amyotrophic lateral sclerosis

Question 1

features?

Answer 1

upper motor neuron signs: spasticity, hyperreflexia, pathologic reflexes (ex: jaw jerk, extensor plantar reflex).
lower motor neuron signs: atrophy, fasciculations.
bulbar symptoms: dysarthria, dysphagia.

Question 2

diagnosis?

Answer 2

clinical evaluation, EMG, MRI of head and spinal cord ( to exclude other causes).

Question 3

treatment?

Answer 3

slow progression and control symptoms: Riluzole ( glutamate inhibitor, reduces glutamate-mediated excitotoxicity) usually combined with edaravone ( an antioxidant that slows progression especially in early disease pts), respiratory support ( ex: NIPPV - noninvasive positive pressure ventilation), Nutritional support ( ex: PEG - percutaneous endoscopic gastrostomy tube).

Question 4

labs?

Answer 4

no confirmatory ones, sometimes mild creatine kinase elevation.