T3 Soriano Objectives

Front 1

Neuromuscular Junction Disorder Symptoms

Back 1

-Purely motor, esp. proximal, w/ fatigue.
-Often abnl eye movement & ptosis (variable diplopia & ptosis)
-Bulbar musculature-dysphagia, dysarthria--Neuro emergency!
-Muscle tone-normal or reduced
-Reflexes-nl or reduced
-Sensory-nl

Front 2

What are the tests for Neuromuscular Junction Disorder?

Back 2

-Special maneuver--prolonged (2 minute) upgaze or arm elevation
-Nerve conductions: usually nl
-Repetitive nerve conductions: decremental response.
-Needle EMG examination: nl

Front 3

What are three examples of NMJ disorders?

Back 3

Myasthenia Gravis, Botulism, Myasthenic Syndrome

Front 4

What are the two types of myopathy?

Back 4

Inherited--Muscle Dystrophy,
Acquired --Polymyositis, Toxic Myopathy.

Front 5

Describe Duchenne Muscular Dystrophy

Back 5

-Mutation in gene for Dystrophin protein.
-Amt of dystrophin determines severity
-X-linked recessive Xp21.
-Gower's sign
-30% w/ new mutation,
-Proximal > Distal wkness of arms & legs, symmetric.
-Onset 3-5 yrs-wkness
-Unable to walk by 13 y/o
-Calf hypertrophy, contractures, scoliosis
-Cardiomyopathy--cause of death.
-Mental retardation
-CRK-very high.

Front 6

How do you diagnose & treat Duchenne Muscular Dystrophy?

Back 6

-Diagnose: Muscule biopsy, DNA testing.
-Steroids: may prolong walking 2 yrs.

Front 7

Describe the clinical presentation of Myasthenia Gravis.

Back 7

Presents in 2 ways:
1. Ocular MG: ptosis, extraocular muscles (purely ocular in 20%,most start w/ this & generalize)
2. Generalized: bulbar, extremities (weakness, swallowing problems)
-Fatigue w/ repeated activity
-Proximal>distal wkness
-Dysphagia-->Myasthenic crisis--Neuro emergency!!

Front 8

Describe the pathophysiology of Myasthenia Gravis.

Back 8

Classic autoimmune d/o.
-Ab's to Ach receptor (Generalized--90% w/ Ab's, Ocular: 50-70% w/ Ab's)
-May be assoc w/ Thymus tumor

Front 9

What are the ways to diagnose MG?

Back 9

-Tensilon Test (short acting cholinesteric inhibitor, rarely causes heart block but use EKG)
-Repetitive nerve stimulation-->decremental response

Front 10

What is the treatment for MG?

Back 10

-Myasthenic crisis--plasma exchange, IV immunoglobulin.
-Cholinesterase inhibitors--Pyridostigmine (Mestinon)
-Immunosuppressant agents: attacks root cause of MG.
-Ocular MG--? need immunosuppreants (if only ocular, don't use)
-Thymectomy-may improve control, needed for thymus tumor.

Front 11

Describe Myasthenic Syndrome (Lambert-Eaton Syndrome)

Back 11

-Legs weaker than arms;
-Fatigability, improves w/ brief exercise.
-Occasional ptosis.
-Autonomic dysfxn--dry mouth & eyes
-Reflexes absent, appears w/ exercise.
-Ab to voltage gated Ca channel
-Exercise increases intracellular Ca.
-Presynaptic disorder (absent reflexes, M. syndrome before synapse, and MG after synapse)
-May be assoc w/ neoplasm, precedes CA >80%, esp w/ small cell lung CA, higher chance of resp failure.
-Tx: immunosuppression, treat CA.

Front 12

Describe Sensory neuronopathy & what conditions its associated with.

Back 12

-Dorsal root ganglia neurons are affected.
-Prominent sensory loss: very poor position sense, areflexia.
-Viral: Sjogren's syndrome.
-Paraneoplastic-anti-Hu antibody, assoc w/ small cell lung CA.

Front 13

In which 4 conditions would you give IV Immunoglobulin and how does it work?

Back 13

Acutely ill patient w/ Guillain-Barre syndrome, Myasthenia Gravis, Chronic Demyelinating Polyneuropathy, Dermatomyositis.
-Pooled, purified, sterilized Ab's, work by blocking antibodies, reducing complement.
-Easily given, widely available, rare side effects, short onset of action.

Front 14

Describe Polyneuropathy.

Back 14

-Symmetric, length dependent, worse in legs than arms, worse distally;
-Sensory, motor, autonomic.
-Nerve pain;lancinating, burning, paresthesias.
-Allodynia: neutral stimuli are painful (ex. bed sheets hurt).
-Asymmetric: mononeuritic multiplex.

