motor neuron disease

Question 1

what is MND?

Answer 1

progressive degenerative disorder of unknown cause

Question 2

site of motor neurons affected?

Answer 2

brainstem, cranial motor neurons, spinal cord, pyramidal neurons,

Question 3

pathology?

Answer 3

loss of neurons in motor cortex, anterior horn and cranial nerve nuclei no inflammation

Question 4

clinical features?

Answer 4

depend upon site which is affected
pseudobulbar palsy: motor fibers from cortex to cranial nerve nuclei
bulbar palsy: loss of cranial nerve nuclei
progressive bulbar palsy: loss of cranial nerve nuclei and its connection with motor cortex
primary lateral sclerosis: corticospinal fibers
progressive musuclar atrophy: loss of anterior horn cells
amyotrophic lateral sclerosis: degeneration of corticospinal and anterior horn cell

Question 5

pattern?

Answer 5

PBP: early involvement of tongur, palate and pharyngeal muscle, wasting and fasiculation
PMA: reflexes absent, wasting, fasciculation
ALS: extensor planter reflex spasticoty

Question 6

D/D?

Answer 6

hyperthyroidism
hyperparathyroidism (muscle wasting hyperreflexia same as MND)
psuedobalbar palsy
spinal muscular atrophy, diabetic amyotrophy

Question 7

investigation?

Answer 7

EMG, nerve conduction test, MRI thryoid and calcium test

Question 8

treatment?

Answer 8

symptomatic treatment like physiotherapy, walking aids, speech therapy, splints