Anterior Horn Cell Disease (Hon)

Question 1

How are motor neuron diseases characterized?

Answer 1

progressive wasting (atrophy) and weakness of the affected muscles without sensory, cerebellar or mental changes

Question 2

What is a metabolic cause of motor neuron disease?

Answer 2

alpha glucosidase (acid maltase) deficiency

Question 3

What is the most common adult motor neuron disease

Answer 3

amyotrophic lateral sclerosis (ALS) 2/3rd of cases

Question 4

Clinical presentation of amyotrophic lateral sclerosis (ALS)?

Answer 4

mixed upper and lower motor neuron findings especially in the same limb; common after 50 yrs; first sign is often hand clumsiness eventually other hand/arm becomes involved and before long the legs become similarly affected

Question 5

amyotrophic lateral sclerosis (ALS) pathophysiology?

Answer 5

unknown etiology; degeneration of Betz cells in (motor nuclei), lower brainstem nuclei, descending corticospinal tracts and anterior horn cells

Question 6

Answer 6

atrophic hands, seen in amyotrophic lateral sclerosis (ALS)

Question 7

Answer 7

atrophic tongue, seen in amyotrophic lateral sclerosis (ALS)

Question 8

What is the typical finding on an EMG in a patient with amyotrophic lateral sclerosis (ALS)?

Answer 8

widespread denervation and re-innervation (listening to the muscle)

Question 9

What imaging should you order for.a patient you suspect has amyotrophic lateral sclerosis (ALS)?

Answer 9

imaging of the brain and cervical spine; looking for cervical compression (can present with mixed UMN and LMN symptoms

Question 10

What are the 6 important “rule of thumb” negatives for amyotrophic lateral sclerosis (ALS)?

Answer 10

1. no sensory symptoms
2. normal mentation
3. no extraocular muscle involvement
4. bowel or bladder symptoms not prominent
5. decubiti rare
6. fasciculations are rare

Question 11

What is the typical cause of death in amyotrophic lateral sclerosis (ALS)?

Answer 11

death from respiratory failure; death within 2 to 5 years

Question 12

Progressive Bulbar Palsy

Answer 12

an adult motor neuron disease with selective involvement of the motor nuclei of the lower cranial nerves; presents with dysarthria, dysphagia, chewing difficulties and drooling; almost always progresses to generalized disease (ALS); the earlier the onset of symptoms the worse the disease progression

Question 13

Spinal Muscular Atrophy (SMA)

Answer 13

an adult motor neuron disease with predominate lower motor neuron deficits; no upper motor neuron involvement; mean onset is 64 years; survival rate is 15 years (better than ALS and PBP); beings with symmetric upper extremity involvement

Question 14

Primary Lateral Sclerosis

Answer 14

an adult motor neuron disease with predominate upper motor neuron deficits; no lower motor neuron involvement; slow progression, survival rate is better than ALS (2-5 yrs)