brachialis
 9. Intractable pain or weakness. Epidural
injections may help with pain control
temporarily, but do not change outcome
RADICULOPATHY continued

10. S1 Radiculopathy - clinical 10. Weakness of gastrocnemius, reduced

ankle jerk, sensory disturbance along the
posterior aspect of the leg, lateral foot and
sole of foot

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
1. Radiation plexopathy Clinical,
EMG, and treatment
2. Posterior cord supplies which nerves?
3. Features that suggest brachial
plexopathy is due to neoplasm rather
than radiation plexopathy.
4. Causes of Erb-Duchenne (upper trunk)
palsy
5. How do you distinguish a lower trunk
plexopathy from a C8-T1 radiculopathy on
EMG?
6. Muscles involved in an upper trunk
plexopathy.
7. Describe idiopathic brachial neuritis
(Parsonage-Turner Syndrome)
8. Branches of lateral cord
9. Spontaneous femoral neuropathy or
lumbosacral plexopathy with flank pain.
What is the diagnosis and treatment?
10. What sensory nerve would be affected
in thoracic outlet syndrome but not
ulnar mononeuropathy at the elbow?
11. Diabetic radiculoplexus neuropathy
1. Myokymia skin twitching and rhythmic
doublets or multiplets on EMG. Treat with
carbamazepine or phenytoin.
2. Radial, axillary, thoracodorsal, and
subscapular nerves. (Not median and ulnar)
3. Pain, lower trunk involvement, Horners
syndrome, mass on CT scan.
4. Traumatic separation of head and
shoulders, birth injury, brachial plexitis.
5. Lower trunk plexopathy:
a. No paraspinal involvement
b. Absent ulnar sensory nerve action
potential (SNAP)
C8-T1 Radiculopathy
a. Fibrillations in paraspinal muscles
b. Normal ulnar SNAP
6. Deltoid, biceps, brachioradialis,
supraspinatus, infraspinatus.
7. Young adults M>F, severe pain followed
by weakness and sensory loss. Immune-
mediated complexes on nerves.
Spontaneous recovery in 6-24 months.
8. Musculocutaneous nerve and part of
median nerve
9. Retroperitoneal hemorrhage. Give Factor
VIII and drain.
10. The medial antebrachial cutaneous
nerve (a branch of the medial cord)
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
2. Radial neuropathy at the spiral groove
3. Radial neuropathy at the axilla
4. Anterior interosseus neuropathy
5. Long thoracic neuropathy
6. Musculocutaneous neuropathy - clinical
7. What is a Martin-Gruber anastomosis?
8. What nerve supplies the first dorsal
interosseus of the hand (FDIH)?
9. What nerve supplies the teres major?
10. What nerve supplies the supinator?
11. Weakness of deltoid and teres minor
and numbness over the shoulder is
most likely due to
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 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.
3. Same as above, plus triceps weakness
4. Weakness of thumb and finger flexion
(digits 2-3) and pronation. No numbness.
Cant make OK sign
5. Scapular winging, inability to abduct
shoulder > 90.
6. Weakness of elbow flexion and
supination (biceps) and numbness in lateral
forearm (the lateral antebrachial cutaneous
nerve).
7. The median nerve sends a branch
across to the ulnar in the forearm and
supplies the ulnar-innervated hand
muscles.
8. Ulnar (unless theres an anastomosis as
in #7!)
9. Subscapular, from the upper trunk
10. Radial
