Motor Neuron Disease Flashcards
what is motor neurone disease?
group of progressive neurodegenerative conditions affecting only upper and/lower motor neurones sparing the nerves relaying sensory and autonomic signalling
what is the most common phenotype in motor neurone disease?
amytrophic lateral sclerosis (ALS)
babinski/hoffmans sign is +ve in UMN or LMN?
UMN
what is muscle tone like if UMN and LMN affected?
UMN- hypertonic
LMN- hypotonic
what are reflexes like if UMN or LMN disease?
UMN- hyperreflexia
LMN- hyporeflexia
who is mostly affected by motor neurone disease?
peak incidence aged 50-75
men>women
how does Amyitrophic lateral sclerosis (ALS) present?
simultaneous UMN and LMN symtpoms
late stages include dysarthria, dysphagia, dyspnoea
which MND presents purely as LMN patholgy e.g. flail arm/foot?
progressive muscular atrophy
primary lateral sclerosis presents purely with UMN or LMN signs?
UMN signs
of pseudobulbar and bulbar palsy which is UMN affected and which is LMN?
bulbar= LMN
pseudobulbar= UMN
which cranial nerves are affected in progressive bulbar/pseudobulbar palsy?
9, 10, 12
what are causes of bulbar palsy?
ALS
Guillian-Barre
myasthenia gravis
what are the causes of pseudobulbar palsy?
ALS
MS
stroke
how is MND managed?
access to specialist MND services and supportive care:
communication needs- SALT
nutrional needs dieticians, parenteral feeding
respiratory needs- BiPAP ventilation at night
what medication can be used to manage MND?
Riluzole
blocks glutaminergic neurotransmission in the CNS
what are some complcations of the drug riluzole?
depression
wgt loss/malnutrition second to dysphagia
respiratory failure due to resp muscle weakness
what causes McArdles disease?
myophosphorylase deficiency leading to impaired glucose release from glycogen in the muscles
how does McArdles disease present?
muscle pain and cramping following exercise
levels of which enzyme are commonly raised in McArdles disease?
Creatinine Kinase
continuing to exercise if you suffer McArdles disease can increase the risk of which complications?
rhabdomyolysis w myoglobinuria
subsequent AKI
