Table 1.

ALS management

Indication	Treatments and interventions*
Disease-modifying treatments
Disease progression	Only two drugs with regulatory approval are available, riluzole and edaravone. They are of marginal efficacy and only in select populations and merely lengthen survival by a few months;(116–118) however, even within select populations, the efficacy of edaravone is contested.(119, 120)
Symptomatic management
Comprehensive care	A multidisciplinary clinic plans the comprehensive, multidisciplinary care needed to manage symptoms in ALS patients. Care spans managing respiration and oral symptoms (speech, swallowing) nutrition and gastrointestinal symptoms, pain and symptoms secondary to muscle loss, and cognition, mood and behavioral changes.
Respiratory and oral symptoms
Bronchial secretions	Cease provoking medications. Administer mucolytics if the patient exhibits sufficient cough flow, which includes N-acetylcysteine, anticholinergic bronchodilator, β-receptor antagonist and nebulized saline, furosemide, and guaifenesin. Mechanical or non-pharmacological approaches are also available, which include manual assisted cough, mechanical insufflator-exsufflator, portable home suction device, and room humidifier. Additionally, patients are encouraged to remain hydrated or drink pineapple or papaya juice to break up secretions.
Dysarthria	Evaluate speech-language regularly and identify language impairments. Provide assistive communication tools, such as electronic writing, voice banking, and voice amplification devices.
Dyspnea	Options include elevating the head of the bed, use of a hospital bed for elevation, noninvasive ventilation, and invasive tracheostomy ventilation.
Sialorrhea	Administer anticholinergics, such as amitriptyline, atropine ophthalmic drops, glycopyrrolate, scopolamine patch. If sialorrhea is refractory to anticholinergics, botulinum toxin injections, external beam radiation therapy, and surgery may be considered. A portable suction device is a less aggressive approach. Dark grape juice and ginger tea are reported to decrease saliva production.
Nutrition and gastrointestinal symptoms
Constipation	Increase fluid and fiber intake or adjust enteral nutrition. Administer an osmotic or stimulant laxative. Increase physical activity.
Dysphagia	Evaluate speech-language pathology regularly and provide swallowing technique education. Gastrostomy feeding tube placement by percutaneous endoscopic gastrostomy, radiologically inserted gastrostomy, or per-oral image-guided gastrostomy. Ideally, gastrostomy placement should occur early in the disease course prior to significant weight loss.
Nutrition	Dietary evaluation by a nutritionist and modify food and fluid consistency accordingly. Administer appetite stimulants and/or high-protein/high-caloric supplements.
Pain and symptoms secondary to muscle loss
Cramps	Administer magnesium, levetiracetam, mexiletine, or quinidine (controversial). Non-pharmacological approaches span physiotherapy, exercise, massage, and hydrotherapy. Hydration with tonic water.
Joint pain	Administer analgesics, including acetaminophen and nonsteroidal anti-inflammatory drugs. Non-pharmacological approaches span physical therapy, especially to limit joint contractures, and repositioning, pressure relief, mechanical support for weak limbs.
Spasticity	Administer spasmolytic drugs (tizanidine, baclofen, intrathecal baclofen pump), benzodiazepines, dantrolene, or botulinum toxin injections. Tetrahydrocannabinol is a novel direction. Non-pharmacological approaches include stretching and range of motion physical therapy.
Venous thromboembolism	Administer anticoagulants. Non-pharmacological preventive approaches span physiotherapy, limb elevation, and compression stockings.
Weakness	To address upper limb weakness, occupational therapy for adaptive equipment and hand splints. To address lower limb weakness, physical therapy, which includes transfer and gait assessments. Fall prevention. Various aids, such as ankle-foot orthoses, Hoyer lift, walker, transport wheelchair, and powered wheelchair.
Mood
Depression and anxiety	Administer antidepressants or anxiolytics. Non-pharmacological approaches span psychotherapy and counseling.
Fatigue	Address related symptoms (e.g., sleep disturbances, depression, cramps, pain, respiratory distress). Encourage patients to conserve energy.
Insomnia	Address related symptoms (e.g., depression, cramps, pain, respiratory distress). Monitor sleep hygiene and address the role of noninvasive ventilation.
Pseudobulbar affect	Administer tricyclic antidepressants, selective serotonin reuptake inhibitors (fluvoxamine, citalopram), or dextromethorphan-quinidine. Educate patients that these are ALS symptoms.
Care planning
Cognitive dysfunction	Encourage early discussions regarding treatment goals while the patient still retains decision-making capacity. Educate family and caregivers. Respite care.
Palliative and end-of-life care	Administer benzodiazepine and/or opioids for symptomatic dyspnea or intractable pain. Encourage early conversations with patients, family members, and caregivers regarding treatment choices. Palliative care referral can occur early in the disease course with hospice care at end of life.

Sources: EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis,(62) The American Academy of Neurology,(111–113) the United Kingdom National Institute for Health and Care Excellence (NICE),(108) ALS Canada(109) and other Canadian guidelines,(114) and Bradley and Daroff’s Neurology in Clinical Practice.(115) These therapies, in most cases, represent good clinical practice as few clinical trials involving patients with ALS exist to provide a robust evidence base for these interventions.