Electrophysiological features of patients with POEMS syndrome

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Abstract

Objective

Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuropathy component is essential for the diagnosis of the disease, the pattern of associated 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 (SNAPs) were not elicited in lower limbs more frequently compared with in upper limbs (P<0.05, in both motor and sensory nerves), and the CMAP amplitude was more attenuated in lower limbs than in upper limbs (P<0.05). Abnormal conduction slowing was frequently observed in motor (95%) and sensory (76%) nerves. Distal motor latencies were abnormally prolonged in 75% of the motor nerves, and terminal latency indices were significantly higher in patients than in normal controls (P<0.05).

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.

Significance

The recognition of these characteristic patterns may be helpful for the early diagnosis of polyneuropathy in POEMS syndrome.

Introduction

Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome was first characterized by Crow in 1956, and was described further by Fukase in 1968 (Nakanishi et al., 1984). This disease is a rare cause of polyneuropathy, but the polyneuropathy component is essential for diagnosis of the disease. Since, polyneuropathy is the most common initial manifestation in POEMS syndrome, recognition of typical electrodiagnostic patterns, if present, may be helpful for early diagnosis of the disease. Electrodiagnostic features of polyneuropathy in this disease, however, have not been characterized in detail. We analyzed the findings of nerve conduction studies (NCS) in patients with POEMS syndrome to elucidate the electrodiagnostic features of polyneuropathy in this rare disorder.

Section snippets

Methods

Twelve consecutive patients (Age, 52.9±11.1 years; Female:Male, 8:4), who were diagnosed as having POEMS syndrome at Seoul National University Hospital, were included in this study (May, 1999–December, 2003). The diagnosis of POEMS syndrome was made according to the criteria suggested by Dispenzieri et al., which requires the presence of sensorimotor polyneuropathy and monoclonal plasmaproliferative disorder as the major criteria (Dispenzieri et al., 2003). Minor criteria include followings: a

Results

Eleven of the 12 patients showed all 5 principal features of the syndrome at the time of diagnostic investigation. Clinical and laboratory features of the patients are summarized in Table 1. Polyneuropathy was the most frequent initial manifestation in our patient group (66.7%), followed by back pain (16.7%), and ascites of unknown etiology (8.3%). Symmetric paresthesia predominantly in the lower extremities was the most common symptom of polyneuropathy (62.5%), followed by distal weakness

Discussion

Our study revealed several characteristic abnormal patterns in POEMS polyneuropathy. First, conduction abnormalities were length-dependent. The frequency of both motor and sensory nerves with no potentials was higher in lower limbs. After excluding the nerves having no potential, CMAP was more severely attenuated in lower limbs. For SNAP amplitude, however, we could not find any significant difference between upper and lower limbs. It could be explained by the fact that the SNAPs are more

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