11. Axillary neuropathy
 12. Suprascapular neuropathy
13. Action of the supraspinatus
14. What muscles are supplied by the median
nerve?
15. If a patient has posterior interosseus
mononeuropathy, what happens when the wrist 15. Extensor carpi radialis is spared, but
is extended?
16. What is the classic clinical picture in a
posterior interosseus mononeuropathy?
17. What muscles supinate the arm?
18. What nerve adducts the thumb?
19. What nerve innervates the latissimus dorsi?
20. What nerve supplies the adductor pollicis, third
lumbrical, and palmar interosseous muscles?
14. 12. Weakness of supraspinatus and
infraspinatus. (NOTE: If the neuropathy
is at the glenohumeral notch, it will
affect the infraspinatus only.)
15.
16. 13. Abduction of the shoulder, first 15
degrees
17. 14. Abductor pollicis brevis, opponens
pollicis, lumbricals 1 and 2, and part of
the flexor pollicis brevis (which also has
some ulnar innervation).
extensor carpi ulnaris is weak. Thus, the
wrist will deviate radially when extended.
16. Finger drop without wrist drop
17. The supinator. When the arm is flexed and
pronated, the biceps is a stronger supinator.
18. Ulnar. Test this by having the patient hold a
piece of paper between the thumb and index
finger. As you try to pull the paper out, the
patient with the ulnar lesion will compensate by
flexing the thumb (Flexor pollicis longus is
median-innervated.)
18. 19. Thoracodorsal (branch of the
posterior cord)
19.
20. 20. Ulnar
MONONEUROPATHY Lower Extremities 11. What nerve can be entrapped under the
piriformis muscle?
1. The sciatic nerve supplies which distal
nerves?
2. What is the only muscle supplied by the
common peroneal branch above the fibular
head?
3. Femoral neuropathy - which muscles are
weak?
4. What are the branches of the common
peroneal and what muscles do they supply?
5. How can you tell a common peroneal
neuropathy apart from an L5 radiculopathy?
6. Tibialis posterior and soleus are innervated by
7. Meralgia Paresthetica
8. Describe the sural nerve
9. The semitendinosus, biceps femoris and other
hamstring muscles are innervated by the
10. Weight loss can lead to mononeuropathy of
which lower extremity nerve?
1. Peroneal, tibial, sural sensory
2. The short head of the biceps femoris
3. Weakness of hip flexion (iliopsoas), knee
extension (quadriceps) and hip internal
rotation (sartorius). NOTE: Hip adductors
are supplied by the obturator, so if it looks
like femoral but adduction is weak, think
lumbosacral radic (L2-4) or plexopathy.
4. a. Deep peroneal - tibialis anterior
(dorsiflexion)
b. Superficial peroneal - peroneus longus
and brevis. (ankle eversion)
5. L5 would have weakness of
aforementioned muscles, plus
a. Weakness of gluteus medius (hip
abduction, internal rotation, superior gluteal
nerve)
b. Weakness of tibialis posterior (foot
inversion, tibial nerve)
6. The tibial nerve
7. Lateral femoral cutaneous neuropathy,
paresthesias on lateral thigh, no weaknes.s.
Often seen in obesity and pregnancy
8. Pure sensory to lateral foot, cell bodies
in DRG outside of S1 nerve root.
9. Sciatic nerve
10. The peroneal. Tell the patient to stop
sitting with legs crossed.
10. 11. The sciatic
MONONEUROPATHY lower extremity
continued

