Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder that affects the motor neurons in the cerebral motor cortex, brainstem, and spinal cord. The first description of ALS dates to the mid-19^th century, when it was reported as progressive muscle weakness accompanied by muscle atrophy [1]. ALS is not a rare disease, with an incidence of approximately 1-2.6 cases per 100,000 persons annually and a prevalence of approximately 6 cases per 100,000 [2]. Although the dominant features of ALS are motor symptoms—such as limb weakness, dysphagia, dysarthria, and respiratory insufficiency that ultimately lead to death—ALS is no longer considered a pure motor disorder. It is now perceived as a multisystem disease with various non-motor symptoms, including cognitive and behavioral changes, neuropsychiatric disturbances, sleep disruption, sialorrhea, hypermetabolism, bladder and bowel dysfunction, changes in bone health, olfactory and somatosensory impairment, and pain [3]. Although new disease-modifying agents, including gene therapies utilizing antisense oligonucleotides, are being actively developed [4], there is currently no cure for ALS, a disease for which palliative care is very important, similar to cancer [5]. However, unlike in cancer care, pain management has not received much attention in patients with ALS.

Pain impairs the quality of life and causes distress to patients and caregivers. Although there are differences among studies, up to 85% of patients with ALS experience pain [6], and a recent meta-analysis estimated the pooled prevalence of pain to be 60% [7]. Pain in ALS is mostly nociceptive, which means that it arises from non-neuronal tissue damage or activation of peripheral nociceptors in response to mechanical stress or other noxious stimuli [8]. Joint pain is common among patients with ALS, particularly in the shoulders and hips, and is often associated with muscle weakness and atrophy, leading to reduced joint support [8]. Lack of support can result in pain and discomfort. Additionally, immobility can lead to skin pressure, which may be perceived as pain in patients with ALS. In patients with ALS, primary pain occurs, including neuropathic pain and pain due to spasticity or cramps [8]. The sources of pain associated with ALS are summarized in Fig. 1, and the management according to its etiologies are summarized in Table 1 [8].

Table 1 . Interventions for the management of pain in people with ALS according to the etiology of pain.

	Self-management	Professional management
Muscle cramps	Massage, stretching	Quinine sulphate
	Aromatherapy	Magnesium
	Drink tonic water (contains quinine)	Vitamin E
	Bananas, oranges, and citrus fruit juices (to address magnesium imbalance)	Vitamin D
	Heat
Spasticity	Positioning	Physiotherapy
	Passive exercise	Tizanidine
	Active within limitations	Baclofen
	Heat	Hydrotherapy
	Pressure above and below joint	Botulinum toxin
	Stretching
Constipation	Review of dietary intake	Laxatives
	Review of fluid intake	Movicol
	Review of current medications	Senna and lactulose
	Exercise-passive	Dulcolax
	Regular review of bowel habits, monitor or change	Laxatives on commencement of opioids
	Abdominal massage
	Linseed seeds
Spasm	Exercise-passive	Baclofen
	Positioning	Dantrolene
		Diazepam
		Sublingual lorazepam
		Tizanidine
Skin pressure	Positioning	Pressure mattresses/cushions
	Regular turning	Appropriate beds/chairs
	Correct moving and handling techniques	Use of hoists
	Preventative measures	Diclofenac sodium
		Morphine
		Amitriptyline
Musculoskeletal	Exercise-passive prior to transfer for immobile individuals, ROM	Physiotherapy
	Preventative measures	Hydrotherapy
	Positioning	NSAIDs
	Correct moving and handling techniques	Paracetamol
		Tramadol
		Joint injections
		Morphine
		Orthotics, splints
		Collars
		TENS
		Complementary/alternative therapy

NSAIDs: non-steroid anti-inflammatory drugs..

Figure 1. Types of pain experienced in amyotrophic lateral sclerosis. NIV: non-invasive ventilation.

Although pain in ALS has attracted more research attention over the past decade, gaps remain in the development of pain assessment tools specific to patients with ALS, effective management strategies, and longitudinal research exploring pain progression changes over time in this population.

Advances in cancer treatment have expanded the scope of cancer-related pain management to include survivors. Similarly, as new disease-modifying treatments and gene therapies for ALS have emerged, effective pain management is more critical than ever. Timely assessments are vital to achieve the ultimate goal of pain management, which is to reduce pain intensity, diminish patient suffering, and enhance the quality of life of patients. In addition, it is necessary to provide treatment tailored to the individual needs of patients based on good clinical practices and information from the management of nonmalignant chronic pain in the current situation, where there are no specific guidelines for controlling pain in ALS.

None.

No potential conflict of interest relevant to this article was reported.