Disease Severity Score of Damy et al for Hereditary Transthyretin Amyloidosis (ATTRv): Motor Function

Description

Damy et al reported a disease severity score for a patient with hereditary transthyretin amyloidosis (ATTRv). A group of findings relates to motor function. The authors are from multiple institutions in Europe.

Patient selection: hereditary transthyretin amyloidosis

Parameters:

(1) atrophy of upper limb

(2) atrophy of lower limb

(3) finger adduction

(4) finger abduction

(5) wrist flexion

(6) wrist extension

(7) elbow flexion

(8) elbow extension

(9) shoulder adduction

(10) shoulder abduction

(11) ankle dorsiflexion

(12) ankle plantarflexion

(13) knee flexion

(14) knee extension

(15) hip abduction

(16) hip adduction

Motor Function		Grade
normal	normal power	4
active movement against gravity and resistance	active MAGR	3
active movement against gravity	active MAG	2
active movement with gravity eliminated	active MWG	1
no contractions	no contractions	0

Parameter	Finding	Points
atrophy of upper limb	absent	0
	present	0.79
atrophy of lower limb	absent	0
	present	0.66
finger adduction	normal power	0
	active MAGR	0.47
	active MAG	0.61
	active MWG	0.74
	no contractions	0.86
finger abduction	normal power	0
	active MAGR	0.50
	active MAG	0.66
	active MWG	0.81
	no contractions	0.95
wrist flexion	normal power	0
	active MAGR	0.58
	active MAG	0.74
	active MWG	0.88
	no contractions	1.02
wrist extension	normal power	0
	active MAGR	0.59
	active MAG	0.75
	active MWG	0.90
	no contractions	1.04
elbow flexion	normal power	0
	active MAGR	0.68
	active MAG	0.84
	active MWG	0.99
	no contractions	1.10
elbow extension	normal power	0
	active MAGR	0.68
	active MAG	0.84
	active MWG	0.99
	no contractions	1.10
shoulder adduction	normal power	0
	active MAGR	0.77
	active MAG	0.88
	active MWG	0.97
	no contractions	1.04
shoulder abduction	normal power	0
	active MAGR	0.75
	active MAG	0.92
	active MWG	1.04
	no contractions	1.13
ankle dorsiflexion	normal power	0
	active MAGR	0.54
	active MAG	0.68
	active MWG	0.84
	no contractions	0.97
ankle plantarflexion	normal power	0
	active MAGR	0.50
	active MAG	0.65
	active MWG	0.81
	no contractions	0.95
knee flexion	normal power	0
	active MAGR	0.61
	active MAG	0.75
	active MWG	0.90
	no contractions	1.02
knee extension	normal power	0
	active MAGR	0.61
	active MAG	0.77
	active MWG	0.92
	no contractions	1.04
hip abduction	normal power	0
	active MAGR	0.74
	active MAG	0.86
	active MWG	0.99
	no contractions	1.08
hip adduction	normal power	0
	active MAGR	0.75
	active MAG	0.88
	active MWG	1.01
	no contractions	1.11

subscore for motor function =

= SUM(points for all of the parameters)

Interpretation:

• minimum subscore: 0

• maximum subscore: 15.86 (15.88 in document)

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Disease Severity Score of Damy et al for Hereditary Transthyretin Amyloidosis (ATTRv): Motor Function

Description

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