12. Muscles supplied by the superior gluteal

nerve.
12. Gluteus medius and tensor fasciae latae.
13. Which branch of the sciatic (peroneal,
tibial, sural) is most susceptible to
injury? 13. Peroneal

14. What are the branches of the tibial nerve

after the tarsal tunnel?
15. Tarsal tunnel syndrome clinical
features
14. Calcaneal, medial plantar, lateral
plantar.
15. Pain around the medial malleolus, sole
paresthesias, worse at night and with weight
bearing.
 MONONEUROPATHY Misc.
12. What nerves supply:
a. The external anal sphincter
b. The jnternal anal sphincter
c. Sensation from the scrotum or labia
13. What is the clinical difference between a
Horners syndrome from an internal carotid
artery dissection and a Horners syndrome from
a lesion in the proximal sympathetic pathway.
14. Treatment of myokymia
12.
a. Pudendal nerve (maintains fecal
continence)
b. Sympathetic fibers in hypogastric plexus
(maintains reflex continence)
c. Perineal nerve
13. Both cause ptosis and miosis. Only the
proximal lesion causes anhidrosis,
because the fibers destined for the sweat
glands are carried on the external carotid
artery.
14. Carbamazepine or phenytoin
POLYNEUROPATHY
1. Heavy metals that can cause neuropathy
2. Toxin that can produce a clinical picture
identical to B12 deficiency
3. Neuropathy and leprosy
4. Hereditary Neuropathy with Liability to
Pressure Palsies (HNLPP) - clinical
5. HNLPP - pathology
6. HNLPP nerve conduction study findings
7. HNLPP genetics
8. CMT1A genetics
9. CMT1B genetics
10. Lead neuropathy
11. Diabetic neuropathy treatment some
random thoughts that seem to come up on the
test repeatedly.
12. Organophosphate toxicity
1. Arsenic, lead, mercury, thallium. (Not
aluminum)
2. Nitrous oxide
3. Tuberculous leprosy (good immune
system, well contained, few organisms),
lepromatous leprosy (poor immune system,
diffuse infiltration of skin.) Both cause
nerve involvement. Includes ears and nose.
4. Episodic entrapment neuropathies (i.e.
wrist drop, foot drop)
5. Tomaculae redundant folds of abnormal
myelin seen on teased fiber preparation.
6. Mild diffuse slowing, conduction block at
entrapment site.
7. AD PMP22 deletion on chrom 17p
8. AD PMP22 duplication on chrom 17p
9. Point mutation in myelin protein 0 (Po)
on chromosome 1
10. Motor > sensory, predilection for the
radial nerve
11. They mention that amitriptyline is
effective, SSRIs are not. Narcotics not first
line. Mexiletine acts locally. Capsaicin
releases substance P, which causes burning
as a side effect.
12. Symptoms of cholinergic excess
acutely. After a few weeks, patients can
develop a motor predominant neuropathy
called OPIDN (organophosphate-induced
delayed neurotoxicity.)
POLYNEUROPATHY, continued
13. Miller Fisher syndrome clinical
14. Miller Fisher syndrome antibody
15. Guillain-Barre syndrome - clinical
16. Guillain-Barre syndrome Lab
17. Which leukodystrophies are associated with
neuropathy?
18. Multifocal motor neuropathy vs. ALS on
EMG. How are they similar? Different?
19. Alcoholic neuropathy
20. Fabrys disease
21. Acrylamide neuroapthy
22. Midodrine
23. Hypoproliferative anemia in autonomic failure
13. Ataxia, ophthalmoplegia, areflexia
14. Anti-GQ1b Ab
15. Rapidly ascending weakness and
areflexia. Autonomic dysfunction common.
True sensory loss is rare. Spirometry is
the best way to assess for resp
dysfunction. Rx: IVIG or plasma exchange
within 2-4 weeks
16. Cell count normal, or at least <50.
Elevated protein. Spinal MRI, nerve
biopsy, electrolytes are often normal
17. Adrenoleukodystrophy, metachromatic
leukodystrophy, Krabbes disease
18. Both have fibrillations, fasciculations,
normal sensory amplitudes, neurogenic
MUAPs. Only MMN has focal conduction
blocks in motor nerves, usually not at
entrapment sites.
19. Axonal. Often small fiber neuropathy.
20. Painful small fiber neuropathy, often
with autonomic dysfunction. Enzyme
replacement is available.
21. Affects dorsal columns and
spinocerebellar tract.
22. Stimulates -adrenergic receptors.
Treatment for orthostatic hypotension.
Does not cause fluid retention.
23. Treatment with erythropoietin can
improve orthostatic tolerance.
POLYNEUROPATHY, continued
25. Polyarteritis nodosa: symptoms, biopsy
26. Diphtheria - clinical
27. Copper deficiency - clinical
28. Friedreichs ataxia - clinical
29. Monoclonal gammopathy (MGUS)
neuropathy nerve conduction study features.
30. Porphyria induced neuropathy - clinical
31. Triggers of porphyria attacks
32. The preganglionic cell bodies for the
sympathetic system are where?
33. How does gabapentin exert its anti-nociceptive
effects?
34. What are the most efficacious treatments for
CIDP?
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.
26. Pharyngeal exudate, palatal neuropathy,
paralysis of papillary accommodation. 10%
get a GBS-like neuropathy 8-12 weeks after
infection.
27. Subacute corticospinal tract and
dorsal column dysfunction. Pancytopenia.
Consider in patients with gastric surgery
or zinc overdose (i.e. denture cream
abuse.)
28. Weakness, ataxia, hyporeflexia,
extensor plantar response. Romberg
positive. Frequently will have high arches,
hammertoes, and scoliosis.
29. Conduction slowing and other
demyelinating features are common.
30. Polyneuropathy, polyradiculopathy,
dysautonomia, ileus, presence of CNS
symptoms.
31. Many drugs. In RITE, there is a case of
sulfonamides inducing an attack.
32. The intermediolateral column of the
spinal cord. These get input from the
posteriolateral hypothalamus.
33. Modulation of pre-synaptic alpha-4-delta
calcium channels.
34. IVIg, plasmapheresis, and steroids
POLYNEUROPATHY, continued
35. Similarities and differences between
CIDP and POEMS
36. Sensory neuronopathy - causes
35. Both have subacute, non-length
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.
36. Sjogrens syndrome, paraneoplastic
syndrome (anti-Hu), pyridoxine
intoxication, variant of AIDP, platinum-
based chemotherapies.
 NEUROMUSCULAR JUNCTION

