COVID-19 ALS and frontotemporal dementia

Amyotrophic Lateral Sclerosis and COVID-19 – Recommendations to patients and caregivers

In patients with Amyotrophic Lateral Sclerosis (ALS), the impact of respiratory infections is expected to be more serious than in the general population, due to the weakness of the respiratory muscles and limitations in coughing

In patients with Amyotrophic Lateral Sclerosis (ALS), the impact of respiratory infections is expected to be more serious than in the general population, due to the weakness of the respiratory muscles and limitations in coughing (1-4). ALS patients with impaired respiratory function and/or rapid clinical deterioration are considered a high risk population for COVID-19 (5), even if no estimates in the different European countries are available.

ENCALS centres and the EAN panel on ALS and Frontotemporal Dementia are working together across countries to optimise support for our patients to protect them during this pandemic. National guidelines specifically concerning patients with ALS can be lacking and current regulations vary between different European countries. Available National guidelines are as follows, some of them addressing the general population:

In the absence of guidelines specifically written to protect ALS patients and their families/ carers during the COVID-19 pandemic period, and acknowledging the various national regulations the consensus of a European Network for the Cure of ALS (ENCALS) and EAN ALS/FTD group of experts* is as follows:

Recommendations to ALS patients and family members/ carers, during the pandemic period of COVID-19 (SARS-CoV-2):

1st recommendation: Patients with ALS should be confined to their homes to prevent becoming infected. If living in a house with a backyard or in the countryside, going out inside the limits of the residence does not present a potential danger. It is advisable to avoid visitors, including family members/young children, even if they are without any symptoms. Hygiene should include more frequent hand washing with soap, avoiding hand-to-face contact (mouth, nose and eyes) and cleaning surfaces with detergent. (18) Carers should sneeze/ cough into the bent elbow or tissue, the tissues should be immediately discarded, and hands should be washed with soap. (18) Check the need to use masks by the patient/ family members/ others contacting the patient with the national authorities of your country and your physician (19-21). When using a mask, best practices should be followed about how to wear, remove, and dispose of it, and for hand hygiene after removal (21).

2nd recommendation: Identification of the principal carer: one principal carer should coordinate care provided to the patient. The principal carer should be someone who is always at home with the patient, in accordance with recommendation 1, to prevent being infected and transmitting the disease to the patient. Food and medication should be bought through online platforms or by ordering home delivery at local shops. Check the need to use masks with the national authorities of your country and the patient’s physician (19-21). When using a mask, best practices should be followed about how to wear, remove, and dispose of it, and for hand hygiene after removal (21).

3rd recommendation: Identification of additional carers: If possible, a second carer should be identified. If necessary, this carer should be available to cover for the principal carer, to prevent leaving the patient alone during unplanned periods, including due to quarantine/ isolation of the principal carer or others. Check the need to use masks with the national authorities of your country and the patient’s physician (19-21).

4rd recommendation: Unnecessary care/ Unavailable carer: If self sufficient but living alone without support, or if no carer is available although needed, the patient is advised to contact the ALS care centre, namely the coordinator or social worker who integrates the ALS team, to report the social situation so that support can be arranged if it becomes necessary.

5th recommendation: Outpatient care services: If support is provided by outpatient care services, a principal carer and an additional carer should be identified for each patient, limiting external contacts to avoid the risk of spreading the infection. This team, if not provided directly by the ALS team, should keep close contact with the ALS team to report on potential needs/ clinical deterioration/ respiratory emergency. The team should take all necessary measures to prevent possible transmission of the infection (20).

6th recommendation: In the case of an unplanned period of physical absence from the principal carer, and if no additional carer is available, the principal carer or the patient him/herself if capable, should report the situation to the ALS health providers, namely the social assistants integrating the ALS teams, local or national ALS association, palliative care team, homecare institution, local fireman association, or others that support community services.

7th recommendation: The principal carer is responsible for monitoring and providing, in the presence of functional limitations/ dependence and/or fronto-temporal dementia, for basic needs of the ALS patient including feeding/ hydration, administration of medication, respiratory support, mobilization and positioning, hygiene, comfort, physical and psychological wellbeing. The principal carer should also take care of him/herself, to maintain physical and psychological well-being. It is important to keep timetables, listen to calm and relaxing music, read books, engage in interesting activities at home, and have some periods for home physical exercise, if possible. Constant following of the news may be avoided.

8th recommendation: If in absolute need to go outside, both patient and carer/ family member should take the advised measures to minimise any risk of infection. Check the need to use masks by the patient, carer/ family member with the national authorities of your country and your physician (19-21). When arriving home, take off clothes (coats, bags, scarves, gloves, shoes) at the entrance to the house. Use disinfectant gel containing alcohol. Take off the mask, by only touching the elastic. Wash the hands with disinfectant products containing alcohol (18). If the patient uses walking aids/ wheelchair, these devices should be left also in the entrance of the house. The carer should help the patient. The walking aids/ wheelchair as well as other surfaces, including at the entrance, where the clothes from the outside are gathered, should be disinfected with detergents or products containing alcohol (22). Clothes should be washed with regular laundry detergent at temperatures above 40º Celsius.

