Table 3 Assessments for monitoring progression in somatic and autonomic neuropathy in recommended order of importance

Somatic neuropathy

10MWT

Change in gait speed 0.05–0.10 m/s [93]

6–12 months

High

OR

Timed Get Up and Go test

An increase of 15% over 6 months (or 30% over 12 months) in the time taken to stand up, walk across the room, and sit down

6–12 months

High

PND score

Change in disease stage

6–12 months

Low in EO V30M

 Not sensitive to small changes in progression but useful to assess during monitoring visits as a change in score indicates increased functional impairment

High in LO V30M

Jamar Hand Dynamometer—both hands grip strength test

Reduction of grip strength of 4–6 kilos over 12 months

In the APOLLO trial, least squares mean grip strength decreased by 43% over 18 months in patients treated with placebo (n = 56, personal communication)

6–12 months

High

R-ODS^b

Worsening of R-ODS score by 3–8 points over 12 months or worsening of the score on 2 consecutive consultations 6 months apart (questionnaire to be filled in before the consultation)

6–12 months

High

SFN-SIQ

Particularly useful for monitoring patients with V30M and EO disease

6–12 months

Medium

Walking perimeter/balance disorders

Onset of balance disorders

6–12 months

High

Falls

Reduction of walking perimeter in daily life (in meters)

6–12 months

Medium

NIS^b

A change of 7–16 points over 12 months or worsening of the score on 2 consecutive consultations 6 months apart

6–12 months

High in LO V30M

 Give more weight to changes in strength and less weight to changes in reflexes

NCS

Decrease of 20% amplitude in several nerves over 12 months when the same nerves are tested using the same methods over time

12 months at most

Medium

Autonomic neuropathy

Sudomotor testing

Using Sudoscan^®, a reduction on 2 consecutive examinations of the feet

12 months

High

Rarely performed but useful for monitoring patients with V30M and EO disease

Heart rate deep breathing

A change from an age-adjusted normal value to abnormal value

12 months

High

CADT questionnaire^b

Reduction of total CADT score by 2 points or reduction of any subscore by 1 point

6 months

Low

OR

COMPASS-31 questionnaire^b

Increase by 1 point in a year

12 months

Low

Orthostatic vital signs

New onset of orthostatic hypotension

6 months

Medium

Onset of orthostatic syncope for patients who already have orthostatic hypotension

Typically manifests in later stages of disease

Valsalva maneuver

A change from an age-adjusted normal value to abnormal value

12 months

High