1. Lambert Eaton Myasthenic Syndrome 1. Proximal weakness, autonomic

(LEMS) clinical. dysfunction (dry mouth, impotence).
60% associated with small cell lung Ca

2. LEMS antibody and mechanism 2. P/Q Calcium antibody, blocks Ca uptake

into presynaptic terminal and inhibits
ACh release.

3. LEMS - EMG 3. Increase in CMAP amplitude after brief

exercise or high frequency repetitive
stimulation.

4. LEMS - treatment 4. 3,4-diaminopyridine (3,4 DAP) blocks

K+ channel in presynaptic membrane.
Pyridostigmine can also be used.

5. Infantile botulism - clinical 5. Hypotonia, poor suck, constipation,

areflexia, ophthalmoplegia, impaired
pupillary reflex. Descending paralysis

6. Infantile botulism - diagnosis 6. Repetitive stimulation at high frequency

(50Hz) shows an increment in the
CMAP (similar to LEMS). Toxin can
also be found in stool.

7. Botulism mechanism 7. Clostridium botulinum toxin causes

inhibition of release of ACh vesicles
from presynaptic terminal.

8. Congenital myasthenic syndromes - 8. Not autoimmune. Defects can be ACh

mechanisms synthesis and packaging, end-plate
deficiency of acetylcholinesterase, ACh
receptor deficiency, or slow channel
syndrome.

