MND

Question 1

What is MND?

Answer 1

It’s a life-limited, rapidly progression neurodegenerative disorder affecting the brain and spinal cord
Not very common - but common for SLT to be involved with
onset 40-60

Question 2

What causes MND?

Answer 2

MND is sporadic and caused by a mixture of genetic predisposition and environmental triggers

Question 3

What are the possible mechanisms of the abnormal motor neuron function?

Answer 3

Accumulation of abnormal protein clumps
Deficiency in antioxidants
Abnormal mitochondria
Glutamate excitotixicity (i.e. damaged cells trying to drive themselves harder)

Question 4

What is the most common form of MND?

Answer 4

ALS (amyotrophic lateral sclerosis)/Lou Gehrig’s disease
Mix of upper and lower motor neurons
Typically starts with weakness and muscle wasting and fasciculations
Usually begins in the limbs
Average survival = 2-5 years

Question 5

What is the hallmark of Progressive Bulbar Palsy?

Answer 5

It begins with the bulbar muscles (therefore dysarthria and dysphagia)

Question 6

How do you diagnose MND?

Answer 6

No single diagnostic test
Neurological history and exam
history of progressive limb weakness - mix of UMN and LMN symptoms and fasciculation - ALS
History of progressive dysarthria and dysphagia = Progressive Bulbar Palsy
Tests to rule out other causes = MRI, EMG, Lumber puncture, blood tests

Question 7

Is the rate of progress constant or fluctuating?

Answer 7

Tends to remain constant (i.e. slow start - slow progress)

Question 8

Is there cognitive impairment in MND?

Answer 8

Yes in approx 1/3 of cases there are mild cognitive impairments. Generally associated with executive function and WFD

15% of cases also have frontotemporal dementia

Question 9

What are the end stage symptoms of MND?

Answer 9

Increasing paralysis 
Increasing dysarthria 
Significant shortness of breath 
Respiratory failure 
Pneumonia (due to aspiration)

Question 10

What is the treatment for MND?

Answer 10

There is no curative treatment. Riluzole is a drug that can reply progression (but only by 2-3 months)
More about symptom management:
Muscle weakness - orthotics
Muscle cramps - baclofen or physio
Drooling - Botox or drugs
Communication - aids/ assistive technology

Question 11

What are the two types of ventilation a person with MND may have?

Answer 11

1. Non-invasive ventilation

2. Invasive Ventilation (big decision)

Question 12

What is the main cause of death in MND?

Answer 12

Respiratory failure due to weakness of respiratory muscles