Abstract
ObjectivePolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare multisystem disease associated with a plasma-cell dyscrasia. Although pachymeningeal involvement has occasionally been described, MRI of the central nervous system (CNS) has not yet been extensively investigated.MethodsWe retrospectively evaluated CNS MRI in Europe’s largest single-center cohort of POEMS syndrome. Of 77 patients who have been formally diagnosed with POEMS, 41 had MRI brain and 29 had MRI spine. A control group of 33 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) was used as this is the major differential diagnosis. Of these CIDP patients, 12 underwent both MRI brain and spine, 7 had solely MRI brain and 14 had MRI spine.ResultsIn 41 POEMS patients with MRI brain, we identified frequent smooth, diffuse meningeal thickening of the cerebral convexities and falx (n = 29, 71%), of which 4 had meningeal collections. 17 (41%) had vascular abnormalities including white-matter disease, of which 4 had established infarcts. Of 29 patients with MRI spine, 17 (59%) had thickening of the brachial and lumbosacral plexus. Conversely in 19 CIDP patients with MRI brain, none had meningeal thickening (p < 0.0001); however, 8 (42%) had vascular abnormalities (p = 0.85). Of 26 patients with MRI spine, 9 (35%) had brachial or lumbosacral plexus thickening (p = 0.06).ConclusionsIn contrast to CIDP, POEMS patients frequently have pachymeningeal thickening. Vascular abnormalities and plexus thickening were also common but not statistically different to CIDP.
Highlights
POEMS syndrome is a rare and disabling multisystem condition characterised by peripheral neuropathy and a monoclonal plasma cell disorder [1]
This study was performed at the POEMS Clinic at University College London Hospital, which treats the largest reported POEMS cohorts in Europe. 77 patients who have attended this clinic from January 2010 to August 2018 have been formally diagnosed with POEMS syndrome according to the current diagnostic criteria [5]
MRI was performed before the diagnosis of POEMS was confirmed in 24 of 41 (58.5%) patients (Supplementary Table 1). 33 age- and sex-matched patients from the University College London Hospital chronic inflammatory demyelinating polyneuropathy (CIDP) database were used as controls, with 19 having brain MRI and 26 having spine MRI available (Supplementary Table 2)
Summary
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes) syndrome is a rare and disabling multisystem condition characterised by peripheral neuropathy and a monoclonal plasma cell disorder [1]. Additional manifestations include papilloedema, extravascular volume overload, sclerotic bone lesions and thrombocytosis. Clinical presentations are heterogeneous making the diagnosis challenging. The major and minor diagnostic criteria were proposed in 2003 [5] but despite this POEMS remains diagnostically elusive. Patients commonly present with only a limited number of the criteria and the subtle bone marrow abnormalities provide false reassurance regarding the plasma cell dyscrasia. Patients are frequently (~ 60%) misdiagnosed with other neuropathies, usually chronic inflammatory demyelinating
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