9. Antibodies for myasthenia gravis (MG) 9. ACh receptor antibody, MUSK antibody

10. Mechanism of pyridostigmine in MG 10. Acetylcholinesterase inhibitor

symptomatic only

11. Time course of immunosuppression and 11. Azathioprine, prednisone,

thymectomy cyclosporine take weeks to months.
Thymectomy may take years.
NEUROMUCULAR JUNCTION cont
12. Some drugs that worsen MG.
13. Infantile myasthenia gravis
14. What happens to patients with ocular
myasthenia when an ice pack is applied to their
face?
15. What is the most common clinical picture in
myasthenia gravis associated with anti-MUSK
antibodies.
16. Contaminated black tar heroin users are
predisposed to what neuromuscular junction
disease?
17. Tick paralysis clinical
18. Tick paralysis pathophysiology
21. Most sensitive test for myasthenia gravis
22. What percent of patients with thymomas
and MG have Striational Ab?
a. Aminoglycosides, beta blockers,
nimodipine, ciprofloxacin other
antibiotics, neuromuscular blockade
agents
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. Ptosis and/or ophthalmoplegia improves
15. Prominent facial, bulbar, and neck
weakness. A subset has relative sparing of
ocular muscles.
16. Wound botulism
17. Painless, progressive weakness in a child
in the spring or summer.
18. Blockade of Ach release and sodium
channels. Normal CSF protein, normal nerve
conduction studies.
19. Single fiber EMG
20. 74%
 MYOPATHY
1. AZT myopathy clinical and
mechanism
2. Myotonic Dystrophy (DM1) clinical
3. Congenital myotonic dystrophy
4. Myotonic Dystrophy (DM1) - genetics
5. Hypokalemic periodic paralysis - clinical
6. Hypokalemic periodic paralysis - genetics
7. Hypokalemic periodic paralysis - treatment
8. Pompes disease what is it?
9. Pompes disease clinical
10. Pompes disease EMG
11. Duchenne and Becker MD - genetics
1. Myalgias, weakness 6-11 months after
initiation. Reverse transcriptase inhibition
leads to depletion of mitochondrial DNA.
Ragged red fibers will be seen on path.
2. Distal > proximal weakness. Myotonia
(difficulty relaxing muscles), associated
with frontal baldness, mental retardation,
temporal wasting, cataracts, testicular
atrophy without sterility.
3. Hypotonia, facial diplegia, weakness, no
myotonia. Maternal inheritance, so examine
mom. CK and EMG may be normal.
4. AD CTG trinucleotide repeat on 19q.
Anticipation. Sequestration of Muscleblind-
like 1 causes abnormal splicing of CLCN1.
5. Episodes of weakness following rest after
exercise, especially after high carbohydrate
meal, emotional stress or cold. Attacks last
hours, spare respiratory muscles.
6. AD, Ca channelopathy
7. Prevent with acetazolamide, K+
supplementation during attacks.
8. Lysosomal glycogen storage disease
caused by acid maltase (alpha-galactosidase
deficiency)
9. Hypotonia, failure to thrive,
cardiomegaly, respiratory failure. Large
tongue. Liver and glucose homeostasis are
relatively normal.
10. May show electrical myotonia without
clinical myotonia
11. X-linked recessive due to absent
dystrophin (Duchenne) and abnormal size
dystrophin (Becker). 1/3 are new
mutations.
MYOPATHY, continued

12. What is dystrophin? 12. A membrane protein along the

intracellular surface of the sarcolemma. It
interacts with actin and may contribute to
the stability of the plasma membrane.

13. Dystrophinopathies clinical and lab 13. Weakness, calf pseudohypertrophy,

cardiac conduction defects, behavioral
changes. High CK, dystrophin gene test.

14. Duchenne MD - treatment 14. Prednisone (walk 2-3 years longer)

15. Oculopharyngeal Muscular Dystrophy 15. Both cause ptosis, ophthalmoplegia,

(OPMD) vs. Kearns-Sayre Syndrome (KSS). dysphagia, proximal weakness. Only KSS
How are they clinically similar? How are they causes hearing loss, ataxia, and cardiac
different? conduction defects.

16. Myotonia congenita. Clinical and EMG 16. Congenital muscle hypertrophy,
stiffness after rest, Cl- channel defect,
myotonia on EMG.

17. Nemaline myopathy 17. Proximal weakness and cardiomyopathy.

Can cause facial and extraocular muscle
weakness. No cognitive problems. No
myotonia on EMG.

18. Central core myopathy 18. Defect in ryanodine receptor,

associated with malignant hyperthermia.
Most benign of the congenital myopathies.

19. Myofibrillar myopathy 19. Desmin-immunopositive inclusions and

cardiac involvement. Can cause facial and
extraocular muscle weakness.

20. Steroid myopathy path 20. Type II fiber atrophy

21. Statin myopathy findings and mechanism 21. Subacute, CK. Attributed to inhibition
of mevalonic acid, a precursor to CoQ10.

22. Dermatomyositis clinical 22. Proximal weakness, erythematous

rash on the dorsum of the hands and
elbows, knees, neck and face.
MYOPATHY, continued
23. Polymyositis or dermatomyositis - EMG 23. Small, polyphasic motor units
(myopathic changes) and fibrillations (seen
in any myopathy with muscle fiber necrosis)

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.

