Part 3 · Treatment & care
Swallowing and feeding tubes
Weak mouth and throat muscles make swallowing slow and less safe, and weight falls. Scrub the timeline to watch one swallow repeat as the muscles weaken, with weight and FVC on the same months. Choose when the feeding tube (gastrostomy) is placed and how, and see what the ProGas study found.
Schematic, not to scale, and not one person's course: the weight and FVC curves and the energy bars are illustrative shapes. Shaded window: from 5% weight loss (a Canadian trigger for nutritional intervention) to FVC 50% (the EFNS wording as quoted by ProGas and Dorst); NICE asks for discussion earlier and at regular intervals. FVC risk bands are those of the AAN 2009 nutrition algorithm. ProGas figures are from a non-randomised cohort. 20,145,332,401,431,470
Gastrostomy timing, guideline by guideline
Published guidance, quoted as written. Every document asks for the tube to be discussed early, and none sets a single moment that applies to every person. 20,84,332,401,470,506
| Document | When the tube is raised or placed | Breathing and the procedure |
|---|---|---|
| AAN 2009 | Discussion after symptom progression or continuing weight loss; no ALS-specific evidence on timing (Level U). PEG "does not eliminate oral feeding". 332 | Risk graded by FVC: above 50% low, 30-50% moderate (anaesthesia review, experienced endoscopist, respiratory support), below 30% high. 332 |
| EFNS 2012 | Before respiratory insufficiency develops (abstract); as quoted by ProGas and Dorst, after 10% or more weight loss from premorbid weight. 20,145,431 | Before VC falls below 50% predicted (quoted second-hand; full text not read). 84,145 |
| NICE NG42 | Discuss early and at regular intervals, explaining the benefits of early placement and the risks of late placement; avoid delay once referred, and accept that some people will not want one. 401 | Late placement risks include low body mass, respiratory complications, dehydration and higher mortality. 401 |
| ESPEN 2018 | Before severe weight loss and before respiratory function is severely impaired. 84 | PEG generally preferred; RIG by an expert team suggested in frailer people or with moderate to severe respiratory impairment. 84 |
| Canada 2020 | Triggers for nutritional intervention, including tube referral: 5-10% or more weight loss, a fall of 1 BMI point or more, or BMI below 18.5; insertion within 4 weeks of the decision. 470 | FVC approaching 50% should prompt referral; FVC below 50% does not necessarily rule a tube out. 470 |
| EAN 2024 | NG feeding may be considered while waiting; parenteral feeding if a gastrostomy is not feasible. 506 | With respiratory insufficiency, NIV is started first and the gastrostomy done with the person established on NIV. 506 |
Weight, energy and diet
Weight loss and survival
- In 92 people in Limoges, a 5% loss from usual weight at diagnosis carried a 30% higher risk of death (RR 1.30, 95% CI 1.08-1.56); each further 5% lost during follow-up raised it by 34%, and each BMI unit lost by 24%. 321
- ESPEN summarises cohort data: at diagnosis each BMI point lost raised the risk of death by 9-23%, more than 5% weight loss roughly doubled it (HR 1.92), and 10% loss raised it by 45%. 84
- In 55 people, 16.4% were malnourished, with a 7.7-fold higher risk of death; in 79 Japanese people a BMI fall of 2.0 kg/m2 a year or more predicted shorter survival. 141,467
- ESPEN suggests aiming for weight gain if BMI is below 25, stability at 25-35 and loss above 35 (good-practice points). 84
Hypermetabolism
- Measured resting energy use was above the predicted value in 47.5% of 61 people, and 80% kept the same metabolic status over time. 71
- Hypermetabolism was found in 41% of 58 people with ALS against 12% of matched controls (adjusted OR 5.4), with faster ALSFRS-R decline (−0.68 against −0.39 points a month) and a higher risk of death (HR 3.2). 485
- The survival link is disputed: Bouteloup 2009 found no correlation, and ESPEN cites a study with no association. 71,84
- ESPEN estimates needs at about 30 kcal/kg a day without ventilation and 25-30 kcal/kg on NIV, which lowered resting energy use by 7% in one study. 84
What the nutrition guideline recommends
- Screen for malnutrition and dysphagia at diagnosis and about every 3 months; videofluoroscopy, videofluoromanometry and FEES can detect early dysphagia. 84
- Texture adaptation and postures such as chin tuck in moderate dysphagia (good-practice points). 84
- Supplements when needs are not met by an enriched diet, while stating there is insufficient evidence that oral supplements improve survival. 84
- Reports PEG placed under NIV: in one study 35 people with FVC below 50% had survival similar to 40 above 50%; spirometry can underestimate breathing in bulbar weakness. 84
Does the tube prolong life?
