Motor neurone disease Flashcards
what is MND?
relentlessly progressive neurodegenerative condition typified by a predilection for upper and lower motor neurones only whilst sparing sensory and autonomic nerves
most common phenotype?
Amyotrophic lateral sclerosis (ALS)
UMN vs LMN?
1) paralysis
2) muscle tone
3) reflexes
4) babinski/ Hoffman’s sign
5) fasciculations
1) spastic vs flaccid
2) hypertonic vs hypotonic
3) hyperreflexia vs hyporeflexia/ absent
4) positive vs negative
5) absent vs present
exact cause of MND is unknown. It occurs as a ___ process in most.
sporadic
age?
sex?
ethnicity?
50-57
M>F
caucasians
ALS
1) UMN or LMN signs?
2) symmetrical or asymmetrical?
3) late stage symptoms?
1) both
2) often asymmetrical in limbs
3) dysphagia, dysarthria, dyspnoea, recurrent aspiration pneumonia
what is this describing: a variant of MND presenting as purely LMN pathology e.g. flail arm/ foot
progressive muscular atrophy
what is this describing: least common phenotype of MND, presents purely with UMN signs
primary lateral sclerosis
Progressive bulbar palsy
1) what is it?
2) causes?
1) LMN disease affecting CN IX, X and XII causing dysphagia, dysarthria, tongue fasciculation and reduced jaw and gag reflex
2) ALS, guillaine-barre, myasthenia gravis
Progressive pseudobulbar palsy
1) what is it?
2) causes?
1) UMN disease affecting CN IX, X and XII causing dysphagia, slow speech and brisk jaw reflex
2) ALS, MS, stroke
mainstay of management for MND?
specialist MND services and supportive care
- communication (SALT)
- nutrition (dietician, parenteral)
- respiratory (BiPAP at night)
pharmacological management of MND?
Riluzole (blocks glutaminergic neurotransmission in the CNS, extends life by average of 3 months)
complications of MND?
depression
weight loss and malnutrition (secondary to dysphagia)
common cause of death: respiratory failure due to resp muscle weakness
