Table 1

Functional domains and stages

ALSFRS domain	Item	Score	Functional score*
Movement (walking/self-care)†	8 Walking	4 Normal	0
		3 Early ambulation difficulties
		2 Walks with assistance
		1 Non-ambulatory functional movement only	1
	OR	0 No purposeful leg movement	1
	6 Dressing and hygiene	4 Normal function	0
		3 Independent and complete self-care with effort or decreased efficiency
		2 Intermittent assistance or substitute methods
		1 Needs attendant for self-care	1
		0 Total dependence	1
Swallowing	3 Swallowing	4 Normal eating habits	0
		3 Early eating problems; occasional choking
		2 Dietary consistency changes
		1 Needs supplemental tube feeding	1
		0 NPO (exclusively parenteral or enteral feeding)	1
Communicating†	1 Speech	4 Normal speech processes	0
		3 Detectable speech with disturbances
		2 Intelligible with repeating
		1 Speech combined with non-vocal communication	1
	AND	0 Loss of useful speech	1
	4 Handwriting	4 Normal	0
		3 Slow or sloppy; all words are legible
		2 Not all words are legible
		1 Able to grip pen but unable to write	1
		0 Unable to grip pen	1
Breathing†	10 Dyspnea	4 None	0
		3 Occurs when walking
		2 Occurs with one or more of: eating, bathing, dressing
		1 Occurs at rest, difficulty breathing when either sitting or lying	1
	OR	0 Significant difficulty, considering using mechanical respiratory support	1
	12 Respiratory insufficiency	4 None	0
		3 Intermittent use of NIPPV	0
		2 Continuous use of NIPPV during the night	1
		1 Continuous use of NIPPV during the night and day
		0 Invasive mechanical ventilation by intubation or tracheostomy
ALS-MITOS	Stage	Functional domains lost
	0	None
	1	1 domain
	2	2 domains
	3	3 domains
	4	4 domains
	5	Death

*Staging determined by the sum of functional score of 1 for each domain.
†Where two items were used, scoring was based on either or both item scores as indicated.
ALFSRS, Amyotrophic Lateral Sclerosis Functional Rating Scale; ALS-MITOS, Amyotrophic Lateral Sclerosis Milano-Torino Staging; NIPPV, nasal intermittent positive pressure ventilation; NPO, nothing by mouth.