POEMS Syndrome in a Juvenile Initially Diagnosed as Treatment Resistant Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a disorder that mainly affects adults. We report a pediatric patient, initially considered to have Guillain-Barré syndrome, who continued to have progression of neuropathic disease leading to the diagnosis of chronic inflammatory demyelinating polyneuropathy. Diagnosis of POEMS was established by an abnormal bone marrow biopsy, prompted by laboratory and imaging findings, which became abnormal later in the course of the disease. POEMS syndrome is extremely rare in children, and neuropathic features in this age group have not been previously described. This case illustrates that "Guillain-Barré syndrome-like" initial presentation for POEMS, which has not been previously reported. It also emphasizes that in children with progressive acquired neuropathies that are treatment unresponsive, POEMS syndrome should be considered.

Bioactive Glass in a Rare Case of Osteomyelitis of the Heel in a Guillain-Barré Syndrome: A Case Report

“…GBS, as well as GBS-like conditions, such as POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes), determine an acute- or subacute-onset polyradiculoneuropathy presenting both sensory symptoms and weakness evolving quickly. 23 The complete resolution, also when it can be obtained without any sequelae, takes a long time to be achieved. During these months or years, the patient continues to complain both sensory impairment, which mimic loss of protective sensation of diabetic neuropathy, and muscular weakness, which creates severe gait alterations, similar to those observed in diabetic motor neuropathy.…”