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Advisor(s)
Abstract(s)
A polineuropatia desmielinizante inflamatória crónica é uma neuropatia adquirida do
sistema nervoso periférico.
A incidência e a prevalência da patologia são baixas. Esta surge mais frequentemente
em adultos e apresenta uma ligeira predileção pelo sexo masculino.
A sua etiologia encontra-se pouco esclarecida, estando descritas inúmeras teorias
acerca da origem dos mecanismos imunes, que cursam com envolvimento dos linfócitos B e T e
culminam em desmielinização nervosa e resposta celular inflamatória.
As manifestações clínicas caracterizam-se por fraqueza simétrica dos músculos
proximais e distais, associada a comprometimento sensorial, num período igual ou superior a
oito semanas. Poderá encontrar-se diminuição ou ausência dos reflexos tendinosos profundos
e, raramente, envolvimento dos nervos cranianos. Encontram-se descritas diversas variantes
clínicas da doença.
O diagnóstico da patologia baseia-se na avaliação clínica do paciente e na realização
de exames eletrofisiológicos, que revelam alterações na velocidade de condução nervosa,
resultados compatíveis com áreas de desmielinização. A análise do líquido cefalorraquidiano
encontra-se alterada, com aumento da concentração de proteínas.
O tratamento de primeira linha envolve a administração de imunoglobulina intravenosa,
de corticóides ou a realização de plasmaferese. No caso dos doentes refratários a estas
terapias, é possível obter algum benefício no uso de fármacos imunossupressores; porém, a sua
efetividade ainda permanece em estudo.
Apesar de constituir uma realidade clínica rara, a polineuropatia desmielinizante
inflamatória crónica é uma neuropatia tratável e com bom prognóstico. No entanto, esta está
frequentemente associada a dificuldades no seu tratamento, o que justifica a pertinência na
realização de uma revisão bibliográfica acerca deste tema.
Os objetivos desta monografia cursam com a realização de uma revisão do estado da
arte da polineuropatia desmielinizante inflamatória crónica, nomeadamente ao nível da sua
epidemiologia, etiologia, fisiopatologia, apresentação clínica e critérios de diagnóstico, assim
como a sistematização das abordagens terapêuticas disponíveis.
A metodologia utilizada para a realização desta monografia baseia-se numa revisão
bibliográfica exaustiva da informação mais atual existente sobre o tema polineuropatia
desmielinizante inflamatória crónica nas bases de dados Pubmed, B-on, o motor de busca
Google Académico e em livros de referência da área da neurologia. A pesquisa foi realizada
com os critérios de seleção linguísticos português, inglês e espanhol e os critérios de seleção
temporais datados dos últimos 10 anos. Os termos-chave de pesquisa utilizados foram “chronic
inflammatory demyelinating polyneuropathy” e “CIDP”, combinados com os termos
“epidemiology”, “etiology”, “physiopathology”, “clinical findings”, “diagnostic criteria”,
“treatment”. A polineuropatia desmielinizante inflamatória crónica é uma das escassas neuropatias farmacologicamente tratáveis e, por isso, é crucial não estagnar o conhecimento acerca desta patologia. Assim, é essencial investir na investigação de marcadores de diagnóstico da doença, em escalas adequadas para a avaliação da incapacidade dos pacientes e no desenvolvimento de novas opções terapêuticas seguras e eficazes.
