MND

Question 1

symptoms that point towards MND

Answer 1

asymmetric limb weakness -> ALS
mixture of lower motor neuron and upper motor neuron signs
wasting of the small hand muscles/tibialis anterior
fasciculations
absence of sensory signs/symptoms

Question 2

diagnosis of MND

Answer 2

clinical
nerve conduction studies show normal motor conduction (excludes neuropathy)
EMG
MRI- useful to exclude cord compression and myelopathy

Question 3

presentation of ALS

Answer 3

typically LMN signs in arms and UMN signs in legs
familial cases the gene is on chromosome 21

Question 4

presentation of primary lateral sclerosis

Answer 4

UMN signs only

Question 5

features of progressive muscular atrophy

Answer 5

LMN signs only
affects distal muscles before proximal
carries best prognosis

Question 6

progressive bulbar palsy presentation

Answer 6

palsy of the tongue, muscles of chewing/swallowing and facial muscles (loss of brainstem motor nuclei)
carries worst prognosis

Question 7

management of MND

Answer 7

riluzole
respiratory care
nutrition

Question 8

role that riluzole plays in MND management

Answer 8

prevents stimulation of glutamate receptors
used mainly in ALS
prolongs life by about 3 months

Question 9

role of respiratory care in MND management

Answer 9

non-invasive ventilation (BIPAP)

Question 10

role of nutrition in MND management

Answer 10

percutaneous gastrostomy tube is preferred
can prolong survival