Motor Neurone Disease Flashcards
how is MND diagnosed?
clinical diagnosis
can do neurophysiology to support this
how does MND often present?
muscle weakness
problems with speech, swallow and breathing
describe the course of disease in MND
focal onset
spreads until finally causing a generalised paresis if left untreated
what is the most common subtype of MND?
amyotrophic lateral sclerosis (ALS)
what is the average survival in MND?
three years
what four conditions are considered to be MND?
amyotrophic lateral sclerosis
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy
how does ALS present?
UMN and LMN features
how does primary lateral sclerosis present?
UMN features
how does progressive muscular atrophy present?
subclinical UMN features
LMN features
name some upper motor neurone signs that may be seen in MND
increased tone hyper-reflexia extensor plantar responses spastic gait exaggerated jaw jerks slowed movements
name some lower motor neurone signs that may be seen in MND
muscle wasting
weakness
fasciculations
absent/reduced deep tendon reflexes
what is one of the main causes of death in MND
weakness of respiratory muscles
what are the red flag respiratory signs in MND?
breathlessness orthopnoea recurrent infection disturbed/non refreshing sleep daytime sleepiness poor concentration
what defines progressive bulbar palsy?
primary bulbar onset of symptoms
who is most commonly affected by progressive bulbar palsy?
women more commonly than men
60-80 years of age
what is the outcome of progressive bulbar palsy?
always undergoes generalisation into ALS
what are possible therapeutic interventions for progressive bulbar palsy?
early communicator
nutritional support
care for the URT
what muscles are affected by progressive bulbar palsy?
tongue muscles
facial muscles
pharyngeal muscles
what is split hand syndrome and what disease is this seen in?
preferential wasting of the thenar muscle group
ALS
what is the pathophysiology behind ALS?
motor neurone degeneration and death
what investigation is done for ALS?
electrophysiology
largely a diagnosis of exclusion - neuroimaging and lab studies to exclude other diseases
what is the diagnostic criteria for ALS?
the el escorial criteria
classifies into clinically definite, clinically probable, probably (lab supported) and clinically possible ALS
what is the only drug available for MND and how beneficial is this?
riluzole
only adds on three months to a patients life
what are the options for managing sialorrhoea in MND?
hyoscine/buscopan
glycopyronium
botox
suction
what are the options for managing muscle cramps in MND?
quinine
baclofen
what are the options for managing muscle spasms in MND?
baclofen
tizanidine
dantrolene
gabapentin
what is the option for managing SOB and anxiety in MND?
lorazepam
