ALS / Motor Neurone Disease

What is ALS / MND?

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neurone disease, a group of progressive neurological conditions in which motor neurones — the nerve cells that control voluntary muscle movement — degenerate and die. As motor neurones stop functioning, the muscles they control weaken, waste, and eventually stop working.

ALS is fatal in most cases; the average survival is 2–5 years from symptom onset, though about 10% of people live more than 10 years. Stephen Hawking, who lived with ALS for over 55 years, is the most celebrated long-term survivor.

ALS affects approximately 5 in 100,000 people worldwide and is slightly more common in men. It typically presents in people between 40 and 70 years of age. Around 90–95% of cases are "sporadic" (no clear genetic cause); 5–10% are familial (inherited).

How It Presents

ALS progresses through the motor system while typically leaving cognition, sensation, and personality intact:

• Early symptoms — weakness in one hand, foot, or leg; slurred speech; fatigue during physical tasks
• Progressive spread — weakness spreads through the body; muscles in the arms, legs, and trunk progressively weaken
• Speech and swallowing — dysarthria (slurred speech) and dysphagia (difficulty swallowing) develop; nutritional support (PEG tube) may be required
• Breathing — respiratory muscles weaken; ventilatory support (non-invasive or invasive ventilation) eventually required
• Cognitive involvement — about 5–15% of people with ALS develop frontotemporal dementia (ALS-FTD); some degree of cognitive change is more common

The loss of communication and physical independence while cognition remains intact creates profound AT needs.

Assistive Technology

AT is central to quality of life in ALS:

• Voice banking — capturing a person's natural voice before significant speech loss, to create personalised synthesised speech for SGDs. Tools include ModelTalker, VocaliD, and ALS TDI's projects. This must be initiated early.
• AAC and Speech Generating Devices — as speech deteriorates, AAC devices become the primary communication tool; eye-gaze systems (Tobii Dynavox, Eye Tribe) provide access when hands can no longer operate devices
• Switch access — for controlling computers, environment, and communication devices with progressively minimal movement
• Environmental control systems — enabling control of home devices (lights, TV, doors) by voice, switch, or eye gaze
• Power wheelchairs — often with head array or breath controls as upper-body function declines
• Non-invasive ventilation (BiPAP/CPAP) — respiratory support that also buys time for voice banking
• Augmented reality and brain-computer interfaces (BCI) — emerging technologies being researched for communication access with very limited motor function

Common Misconceptions

• "People with ALS lose their minds." In most cases, ALS spares cognition entirely. People with ALS remain fully intellectually capable of making decisions, engaging in relationships, and pursuing interests.
• "ALS always progresses the same way." Rate of progression and pattern of symptom development varies considerably; bulbar-onset ALS (starting with speech/swallowing) and limb-onset ALS have different trajectories.
• "There is nothing you can do once diagnosed." While ALS has limited disease-modifying treatments, proactive AT planning — especially early voice banking and AAC — dramatically affects quality of life.

Language and Identity

Person-first language is most common ("person with ALS"). Many in the ALS community have become passionate advocates for disability rights, research funding, and AT access — demonstrating that a terminal prognosis does not diminish advocacy or identity.