MND

Question 1

What is motor neurone disease?

Answer 1

A progressive degenerative disorder of the CNS, affecting the anterior horn cells of spinal cord, corticospinal tracts and nuclei of brain stem. Resulting in lesions in upper and lower motor neurones.

Question 2

Define the types of MND

Answer 2

1. Amyotrophic lateral sclerosis (ALS) - affecting both upper and lower motor neurones.
2. Primary lateral sclerosis (PLS) - affects upper motor neurones.
3. Progressive Muscular Atrophy (PMA) - affects lower motor neurone.
4. Pseudobulbar palsy - bulbar region of upper motor neurones.
5. Progressive bulbar palsy (PBP) - bulbar region of lower motor neurones.

Question 3

Pathology of MND

Answer 3

Death of both upper and lower motor neurones, more specifically anterior horn cells of spinal, corticospinal tracts of cerebral cortex and nuclei of brainstem. Prior to their destruction motor neurones develop protein rich inclusions in their cell bodies and axons. These inclusions often contain ubiquitin and ALS - associated proteins.

Question 4

MND symptoms

Answer 4

1. Dysphagia
2. Dysarthria
3. Weakness
4. Spasticity/Hypereflexia
5. Clonus
6. Muscular atrophy
7. Fasiculation
8. Dyspnoea

Question 5

Management of MND

Answer 5

Riluzole is only medication shown survival benefits, slowing down damage to neurones Physio can help reduce muscle tone and stiffness, increase ROM. Baclofen relaxes muscles Respiratory difficulties may be helped with NIV ( non invasive mechanical ventilation), May result in tracheostomy