25. Isaac Syndrome (neuromyotonia) clinical 25. Progressive muscle stiffness,

vibrating/twitching muscles, delayed
relaxation, even during sleep and
anesthesia. Paresthesias and
dysautonomia.

26. Isaac Syndrome associated antibody 26. Voltage gated K+ antibody seen in
about 40%. Can be autoimmune or
paraneoplastic

27. Isaac Syndrome EMG 27. Neuromytonic discharges, myokymic

discharges (doublets and triplets)

28. Isaac Syndrome treatment 28. No known treatment. Phenytoin and

carbamazepine may relieve stiffness. Plasma
exchange may provide short-term relief

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.

31. Fascioscapulohumeral dystrophy (FSHD) 31. Asymmetric weakness, especially of

clinical and genetics. face and shoulder girdle. AD chrom 4q

32. Mitochondropathies such as cytochrome oxidase 32. Muscle and nerve

type 1 deficiency affect what part of the nervous
system.
33. Na channel blockers such as
33. Treatment of myotonia in myotonic mexiletine, phenytoin, or carbamazepine.
dystrophy and myotonia congenita.
34. Removal of offending agent,
34. Treatment of malignant hyperthermia hyperventilation, and dantrolene.
MYOPATHY, continued
35. Merosin deficient muscular dystrophy
(Merosin is the same thing as lamin 2)
36. According to the AAN practice parameter,
what drug has a class II evidence for treating
muscle cramps?
37. What organs are affected in different
glycogen storage diseases?
38. Critical illness myopathy risk factors
39. Critical illness myopathy how do you
distinguish it from critical illness neuropathy or
NMJ disease?
40. Nemaline myopathy
Biopsy
CK
EMG
Most common genetic mutation
41. CoQ 10 deficiency primary
42. CoQ10 deficiency - secondary
43. Paramyotonia congenita - clinical
44. Increased internalized nuclei are a prominent
feature of which myopathy?
45. Hydroxychloroquine myopathy path
46. Drugs that increase risk of statin-induced
myopathy.
35. Most common congenital dystrophy
Moderately high CK
Proximal weakness and contractures
Cerebral white matter changes, often
asymptomatic
36. Diltiazem
37. Myophosphorylase def muscle only
Pompes muscle and liver
38. Critically ill patient treated with steroids
39. Preservation of deep tendon reflexes,
normal nerve conduction studies, normal
repetitive stimulation.
40.
Rod-like inclusions on Gomori trichrome
Normal or mildly elevated
May be normal
Alpha-actin gene (ACTA1)
41. Can cause myopathy or cerebellar
degeneration.
42. Statin myopathy.
43. Stiffness worse with activity and cold,
no weakness.
44. Myotonic dystrophy. DM1 has more ring
fibers and targetoid fibers than DM2.
45. Vacuolar changes and ultrastructural
curvilinear bodies.
46. Drugs that interact with CYP3A4,
especially Ca-channel blockers.
NERVE AND MUSCLE PHYSIOLOGY

1. Which myelinates first CNS or PNS? Motor 1. PNS before CNS

roots or sensory roots? Motor roots before sensory roots.

2. What causes depolarization of a resting neuron? 2. Increased sodium permeability

3. What limits the duration of an action potential? 3. K+ influx

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.

6. Saxitoxin (paralytic shellfish) and tetrodotoxin 6. Reversibly bind to membrane voltage-

(puffer fish) mechanism gated sodium channels. Can cause severe
neuropathy and respiratory failure.

7. What do fusimotor fibers do? 7. Transmit impulses to muscle spindles.

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).

16. Painful paresthesias and myalgias after 9. Ciguatera poisoning

ingestion of large reef fish.

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.

