Living with ALS

How common it is

In a population-based Irish cohort of 160 people with newly diagnosed ALS, 13.8% met criteria for frontotemporal dementia (FTD). Another 34.1% of those without dementia met consensus criteria for cognitive impairment, and 46.9% had no detectable cognitive abnormality. ⁴²²

In the Piemonte (Italy) population-based series of 183 people, 49.7% were cognitively normal and 12.6% had ALS-FTD. The authors read this as about half having some cognitive impairment. ³⁴⁵

The 2017 revised criteria describe a spectrum, ALS-frontotemporal spectrum disorder (ALS-FTSD), and state that deficits affect "over 50%" of people with ALS. ⁴⁸⁶

The figures depend on the criteria and tests used, so they are not directly comparable between studies. ^345,422,486

Why it matters for survival

In the Irish cohort, FTD was associated with shorter survival (HR 2.67, 95% CI 1.04-6.85). In people without dementia, executive dysfunction independently predicted shorter survival (HR 3.44, 95% CI 1.45-8.18). ¹⁵⁶

In a Torino cohort of 128 people, 32.0% had neurobehavioural dysfunction on the family-rated FrSBe. Median survival was 3.3 years with dysfunction and 4.3 years without. ¹⁰⁸

An Irish population-based study of 317 people found no association between behavioural category and survival (P = 0.198). ⁸⁵

Which behaviours change

On the Beaumont Behavioural Inventory (317 people), 57% had no behavioural change, 30% mild-moderate change and 13% clinically severe change. The most frequent items were reduced concern for hygiene (36.8%), irritability (36.2%), new unusual habits (33.4%) and increased apathy (31.1%). ⁸⁵

On the FrSBe (225 people with sporadic ALS), 39.6% had impairment in at least one domain, and apathy was the most common (31.1%). ⁵⁴¹

So apathy is the commonest change on one scale (FrSBe) but ranks fourth by item frequency on another (BBI). The answer depends on the instrument. ^85,541

In a longitudinal study of 49 people, initiation apathy was the most common subtype at baseline (38.8%), and it increased over time. ⁴⁴⁰

Screening, as published

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) takes 15-20 minutes and was designed for people with physical disability. Its ALS-Specific score covers executive function, social cognition, fluency and language; its ALS Non-specific score covers memory and visuospatial function; a carer interview covers five behavioural domains. ²

NICE recommends exploring cognitive or behavioural change at diagnosis and when concern arises, and advises awareness that people with MND and FTD may lack mental capacity for some decisions. The AAN 2009 practice parameter says screening should be considered (Level B). ^333,401

Mood

Self-report scales with bodily (somatic) items, such as the BDI, give much higher depression figures than structured interviews or the HADS. The spread from about 10% to 50% reflects method as much as mood. ^234,277

In 80 hospice-eligible people with late-stage ALS interviewed monthly, 81% had no depressive disorder at baseline, 57% never had a depression diagnosis at any visit, and depression did not increase as death approached. ⁴³⁸

In 433 people without dementia, clinically significant state anxiety was found in 18.2% and trait anxiety in 13.9%, driven mainly by depressive features and independent of motor status. ⁴

Quality of life

In 96 people with ALS, self-rated overall quality of life did not correlate with strength or physical function; it appeared to depend on psychological, existential and spiritual factors. ⁴⁷³

Followed over 6 months, quality of life was maintained as physical function declined. ⁴⁵⁰

In 80 consecutive people, perceived quality of social support was the most important explanatory variable, and physical status was not. ¹⁰²

In 19 people in a locked-in state who answered by eye-tracking, most reported a positive quality of life and no clinically relevant depression, and caregivers underestimated their well-being. ²⁷⁸

The "disability paradox" describes many people with serious disabilities reporting good quality of life; the original study was not specific to ALS. ⁷

Suicide risk

In a Swedish population-based cohort of 6,642 people with ALS (1965-2004), suicide risk was almost six times that of the general population (SMR 5.8, 95% CI 3.6-8.8), with 21 suicides observed against 3.6 expected. Risk was highest in the first year after first hospitalisation (SMR 11.2). The study was published in Brain. ¹⁶⁷

In a Korean nationwide cohort of 2,955 people, 47 attempted suicide; 42.6% of attempts came within 3 months of diagnosis, and prior psychiatric hospitalisation was linked to about three times the risk (HR 3.17). ²⁶¹

Absolute numbers are small. Support is available through the person's care team.

