Table 2 Neuropathic features of POEMS syndrome
CaseInitial symptomDuration until biopsy (m)Motor signsSensory signs*Modified Rankin Scale‡
WeaknessMuscle atrophySpontaneous painPain†VibrationJoint sense
UE (P/D)LE (P/D)
1N12+1/+2+2/+1+10+1−3−22
2N5+1/+2+2/+300−2−3−24
3N3+1/0+2/+1+1+2+2−103
4N24+2/+1+2/+1+1+3+3−3−22
5W120/0+2/+30+2+2−2−23
6N70/+1+1/+300−2−3−13
7N100/0+2/+10+3−3§−3−32
8W10/+1+2/+3000−2−13
9N6+1/+1+2/+3+1+3+3−2−23
10N40/+2+1/+3+1+2+2−3−23
11N40/+2+3/+3+20−2−3−33
12W9+2/+3+3/+3+200−204
13W5+1/+1+2/+300+1−1−13
14W70/0+2/+3000−202
15N6+1+2+1/+10+3+3−3−32
16N1+1/+1+1/+2000−3−12
17N12+1/+1+2/+3+1+1+2−3−13
18N3+1/+1+1/+1+10+2−3−22
19N70/0+2/+30+3+2−3−23
20W20/0+2/+30+1+3−2−23
21N120/00/00+1+1−2−12
22N3¶0/0+1/+3+1+2+2−102

  • +, positive findings; −, negative findings; 0, none.

  • +1, +2, and +3 represent mild, moderate and severe degree of muscle weakness, atrophy and sensory signs.

  • −1, −2, and −3 represent mild, moderate and severe reduction for sensory signs.

  • D, distal portion; LE, lower extremities; N, numbness of the lower extremities; P, proximal portion; POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes; UE, upper extremities; W, weakness of the lower extremities.

  • *Sensory signs were assessed in the distal portion of the lower limbs.

  • †Pain is devided into two categories; hyperalgesia (+1, +2 or +3) and hypoalgesia (−1, −2 or −3).

  • ‡Modified Rankin Scale:14 0, asymptomatic; 1, non-disabling symptoms not interfering with lifestyle; 2, minor disability from symptoms leading to some restriction of lifestyle but not interfering with patients’ capacity to look after themselves; 3, moderate disability from symptoms significantly interfering with lifestyle or preventing totally independent existence; 4, moderately severe disability from symptoms clearly precluding independent existence, although not requiring 24-hour attention from a caregiver; and 5, severe disability and total dependence, requiring constant attention day and night.

  • §Painful sensation in patient 7 was so severe that the response to mechanical stimuli was not obvious.

  • ¶Duration until first referral to the hospital.