RESUMO
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome is a rare plasma cell disease. Vascular endothelial growth factor (VEGF) seems to play a pathogenic role. Peripheral neuropathy is the main neurological feature. Cranial pachymeningitis has occasionally been reported, but no histopathological studies have been performed. The authors extensively evaluated the central nervous system MRI in 11 patients (seven men, four women; mean age at diagnosis 54.45 years) with POEMS syndrome. In two patients, meningeal histopathology with staining for VEGF and VEGF receptor was performed, and pachymeningeal involvement characterised at histopathological, immunohistochemical and confocal microscopy levels. Nine patients presented with cranial pachymeningitis. One patient suffered from migraine, and none complained of cranial nerve palsies or visual loss. None showed any MRI signs of spinal pachymeningitis. No correlation was found with disease duration and VEGF serum level. Histopathology showed hyperplasia of meningothelial cells, neovascularisation and obstructive vessel remodelling, without inflammation. VEGF and VEGF receptor were strongly coexpressed on endothelium, smooth-muscle cells of arterioles and meningothelial cells. In conclusion, POEMS patients present a high prevalence of meningeal involvement. The histological changes, different from those present in chronic pachymeningitis of other aetiology, suggest a possible VEGF role in the pathogenesis of the meningeal remodelling.
Assuntos
Meninges/patologia , Síndrome POEMS/patologia , Encéfalo/patologia , Feminino , Imunofluorescência , Humanos , Imageamento por Ressonância Magnética , Masculino , Meningite/etiologia , Meningite/patologia , Pessoa de Meia-Idade , Síndrome POEMS/complicações , Receptores de Fatores de Crescimento do Endotélio Vascular/metabolismo , Medula Espinal/patologia , Fator A de Crescimento do Endotélio Vascular/metabolismoRESUMO
Colchicine, a long established anti-inflammatory agent now used in several rheumatologic conditions, acts by inhibiting microtubular polymerization, as it binds equimolarly to tubulin molecules. Cytoskeletal microtubules are crucial in processes of cell viability, such as mitosis and intracellular vesicle motility.Gastrointestinal side effects are quite common and often minor in nature or duration, whereas neuromuscular toxicity is rare. We report 2 cases of colchicine myopathy in the context of very different clinical settings.
Assuntos
Colchicina/efeitos adversos , Doenças Musculares/induzido quimicamente , Moduladores de Tubulina/efeitos adversos , Membro 1 da Subfamília B de Cassetes de Ligação de ATP/metabolismo , Adolescente , Artrite Gotosa/tratamento farmacológico , Colchicina/metabolismo , Colchicina/uso terapêutico , Citocromo P-450 CYP3A/metabolismo , Febre Familiar do Mediterrâneo/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/induzido quimicamente , Debilidade Muscular/diagnóstico , Doenças Musculares/diagnóstico , Moduladores de Tubulina/metabolismo , Moduladores de Tubulina/uso terapêuticoRESUMO
Celiac disease is a rare cause of neuropathy that most commonly presents with symmetric distal sensory disturbances. We describe two patients with celiac disease in whom neuropathy presented unusually with progressive weakness of the limbs. In both patients a gluten-free diet induced a significant improvement of muscle strength and neurophysiological abnormalities, suggesting a direct pathogenetic role of sensitivity to gluten. Celiac disease should be considered in patients with idiopathic neuropathy even when gastrointestinal symptoms are absent.