MS and MND

Question 1

Describe MS

Answer 1

Inflammatory plaques of demyelination in CNS which are disseminated in time and space

Question 2

Subtypes of MS

Answer 2

Relapsing remitting
Primary progressive
Secondary progressive (secondary to relapsing remitting)

Question 3

Age of onset in MS and MND

Answer 3

MS: ~30 years
MND: >40 years

Question 4

Common presentation of MS

Answer 4

Unilateral optic neuritis (20%)
Corticospinal involvement
Urine retention or incontinence
Symptoms worsen with heat

Question 5

Clinical features of MS (11 points)

Answer 5

Sensory loss
Neuropathic pain
Spastic muscle weakness
Urine retention/incontinence
Constipation
Sexual dysfunction
Dysphagia
Optic neuritis
Internuclear ophthalmoplegia 
Cerebellar signs
Cognitive decline

Question 6

Criteria for MS

Answer 6

McDonald:
At least 2 attacks lasting >1 hour and are >30 days apart
AND
Disseminated in time and space (clinical or MRI evidence)

Question 7

Diagnosis of MS

Answer 7

T2 weighted MRI brain and spinal cord with contrast
LP for oligoclonal IgG bands on electrophoresis of CSF
Visual evoked response

Question 8

Effect of pregnancy on MS

Answer 8

During pregnancy relapses may reduce
After giving birth relapses may increase for 3-6 months
Frequency returns to previous rate

Question 9

Management of MS

Answer 9

Lifestyle: exercise, stop smoking, vitamin D supplements
Disease modifying drugs: dimethyl fumarate, monoclonal antibodies (alemtuzumab)
Relapse drugs: methylprednisolone for 3-5 days
Symptom control (bladder, bowel, pain, spasticity)

Question 10

Treatment for spasticity

Answer 10

Baclofen

Gabapentin

Question 11

Treatment for tremor in MS

Answer 11

Botulinum toxin injections

Question 12

Management of urinary symptoms in MS

Answer 12

Self catheterisation if RV >100ml

Tolteridone if RV <100ml

Question 13

Describe MND

Answer 13

Loss of neurones in:
Motor cortex
Cranial nerve nuclei
Anterior horn of spinal cord 
(Mixed UMN and LMN signs)

No sensory loss
No sphincter disturbance

Question 14

What are the 4 different clinical patterns of MND

Answer 14

ALS
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis

Question 15

What does ALS stand for

Answer 15

Amyotrophic lateral sclerosis

Question 16

Describe ALS

Answer 16

Loss of motor neurones in motor cortex and anterior horn of spinal cord leading to UMN and LMN signs

Question 17

Describe progressive bulbar palsy

Answer 17

LMN signs affecting cranial nerves 9-12

Results in difficulty talking and swallowing

Question 18

Describe progressive muscular atrophy

Answer 18

Anterior horn cell lesion so LMN signs

Distal muscles affected earlier than proximal

Question 19

Describe primary lateral sclerosis

Answer 19

Loss of neurones in motor cortex so UMN signs

Spastic leg weakness and pseudobulbar (damage to corticobulbar tracts)

Question 20

Contrast bulbar and pseudobulbar palsy

Answer 20

Bulbar is LMN lesion of muscles of talking and swallowing, pseudobulbar is UMN lesion of muscles of talking and swallowing
Pseudobulbar has increased jaw jerk, slow speech and mood incongruence

Question 21

What other progressive condition is associated with MND

Answer 21

Frontotemporal dementia in 25%

Question 22

Diagnosis of MND

Answer 22

No diagnostic test:
MRI excludes structural causes
LP excludes inflammatory causes
Myasthenia antibodies

Question 23

Disease modifying drugs for MND

Answer 23

Riluzole - NMDA antagonist

Question 24

Symptom management for MND

Answer 24

Salivation - antimuscurinics (glycopyrronium bromide)
Dysphagia - blend food, gastrostomy 
Spasticity - baclofen or gabapentin
Alternative communication equipment
Non invasive ventilation overnight

Question 25

Prognosis of MND

Answer 25

50% <3 years