Table 6.

Staging of ATTRv amyloidosis with PN, scales, and tools at baseline

Locomotion stage description [7]	Duration of stage, years		PND score [88]
	Early-onset Val30Met [7]	Late-onset Val30Met Other variants [8, 72]
Stage 1  Disease limited to the lower limbs  Walking without help  Slight weakness of the extensors of the big toes	5.6 ± 2.8	2–4	PND I  Sensory disturbances in extremities  Preserved walking capacity PND II  Difficulty walking but no need for a walking stick
Stage 2  Progression of motor signs in lower limbs with steppage and distal amyotrophies; muscles of the hands becoming wasted and weak  Patient obviously disabled but can still move around with help	4.8 ± 3.6	2–3	PND IIIa  1 stick or 1 crutch required for walking
			PND IIIb  2 sticks or 2 crutches required for walking
Stage 3  Patient confined to a wheelchair or a bed, with generalized weakness and areflexia	2.3 ± 3.1	1–2	PND IV  Patient confined to a wheelchair or a bed

Reprinted with permission from Adams [18]

ATTRv hereditary transthyretin amyloidosis, PND polyneuropathy disability