Chapter 05: Emg - Motor Neuron Disease

Front 1

LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
Spinal Muscular Atrophy

Back 1

LOWER

*UPPER too (brainstem motor nuclei)

Front 2

LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
POLIOmyetlitis/PostPOLIO syndrome

Back 2

LOWER

Front 3

LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
AMYOTROPHIC LATERAL SCLEROSIS

Back 3

BOTH

Front 4

LOWER motor neuron lesion, UPPER motor neuron lesion, or BOTH?
Primary Lateral Sclerosis

Back 4

UPPER

Front 5

UPPER motor neuron sign or LOWER motor neuron sign?
HYPOreflexia

Back 5

LOWER

Front 6

UPPER motor neuron sign or LOWER motor neuron sign?
Spasticity

Back 6

UPPER

Front 7

UPPER motor neuron sign or LOWER motor neuron sign?
Flaccidity

Back 7

LOWER

Front 8

UPPER motor neuron sign or LOWER motor neuron sign?
UP-going toes

Back 8

UPPER
Positive Babinski

Front 9

UPPER motor neuron sign or LOWER motor neuron sign?
FASCICULATIONS

Back 9

LOWER

Front 10

How do you test for recruitment of the DIAPHRAGM?

Back 10

- MONOpolar needle
- Between 8th-9th INTERCOSTAL SPACE (anterior axillary line)
- Intercostals are recruited during EXPIRATION
- Diaphragm recruited during INSPIRATION

Front 11

FASCICULATIONS on EMG are indicative of which MOTOR NEURON DISEASES?

Back 11

ALS
- also, poliomyelitis or postpolio syndrome
- SMA in the TONGUE

Front 12

Which MOTOR NEURON DISEASE would you see CRD's in?

Back 12

SMA type THREE

- may also see fibs/PSW's

Also in ALS

Front 13

Pattern of inheritance for SMA type I?

Back 13

Autosomal RECESSIVE
(Acute Werdnig Hoffman)

Front 14

Pattern of inheritance for SMA type II?

Back 14

Autosomal RECESSIVE
(Chronic Werdnig Hoffman)

Front 15

Pattern of inheritance for SMA type III?

Back 15

Autosomal recessive or dominant
(Kugelberg Welander)

Front 16

Age of onset for SMA type I?
Disease progression?

Back 16

3-6 months
- death by 2-3 years old
(acute Werdnig Hoffman)

Front 17

Age of onset for SMA type II?
Disease progression?

Back 17

2-12 months
- death by 10 years old
- WC by 2-3 years old
(chronic Werdnig Hoffman)

Front 18

Age of onset for SMA type III?
Disease progression?

Back 18

2-15 years
- NORMAL life expectancy
- WC by age 30
(Kugelberg-Welander)

Front 19

Death from SMA type I & II is usually due to what?

Back 19

RESPIRATORY FAILURE
(acute & chronic Werdnig Hoffman)

Front 20

Tongue fasciculations should cause you to include what in your differential diagnosis?

Back 20

SMA

Front 21

Patients with SMA show what abnormality in blood work?

Back 21

Increased CPK

Front 22

What will you see on muscle Bx with SMA?

Back 22

Hypertrophic/atrophic fibers

Front 23

An infant that NEVER sits independently has HYPOtonia, is not reaching his/her milestones, FROG-LEGGED position, with tongue fasciculations. What does he/she have?

Back 23

SMA I (acute werdnig hoffman)
*facial muscles are affected the least

Front 24

An infant that CAN sit independently but has HYPOtonia, progressive weakness, KYPHOSCOLIOSIS, EQUINUS deformity of the feet, with tongue faciculations. What does he/she have?

Back 24

SMA II (chronic werdnig hoffman)
*watch for progressive PULMONARY involvement
*will likely need assistive devices for standing/walking

Front 25

What is the pattern of weakness seen in Kugelberg-Wellander disease?

Back 25

SMA II

- SYMMETRIC: lower then upper

Front 26

Which motor neuron disease may mimic DUCHENNE clinically?

Back 26

SMA III (Kugelberg Wellander)
- Gower's sign d/t lower limb weakness
- Calf pseudohypertrophy

*in contrast SMA III has good long-term survival -dependent mostly on respiratory function

Front 27

Typical onset of ALS?
Which sex is more prone to develop it?

Back 27

60's
MEN

Front 28

In terms of survival is it better to develop ALS at a YOUNGER age or an OLDER age?
- What other factors predict survival?

Back 28

YOUNGER
- SEVERITY of onset
- Pulmonary function

Front 29

What are the first signs of ALS typically?

Back 29

ASYMMETRIC weakness, atrophy, FASCICULATIONS

Front 30

3, 5, & 10 year mortality for ALS?

Back 30

3 years - 50%
5 years - 70%
10 years - 90%

Front 31

Sensation& what two muscle groups are typically spared in ALS?

Back 31

Extrocular muscles
Bowel/Bladder

Front 32

What electrodiagnostic signs will point to a diagnosis of ALS?

