Papilledema in a patient with POEMS syndrome

Abstract

Introduction: POEMS syndrome is a rare plasma cell disorder considered a paraneoplastic manifestation. In some of these cases, the presence of papilledema, produced by increased intracranial pressure, idiopathic or caused by expansive intracranial lesions, venous sinus thrombosis, inflammatory processes, or trauma, is reported. POEMS Syndrome is not usually suspected when evaluating a patient with papilledema, which is why this case report is made. Clinical case: A 47-year-old woman, with a history of diabetes mellitus and thrombocytosis, who presented with darkening of the skin and weight loss, followed by tingling and a sensation of numbness in the lower limbs and hands for two months. The symptoms progressively worsened, accompanied by transient visual obscurations. On eye fundus examination, edema of both optic nerves was found. Although visual acuity was normal, the automated visual field study showed an increase in the blind spot in both eyes and decreased sensitivity in the nasal sector of the right eye. The diagnostic possibility of papilledema was raised, but given the varied systemic manifestations, she was sent to a general hospital for a multidisciplinary evaluation, where a diagnosis of POEMS Syndrome was made. After treatment of the cause, the patient evolved favourably, only discreet pallor of the optic disc and decrease in ganglion cells persisting in the right eye.

Conclusions: Although POEMS syndrome is a rare entity, it should be considered in a patient with papilledema, particularly when accompanied by other systemic manifestations such as peripheral neuropathy and skin discoloration. Adequate and timely treatment of the disease will prevent the impairment of visual function.