ALS/MND

Question 1

UMN or LMN signs?

Answer 1

Both

Question 2

What is bulbar ALS?

Answer 2

Atrophic tongue

Inability to speak/swallow

Question 3

Motor degeneration:

Answer 3

Fasciculation
Atrophy
Spasticity
Hyperreflexia

Question 4

Non-motor degeneration:

Answer 4

Development of front-temporal dementia

Emotional instability

Question 5

Differentials:

Answer 5

Structural lesions
Metabolic syndrome: B12, copper, white cell enzymes
AI: multifocal motor neuropathy
Infections: HIV, syphilis

Question 6

UMNS differentials:

Answer 6

Progressive supra nuclear palsy
Primary progressive multiple sclerosis
Hereditary spastic paraplegia (gait disorder)

Question 7

LMNS differentials:

Answer 7

Myasthenia/muscle disease
Motor dominant chronic inflammatory demyelinating polyneuropathy/multifocal motor neuropathy
Inclusion body myelitis
Lead poisoning

Question 8

4 domains of ALS:

Answer 8

Bulbar
Upper limb (fine motor)
Lower limb (gross motor)
Respiratory

Question 9

Body regions in El Escorial ALS criteria:

Answer 9

Bulbar, cervical, thoracic and lumbar

UMN and LMN in 1 region = possible, 2 = probable, 3 = definitely

Question 10

What is stage 4A?

Answer 10

Gastrostomy

Question 11

What is stage 4B?

Answer 11

Non-invasive ventilation

Question 12

What is the disease modifying drug?

Answer 12

Riluzole
Prolongs life by 3 months
A glutamate antagonist that treats hyper-excitability

Question 13

Symptomatic treatments:

Answer 13

Neudexta and antidepressants for emotional lability
Quinine for cramps
Baclofen or tizanidine for spasticity
Hyoscine, atropine, amitryptiline, botox or RT for salivation

Question 14

Classic features:

Answer 14

No sensory signs
Fasciculations
Wasting of intrinsic hand muscles and tibialis anterior
Doesn't affect external ocular muscles
No cerebellar signs
Sphincter dysfunction is a late feature

Question 15

Why might you do MRI?

Answer 15

To exclude myelopathy and spinal cord compression

Question 16

Conduction and EMG?

Answer 16

Normal nerve conduction (excludes neuropathy)

EMG will show less APs with smaller amplitudes

Question 17

Features specific to ALS:

Answer 17

Typically UMN in upper limb and LMN in lower limb

Question 18

Features specific to primary lateral sclerosis:

Answer 18

UMN signs only

Question 19

Features specific to progressive muscular atrophy:

Answer 19

LMN signs only
Affects distal muscles before proximal
Best prognosis

Question 20

Features specific to progressive bulbar palsy:

Answer 20

Palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
Worst prognosis