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100 Characterisation of peripheral neuropathy in patients with POEMS with and without associated multicentric castleman disease

jnnp · 2025-11-26 · canonical JSON source

12 visible annotations · policy: published · automated confidence ≥ 75.00%

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Introduction POEMS syndrome is a rare multisystem disorder where peripheral neuropathy is a hallmark. Multicentric Castleman Disease (POEMS-MCD), a rare lymphoproliferative disorder with characteristic lymphadenopathy and histology, is a major diagnostic criterion. POEMS causes typical, length-dependent sensorimotor neuropathy. Aim: to summarise neuropathy characteristics in patients with POEMS-MCD and POEMS from UCLH, the national referral centre/registry.Methods We retrospectively reviewed 18 patients with POEMS-MCS compared to 36 randomly selected patients with POEMS syndrome referred from 1998-2024. We compared demographics, clinical features (including Rasch&noentity8208;built overall disability scale-POEMS (RODS-POEMS)), examination findings and neurophysiology. Neuropathy Impairment Score (NIS) and Composite Amplitude Score (CAS) of sural sensory, tibial-AH and peroneal-EDB motor amplitudes were calculated.Results Median symptom onset age was significantly lower in POEMS-MCD than non-MCD POEMS (44 vs 56-years-old, p=0.0025), as was age at diagnosis (45 vs 56-years-old, p=0.0038). No significant differences were noted in gender, time from symptom to diagnosis, or RODS.NIS in POEMS was significantly higher than in POEMS-MCD (57.5 vs 26.5, p=0.00447). CAS in POEMS was significantly lower (worse) than in POEMS-MCD (0 vs 1.35, p=0.008).Conclusions Peripheral neuropathy in POEMS is more severe than in POEMS-MCD, warranting consideration of POEMS-MCD in patients with minimal/no neuropathy but other POEMS features.zara.markovic-obiago@nhs.net