Degeneration of motor neurons in the cortex, brainstem (corticobulbar tracts), cranial motor nuclei and anterior horn cells in the spinal cord resulting in various neurological symptoms

Upper motor neuron: Spasticity, brisk reflexes, positive Babinski

Lower motor neuron: Fasciculations, muscle wasting and weakness.

Imaging: MRI brain and spine to exclude other diagnoses

Special tests: Nerve conduction studies/electromyography show signs of chronic denervation in muscles

Medical: Riluzole (anti glutamate antagonist) only licensed treatment available

Supportive: Non-invasive ventilation, PEG feeding and communication aids

Average survival is usually between 2 – 5 years from onset 

5 year survival is 25%.

Infection, incontinence, spasticity, depression, dysphagia