Motor neuron disease Flashcards
Define motor neuron disease
MND refers to a cluster of degenerative disease with selective loss of neutrons in cortex–no sensory loss (not like MS), and never affects eyes (unlike myasthenia graves)
ALS (50% of NMD), Progressive bulbar palsy (10%), presgressive muscular atrophy (10%), Primary lateral sclerosis
Aetiology and risk factors of motor neurone disease
Progressive bulbar palsy-only affects cranial nerve IX-XII
Progressive muscular atrophy-anterior horn cell-
Primary lateral sclerosis-betz cells in cortex
ALS-motor neuron in cortex AND neurons in anterior horn (like PMA + PLS)
very genetic or unclear reason
comes early (60yo onset, fatal in 2-4 years
1.5x more men
Epidiemology of MND
think of MND if young and stumbling spastic gains, foot drop, weak grip, aspiration pneumonia
Signs and symptoms of motor neurone disease
PBP-swallowing and cranial nerve related. LMN of tongue, absent jaw jerk, absent gag reflex, speech is quiet/hoarse/nasal-slow
Pseudobulbar is the UMN of this-high reflex
both can be there in PBP
ALS- PMA +PLS
UMN signs (big plantars) + LWN -dysarithia,
wasting/fasciculation, get worse until swallowing is affected (bulbar)->death
pseudobulbar sx- crying, labile emotions
Investigations of Motor neurone disease
Mainly clinical diagnosis
Suspected-UMN/LWN signs in more than 1 region
Possible -UMN and LWN in same region
Probably with lab-LWN+UWN in one region + EMG shows acute denervation
probably -UMN+LWN in 2 region
Definite-3 regions
Suspect MND when combinations of UMN and LMN disorders
MRI/CT should be normal
Management of ALS/MND
Riluzole for ALS- can improve prognosis time ( care Hepatic failure, leukopenia)-monitor FBC/LFT
and physio, SALT, OT
and supportive
Non invasie Mechanical ventilation for resp
PEG for swallow
Carbocysteine for mucous
drooling- atropine, amytryptilne, hocyamine
spastic- baclofen
pseudobulbar- reassurance mainly, SSRI
