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1.
Zh Nevrol Psikhiatr Im S S Korsakova ; 123(7. Vyp. 2): 15-21, 2023.
Artigo em Russo | MEDLINE | ID: mdl-37560829

RESUMO

POEMS-syndrome (polyneuropathy - P, organomegaly - O, endocrinopathy - E, M-protein - M, skin changes - S) is a paraneoplastic syndrome caused by underlying dyscrasia of plasma cells. The main criteria of the syndrome are polyradiculoneuropathy, clonal proliferation of plasma cells, sclerotic bone lesions, elevated levels of vascular endothelial growth factor and the presence of Castleman disease. Additional signs include organomegaly, endocrinopathy, characteristic skin changes, edema of the optic disc, extravascular volume overload (edema) and thrombocytosis. The diagnosis is often made late, because the syndrome is rare and is often mistaken by specialists for other neurological disorders, most often for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We present two cases of POEMS-syndrome. The description is based on the principle of differential diagnosis with a number of similar neurological disorders. The goal facing the neurologist is to carry out the most complete diagnostic measures for early diagnosis, which further determines timely therapeutic tactics. Hematologists are engaged in specific therapy of POEMS-syndrome. A brief description of possible therapeutic options is presented. On the example of these cases, we demonstrate possible variants of the therapeutic response based on the developed system of risk stratification of patients with POEMS-syndrome.


Assuntos
Hiperplasia do Linfonodo Gigante , Síndrome POEMS , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Humanos , Fator A de Crescimento do Endotélio Vascular , Síndrome POEMS/diagnóstico , Síndrome POEMS/patologia , Síndrome POEMS/terapia , Diagnóstico Diferencial , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/terapia
2.
Zh Nevrol Psikhiatr Im S S Korsakova ; 119(2. Vyp. 2): 52-57, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31156242

RESUMO

Susac syndrome (SS) is an extremely rare neurological disorder characterized by symptoms of a clinical triad: encephalopathy, retinopathy and hearing loss. These problems arise from the microangiopathy of blood vessels that supply blood to the brain, the retina and the cochlea. SS is more common in young women. The authors present a literature review on the diagnosis and treatment of SS and the clinical observation of patients with classic signs of the disease triad.


Assuntos
Encefalopatias , Doenças Retinianas , Síndrome de Susac , Doenças Vasculares , Feminino , Humanos , Síndrome de Susac/complicações , Síndrome de Susac/diagnóstico
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