Neuromuscular disease Flashcards
What is the clinical pattern of motor neurone disease?
painless and absence of sensory symptoms
florid fasciculations
strong triceps and finger flexors
split hand - sparing of hypothenar eminence
combination of UMN and LMN lesions
MND bulbar signs
dysphagia
difficulty in chewing
nasal regurgitation
slurring of speech
difficulty in handling secretions
choking on liquids
dysphoria
dysarthria
Why do motor neurons die in ALS?
mitochondrial dysfunction
protein aggregation
axonal transport
excitotoxicity
oxidative stress
What does ALS stand for?
Amyotrophic lateral sclerosis
How can thyrotoxicosis present in muscles?
weakness
wasting fasciculation
What is Kennedy’s disease?
males only
pure LMN disorder
proximal symmetrical weakness
tongue wasting
perioral fasciculation
slow progression
sensory changes
gynaecomastia
What is mononeuritis multiplex?
individual nerves picked off randomly
subacute presentation
Mononeuritis multiplex causes
vasculitides
connective tissue disorders
What is Chronic inflammatory demyelinating polyneuropathy (CIDP)?
chronic equivalent of inflammatory demyelinating polyradiculoneuropathy (GBS)
Chronic inflammatory demyelinating polyneuropathy (CIDP) treatment
corticosteroids
immunosuppressants
plasmapheresis (if severe)
IV immunoglobulins
Myasthenia gravis diagnosis
acetylcholine receptor antibodies
anti-MuSK antibodies
single fibre EMG
repetitive stimulation
tensilon test/ice pack
CT chest (to look for thymoma)
Myasthenia gravis treatment
acetylcholinesterase inhibitors
corticosteroids
immunosuppressants
plasmapheresis
IV immunoglobulins
thymectomy