9th recommendation:

If the patient develops fever, a non-productive cough, headache, muscle pain or dyspnoea, in the context of an external contact with someone who could be infected, the patient/ the principal carer should: 1- Stay calm. Do not go to the hospital or other healthcare centres. Stay at home; 2– Both use masks (18,21); 3- Measure the temperature and write down symptoms. Maintain a medication list, including dosing and administration times; 4- Contact the physician or medical team that follows you/ the patient, by phone, mail or available internet platforms.; 5- If no contact is available, phone the national healthcare line, or the COVID-19 line if available in your country. If nobody answers, ring again. Follow instructions. If the patient has already been adapted to non-invasive ventilation, a face mask without expiratory holes (or with the expiratory holes closed with tape), connected directly to a filter and to an expiratory piece should be used, to filter the expiratory air. In case of tracheostomy, a filter and the expiratory piece should be connected to the tube. If assistance is needed to remove bronchial secretions, for example by using a mechanical insufflator-exsufflator device, the carer should use FFP2 mask, goggles, gloves and gown, discard all biological material immediately and have a shower afterwards. As the coronavirus can be detected for at least 3 hours after aerosolization, mask and goggles should be used (22).

If the carer/family member is the one developing the symptoms: 1- Stay calm. Do not go to the hospital or other healthcare centres. Stay home; 2-Use masks (both) (18,21); 3- Record your symptoms as well as any symptoms that the patient may have. Have an actualized medication list; 4- Contact the additional carer so that he/she can be prepared to substitute if indicated when phoning the health providers; 5- Contact the COVID-19 line/ the national healthcare line as well as the ALS physician who follows the patient. Follow the instructions.

10th recommendation: In the case of respiratory decompensation, in ALS patients under home ventilatory support or initial respiratory symptoms in the context of ALS, the patient or the caregiver should contact the homecare/ palliative team/ caring ALS centre and inform the physician who regularly cares for the patient. If needed, call the 24/7 health support lines that may have been provided and link with the ALS care centre’ss support. Follow instructions.

11th recommendation: In case of respiratory emergency, first call the neurologist and respiratory physician in charge to finalize the required intervention. If needed, call the 24/7 health support lines that may have been provided and articulate it with the ALS care centres support.

12th recommendation: Keep well informed, by reading the news/ listening to reliable sources. Avoid fake news and misleading social media. Rely on official sources from your country. However limit the time that you spend listening to or reading the news. Be positive. This is a transitory period and it will end.

The present recommendations will be reviewed whenever pertinent, to adjust to the constant evolving evidence generated during the COVID-19 pandemic.

Andrà tutto bene. Vai ficar tudo bem. Wszystko będzie dobrze. Allt ska bli bra. Everything will be alright.


*ENCALS/ EAN expert panel

Susana Pinto (lead and draft of the recommendations)
Translational and Clinical Physiology Unit, Instituto de Medicina Molecular,
Faculty of Medicine, University of Lisbon
Lisbon, Portugal

Vincenzo Silani
Department of Neurology-Stroke Unit and Laboratory of Neuroscience
Department of Pathophysiology and Transplantation
Center for Neurotechnology and Brain Therapeutics
Università degli Studi di Milano
Milan, Italy

Adriano Chiò
Ferdinando Rossi School of Advanced Studies of Turin
ALS Regional Expert Center of Turin
Rita Levi Montalcini Department of Neuroscience, ALS Center
Torino, Italy

Andrea Calvo
Azienda Ospedaliera Città della Salute e della Scienza,
Rita Levi Montalcini Department of Neuroscience, ALS Centre
University of Torino
Turin, Italy

Francois Salachas
Paris ALS centre, Department of Neurology
Hôpital de la Pitié-Salpêtrière
Paris, France

William Camu
ALS Center, CHU Gui de Chauliac,
University of Montpellier
Montpellier, France

Susanne Petri
Department of Neurology,
Hannover Medical School
Hannover, Germany

Julian Grosskreutz
Hans-Berger Department of Neurology
Jena University Hospital
Jena, Germany

Magdalena Kuzma-Kozakiewicz
Department of Neurology
Neurodegenerative Disease Research Group
Medical University of Warsaw
Warsaw, Poland

Philip van Damme
KU Leuven – University of Leuven, Department of Neurosciences, Experimental Neurology and VIB Center for Brain & Disease Research and University Hospitals Leuven,
Department of Neurology
Leuven, Belgium

Orla Hardiman
Academic Unit of Neurology, Trinity Biomedical Sciences Institute,
Trinity College Dublin
Dublin, Republic of Ireland

Markus Weber
Neuromuscular Diseases Center/ALS Clinic,
Kantonsspital St. Gallen
St.Gallen, Switzerland

Pamela Shaw
Sheffield Institute for Translational Neuroscience and Sheffield NIHR Biomedical Research Centre for Translational Neuroscience and Neuroscience Research Institute,
Sheffield, UK

Ammar Al-Chalabi
Maurice Wohl Clinical Neuroscience Institute, Department of Basic and Clinical Neuroscience, King’s College London*
London, UK

Mamede de Carvalho
Translational and Clinical Physiology Unit, Instituto de Medicina Molecular Faculty of Medicine, University of Lisbon
Neuroscience Department, Hospital de Santa Maria,
Centro Hospitalar Lisboa Norte
Lisboa, Portugal



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