POEMS neuropathy: optimising diagnosis and management

Abstract

POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The acronym ‘POEMS’ represents the complex and multisystem features of the disease, including polyneuropathy, organomegaly, endocrinopathy, a monoclonal plasma cell disorder and skin disease. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made. University College London Hospitals sees one of the largest reported POEMS cohorts in Europe, and runs a multispecialist clinic to assist with diagnosis, treatment and ongoing support. This review draws upon our experience to present the typical features of POEMS syndrome and highlight diagnostic conundrums commonly experienced, supplemented with clinical cases. We provide an investigative guide for clinicians when considering POEMS as the diagnosis, and propose a treatment algorithm that centres on the site and degree of monoclonal cell proliferation.

An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction

“…The latter can be predominantly distal or, in the case of CIDP, show symmetrical proximal distribution [3,7]. Both conditions involve albuminocytologic dissociation in CSF and electrophysiological evidence of demyelinating f wave prolongation, slow conduction velocities and temporal dispersion [5]. The electroneuromyography of POEMS syndrome patients exhibits heightened axonal loss [2].…”

Laboratory Evaluation of Peripheral Neuropathy

“…Laboratory testing often reveals elevated serum vascular endothelial growth factor (VEGF) several times above the normal limit. 45 In GALOP, disabling gait disturbances and sensory abnormalities develop in elderly patients. 29 Antibodies to sulfatide and galopin may be positive.…”

51-year old man with tingling, burning and progressive limb weakness

“…However, unlike CIDP, POEMS polyneuropathy is a length-dependent sensory and motor neuropathy 24. It usually starts with distal sensory symptoms, that evolve proximally over weeks or months, followed by motor disturbances.…”

“…About 65% of people report pain 25. Associated multisystem involvement, particularly glomeruloid haemangiomata is an important pointer towards POEMS 24. Electrophysiology shows more uniform slowing of conduction velocities and relatively greater axonal loss as compared with CIDP 26.…”