MND

Question 1

What is the other names for MND?

Answer 1

ALS (amyotrophic lateral sclerosis)

Lou Gehrig disease

Question 2

What is required for a diagnosis of ALS?

Answer 2

Mixed UMN and LMN signs with an ABSENCE of sensory signs

Question 3

What is the median survival for MND?

Answer 3

3 years

Question 4

What is an UMN predominant form of MND?

Answer 4

PLS; primary lateral sclerosis

Question 5

What is a LMN predominant form of MND?

Answer 5

PMA; progressive muscular atrophy

Question 6

Which neurodegenerative condition is MND associated with?

Answer 6

FTD

Question 7

Where can MND begin?

Answer 7

Predominantly spinal/ limbal
Bulbar; speech and swallowing
Cognitive; FTD
Respiratory

Question 8

Which genes are assoc with MND?

Answer 8

C9orf

SOD1

Question 9

What are the different forms of motor neurone disease?

Answer 9

ALS
PLS
Progressive bulbar palsy
PMA
FTD

Question 10

Which is the best form of MND in terms of survival?

Answer 10

PLS

Question 11

Describe an upper motor neurone lesion

Answer 11

Increased tone
Hyperreflexia
Extensor plantar response
Spastic gate
Exaggerated jaw jerk
Slowed movement
Pseudobulbar effect

Question 12

Describe a lower motor neurone lesion

Answer 12

Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes

Question 13

What is predominantly affected in a bulbar onset MND?

Answer 13

Wasting, fasciculations of tongue
Facial muscles affected
Pharyngeal muscles affected; difficult phonation

Question 14

What are the therapeutic interventions utilised in bulbar onset MND?

Answer 14

Early communicator
Nutritional support
Care for URT
Ensure not malnutritioned

Question 15

What will spinal dysfunction result in?

Answer 15

Muscle wasting
Loss of tone OR contractures
Preferential wasting of thenar group

Question 16

What interventions can be put into place in the context of spinal dysfunction in MND?

Answer 16

Consider help with washing, dressing, feeding and turning in bed
Mobility aids or hoists required

Question 17

What investigations are performed for diagnosis of MND?

Answer 17

EMG

Question 18

What criteria is utilised in the diagnosis of MND?

Answer 18

El Escorial

Question 19

Diagnosis of MND

Answer 19

Consensus clinical criteria with UmN/LMN signs and absence of sensory symptoms
Diagnosis of exclusion
Limb onset, bulbar onset and/or cognitive
Clinical neurophysiology; el escorial criteria inclusion of EMG findings
Neuroimaging and lab studies to rule out other diseases

Question 20

What are frequent misdiagnoses of MND?

Answer 20

Carpal tunnel syndrome
Stroke
Neuropathy

Question 21

What are common false positives for MND?

Answer 21

Multifocal motor neuropathy
Kennedy’s disease
Myopathy (inclusion body myopathy)
Cervical spondylotic radiculomyelopathy

Question 22

What is the tx for MND?

Answer 22

Riluzole
BiPAP
Gastrotomy; PEG, RIG, PIGG or NG

Question 23

What are the specific treatments for bulbar MND?

Answer 23

Dysarthria: communication aids
Dysphagia: hydration, nutrition, saliva, gastronomy

Question 24

What is the association between weight loss and prognosis in MND?

Answer 24

More than 10% weight loss is indicator of mortality within 6 months

Question 25

What is the treatment for sialorrhoea?

Answer 25

Hyoscine/buscopan
Glycopyrronium
Botox
Suction

Question 26

What is the treatment for muscle cramps in MND?

Answer 26

Baclofen

Quinine (not commonly used now)

Question 27

What is the treatment for muscle spasms in MND?

Answer 27

Baclofen
Tizanidine
Dantrolene
Gabapentin
Medicinal cannabis

Question 28

What are the red flag resp signs in MND?

Answer 28

Breathlessness
Orthopnoea 
Recurrent chest infections
Disturbed sleep
Nightmares
Daytime sleepiness
Poor concentration 
Non-refreshed sleep

Question 29

What is the treatment for SOB/anxiety in MND?

Answer 29

Lorazepam

Question 30

What is once of the main causes of death in MND?

Answer 30

Weakness of respiratory muscles