Motor Neurone Disease Flashcards
Which types of symptoms such make you suspicious of MND?
Upper and lower motor neurone symptoms
NO sensory symptoms or sphincter disturbance
Describe some of the clinical signs seen in MND
UMN signs: spasticity, increased reflexes and plantars
LMN signs: weakness, fasciculations of tongue, abdomen, back and thighs
Bulbar signs –> speech and swelling difficulties
Frontotemporal dementia in 25%
What are the 4 different types of MND?
Amyotrophic lateral sclerosis (ALS)
Progressive bulbar palsy
Primary lateral sclerosis
Progressive muscular atrophy
Where are motor neurones lost in ALS?
Motor cortex + anterior horn of cord
Which hand sign is suggestive of ALS?
Split hand sign
- thenar wasting (thumb side of hand)
- hypothenar less affected
Which gene should be tested for if ALS + front-temporal dementia?
C9orf72
How is ALS diagnosed?
Clinical diagnosis
MRI/LP to exclude structural/inflammatory causes
Which nerves are implicated in progressive bulbar palsy?
CN 9-12
What is the pathology in primary lateral sclerosis?
loss of Betz cells in motor cortex
–> mainly UMN signs
Which area is affected in progressive muscular atrophy?
Anterior horn cells only
–> no UMN signs
Which muscle groups are affected first in progressive muscular atrophy?
Distal muscle groups
Which drugs can be used for drooling/salivation in MND?
Hyoscine/Buscopan
Glycopyrronium
Botox
Which drugs can be used to treat muscle cramps?
Quinine
Baclofen
Which drugs can be used to treat muscle spasms?
Baclofen
Tizanidine
Dantrolene
Gabapentin
What is the main cause of death in MND?
Weakness of respiratory muscles –> type 2 respiratory failure