2. What are the neurologic manifestations of 2. Peripheral neuropathy, psychosis,

disulfiram toxicity? choreathetosis, parkinsonism, catatonia
 EMG / NERVE CONDUCTION
STUDIES
1. What is conduction block and what conditions
are associated with it?
2. Where are the cell bodies of sensory nerves
and how does this affect nerve conduction
studies?
3. What are fasciculations and when are they
seen?
4. What is the first abnormality to be seen on
needle EMG after an acute injury to a nerve?
5. What does the compound motor action potential
(CMAP) correlate with?
6. What is the most sensitive study to diagnose
carpal tunnel syndrome?
7. What is the affect of cold skin on nerve
conduction studies?
8. What is the normal conduction velocity of an
upper extremity motor nerve in a 6-month-old?
9. Describe the F-response
1. A large drop (usually >50%) between
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. In the dorsal root ganglion (DRG). Any
lesion distal to the DRG (i.e. plexopathy,
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. Irritation of the alpha motor neuron. Seen
in ALS (as a later finding), radiculopathy,
cramp-fasciculation syndrome , or in
normal muscle.
4. Decreased recruitment in affected muscle
(immediately.) Fibrillations begin after 7-10
days.
5. The number of functional axons in the
nerve.
6. Median palmar sensory studies show
prolonged latency.
7. It artificially prolongs the latency and
increases the amplitude.
8. 20-36 ms/s
9. A supramaximal stimulus is given to a
motor nerve. It is motor nerve anterior
horn cells motor nerve without a synapse.
EMG/NERVE CONDUCTION STUDIES cont
10. Describe the H-reflex
11. CMAP facilitation >100% after exercise?
12. Decremental response to 2-3 Hz?
13. What are the limbs of the blink reflex?
14. What would you see on a blink reflex in a
patient with a CN VII palsy on the left?
15. What are the blink reflex findings in a left
trigeminal lesion?
16. What causes myotonic potentials?
17. What causes complex repetitive discharges
(CRDs)?
18. In what conditions are CRDs seen?
19. What would you see on EMG in a patient
who had had polio as a child?
20. Abnormal jitter and blocking on single fiber
EMG?
21. What is seen on EMG in an upper motor neuron
process?
22. What are the nerve conduction findings in
CIDP?
10. A submaximal stimulus is given,
exciting 1a afferent nerves. Monosynaptic
from sensory nerve motor neuron. It is
the equivalent of the ankle jerk reflex.
11. LEMS. Note that you will also see
facilitation after rapid repetitive stimulation.
12. Classically, this is myasthenia gravis.
However, this can also be seen in LEMS,
botulism, or even severe denervation.
13. Afferent is carried by the CN V ipsi to
the stimulus. Efferent is CN VII bilaterally.
14. Stim Left absent R1 and R2
Stim Right absent R2
15. Stim Left prolonged ipsi R1, bil R2
Stim Right normal
16. Repetitive discharge of the muscle
membrane.
17. Ephaptic transmission of impulses
from muscle fiber to muscle fiber. Can be
neurogenic or myopathic.
18. Same as fibrillations. Chronic
neuropathic process and some myopathies
(especially inflammatory myopathies.)
19. A decreased number of very large motor
units firing rapidly.
20. Seen in myasthenia gravis, but not
specific. Would not be seen in metabolic or
steroid-induced myopathies.
21. Decreased activation, or a decreased
number of normal motor units, firing slowly.
22. First: Decreased recruitment and
prolonged F-waves. Later: Slow motor
conduction velocities and conduction block.
EMG/NERVE CONDUCTION STUDIES cont

23. EMG/NCS findings in Guillain-Barr 23. Prolonged or absent F-responses, slowed

conduction velocities, prolonged latencies,
conduction block and temporal dispersion.

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

25. Fasciculations without weakness or other 25. Benign fasciculation syndrome. Rx =

clinical symptoms is diagnostic of withdraw caffeine and treat anxiety.

26. What is the effect of each of these on nerve 26.

conduction velocities and amplitudes
a. Low temperature a. Larger amplitude, slower CV
b. Increased patient age b. Smaller amplitude, slower CV
c. Arms (compared to legs) c. Faster CV
d. Myelinated fibers d. Faster CV

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.)

32. EMG changes in myopathy 32. Small polyphasic motor units

EMG/NERVE CONDUCTION STUDIES cont

33. Nerve conduction studies in small fiber 33. Normal

neuropathy.

