Neuromuscular disorders

Question 1

Give 5 conditions of anterior horn cell disorders

Answer 1

poliomyelitis and ALS Amyotrophic lateral Sclerosis
Progressive Bulbar Palsy
Progressive/Spinal Muscular Atrophy
Primary lateral sclerosis

Question 2

As for UMN signs, is there up-going or down-going plantars?

Answer 2

Up going plantars in UMN sign

Question 3

What is ALS?

Answer 3

ALS is Amyotrophic Lateral Sclerosis.

There is a loss of motor neurones in motor cortex and the anterior horn of the cord.

Question 4

What is the clinical presentation of ALS?

Answer 4

Weakness, UMN signs such as up-going plantars, and LMN signs like wasting and muscle fasciculations.
More wasting of thenar muscles than hypothenar muscles, resulting in the split hand sign.

Question 5

What happens leading up to a split hand sign?

Which condition may present with a split hand sign?

Answer 5

Disproportionate muscle wasting of the thenar muscles more than the hypothenar muscles, resulting in a split hand sign.
This is seen in ALS

Question 6

How is ALS different from Progressive Muscular Atrophy?

Which condition has a better prognosis?

Answer 6

ALS affects both the UMN and LMN.
Progressive Muscular Atrophy only has LMN signs.

Progressive Muscular Atrophy has a better prognosis.

Question 7

In Progressive Muscular Atrophy, which muscle groups are affected first - Proximal or distal?

Answer 7

Distal muscle groups are affected first in Progressive Muscular Atrophy.

Question 8

What is affected in Progressive Bulbar Palsy?

Answer 8

In Progressive Bulbar Palsy, only cranial nerves VII, IX, X and XII in the medulla are affected.

Question 9

What are the signs of Progressive Bulbar Palsy?

Answer 9

LMN lesion signs affecting the tongue and bulbar muscles (involved in chewing and swallowing).

• Flaccid, fasciculating tongue (like a sack of worms)
• Jaw jerk is absent or normal
• Speech is quiet and hoarse, or nasal

Question 10

In Guillain-Barre syndrome, which muscle groups are more affected - proximal or distal?

Answer 10

In Guillain-Barre syndrome, proximal muscle groups are more affected.

Question 11

What is the first-line symptomatic treatment for ALS?

Answer 11

Riluzole.

Riluzole is a sodium channel blocker which inhibits glutamate release. Glutamate is a neuro-excitatory neurotransmitter.