[Bilateral optic disk edema in polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins and skin changes (POEMS syndrome)].
キーワード
概要
BACKGROUND
POEMS occurs only in about 1% of plasmocytoma cases, but in more than 50% of the rare osteosclerotic subtypes and plasma cell dyscrasias. The estimated frequency is 20 cases per year in Germany. Swelling of the optic disks is an early sign of the syndrome.
METHODS
An osteosclerotic plasmocytoma type IgA lambda was known in a 63-year-old farmer for 4 years. The primary lesion in the left femur was irradiated at diagnosis. Half a year prior to our examination the patient experienced edema of the legs and recurrent diarrhea. A staging confirmed the earlier diagnosis of a POEMS syndrome. The patient was presented for ophthalmologic examination because of optic disk swelling and progressive visual field defects: raster perimetry revealed enlarged blind spots and increased thresholds. Neurologic examination revealed polyradiculitis, increased protein levels in the cerebrospinal fluid and intracranial hypertension. The patient was treated with oral steroids which entailed improvement of the visual fields and decrease of optic disk swelling.
CONCLUSIONS
Ophthalmologists play an active role in the staging, in the ruling out of other causes, and in the treatment. Symptomatic treatment with oral steroids should be monitored with visual acuity, raster perimetry and fundus examinations.