34. What causes increased fiber density on single-
fiber EMG?
34. Denervation and reinnervation. The most
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.

35. What are normal upper extremity conduction 35.

velocities in
An adult >40 m/s
A six month old child 20-36 m/s
 AUTONOMIC NEUROPATHY AND
TESTING
1. QSART
2. Valsalva maneuver
3. Innervation of muscles involved in
urination.
4. Autoimmune autonomic ganglionopathy.
Presentation, serum test, treatment.
5. Neurotransmitter of postganglionic
fibers to:
a) sweat glands
b) all other sympathetic synapses
1. Correlates with postganglionic
sympathetic sudomotor fibers. Abnormal
in small fiber neuropathy.
2. Evaluates the baroreflex arc and its
sympathetic and parasympathetic responses.
The mean blood pressure can increase by
more than 10 mm Hg. If it does, there is
dysfunction of the sympathetic response
.
6. 3. Detrussor muscle parasympathetic
(S2-S4)
7. Internal urethral sphincter
sympathetic
8. External sphincter somatic fibers
from pudendal nerve
9.
10. 4. Isolated dysautonomia
Ganglionic acetylcholine receptor antibodies
Treat with IVIG or prednisone
5. a) Acetylcholine, b) Norepinephrine
 PATHOLOGY

11. Muscle biopsy in Duchenne muscular

dystrophy 1. Excessive fibrosis, proliferation of
connective tissue, variation in fiber size.
Absent dystrophin immunostaining but not
dystroglycan.

2. Necrotizing vasculitis with fibrinoid necrosis 2. Polyarteritis nodosa, Wegeners. NOT

in vessel walls polymyalgia rheumatica, which will have
a normal biopsy (or show Type II
atrophy)

3. Findings in denervated muscle 3. Target fibers (reinnervation), angulated

fibers, fiber type grouping, group atrophy.

4. Muscle biopsy in Pompes disease 4. Vacuolar myopathy from glycogen

deposition.

5. Muscle biopsy in centronuclear myopathy 5. Centrally located nuclei with

perinuclear halo, type I predominance.

6. Muscle biopsy changes in mitochondrial 6. a. Ragged red fibers

myopathies (i.e. Kearns-Sayre, MELAS, b. Deficient cytochrome C oxidase.
MERRF, etc.) c. subsarcolemmal staining on SDH

7. Muscle biopsy changes in dermatomyositis 7. Peri-fascicular atrophy

8. Verocay bodies 8. Peripheral nerve schwannoma

9. What are the end organs for sensing vibration and 9. Pacinian corpuscles, in the subcutaneous
where are they? connective tissues and periosteum.

10. Amyloid neuropathy 10. Amyloid deposition on congo red stain.

Apple green when viewed with polarized
light. Loss of small myelinated axons with
sparing of large myelinated axons.

11. Thickened blood vessels with basement 11. Diabetic neuropathy

membrane duplication

12. Lymphoid infiltrates and abnormal 12. Polymyositis

expression of MHC
OTHER PICTURES TO LOOK FOR

1. A teased nerve fiber with round

swellings of myelin. Tomaculous
neuropathy. Seen in HNLPP.

2. L5-S1 disc herniation distinguish

from meningioma, metastatic disease,
neurofibroma, lymphoma. Of all of those
lesions, only herniated disc arises within
the disc space and extends posteriorly.

3. Syringomyelia patients have muscle

atrophy, decreased pin/temp with sparing
of crude touch, vibration and position
sense.

4. A picture of two CMAPs, with one

having a later onset, a lower amplitude,
and a spread-out waveform. This is
conduction block. Think CIDP or
multifocal motor neuropathy, but not
CMT.

5. A picture of a nerve biopsy with onion

bulbs. Suggests demyelination and
remyelination, like CMT 1A.

6. A muscle biopsy with large groups of

large muscle fibers and large groups of
small, atrophic fibers. This is called
large group atrophy, and generally
represents severe denervation, like ALS
or spinal muscular atrophy.

7. A muscle biopsy showing rimmed

vacuoles. Most suggestive of inclusion
body myositis.