Back 32

SINGLE FIBER = increasd JITTER & fiber DENSITY
Low rate rep stim = increased DECREMENT
NCS = normal
EMG = membrane instability, LDLA MUAPs, CRDs (NEUROPATHIC)

Front 33

What agent slows disease PROGRESSION in ALS?

Back 33

Rilutek - riluzole antiglutamate

Front 34

What pathologic organism is responsible for Poliomyelitis?

Back 34

PICORNAVIRUS

Front 35

What are some PSEUDOBULBAR signs associated with ALS?

Back 35

- Difficulty chewing
- Dysphagia
- Difficulty speaking
- Emotional outbursts

Front 36

What percent of patients with Poliomyelitis have SEVERE disability, MILD disability, & COMPLETE RECOVERY?

Back 36

Severe = 25, Mild = 25%, Complete recovery = 50%

Front 37

What factors predict increased mortality due to poliomyelitis in ADULTS?

Back 37

10% mortality in adults with
- BULBAR signs
- RESPIRATORY involvement

Front 38

What electrodiagnostic signs will point to a diagnosis of POLIOMYELTIS?

Back 38

Normal SNAP (sensory is spared)
Normal or decreased CMAP
NEUROPATHIC EMG = LDLA MUAPs, membrane instability

Front 39

What is the pathologic difference between Poliomyelitis & Post Polio Syndrome?

Back 39

Polio = degeneration of anterior horn cell
Post Polio = death of anterior horn cell (burned out)

Front 40

The Halstead-Ross Criteria are used to diagnose which Motor Neuron disease? What are they?

Back 40

POST POLIO SYNDROME
1. Previous Dx of polio
2. Recovery of fxn
3. 15 year disease stability
4. Return of Sx (weakness, atrophy)
5. No other medical problems to explain

Front 41

What electrodiagnostic signs will point to a diagnosis of POLIOMYELTIS?

Back 41

SNAP = normal
CMAP = abnormal
EMG = GIANT MUAPs, membrane instability
SINGLE FIBER = increased jitter, density, & blocking (heavy collateral sprouting)

Front 42

GIANT MUAPs on EMG point to a diagnosis of which motor neuron disease?

Back 42

POST POLIO
- severe loss of anterior horn cells - remaining motor units become huge due to heavy collateral sprouting

Front 43

ALS or Post polio?
Low rate rep stim shows INCREASED decrement?

Back 43

ALS
*LRRS is normal in post polio

Front 44

Weakness + INTACT sensory + UPPER motor neuron signs = ?

Back 44

ALS

Front 45

Weakness + INTACT sensory + LOWER motor neuron signs + defect in MUSCLE + NO PAIN =

Back 45

Myopathy or inclusion body myositis

Front 46

Weakness + INTACT sensory + LOWER motor neuron signs + defect in MUSCLE + PAIN =

Back 46

Polymyositis/dermatomyositis

Front 47

Weakness + INTACT sensory + LOWER motor neuron signs + defect in ANTEROR HORN CELL =

Back 47

Poliomyelitis

Front 48

Weakness + INTACT sensory + LOWER motor neuron signs + defect in NMJ =

Back 48

MG or lambert eaton

Front 49

Weakness + ALTERED sensory + UPPER motor neuron signs =

Back 49

Tumor, syrinx, MS (cerebrum, brain stem, spinal cord)

Front 50

Weakness + ALTERED sensory + LOWER motor neuron signs =

Back 50

peripheral neuropathy, plexopathy, radiculopathy

Front 51

Critical illness polyneuropathy is primarily axonal or demyelinating?
Motor or sensory?

Back 51

AXONAL LOSS
Both motor and sensory PERIPHERAL POLYNEUROPATHY

Front 52

1 month old with weak cry, generalized hypotonia, symmetric weakness of lower>upper limbs, frog legged position when supine, diaphragmatic breathing (BELL-SHAPED torso), facial weakness, & tongue fasciculations

Back 52

SMA I

*preserved MSR's does NOT exclude the diagnosis, although they are usually absent

Front 53

What type of SMA has a characteristic BASELINE TREMOR on ekg?

Back 53

SMA II

*especially in limb leads

Front 54

Caused by abnormality in FRATAXIN (protein)

Back 54

Friedreich's ataxia

- both upper and lower motor neuron lesions

- sparing of motor neurons of spinal cord

Front 55

What is the liklihood that a patient with Friedreich's ataxia will develop scoliosis?

Back 55

almost 100%!!!

*usually before age 10

Front 56

Wheelchair reliance is imminent when what muscle group becomes less than 3/5 and time to ambulate 30 feet is greater than ___ seconds?

Back 56

KNEE EXTENSION

time to ambulate >12 seconds

Front 57

If surgical management of scoliosis is to be pursued it must occur prior to the patients FVC reaching what level?
Why?

Back 57

A forced vital capacity <35% of normal is a contraindication to scoliosis surgery d/t increased perioperative morbidity.

Front 58

In general what is the most effective treatment for progressive scoliosis?

Back 58

spinal ARTHRODESIS (fusion)

Front 59

When should you start using spinal orthotics to manage spinal deformity in Duchenne Muscular Dystrophy?

Back 59

NEVER -they are are ineffective and do not change the natural history of the curve