AH Cell Disease

Question 1

Diseases that cause degeneration of the motor neurons, affect which cells in the spinal cord?

Answer 1

Anterior horn cells

Question 2

Anterior Horn Cell Diseases often just have what symptom alone?

Answer 2

Weakness of the affected muscles

Question 3

What is the most common Motor Neuron Disease?

Answer 3

ALS (amyotrophic lateral sclerosis)

Question 4

Besides ALS, what are some other Motor Neuron Diseases?

Answer 4

• Progressive Bulbar Palsy
• Spinal Muscular Atrophy
• Primary Lateral Sclerosis

Question 5

What motor neurons are affected with ALS?

Answer 5

BOTH UMN and LMN!

Question 6

What are the symptoms of ALS?

Answer 6

UMN: spasticity, babinski sign, hyperreflexia
LMN: atrophy, weakness, fasciculations

Question 7

What are the symptoms of ALS?

Answer 7

UMN: spasticity, babinski sign, hyperreflexia
LMN: atrophy, fasciculations, weakness

Question 8

With ALS, what specifically degenerates (4)?

Answer 8

Betz cells
Lower brainstem nuclei
Descending cortical spinal tracts
Anterior horn cells

Question 9

With ALS, what specifically degenerates? (4)

Answer 9

Betz cells
Corticospinal tracts
Anterior horn cells
Lower brainstem nuclei

Question 10

What can be the first sign of ALS?

Answer 10

Hand clumsiness/weakness

Question 11

What will be seen on the EMG with ALS?

Answer 11

Denervation and re-innervation

Question 12

Are there sensory symptoms or mental status changes with ALS?

Answer 12

NO

Question 13

How do ALS patients usually die?

Answer 13

Respiratory failure

Question 14

What does Progressive Bulbar Palsy affect?

Answer 14

Lower cranial nerve motor nuclei – 9, 10, 11, 12

Question 15

What are the signs of Progressive Bulbar Palsy?

Answer 15

Dysarthria, Dysphagia, chewing difficulty, drooling, etc.

Question 16

Does Progressive Bulbar Palsy usually progress?

Answer 16

Yes usually to ALS

Question 17

The prognosis is ____ with Progressive Bulbar Palsy

Answer 17

WORSE

Question 18

What is affected with Spinal Muscular Atrophy?

Answer 18

LMN

Question 19

LMN only

Answer 19

Spinal Muscular Atrophy

Question 20

What is affected with Primary Lateral Sclerosis?

Answer 20

UMN

Question 21

UMN only

Answer 21

Primary Lateral Sclerosis

Question 22

Is there pain with ALS?

Answer 22

No, no sensory changes either

Question 23

What 2 main things are affected with ALS?

Answer 23

Anterior horn cells

Corticospinal tracts

Question 24

What is another condition that causes degeneration of UMN and LMN besides ALS?

Answer 24

B12 deficiency = Subacute Combined Degeneration

Question 25

With B12 deficiency, what part of the spinal cord is affected and how does that manifest?

Answer 25

Posterior columns –> abnormal Romberg test