Chronic inflammatory demyelinating polyneuropathy is an acquired neuropathy of the peripheral nervous system. Disease’s incidence and prevalence are low. This affects adults more often and has a slight predominance on the male sex. Etiology is poorly understood and several theories are described about the origin of immune mechanisms that occur with involvement of B and T lymphocytes and culminate in nervous demyelination and inflammatory cell response. Clinical manifestations are characterized by symmetric weakness of the proximal and distal muscles, associated with sensory impairment, in a period not less than eight weeks. Decreased or absent deep tendon reflexes may occur and, rarely, there is the involvement of the cranial nerves. Several clinical variants of the disease are described. The diagnosis of the disease is based on patient’s clinical evaluation and on conducting electrophysiological tests, which show changes in nerve conduction velocity, consistent findings with areas of demyelination. The analysis of cerebrospinal fluid is altered with increasing protein concentration. The first-line treatment involves administration of intravenous immunoglobulin, steroids or implementation of plasma exchange. In the case of refractory patients to these therapies, it is possible to get some benefit from the use of immunosuppressive drugs; however, its effectiveness is still being studied. Although this is a rare clinical reality with a good prognosis, chronic inflammatory demyelinating polyneuropathy is the most treatable neuropathy. Nevertheless, it is often associated with difficulties in its treatment, which justifies the relevance in carrying out a literature review on the subject. The aims of this monograph are to review the state of the art of chronic inflammatory demyelinating polyneuropathy, especially in terms of epidemiology, etiology, pathophysiology, clinical presentation and diagnostic criteria, as well as the systematization of therapeutic approaches available. The methodology used for the elaboration of this monograph is based on an exhaustive literature review of the most up-to-date information available on the topic chronic inflammatory demyelinating polyneuropathy in Pubmed, B-on, the search engine Google Scholar and in the neurology’s reference books. The search was under Portuguese, English and Spanish language selection criteria and temporal selection criteria dated within the last 10 years. The key search terms used were “chronic inflammatory demyelinating polyneuropathy” and “CIDP”, combined with the terms “epidemiology”, “etiology”, “physiopathology”, “clinical findings”, “diagnostic criteria”, “treatment”. Chronic inflammatory demyelinating polyneuropathy is one of the few pharmacologically treatable neuropathies and, therefore, it is crucial not to neglect the knowledge of this condition. Thus, it is essential to invest in research on disease’s diagnostic markers, on appropriate scale for assessing the inability of the patients and on the development of safe and effective new treatment options.
Chronic inflammatory demyelinating polyneuropathy is an acquired neuropathy of the peripheral nervous system. Disease’s incidence and prevalence are low. This affects adults more often and has a slight predominance on the male sex. Etiology is poorly understood and several theories are described about the origin of immune mechanisms that occur with involvement of B and T lymphocytes and culminate in nervous demyelination and inflammatory cell response. Clinical manifestations are characterized by symmetric weakness of the proximal and distal muscles, associated with sensory impairment, in a period not less than eight weeks. Decreased or absent deep tendon reflexes may occur and, rarely, there is the involvement of the cranial nerves. Several clinical variants of the disease are described. The diagnosis of the disease is based on patient’s clinical evaluation and on conducting electrophysiological tests, which show changes in nerve conduction velocity, consistent findings with areas of demyelination. The analysis of cerebrospinal fluid is altered with increasing protein concentration. The first-line treatment involves administration of intravenous immunoglobulin, steroids or implementation of plasma exchange. In the case of refractory patients to these therapies, it is possible to get some benefit from the use of immunosuppressive drugs; however, its effectiveness is still being studied. Although this is a rare clinical reality with a good prognosis, chronic inflammatory demyelinating polyneuropathy is the most treatable neuropathy. Nevertheless, it is often associated with difficulties in its treatment, which justifies the relevance in carrying out a literature review on the subject. The aims of this monograph are to review the state of the art of chronic inflammatory demyelinating polyneuropathy, especially in terms of epidemiology, etiology, pathophysiology, clinical presentation and diagnostic criteria, as well as the systematization of therapeutic approaches available. The methodology used for the elaboration of this monograph is based on an exhaustive literature review of the most up-to-date information available on the topic chronic inflammatory demyelinating polyneuropathy in Pubmed, B-on, the search engine Google Scholar and in the neurology’s reference books. The search was under Portuguese, English and Spanish language selection criteria and temporal selection criteria dated within the last 10 years. The key search terms used were “chronic inflammatory demyelinating polyneuropathy” and “CIDP”, combined with the terms “epidemiology”, “etiology”, “physiopathology”, “clinical findings”, “diagnostic criteria”, “treatment”. Chronic inflammatory demyelinating polyneuropathy is one of the few pharmacologically treatable neuropathies and, therefore, it is crucial not to neglect the knowledge of this condition. Thus, it is essential to invest in research on disease’s diagnostic markers, on appropriate scale for assessing the inability of the patients and on the development of safe and effective new treatment options.
Description
Keywords
Desmielinização Nervosa Estudos de Condução Nervosa Imunoglobulina Intravenosa Polineuropatia Desmielinizante Inflamatória Crónica Sistema Nervoso Periférico