Front 15

Describe Polymyositis.

Back 15

Endoymsial inflammation (around muscle FIBER). Its a type of inflammatory myopathy: symmetric, proximal>distal wkness, dysphagia, posterior neck wkness (hold up head from behind), quads very weak, age: >20, fatigue, anorexia, muscle pain. Slightly inc risk of malignancy (lung, breast), Assoc w/ autoimune, Thyrotoxicosis, arhythmias, muscle bx important b/c justifies use of immunosuppressants, EMG w/ small MUPs w/ inc insertional activiity.

Front 16

What are the 5 signs of Acute cord Lesion?

Back 16

Ascending weakness, Hypotonia, Hyporeflexia or areflexia, Babinski sing not reliable, reduced rectal sphincter tone

Front 17

What does and Acute cord lesion resemble?

Back 17

An acute peripheral neuropathy - "spinal Shock"

Front 18

Define Myopathy? What is the effect on Motor system and what is the effect on sensory system?
Reflexes?

Back 18

Muscle pain with movement, palpation. Motor: Normal, hyper-, atrophy, normal or reduced muscle tone, proximal weakness> distal, Dysphagia possible, eye movemnt normal, fce normal.
Sensory: Normal
Reflexes: Normal or reduced

Front 19

What is the time course of a acute vs chronic myopathy?

Back 19

Acute (toxic, infectoins) or chronic (inflammatory, inherited)

Front 20

WHat do you see on an EMG and a Needle EMG for a myopathy?

Back 20

EMG- Normal Sensory nerves
- normal or reduced motor amplitude
- NEEDLE EMG: increased spontneous activity (indicates abnormality), Small MUP and increased recruitment (both indicate myopathy)

Front 21

What are the three types od Peripheral Neuropathy

Back 21

-Axonal or Demyelinatin
-Acute or chronic
-Critical care neuropathy and Myopathy

Front 22

What is Beckers MD

Back 22

Its a type of inherited myopathy
New mutations are rare
Onset >7 yrs, still walking past 13 (unlike Duchene MD)
Proximal> Distal
Calf hypertrophy, contracturee, scoliosis
Cardiomyopathy, mental retardation
CPK- very high

Front 23

What is Statin Induced Myopathy? aka Lipid Lowering Agents Myopathy

Back 23

-1 to 6/10,000
- Older females with renal or liver disease, DM, debilitated
- Starts after 2-3 mo of starting the drug
- Early- resting oain, worse with exertion
- Late- weakness, myoglobinuria
-CPK- normal to high
- Recovery- Days to months after stoppin (pts can restart the drug for short time then stop again)

Front 24

What is Botulism?
- WHat agent causes it
- What is the MOA of the Toxin
- What effects does it have?
- What is its Txt?

Back 24

It is a NMJ disorder
- prod. by Clost. botulinum (gram + bacilli, anaerobic, spores)
-7 types (A to G)- A & B (medical uses)
-Degrades a specific SNARE protein in the axon terminal- nNT vesicles cannot bind with cell membrane (cant release ACH)
-Presynaptic defect
- 18-38 hrs latency
-Weakness- diffuse, symmetric, proximal worse than distal
- Bulbar - Dysphagia and dysarthis
-Ptosis, extraocular muscle weakness
-Reflexes reduced
-Autonomic-dilated pupils , dec HR, dec BP
-Txt- supportive, antitoxin

Front 25

WHat is CIDP? Chronic Inflammatory Demylinating Polyneuropathy
What does the EMG show?
What is its treatment?

Back 25

-SLow progressive weakness> moths
-Proximal and distal weaknes
-Sensory Loss- not prominent
-Abscent reflexes
-EMG: SLowed conduction + blocks
-Treatment: Long term immunosuppressant agents

Front 26

What are two types of Acute inflammatory Polyneuropathy (AIP) and what is an example of the "classic demyelinating disease"

Back 26

- Can be either Axonal or demyelinating
- Guillian -Barre syndrome

Front 27

What are the 2 types of GBS?
Decribe each type.

