motor neurone Flashcards
in MND where is there loss of motor neurones?
motor cortex
cranial nerve nuclei
anterior horns on spinal cord
what type of dementia does MND have a link too?
fronto-temporal
what is the primary symptom in MND?
muscle weakness
what is the pattern of weakness in MND?
usually focal and progressively spread to become generalised
what other symptoms are there in MND?
fascinations
mixed UMN and LMN symptoms
wasting of small hand muscles and tibialis anterior common
where does MND most commonly affect first?
limbs first - upper limb
can start in bulbar or thorax muscles
what motor neurones are most commonly degenerated first?
lower motor neurones then upper motor neurones (patients commonly present with a mix)
how can MND be distinguished from other neurological conditions?
doesn’t affect the eyes (myasthenia)
no sensory loss or sphincter dysfunction (MS and neuropathies)
what is the most common type of MND?
amyotrophic lateral sclerosis
what is the presentation of amyotrophic lateral sclerosis?
mixed motor neurone deficit - LMN in arms and UMN in legs
where does amyotrophic lateral sclerosis start?
starts in hands before spreading to affect all the limbs and bulbar/thorax muscles
what other symptoms are associated with amyotrophic lateral sclerosis?
weak hand grip
weak shoulder abduction
stumbling gait
foot drop
which type of MND has the worst prognosis?
progressive bulbar
what are the symptoms associated with progressive bulbar MND?
drooling
dysphagia
progressive dysarthria
what are the symptoms seen with progressive spinal muscular atrophy MND?
similar to amyotrophic lateral sclerosis but purely LMN
what is primary lateral sclerosis MND?
rare form - loss of benz cells in the motor cortex
what symptoms are seen with primary lateral sclerosis MND?
purely UMN
how is MND diagnosed?
clinical
nerve conduction studies to rule out other neuropathies - normal in MND
what can be used to treat MND? which type is in mainly used in?
riluzole
mainly in ALS
how long does riluzole prolong a MND patient’s life by?
3 months
what other management can be used to help treat MND?
analgesia
anti-cholinergic for drooling
BiPAP at night for breathing
baclofen to relax muscles
what is Kennedy’s disease?
may be misdiagnosed as MND
lower motor neurone limb and bulbar weakness
what is the inheritance of Kennedy’s disease?
x-linked genetic disorder
what other symptoms are associated with Kennedy’s disease?
erectile dysfunction
infertility
gynaecomastia
atrophic testes
