Fig. 12.1
Fundal photographs showing haemorrhagic papilloedema typical of POEMS syndrome (cheese and tomato appearance)
Examination of the limbs revealed distal weakness and wasting in the upper and lower limbs. He was areflexic. Joint position, vibration, pinprick sensation were reduced to the mid forearms and groin.
Investigations
Blood and urine tests – Hb 185 g/l (130-170), platelets 652 × 109/L (150-400), primary hypogonadism, hypothyroidism and mild cortisol deficiency. IgG lambda paraprotein. Kappa/Lambda light chain ratio 0.23 (0.26-1.65). No urinary Bence- Jones protein. Serum VEGF 10728 pg/ml (<771)
CSF – <1 WBC/mm3, protein 2.72 g/L (0.25-0.4) with normal glucose, negative cytology and negative oligoclonal bands.
MRI lumbosacral spine – L3 osteolytic lesion.
CT body – multiple osteosclerotic and osteolytic lesions throughout the axial spine and ribs. Hepatomegaly.
Bone marrow biopsy and trephine – <5 % lambda restricted CD20+ CD138 weak + cells and no evidence of plasma cell dyscrasia on trephine.
Neurophysiology (Tables 12.1, 12.2 and 12.3)
Table 12.1
Sensory and mixed nerve conduction studies
Right | Left | |||
---|---|---|---|---|
μV | m/s | μV | m/s | |
Radial (forearm-wrist) | 8 | 38 | ||
Median (F2-wrist) | 2 | 30 | 2 | 34 |
Median (F3-wrist) | No response | 2 | 34 | |
Median (palm-wrist) | 3 | 26 | 4 | 30 |
Ulnar (F5-wrist) | 2 | 36 | 1 | 35 |
Ulnar (palm-wrist) | 3 | 35 | 1 | 34 |
Sural (calf-ankle) | No response | No response |
Table 12.2
Motor nerve conduction studies
Right | Left | |
---|---|---|
Median (SE on APB) | ||
DML | 8.4 ms | 7.4 ms |
TLI | 0.41 | 0.36 |
CV (wrist-elbow) | 23 m/s | 30 m/s |
CMAP (wrist) | 1.7 mV | 0.4 mV |
CMAP (elbow) | 1.2 mV | 0.1 (dispersed) |
Minimal F-wave latency (wrist) | Absent | Absent |
Ulnar (SE on ADM) | ||
DML | 5.0 ms | 5.2 ms |
TLI | 0.61 | 0.59 |
CV (wrist-below elbow)
Stay updated, free articles. Join our Telegram channelFull access? Get Clinical TreeGet Clinical Tree app for offline access |