Motor neurone disease Flashcards
What is MND
a neurological condition of unknown cause which can present with both upper and lower motor signs
What is amyotrophic lateral sclerosis
most common form of MND
-sometimes familial cause- usually idiopathic
How does ALS usually present
LMN signs in arms
UMN in legs
What is primary lateral sclerosis
Upper motor neuron signs only
How does progressive muscular atrophy present
LMN signs only
affects distal muscles before proximal
carries best prognosis
How does progressive bulbar palsy present
palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
worst prognosis
What are the types of MND
ALS
primary lateral sclerosis
progressive muscular atrophy
progressive bulbar palsy
What are symptoms of ALS
asymmetric limb weakness
mixture of LMN and UMN signs
wasting of small hand muscles/tibialis anterior
fasciculation
absence of sensory signs
How else does progressive bulbar palsy present
dysarthria
dysphagia
wasted fasciculating tongue
brisk jaw jerk reflex
What are some general signs you do not see in any MND
DOES NOT AFFECT
external ocular muscles
no cerebellar signs
preserved abdominal reflexes
What investigations are required
Clinical diagnosis
Nerve conduction studies will show normal motor conduction
EMG - reduced number of APs with increased amplitude
MRI to exclude other conditions
What is management for ALS
Riluzole
What other management is used
NIV at night - usually BiPaP
percutaneous gastrostomy tube for nutrition
