Summary

• Progressive disease characterised by degeneration of the motor neurons with cortical, brainstem, and ventral cord location.
• Usually presents as a combination of upper motor neuron (UMN) and lower motor neuron (LMN) symptoms and signs, involving initially 1 segment of the neuroaxis (i.e., cranial, cervical, thoracic, or lumbosacral), and then progressively spreading to contiguous areas.
• Typical presentations include limb-onset and bulbar-onset ALS, and, less frequently, respiratory-onset ALS. Generalisation of the symptoms follows in time, without intervals of remission, exacerbation, or stabilisation, resulting in progressive disability and death.
• There is no cure for ALS at the present time. The focus of medical care is to provide supportive and palliative intervention, aiming to improve the quality of life for patients. The only drug currently used for modifying the disease course is riluzole, which should be offered to patients at the time of diagnosis.
• Continued discussions regarding advance directives as well as methods of respiratory and nutritional support are critical; palliative care options should also be presented and discussed prior to the need for their initiation.