| [PMID]: | 27000248 |
| [Au] Autor: | Cortese A; Franciotta D; Alfonsi E; Visigalli N; Zardini E; Diamanti L; Prunetti P; Osera C; Gastaldi M; Berzero G; Pichiecchio A; Piccolo G; Lozza A; Piscosquito G; Salsano E; Ceroni M; Moglia A; Bono G; Pareyson D; Marchioni E |
| [Ad] Endereço: | C. Mondino National Neurological Institute, Pavia, Italy. Electronic address: andrea.cortese@mondino.it. |
| [Ti] Título: | Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment. |
| [So] Source: | J Neurol Sci;363:182-7, 2016 Apr 15. |
| [Is] ISSN: | 1878-5883 |
| [Cp] País de publicação: | Netherlands |
| [La] Idioma: | eng |
| [Ab] Resumo: | Combined central and peripheral demyelination (CCPD) is rare, and current knowledge is based on case reports and small case series. The aim of our study was to describe the clinical features, diagnostic results, treatment and outcomes in a large cohort of patients with CCPD. Thirty-one patients entered this retrospective, observational, two-center study. In 20 patients (65%) CCPD presented, after an infection, as myeloradiculoneuropathy, encephalopathy, cranial neuropathy, length-dependent peripheral neuropathy, or pseudo-Guillain-Barré syndrome. Demyelinating features of peripheral nerve damage fulfilling European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria for CIDP were found in 23 patients (74%), and spatial dissemination of demyelinating lesions on brain MRI fulfilling the 2010 McDonald criteria for multiple sclerosis (MS) in 11 (46%). Two thirds of the patients had a relapsing or progressive disease course, usually related to the appearance of new spinal cord lesions or worsening of the peripheral neuropathy, and showed unsatisfactory responses to high-dose corticosteroids and intravenous immunoglobulins. The clinical presentation of CCPD was severe in 22 patients (71%), who were left significantly disabled. Our data suggest that CCPD has heterogeneous features and shows frequent post-infectious onset, primary peripheral nervous system or central nervous system involvement, a monophasic or chronic disease course, inadequate response to treatments, and a generally poor outcome. We therefore conclude that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of CCPD, whose pathogenesis remains largely unknown. |
| [Mh] Termos MeSH primário: |
Doenças Desmielinizantes/diagnóstico por imagem
Doenças Desmielinizantes/terapia
|
| [Mh] Termos MeSH secundário: |
Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Estudos de Coortes
Feminino
Seguimentos
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Polirradiculoneuropatia/diagnóstico por imagem
Polirradiculoneuropatia/terapia
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico por imagem
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia
Estudos Retrospectivos
Resultado do Tratamento
Adulto Jovem
|
| [Pt] Tipo de publicação: | JOURNAL ARTICLE; MULTICENTER STUDY; OBSERVATIONAL STUDY; RESEARCH SUPPORT, NON-U.S. GOV'T |
| [Em] Mês de entrada: | 1612 |
| [Cu] Atualização por classe: | 161231 |
[Lr] Data última revisão:
| 161231 |
| [Sb] Subgrupo de revista: | IM |
| [Da] Data de entrada para processamento: | 160323 |
| [St] Status: | MEDLINE |
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