Motor neuron disease

Question 1

Definition of MND

Answer 1

Mixture of UMN + LMN signs

Question 2

Signs in MND

Answer 2

Muscle wasting + fasiculations (LMN)
Hypertonia + spasticity (UMN)
Hypereflexia + upgoing plantars (UMN)
Reduced power (Distal > proximal)
Sensation is PRESERVED

Question 3

DDx of MND

Answer 3

Frontal lobe dementia -> Cognitive impairment

Question 4

Investigations in MND

Answer 4

Neurophysiology -> EMG -> Fasiculations
MRI Brain -> exclude DDx

Question 5

Management of MND

Answer 5

Supportive -> respect autonomy + choice

MDT approach:
Specialist neurologist + nurses
SALT -> Swallow ax
PT/OT -> Walking aids + home adaptations
Resp -> monitor FVC ? resp failure

Riluzole -> Glutamate antagonist -> slows progression by 3/12