Clinical and Electrophysiological Features of the Patients with POEMS Syndrome

POEMS 증후군의 임상적, 전기생리학적 특성

Backgrounds and objectives: POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is the rare cause of polyneuropathy. Although the polyneuropathy is essential for the diagnosis of the disease, the pattern of electrodiagnostic abnormalities has not been characterized in detail. The purpose of this study was to elucidate the features of nerve conduction abnormalities in POEMS syndrome. Methods: We reviewed the medical records and nerve conduction studies (NCS) of 12 consecutive patients with POEMS. Results: A total of 68 motor and 46 sensory nerves were examined. Compound muscle action potentials (CMAPs) and sensory nerve action potentials were abnormally attenuated or not elicited in majority of motor and sensory nerves (80.88% in motor, and 82.6% in sensory nerves). Frequency of the nerves with no potential was significantly higher in lower limbs than in upper limbs (p<0.01 in both motor and sensory nerves), and CMAP amplitude was more reduced in lower limbs than in upper limbs (p<0.01). Conduction slowing was very frequently observed with 95% and 76% of motor and sensory nerves, respectively, having the abnormally reduced values of conduction velocity. Distal motor latencies were abnormally prolonged in 75% of motor nerves, and terminal latency indices were significantly higher in patients than in normal controls (p < 0.05). Conduction block was observed only in 5% of motor nerves. Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predominantly in the intermediate nerve segments than in the distal portions. The recognition of these characteristic patterns may be helpful in early diagnosis of polyneuropathy in POEMS syndrome.