Motor Neurone Disease

Question 1

what does motor neurone disease encompass?
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Answer 1

Motor neurone disease is an umbrella term that encompasses a variety of specific diagnoses

Question 2

Motor neurone disease is a …….. (description)

Answer 2

Motor neurone disease is a progressive, ultimately fatal condition where the motor neurones stop functioning

Question 3

does motor neurone disease effect sensory neurones too

Answer 3

There is no effect on the sensory neurones and patients should not experience any sensory symptoms.

Question 4

what is ALS?

Answer 4

Amyotrophic lateral sclerosis (ALS) is the most common and well-known specific motor neurone disease. Stephen Hawking had amyotrophic lateral sclerosis.

Question 5

Progressive bulbar palsy is ….

Answer 5

Progressive bulbar palsy is the second most common form of motor neurone disease. It affects primarily the muscles of talking and swallowing.

Question 6

as well as ALS, and progressive bulbar palsy, give 2 others to be aware of

Answer 6

Other types of motor neurone disease to be aware of are progressive muscular atrophy and primary lateral sclerosis.

Question 7

in MND, there is degeneration of which neurones?

Answer 7

There is a progressive degeneration of both upper and lower motor neurones. The sensory neurones are spared.

Question 8

do we know the cause of MND?

Answer 8

No, not exactly.
thought to be genetic component
5-10% of cases are inherited
smoking increases risk, as well as heavy meta exposure, and some pesticides

Question 9

typical presentation of MND

Answer 9

The typical patient is a late middle aged (e.g. 60) man, possibly with an affected relative. There is an insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising. They may complain of clumsiness, dropping things more often or tripping over. They can develop slurred speech (dysarthria).

Question 10

the weakness in MND is often first noticed in the?

Answer 10

upper limbs

Question 11

can MND patients develop slurred speech?

Answer 11

Yes

Question 12

what are the signs of Lower MND

Answer 12

Signs of lower motor neurone disease:

Muscle wasting
Reduced tone
Fasciculations (twitches in the muscles)
Reduced reflexes

Question 13

what are the signs of upper MND

Answer 13

Signs of upper motor neurone disease:

Increased tone or spasticity
Brisk reflexes
Upgoing plantar responses

Question 14

how do we diagnose MND?

Answer 14

needs to be made carefully
based on presentation, and once other conditions have been excluded.
only be made by a specialist
often delayed though - causing anxiety and stress.

Question 15

how do we manage MND

Answer 15

no effective treatments for halting or reversing the progression of the disease.
Drugs:
Riluzole
Edaravone
Non-invasive ventilation

Question 16

how does Riluzole help NMD patients?

Answer 16

Riluzole can slow the progression of the disease and extend survival by a few months in ALS. It is licensed in the UK and should be initiated by a specialist.

Question 17

how does Edaravone help MND patients?

Answer 17

Edaravone is currently used in the United States but not the UK. Recent studies suggest it has the potential to slow the progression of the disease and it may come in to use in the future.

Question 18

how is NIV used to help MND patients?

Answer 18

Non-invasive ventilation (NIV) used at home to support breathing at night improves survival and quality of life.

Question 19

give some important skills / management things which are important in helping manage MND patients?

Answer 19

Effectively breaking bad news
Involving the multidisciplinary team (MDT) in supporting and maintaining their quality of life
Advanced directives to document the patient’s wishes as the disease progresses
End of life care planning
Patients usually die of respiratory failure or pneumonia