Motor Neurone Disease

Question 1

what is MND

Answer 1

cluster of untreatable and rapidly progressive neurodegenerative conditions

Question 2

Sx of MND

Answer 2

muscle weakness
problems with speech, swallowing or breathing
UMN/LMN signs

Question 3

what is not seen in MND

Answer 3

NO sensory loss or sphincter disturbance

helps distinguish MND from MS and polyneuropathies

Question 4

how can MND be distinguished from myasthenia gravis

Answer 4

MND does not affect eye movements

Question 5

what are the 4 main types of MND

Answer 5

ALS/amyotrophic lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis

Question 6

what is the epidemiology of MND

Answer 6

90% sporadic and 10% familial

Sporadic MND peaks at the ages of 50-75 years and declines after the age of 80.

Question 7

what are UMND signs

Answer 7

increased tone
hyper-reflexia
extensor plantar response
spastic gait
exaggerated jaw jerk 
slowed movements

Question 8

what are LMND signs

Answer 8

Muscles wasting
weakness
fasciculations
absent or reduced deep tendon reflexes

Question 9

what is ALS (and MND to a lesser degree) linked with

Answer 9

frontotemporal dementia

Question 10

ALS pathology

Answer 10

loss of motor neurones in motor cortex and the anterior (ventral) horn of the cord

Question 11

Sx of ALS

Answer 11

weakness
UMN signs
LMN wasting/fasciculations

Question 12

what is suggestive of a worse prognosis in ALS

Answer 12

bulbar onset
increased age
decreased FVC

Question 13

what sign may be present in ALS

Answer 13

Split hand sign

- wasting of the thenar eminence, with the thumb seemingly cast adrift, but sparing of the hypothenar

Question 14

pathology of progressive bulbar palsy

Answer 14

disease of the nuclei of cranial nerves IX to XII in the medulla

Question 15

causes of progressive bulbar palsy

Answer 15

MND
Guillian-Barre 
Polio
Myasthenia graves
Brainstem tumours

Question 16

Sx of progressive bulbar palsy

Answer 16

tongue - weak, wasting, fasciculations
drooling
dysphagia
quiet/hoarse/nasal speech
dysphonia

Question 17

Signs of progressive bulbar palsy

Answer 17

jaw jerk normal or absent

Question 18

why does progressive muscular atrophy have a better prognosis than ALS

Answer 18

anterior horn cell lesion only

thus no UMN signs

Question 19

what does progressive muscular atrophy more commonly affect

Answer 19

distal muscle groups more than proximal

Question 20

what is flail arm syndrome

Answer 20

atypical presentation of ALS characterized by progressive, proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles

Question 21

what is flail leg syndrome

Answer 21

same as flail arm syndrome but with involvement in the lower limbs and not else where.

Question 22

pathology of primary lateral sclerosis

Answer 22

loss of Betz cells in motor cortex

Question 23

Sx of PLS

Answer 23

UMN signs 
affects distal muscle before proximal
spastic leg weakness
pseudo-bulbar palsy 
no cognitive decline

Question 24

what is the difference between bulbar palsy and pseudo-bulbar palsy

Answer 24

bulbar - lower motor neurone lesion

pseudo-bulbar - upper motor neurone

Question 25

what helps make the diagnosis of MND

Answer 25

• UMN/LMN signs
• absence of sensory symptoms
• MRI of brain/cord excludes structural causes
• LP excludes inflammatory ones

Question 26

what are clinical signs of an UMN

Answer 26

Babinski sign

Bulbar UMN signs: exaggerated snout reflex, clonic jaw jerk

Preserved reflex in a weak, wasted limb
Hoffmann reflex
hyperreflexia

Question 27

why do you need to be careful about a positive Hoffman reflex

Answer 27

not necessarily pathological cause

can be seen in normal patients

Question 28

what is the treatment of MND

Answer 28

MDT
palliative care
neurologists