Electrophysiological features of patients with 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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