MND

Question 1

What are some sub-types of MND?

Answer 1

Amyotrophic Lateral Sclerosis (ALS) - Most common, highest Mortality.

Progressive Bulbar Palsy - Primarily effects muscles of talking and swallowing (second most common) 20% cases.

Others;
- Progressive Muscular Atrophy - 10% cases
- Primary lateral Sclerosis - 1-3% cases

Question 2

What Neurons are degenerated in MND?

Answer 2

Both Upper and Lower moor neurons are progressively degenerated. Sensory neurons are spared.

Question 3

How does MND present? And where?

Answer 3

Muscle weakness and wasting secondary to motor neuron degeneration.

Typically presents first in Extremities (upper > Lower)

Question 4

What is MND Katabolism?

Answer 4

Occurs in 40-50% patients.
This describes a rapid increase in metabolic rate causing the patient to lose weight rapidly, typically just before death.

Question 5

What is Split Hand Syndrome?

Answer 5

Preferential wasting of the Thenar Group. This is a typical pattern of atrophy seen in ALS.

Question 6

Which MND subtype has the best Prognosis? which has the worst?

Answer 6

• PLS has best survival rate (survival > 5 yrs).
• ALS has a poor survival rate (Median survival 3-5yrs)

Question 7

What are the Upper motor neuron signs in MND?

Answer 7

• Increased tone
• Hyper-reflexia
• Extensor Plantar responses
• Spastic gait
• Exaggerated Jaw-jerk
• Slowed movements

Question 8

What are the lower motor neuron signs in MND?

Answer 8

• Muscle wasting
• Weakness
• Fasciculations
• Absent or reduced deep tendon reflexes.

Question 9

What Indicates a more benign prognosis of ALS?

Answer 9

Presence of the following:
- Flail arm syndrome
- Flail Leg Syndrome
- Primary Lateral Sclerosis
- Focal Distal Spinal Muscular Atrophy
- Kennedy’s disease (SMA Variant)

Question 10

How is MND managed?

Answer 10

On-going management:
- Communication needs (SALT, Voice banking)
- Nutritional needs (Dieticians, Gastrostomy)
- Respiratory needs (Assessment, Home Ventilation)

Question 11

What is Riluzole?

Answer 11

Medication used in the Tx of ALS and other forms of MND - Used to try and slow progression dependence on ventilators for patients.

Question 12

What are some Pharmacological Symptomatic Treatments for MND?
- Sialorrhoea Tx
- Muscle Cramps Tx
- Muscle Spasms Tx

Answer 12

Sialorrhoea (excessive Saliva)
- Hyoscine/Buscopan
- Glycopyrronium (especially if cognitive impairment)

Muscle Cramps
- Baclofen
- Quinine

Muscle Spasms
- Baclofen
- Tizanidine
- Gabapentin

Shortness of breath / Anxiety
- Lorazepam

Question 13

What is the main cause of Death in MND and how is it managed?

Answer 13

Respiratory weakness.
Non-Invaisive ventilation (NIA) is offered to support type 2 resp failure.
- Is commenced at night and gradually increased as disease Progresses.

Question 14

What is the most common genetic cause of familial ALS?

Answer 14

SOD1 mutation.