YearClub 2026 Neurology session (MND) Flashcards
What is MND?
Motor neurone disease is a term that encompasses a variety of specific diseases affecting the motor nerves.
Motor neurone disease is a progressive, eventually fatal condition where the motor neurones stop functioning.
There is no effect on the sensory neurones. Sensory symptoms suggest an alternate diagnosis.
Most common form of Motor neurone disease?
ALS (amyotrophic lateral sclerosis)
Summary of ALS?
Pathology = Upper + lower motor neuron degeneration (within spinal cord) -> symptoms
Focal onset -> continuous spread -> generalised paresis
Relentless progression
Why is still unknown
Other forms of MND and where does it affect?
Primary lateral sclerosis = upper motor neuron
Progressive muscular atrophy = lower motor neuron
Progressive bulbar palsy = cranial nerve + supranuclear symptoms -> ALS
FTD-associated = ALS + FTD
How is MND diagnosed?
Primarily clinical diagnosis, but EMG (showing denervation) and absence of signs on neuroimaging can help support the diagnosis
There can be weakness/paralysis in the strength of the muscle for both UMN and LMN lesions. True/false?
True
LMN lesion muscle tone and UMN lesion muscle tone?
UMN = hypertonia (increased muscle tone)
LMN = absent/hypotonia (decreased muscle tone)
Reflex strength for both UMN and LMN lesions?
UMN = hyperreflexia (increased reflex strength) + Babinski’s sign
LMN = decreased/absent reflexes
Muscle wasting is experienced in UMN lesions. True/false?
False. Muscle mass is maintained in UMN lesions.
Muscle wasting occurs more with LMN lesions.
Examples causes of UMN lesions?
Stroke
Cord section
MND (ALS + PLS)
Example causes of LMN lesions?
Muscular dystrophies i.e. Duchenne’s
Myasthenia gravis
Peripheral nerve dysfunction
Management of MND?
Average prognosis is 3 years after diagnosis
Get close monitoring for first few months -> then can workout how fast progression is
NO treatment/cure apart from experimental therapies - symptom management is key:
Nutritional support
Prepare for future - advanced directives, ‘voice banking’, AAC
Cramps + spasms - baclofen
Weakness - physio + OT, splinting, mobility aids
What is the only drug shown to improve patient survival in MND?
Riluzole
What is an EMG?
Technique for evaluating and recording the electrical activity produced by skeletal muscles. Helping to diagnose a variety of muscle and nerve disorders.