Back 27

Two types are Demyelinating or Axonal

Demyelinating: distal paresthesias, weakness: progressive "ascending", Weakness more than sensory loss, Abscent reflexes early, CN VII- upper and lower face

AXONAL type- worse prognisi, slow recover, Campylobacter jejuni coinfecton, Quadriplegia in 30%, Mechanical ventilaton in 1/3, NEUROLOGIC EMERGENCY- watch resipiration function

Front 28

What do you see on Lab studies for GBS

Back 28

EMG: NL for 1st 7-10 days
-Nerve conduction study- demyelnating -slowed conduction
Axonal- Reduced amplitude
-Needle EMG- normal first 3 weeks
-CSF- inc protein, NL WBC
- If elevated CSF whit cell- ?HIV

Front 29

What would you get is the ulner N. is entrapped/Lesioned in the following locations: Axilla, Elbow, Forearm, wrist? What are the symptoms? How do you diagnose it? How do you treat it?

Back 29

-Axilla- cruches, usually with median n.
-Elbow- Cubital tunnel syndrome (2nd most common nerve compression site, leprosy)
-Forearm- trauma, hematoma,shunts
- Writst- Guyon's cannal (Hamate and pisiform)-0 distal to this- sensation spared
Symptoms- parathesis and numbness in ulnar n sensory distribustion
-DX- EMG examination
Tx: Cubital Tunnel- ant. transposition

Front 30

What is the sensory innervation of ulnar nerve?

Back 30

C8-T1 and covers the pinky and half of 4th finger on each side of the hand.

Front 31

Describe Carpel Tunnel Syndrome

Back 31

F>M, middle aged.
-Most common nerve entrapment syndrome.
-Intermittent palmar finger/hand paresthesias
-Wakes up pt @ night or on waking up
-Better after flicking wrist.
-Tinel's sign--tap wrist
-Phalen's test--forced wrist flexion x 30 sec
-EMG: slowed conductions across wrist
-Tx: splinting, surgery.

Front 32

What is the sensory innervation of median nerve (C6-T1)?

Back 32

Tips of fingers on dorsum (2-4), palmar surfaces of your hand (thumb thru 1/2 of fourth).

Front 33

What are the causes of foot drop?

Back 33

Damaged muscles: Tib Ant, Pero Long, TibPos, GasSol.
-If peroneal N cause-->TibAnt & PerLon involved.
-If Sciatic N-->All muscles involved.
-If L5, Tib Ant, PerLon, Tib Pos involved.

Front 34

What is the fxn of PerLon?

Back 34

Foot down & out

Front 35

What is the fxn of Gas Sol?

Back 35

Down like on Gas Peddle

Front 36

What is the fxn of Tib Ant?

Back 36

Picks foot up

Front 37

What is the fxn of Tib Posterior?

Back 37

Down and In.

Front 38

What are some causes of lumbosacral radiculopathy?

Back 38

-Immune-elevated sed rate.
-Diabetic amyotrophy (severe pain in anterior thigh, knee reflex decreases)--actually upper lumbar plexitis.
-Radiation to pelvis.
-Cancer or hemorrhage in pelvis.
-Lyme disease, vasculitis, heroin.

Front 39

Describe the symptoms and causes of Polymyelitis.

Back 39

Causes: -viral infection w/ an enterovirus, major illness in 10% infected & paralytic dz in 50% w/ major illness; Legs >arms >bulbar, may have acute dysautonomia. Post-polio syndrome after >15 yrs (suddenly develop worsening symptoms)

Front 40

What are the symptoms of radiculopathy?

Back 40

-Pain in N distribution, esp sharp shooting.
-wkness & absent reflexes in N distribution.
-Rarely w/ sensory abnormalities
-In legs, radiates past knees (+straight leg raising test)
-Dx: MRI:anatomical study, loss for compressive N lesions.
-EMG: physiologic study.
-Sensory: ALWAYS NL in radic;
-Needle EMG: best @ localizing.
-Paraspinal muscles affected.

Front 41

What is the tx for radiculopathy?

Back 41

Conservative management vs. surgery.

Front 42

What is Amyptrophic lateral Sclerosis (ALS) aka Lou gehrig's disease) and what is its Hallmark?

Back 42

-Weakness esp in distal extremisties
-Hallmarck- Combined upper and lowe motor neuron symptoms and signs
- Also get hyperreflexia and increased tone combined with atrophy and fasciculations
-often in same limb
- Cranial nerves involved- tongue fasciculations
- Paraspinal muscle involved- EMG
-Overall survival 3-4 yrs

Front 43

a) C4 is sensory nerve root for-
b) C6 is sensory nerve root for-
c) C7 is sensory nerve root for-
d) C8 is sensory nerve root for-
e) L1 is sensory nerve root for-
f)L2 is sensory nerve root for-
g)S2 is sensory nerve root for-

Back 43

C4- Cape
C6- rdial forearm + thumb
C7- Middle finger
C8- Ulnar forearm + thumb
L1- inguinal region
L2- Anterior thigh
S2- coccygeal- target around anus