motor neurone disease

Question 1

cause of MND

Answer 1

unknown but may be congenital

Question 2

presentation of spinal ALS

Answer 2

-classic
-upper and lower usually in one limb but then spreads to other limbs and trunk muscles
-split hand syndrome
-flail arm/leg syndrome

Question 3

presentation of bulbar ALS

Answer 3

-dysarthria
-dysphagia
-nasal regurgitation of fluids
-choking

Question 4

presentation of progressive muscular atrophy

Answer 4

-only lower
-weakness
-muscle wasting
-fasciculations
-starts in one limb and spreads

Question 5

presentation of primary lateral sclerosis

Answer 5

-only upper
-tetraparesis and pseucobulbar palsy

Question 6

investigations for MND

Answer 6

-largely clinical
-diagnosis of exclusion
-EMG (denervation of muscles)
-electrophysiology

Question 7

what indicates a very poor prognosis

Answer 7

weight loss of >10%

Question 8

symptomatic treatment

Answer 8

muscle cramps - quinine, baclofen
muscle spasms - baclofen, tizanidine
emotional liability - sometimes antidepressants

Question 9

ongoing management of MND

Answer 9

-key worker (MND nurses)
-communication needs (speech therapy, AAC)
-nutritional needs (dieticians, gastrostomy)
-respiratory needs (home ventilation)
-Riluzole increases life expectancy by 3-4 months but only to be used in the last stages of life

Question 10

red flags

Answer 10

any sign of respiratory failure

Question 11

life expectancy

Answer 11

3 years

Question 12

what is the most common type

Answer 12

ALS
-is also the most aggressive