Driving (UK rules as an example)

In the UK a person with MND (also known as ALS) must notify the DVLA, and failing to report a condition that affects driving can bring a fine of up to GBP 1,000. Car and motorcycle (Group 1) drivers may continue as long as safe vehicle control is kept at all times, with a licence of 1, 2, 3 or 5 years that may require adapted controls. Bus and lorry (Group 2) licences are refused or revoked if the condition is progressive or disabling. ¹⁵⁰

Driving rules differ widely between countries, and a 2026 review argues that cognitive, behavioural and breathing changes, not only weakness, affect driving safety. ²⁶⁶

In a small Norwegian study of 31 people, 61% were driving within 4 months of diagnosis, and baseline cognition did not predict who had stopped 4 months later. ⁴⁹¹

Work, money and support

NICE lists information to give at diagnosis, including social care support, employment rights, benefits and duties such as notifying the DVLA. Psychological and social support should include financial advice, support to keep engaging in work and hobbies, and respite care. ⁴⁰¹

Home and equipment

NICE recommends prompt assessment for funding of home adaptations, and joining up equipment such as communication aids and environmental controls with wheelchairs. ⁴⁰¹

Pregnancy

A systematic review found 38 published cases of ALS overlapping with pregnancy. Progression during pregnancy was rapid or severe in 55%, 95% completed the pregnancy with a live birth (preterm delivery was common) and 86% of newborns were healthy. These are case reports, open to publication bias. ²¹⁶

Multidisciplinary clinics

In the Irish ALS register (1996-2000), people who attended the multidisciplinary clinic had median survival 7.5 months longer than those seen in general neurology clinics. ⁴⁹⁸ Across Ireland and Northern Ireland (2005-2010), clinic attendance was associated with longer survival (HR 0.59, 95% CI 0.49-0.71). ⁴⁵⁴

The clinic group was about five years younger and more often took riluzole (99% vs 61%), so part of the gain may reflect who attends. ⁴⁹⁸

NICE recommends coordinated team assessments, usually every 2 to 3 months, and a face-to-face follow-up within 4 weeks of diagnosis. The AAN says referral to a multidisciplinary clinic should be considered (Level B). ^333,401

A palliative approach from diagnosis

A review of international guidelines states that optimal ALS care needs a palliative approach from diagnosis, and notes that late referral to palliative services is common. ⁴⁵

NICE: offer the chance to discuss end-of-life preferences at diagnosis, at a significant change in breathing, and when gastrostomy or NIV is needed, and be ready to discuss these whenever the person wishes. ⁴⁰¹

What planning covers

NICE lists how death may occur, anticipatory medicines at home, Advance Decisions to Refuse Treatment, DNACPR orders and Lasting Power of Attorney. It advises discussing these earlier if communication, cognition or capacity is expected to worsen. ⁴⁰¹

The EFNS 2012 guideline says advance directives should be discussed early, respecting social and cultural background. ²⁰ The EAN 2024 guideline adds invasive ventilation as part of planning (aiming to avoid emergency invasive ventilation), palliative sedation and withdrawal of treatment, and a recommendation to be prepared to discuss euthanasia and assisted suicide. ⁵⁰⁶

How often it happens

A 2024 meta-analysis of 26 studies found signed advance directives in 60.4% of records, lower in 2012-2024 studies (42%) than in 1999-2011 studies (78%). ³¹⁷

At one US academic ALS centre (513 people), 30% had any advance care planning document and 5.1% saw a palliative care physician. ⁴⁸⁹

In a Scottish national cohort, advance care planning (OR 3.94) and specialist palliative care input for 28 days or more (OR 2.66) were associated with dying outside hospital. ⁴³⁶

Decisions about tubes and ventilation

NICE notes that some people will not want a gastrostomy, and advises explaining the benefits of early placement and the risks of late placement. It recommends regular chances to talk about continuing or withdrawing NIV. ⁴⁰¹

In an Australian service, 73% of 33 people recommended a gastrostomy accepted it, after decisions that took days to months. ²⁸¹

Living longer

In a population-based Lombardy cohort of 483 people, 76.2% were alive 1 year after diagnosis, 23.4% at 5 years and 11.8% at 10 years. Younger age, spinal onset, male sex and a less certain diagnostic category at diagnosis predicted longer survival. ⁴³³

