MND

Question 1

what happens in MND

Answer 1

progressive weakness and eventually death either from respiratory failure or aspiration

Question 2

which age of people does this affect

Answer 2

50-75

Question 3

what is affected

Answer 3

upper and lower motor neurones in the spinal cord, cranial nerve motor nuclei and the cortex

Question 4

what are the 4 types of MND

Answer 4

ALS (amyotrophic lateral sclerosis); progressive muscular atrophy; progressive bulbar and pseudobulbar palsy (20%); primary lateral sclerosis (1-2%)

Question 5

what are the features of ALS

Answer 5

involvement of upper and lower motor neurones, usually starts in one limb then spreads.can present as asymmetric spastic paraparesis and then lower MN signs appear months later. progressive muscle wasting and weakness and fasciculations

Question 6

what are the features of progressive muscular atrophy

Answer 6

only lower motor neurone signs. weakness, wasting, fasciculations usually starting in one limb then spreading

Question 7

what are the features of progressive bulbar and pseudobulbar palsy

Answer 7

lower cranial nerve nuclei and their connections initially involved. dysarthria, dysphagia, nasal regurgitation of fluids and chokin (bulbar); emotional incontinence, pathological laughter and crying (pseudobulbar)

Question 8

what are the features of primary lateral sclerosis

Answer 8

upper motor neurones. slowly progressive tetraparesis and pseudobulbar palsy

Question 9

how is diagnosis met

Answer 9

clinically. investigations to exclude other causes eg EMG

Question 10

what can be DDX in MND

Answer 10

cervical spondylosis causing radiculopathy; motor neuropathies

Question 11

prognosis

Answer 11

survival > 3 years is unlikely.

Question 12

treatments

Answer 12

riluzole (sodium channel blocker) slows progression slightly but only extends life expectancy by 3-4 months. non invasive ventilatory support and feeding via gastrostomy. MDT with access to palliative care