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Flashcards in MND Deck (30):
1

What is the other names for MND?

ALS (amyotrophic lateral sclerosis)
Lou Gehrig disease

2

What is required for a diagnosis of ALS?

Mixed UMN and LMN signs with an ABSENCE of sensory signs

3

What is the median survival for MND?

3 years

4

What is an UMN predominant form of MND?

PLS; primary lateral sclerosis

5

What is a LMN predominant form of MND?

PMA; progressive muscular atrophy

6

Which neurodegenerative condition is MND associated with?

FTD

7

Where can MND begin?

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

8

Which genes are assoc with MND?

C9orf
SOD1

9

What are the different forms of motor neurone disease?

ALS
PLS
Progressive bulbar palsy
PMA
FTD

10

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

PLS

11

Describe an upper motor neurone lesion

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

12

Describe a lower motor neurone lesion

Muscle wasting
Weakness
Fasciculations
Absent or reduced deep tendon reflexes

13

What is predominantly affected in a bulbar onset MND?

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

14

What are the therapeutic interventions utilised in bulbar onset MND?

Early communicator
Nutritional support
Care for URT
Ensure not malnutritioned

15

What will spinal dysfunction result in?

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

16

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

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

17

What investigations are performed for diagnosis of MND?

EMG

18

What criteria is utilised in the diagnosis of MND?

El Escorial

19

Diagnosis of MND

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

20

What are frequent misdiagnoses of MND?

Carpal tunnel syndrome
Stroke
Neuropathy

21

What are common false positives for MND?

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

22

What is the tx for MND?

Riluzole
BiPAP
Gastrotomy; PEG, RIG, PIGG or NG

23

What are the specific treatments for bulbar MND?

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

24

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

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

25

What is the treatment for sialorrhoea?

Hyoscine/buscopan
Glycopyrronium
Botox
Suction

26

What is the treatment for muscle cramps in MND?

Baclofen
Quinine (not commonly used now)

27

What is the treatment for muscle spasms in MND?

Baclofen
Tizanidine
Dantrolene
Gabapentin
Medicinal cannabis

28

What are the red flag resp signs in MND?

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

29

What is the treatment for SOB/anxiety in MND?

Lorazepam

30

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

Weakness of respiratory muscles