Introduction: Oedema of the papilla, or papilloedema, is usually due to intracranial hypertension, but can sometimes be the initial symptom of systemic diseases like Guillain-Barre syndrome, neurosarcoidosis, Leber's disease, Lyme disease or POEMS syndrome, among others. We report on a case that occurred at the Hospital General Universitario in Alicante, in which papilloedema was the initial symptom of POEMS syndrome.
Case report: A 36-year-old female, with no relevant past history, who visited after suffering a left-side frontal oppressive headache for five days; onset was sudden, there was no concomitant nausea or vomiting, it did not get worse when Valsalva manoeuvres were performed and there were no other accompanying symptoms. The examination revealed nasal blurring in the visual field of the right eye and papilloedema in the left eye, hyperpigmentation of the skin on the face and upper chest area that respected creases in the skin, painful distal tactile hypaesthesia in the lower limbs and bilateral areflexia of Achilles' tendons and patellar hyperreflexia. After conducting a number of different complementary tests intracranial space-occupying lesions and intracranial hypotension were both ruled out and the patient was diagnosed with mixed-type sensory-motor chronic polyneuropathy in the upper limbs, and in the lower extremities with predominantly distal involvement, bilateral oedema of the papilla and skin photosensitivity. Two years later the patient had hepatomegaly, subclinical hypothyroidism and sclerodermiform alterations of the skin, which suggested a probable diagnosis of POEMS syndrome that was later confirmed by the presence of monoclonal component in blood and urine.
Conclusions: Papilloedema is rarely seen as the initial symptom of POEMS syndrome, but it must be included in the differential diagnosis of this pathology due to the diagnostic and therapeutic implications it entails.