Table 4.
Patient | Male/female | Clinical presentation | NCS | Electrodiagnostic criteria* | Liquor protein in g/L (normal 0.00–0.40) |
Treatment and dosage | Response to treatment |
---|---|---|---|---|---|---|---|
1 | Male | Symmetrical weakness in proximal and distal arm and leg muscles; loss of vibration, touch and position sense in arms and legs; areflexia | CMAP↓ right median, right peroneal and left tibial nerve. ↑DML bilateral median nerves. ↓SNAP bilateral median, ulnar, radial and sural nerves | Not compatible | 0.39 | Intravenous immunoglobulins, 40 g every 3 weeks | Improvement of pinch force right hand from 55 to 100 kPa, improvement pinch force left hand of 30 kPa to 98 kPa, measured with Martin vigorimeter |
2 | Male | Asymmetrical weakness in proximal and distal right arm muscles and right leg muscles; loss of vibration sense distal from knees; low reflexes in the arms, areflexia in the legs | CMAP↓ bilateral median, right ulnar, bilateral peroneal and left tibial nerve. ↑DML bilateral median nerves. Normal SNAP’s | Not compatible | 0.48 | Intravenous immunoglobulins, 30 g every 3 weeks | Improvement of dorsal flexion of right foot, measured with myometry by physiotherapist |
3 | Male | Asymmetrical weakness in proximal and distal right arm; tremor; loss of vibration sense in feet; areflexia | CMAP↓ right median nerve. ↑DML left median nerve. ↓SNAP bilateral median and sural nerves | Not compatible | 0.61 | Intravenous immunoglobulins, 40 g every 4 weeks | Improvement of MRC 4 to 5 in right arm, measured by treating physician |
4 | Male | Symmetrical weakness in proximal and distal leg muscles; fluctuating pain in legs; loss of vibration sense in feet; areflexia | CMAP↓ right peroneal and left tibial nerve. ↑DML right median and left ulnar nerve. ↓SNAP bilateral sural nerves | Not compatible | 0.70 | Methylprednisolone 1000 mg every 4 weeks | Improvement of MRC 3 to 4 (right) and MRC 4 to 5 (left) in quadriceps muscles, improvement of MRC 0 to 4 in left anterior tibial muscle, measured by treating physician. For 3 years ago in wheel chair, currently walks an hour (with walking stick) |
5 | Male | Symmetrical weakness in extensor hallucis longus muscle; loss of vibration and touch sense in feet up to the knees; low reflexes in the arms, areflexia in the legs | CMAP↓ left median, bilateral ulnar and peroneal, right tibial nerve. ↑DML right median nerve. ↓SNAP bilateral median, right ulnar, left tibial, bilateral sural | Not compatible | 0.42 | Single therapy of intravenous immunoglobulins, 40 g during 5 days | Improvement of touch sense (currently only persistent in feet), better balance, observed by treating physician |