- The 2023 Cochrane review found no randomised or quasi-randomised trials comparing tube feeding with continued oral feeding, and judged such trials unlikely for ethical reasons. 487
- AAN 2009 graded PEG Level B to stabilise weight and prolong survival, from 2 Class II and 7 Class III studies; 4 Class III studies found no survival benefit. 332
- ESPEN reports that EFNS found no convincing survival evidence. 84
- A 2013 meta-analysis suggested RIG or PIG might be safer than PEG; the larger, prospective ProGas found similar 30-day mortality, but its groups differed in FVC and weight loss. 431,483
High-calorie diets
Phase 2, double-blind, 24 people already tube-fed (20 started). The high-carbohydrate hypercaloric group had fewer serious adverse events (0 against 9) and no deaths against 3 of 7 controls over 5 months (p=0.03); the authors called this preliminary. 539
Early evidence201 people, a 405 kcal/day fatty supplement or placebo with riluzole: survival probability at 28 months 0.37 against 0.39 (HR 0.97, p=0.44), no benefit overall. 304
Established: no overall benefitA survival benefit in fast progressors was found only in a post hoc analysis (effect size not read). 304
HypothesisAn ultra-high-calorie fatty diet against placebo, survival as the primary outcome, in people with disease under 24 months and ALSFRS-R loss of 0.33 points a month or more; 399 enrolled, active and not recruiting, estimated completion April 2028. EAN 2024 makes no recommendation specific to high-calorie diets. 297,506
Texture modification and IDDSI
- The IDDSI framework (released November 2015) grades drinks and foods on an 8-level continuum, 0 to 7, each level with a number, text label, colour code, definition and a way to test it. 113
- It was built from surveys of 2050 respondents in 33 countries and 3190 in 57 countries. 113
- Videofluoroscopy may identify textures a person can handle; in a FEES cohort, 44% refused thickeners or PEG. 332,408
Background: IDDSI is a shared vocabulary for textures, not a treatment, and it is used across conditions, not only in ALS.
Symptoms around the mouth, throat and muscles
Published guidance and trial results, compressed. Doses are as published, not instructions. Living with ALS covers pain, fatigue, sleep and mood. 401,506
| Symptom | What guidelines list | Trial and label evidence |
|---|---|---|
| Sialorrhoea (drooling) | NICE: an antimuscarinic first line (off-label in 2016), glycopyrronium first with cognitive impairment, botulinum toxin A as first or second line; Xeomin is recommended in NICE TA605. EAN 2024: anticholinergics first, dextromethorphan-quinidine especially with emotional lability, botulinum toxin when drugs fail, then radiotherapy. AAN 2009: botulinum toxin B for refractory cases (Level B). 333,401,506 | Normal saliva output is 0.5-1.5 L a day; drooling in MND comes from poor clearance, not excess saliva. Cochrane 2022 (4 RCTs, 110 people): low to moderate certainty for botulinum toxin B, moderate for dextromethorphan-quinidine. The Xeomin label: 100 units per adult session (30 per parotid, 20 per submandibular gland), no sooner than every 16 weeks. 179,247 Early evidence |
| Thick secretions | NICE: review medicines (especially saliva treatments), hydration, posture and oral care; consider humidification, nebulisers and carbocisteine. EAN 2024 adds beta-blockers (propranolol, metoprolol) and mucolytics (acetylcysteine, guaifenesin). 401,506 | Secretion problems are reported by 40-60% of people with ALS. For cough assistance see Breathing and NIV. 60 |
| Pseudobulbar affect (uncontrollable crying or laughing) | EAN 2024: SSRIs, tricyclics or dextromethorphan-quinidine, after telling it apart from depression and FTD. AAN 2009: dextromethorphan-quinidine Level B "if approved by the FDA". 333,506 | RCT in ALS (140 people, 28 days): dextromethorphan 30 mg plus quinidine 30 mg twice daily improved CNS-LS by 3.3 points more than dextromethorphan alone. STAR (326 people with ALS or MS): episodes 46.9-49.0% lower than placebo. The Nuedexta label: 20 mg/10 mg capsules, one daily for 7 days then every 12 hours. 76,174,423 Established |
| Cramps | NICE: quinine first, then baclofen, then tizanidine, dantrolene or gabapentin. EAN 2024: sodium-channel blockers (ranolazine, quinine, mexiletine, carbamazepine), gabapentin, pregabalin or baclofen, with low-dose quinine (100-200 mg/day) and cardiac monitoring. Canada: mexiletine second line. 401,470,506 | Mexiletine (60 people, 12 weeks): cramp frequency 31% of placebo at 300 mg/day and 16% at 900 mg/day, with 32% stopping the higher dose; a 20-person crossover found 5.3 to 3.5 cramps a day. Cochrane 2017: memantine and THC probably ineffective. 398,411,531 Early evidence |
| Spasticity | NICE: baclofen, tizanidine, dantrolene or gabapentin, and a specialist service for severe spasticity. EAN 2024 adds physiotherapy, cannabinoids and focal botulinum toxin. Canada: insufficient evidence for intrathecal baclofen. 401,470,506 | The only RCT in the Cochrane review tested endurance exercise in 25 people, too small to show benefit or harm; no spasticity drug has an included RCT in ALS. 398 |
Where the sources disagree
The FVC 50% rule. AAN (one Class III study) and EFNS link higher risk to FVC below 50%, but ESPEN, Canada and EAN cite procedures under NIV, RIG or PIG with similar outcomes below 50%, and note that spirometry is unreliable in bulbar weakness. 84,332,470,506
PEG, RIG or PIG. A 2013 meta-analysis suggested RIG and PIG might be safer; ProGas found similar 30-day mortality, but it was not randomised and its groups differed in FVC and weight loss. 431,483
Hypermetabolism and survival. Steyn 2018 linked it to shorter survival; Bouteloup 2009 did not. 71,84,485
Multidisciplinary clinics (observational evidence). Attending a specialist clinic was linked to longer survival in Ireland (median 7.5 months longer; HR 0.59 across Ireland and Northern Ireland) and better mental quality of life in the Netherlands, but Cochrane found no RCT, so the evidence is observational. 398,454,498,507