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
