The ALS Score of Jablecki et al for Disability in Amyotrophic Lateral Sclerosis

Description

Jablecki et al developed a score for evaluating disability in patients with amyotrophic lateral sclerosis (ALS). This can be used to monitor a patient's decline in ability over time and can be used to estimate survival. The authors are from the University of California and Children's Hospital in San Diego.

Parameters:

(1) speech

(2) swallowing

(3) respiration

(4) ambulation

(5) upper extremity strength, right

(6) upper extremity strength, left

(7)upper extremity survey score, right

(8)upper extremity survey score, left

(9) lower extremity strength, right

(10) lower extremity strength, left

(11) lower extremity survey score, right

(12) lower extremity survey score, left

The responses for nonstrength items range from normal (0 points) to 2/4/5/6

Muscle in Upper Extremity, Left or Right	Finding	Points
deltoid	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
biceps	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
flexor pollicis longus	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
first dorsal interosseous	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4

Muscle in Lower Extremity, Left or Right	Finding	Points
iliopsoas	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
quadriceps	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
tibialis anterior	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4
tibialis posterior	normal strength	0
	mild weakness (1 – 25% reduction)	1
	moderate weakness (26 – 50% reduction)	2
	severe weakness (51 – 75% reduction)	3
	useless (76 – 100% reduction)	4

muscle strength score for a limb =

= (SUM(points for all 4 muscles)) / 4

ALS score =

= SUM(points for all 12 parameters)

Interpretation:

• minimum score: 0

• maximum score: 40

• The higher the score, the greater the disability.

• The score can be used to predict survival time when combine with the patient's age and duration of weakness, as discussed in the chapter on neurology.

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Description

Specialty

ICD-10