Local Treatment with Triamcinolone Acetonide and Bevacizumab for Ocular Symptoms in a Patient with POEMS Syndrome.

PURPOSE: To report our experience in managing a case of bilateral optic disc oedema and unilateral cystoid macular oedema with a posterior subtenon (PST) injection of triamcinolone acetonide and intravitreal bevacizumab (IVB) in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. CASE REPORT: A 50-year-old healthy woman complaining of bilateral blurred vision for several days was referred to the ophthalmologist. An eye examination showed a bilateral optic disc swelling and small preretinal haemorrhages. In addition, a peripheral neuropathy was found. Laboratory tests showed elevated levels of M-protein type IgA of 1.4 g/l and a vascular endothelial growth factor (VEGF) concentration of 6.1 ng/ml. The cerebrospinal fluid protein content was 131 mg/dl. An abdominal CT scan revealed hepatosplenomegaly. The diagnosis of POEMS syndrome was made based on the polyneuropathy, hepatosplenomegaly, IgA lambda monoclonal proteinemia, and optic disc swelling. As vision declined, 40 mg PST injection of triamcinolone was given bilaterally. Ten weeks thereafter, 2 IVB injections of 2.5 mg were administered to the left eye, with a 4-week interval. Because of an incomplete recovery of her visual functions, diarrhoea and general malaise, melphalan, followed by autologous peripheral blood stem cell transplantation, was initiated. After systemic treatment, she had no visual complaints anymore, the optic disc oedema had resolved, and the VEGF serum concentration had normalized. DISCUSSION: Local treatment with IVB and PST injection of triamcinolone can be an option for ocular symptoms in POEMS syndrome. However, given the incomplete recovery of the ocular abnormalities and the other symptoms, systemic treatment remains standard.

Genotypes of JC virus in East, Central and Southwest Europe.

Distinctive genotypes of JC virus have been described for the major continental landmasses. Studies on European-Americans and small cohorts in Europe showed predominantly Type 1. Types 2 and 7 are found in Asia, and Types 3 and 6 in Africa. These genotypes differ in sequence by about 1--3%. Each genotype may have several subtypes which differ from each other by about 0.5--1%. The genotypes can be defined by a distinctive pattern of nucleotides in a typing region of the VP1 gene. This genotyping approach has been confirmed by phylogenetic reconstruction using the entire genome exclusive of the rearranging regulatory region. In this first large European study, we report on the urinary excretion of JCV DNA of 350 individuals from Poland, Hungary, Germany and Spain. We included Gypsy cohorts in Hungary (Roma), Germany (Sinti), and Spain (Gitano), as well as Basques in Spain. We show that while Type 1 predominates in Europe, the proportions of Type 1A and 1B may differ from East to Southwest Europe. Type 4, closely related to the Type 1 sequence (only approximately 1% difference) was a minor genotype in Germany, Poland and Spain, but represented the majority in Basques. The Gitanos in Spain showed a variant Type 4 sequence termed 'Rom-1'. Interestingly, neither the Gitanos in Spain, nor Sinti or Roma in Germany or Hungary showed the Type 2 or Type 7 genotype that might be expected if their origins were in an Asian population.