Motor Neuron Disease Flashcards
Is MND treatable?
No.
It is a rapidly progressive neurodegenerative illness.
How does MND initially present?
Begins focally, before spreading out and becoming a generalised paresis.
Are most cases of MND sporadic or genetic?
Around 90% of cases are sporadic.
What are the 4 types of MND?
PLS
PMA
ALS
Bulbar onset MND
What is the most common form of MND?
ALS
What is the most common site of MND onset?
Limbs
Is bulbar onset MND more common in males or females?
Females
Which form of MND has the poorest prognosis?
ALS
What is affected in bulbar MND?
The muscles supplied by CNVII-CNXII.
Affects tongue muscles, facial muscles and pharyngeal muscles.
Is cognitive impairment a feature seen in MND?
Yes
MND has an association with frontotemporal dementia.
What are examples of UMN signs in MND?
Increased tone Hyper-reflexia Extensor plantar response Spastic gait Exaggerated jaw-jerk Slowed movement
What are examples of LMN signs in MND?
Muscle wasting Weakness Fasciculations Absent/reduced reflexes Loss of tone
What does MND do to the metabolic rate?
Increases it. This is called hypercatabolism.
It is linked to the weight loss experienced in MND.
What is split-hand syndrome?
Where a region of the hand experiences disease, whilst another area of the same hand remains functional.
What is the name of the diagnostic criteria used for MND?
El Escorial criteria.
What is the name of the only drug treatment available for MND?
Riluzole
Why do patients not like riluzole therapy?
Causes many liver/kidney issues.
Only lengthens life for 3 months, and this is during the period in which the patient is most unwell.
What is sialorrhoea?
Excessive salivation.
Treat with hyoscine. If not enough then botox injection of the salivary glands is an option.
What is the treatment for muscle spasms?
Gabapentin
What is the treatment for muscle cramps?
Baclofen
What is a major cause of death in MND?
Weakening of the respiratory muscles.
Treat with non-invasive ventilation.