How people with ALS die

In a study from Munich (relatives of 121 people) and a UK hospice (records of 50 people), most died peacefully (Germany 88%, UK 98%), and no patient choked to death. The commonest symptoms in the last 24 hours were breathlessness, coughing, anxiety and restlessness. About half died at home (55% and 52%). ³⁹¹

In a French prospective study of 302 deaths, respiratory failure was the most frequent reported cause (77%), and 63% died in a medical facility. ²⁰⁴

In 100 autopsies in Paris, clinical and pathological causes of death agreed in only 20%; pneumonia was the main cause at autopsy. ¹¹⁸

Easing symptoms

NICE advises considering opioids to relieve breathlessness, and benzodiazepines when anxiety makes breathlessness worse (both off-label in February 2016), with prompt access to anticipatory medicines, specialist palliative care and bereavement support for families. ⁴⁰¹ The EAN 2024 guideline repeats these options. ⁵⁰⁶

In a 6-person pilot in terminal ALS, morphine lowered breathing rate and breathlessness scores without a significant rise in CO₂. ¹¹⁵

Morphine was used in the final phase in 27% (Germany) and 82% (UK) in the Neudert study. ³⁹¹

Stopping ventilation

In a UK evaluation of 46 ventilation withdrawals (42 NIV, 4 tracheostomy), 43% took place at home and 48% in a hospice, and 89% received opioid or sedative medicine at the start. People on tracheostomy ventilation died within 30 minutes; after NIV withdrawal the most common time to death was 15 minutes (range under 15 minutes to 54 hours). ¹⁷⁰

In Denmark, 12 people on invasive home ventilation chose to withdraw it, under deep sedation. ¹⁴⁶ In Italy, Law 219/2017 set out the right to request withdrawal of life-sustaining treatment including ventilation, and withdrawals increased afterwards. ³⁴⁰

Tracheostomy ventilation

In the Piemonte and Valle d'Aosta register (1995-2004), 10.6% of 1,260 people had a tracheostomy, with median survival of 253 days afterwards. ¹⁰⁶

In a Japanese multicentre registry (2006-2018), propensity-matched median survival was 11.33 years with tracheostomy ventilation and 4.61 years without. ²²³

Rates and survival after tracheostomy differ between countries, partly for sociocultural reasons, and the studies are not directly comparable. ^106,223

Place of death

US death certificates for 30,066 MND deaths showed home deaths rising from 45.5% before COVID-19 to 54.4% during the pandemic. ⁴⁴⁶

Among 77 ventilated people in five European hospitals, 57% died at home; the authors argue the conditions of dying may matter more than the place. ¹⁶⁵

Reading these figures

The Dutch figures run from 25% (all ALS deaths reported to the review committees) to 40% (a survey with a 49% clinician response rate). They measure different things. ⁵¹⁰

Oregon year totals are revised as late reports arrive: 2024 deaths were 376 in the 2024 report and 421 in the 2025 report. ⁴⁰⁶

Canadian national figures are not shown here because they could not be checked against the original report.

What guidelines say

The EAN 2024 guideline adds a recommendation to be prepared to discuss euthanasia and assisted suicide. ⁵⁰⁶

Across all conditions in Oregon, losing autonomy was the end-of-life concern reported most often (89.9%). ⁴⁰⁶

Who gives care, and how much

In an Irish cohort of 81 family caregivers, 70% were women, 72% were spouses or partners and 83% lived with the person with ALS. They gave an average of 47 hours of care a week (median 26.5). ¹⁹²

In a 1997 US survey, primary caregivers spent a median of 11 hours a day caring even with home care in place. ²⁷²

Burden

The mean Zarit Burden Interview score in the Irish cohort was 27.1 (SD 14.7), and 52% were in the high-burden range (24 or more). Burden was linked with hours of care, lower quality of life and psychological distress. ¹⁹²

In 140 Australian caregivers, 48% had high burden, and the person's behaviour change predicted it more strongly than physical disability. ²⁹² A 2018 systematic review found high-quality evidence linking burden to behavioural impairment. ¹⁴²

In 60 Italian caregiver-patient pairs, burden rose with worsening disability. The studies used different instruments and populations. ¹⁰³

After a death, and children

Of 16 spousal carers interviewed 1-4 years after the death, 6 met criteria for prolonged grief disorder; those 6 had accessed palliative care at a later stage. ²³

Children aged 7-18 with a close relative with ALS showed more negative affect and hyperactivity than controls, and research on this group is scarce. ⁴⁹³