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A Esclerose Múltipla (EM) e o Síndrome Guillain-Barré (SGB) são ambos doenças autoimunes e desmielinizantes que afetam, respetivamente, o Sistema Nervoso Central (SNC) e o Sistema Nervoso Periférico (SNP), pertencendo ainda a um grupo de doenças neurodegenerativas que envolvem lesões inflamatórias associadas a desmielinização, induzindo dano no axónio e consequente neurodegeneração, o que leva a uma perda de função progressiva. A EM é uma doença inflamatória crónica do SNC sendo a causa mais frequente de distúrbios neurológicos em jovens adultos. É uma doença que consiste na inflamação, desmielinização e uma variável perda axonal. A sua etiologia ainda não é completamente conhecida, mas presume-se que envolva a interação entre fatores genéticos e ambientais, estimulando um ataque autoimune e consequentes danos na mielina e nos axónios. Clinicamente, a maior parte dos doentes tem uma fase recidivante-remitente, caracterizada pela presença de surtos seguida de recuperação. Destes doentes, a maioria progride para uma doença secundária progressiva e os restantes doentes desenvolvem uma Esclerose Múltipla primária progressiva. Alguns doentes têm ainda um síndrome clinicamente isolado que corresponde a um primeiro episódio de sintomas neurológicos no SNC, sendo que estes podem ou não evoluir para Esclerose Múltipla. Em termos de tratamento, estão aprovados os medicamentos modificadores de doença, especialmente no caso de doença recidivante-remitente. A SGB é uma doença inflamatória, mas do SNP, sendo a causa mais frequente de paralisia flácida aguda. Esta doença autoimune é antecedida por uma infeção viral ou bacteriana, como vírus Influenza ou Campilobacter jejuni, que são capazes de desencadear uma resposta imune anormal direcionada contra os componentes dos nervos periféricos, por mimetismo molecular. As formas mais frequentes são polineuropatia desmielinizante inflamatória aguda e neuropatia motora axonal aguda, existindo ainda a neuropatia motora sensorial axonal aguda e a síndrome de Miller Fisher. Os doentes com SGB têm insuficiência respiratória e disfunção autónoma como complicações associadas. O tratamento é composto por uma abordagem multidisciplinar que inclui cuidados médicos gerais e imunoterapia. As prioridades na investigação da EM e da SGB incluem o desenvolvimento de biomarcadores e um melhor conhecimento da imunopatogénese, para que haja medicina personalizada.
Multiple Sclerosis (MS) and Guillain-Barré Syndrome (GBS) are both demyelinating and autoimmune disorders affecting, respectively, the central nervous system (CNS) and the peripheral nervous system (PNS), which means they belong to a group of neurodegenerative diseases that involve inflammatory lesions associated with demyelination, inducing axonal damage and consequent neurodegeneration, leading to progressive loss of function. MS is a chronic inflammatory disorder of the CNS and is assumed to be the most frequent cause of neurological disability in young adults. This disorder consists in inflammation, demyelination and variable levels of axonal loss. The etiology is still unknown but it is presumed to involve interaction between genetic and environmental factors that triggers an autoimmune attack, resulting in damaged myelin and axons. Clinically, most of the patients experience a relapsing-remitting phase, characterized by relapses followed by recovery. The majority of them, late on enter in a progressive phase called secondary progressive MS. The remaining patients pursue a progressive course that is called primary progressive MS. There is also clinically isolated syndrome corresponding to a first episode of neurologic symptoms in the CNS, and people who experience it may or may not develop MS. In terms of therapeutic options, disease-modifying treatments are approved specially to treat relapsing remitting form of the disease. GBS is also an inflammatory demyelinating disease of the PNS and it is the most frequent cause of acute flaccid paralysis. This autoimmune disorder is, in most cases, preceded by viral or bacterial infections, such as Campylobacter jejuni or Influenza virus, that are capable of triggering an abnormal immune responses directed against components of the peripheral nerves by molecular mimicry. Clinically, the most frequent forms of GBS is acute inflammatory demyelinating polyradiculoneuropathy and acute motor axonal neuropathy, but there is also acute motor-sensory axonal neuropathy and Miller-Fischer syndrome. Patients with GBS commonly have respiratory insufficiency and autonomic dysfunction as associated complications. The treatment of this syndrome is composed by a multidisciplinary approach that includes general medical care and immunotherapies. The priorities for MS and GBS investigation include establishment of biomarkers and an improved knowledge of the immunopathogenesis, to go towards personalized medicine.
Multiple Sclerosis (MS) and Guillain-Barré Syndrome (GBS) are both demyelinating and autoimmune disorders affecting, respectively, the central nervous system (CNS) and the peripheral nervous system (PNS), which means they belong to a group of neurodegenerative diseases that involve inflammatory lesions associated with demyelination, inducing axonal damage and consequent neurodegeneration, leading to progressive loss of function. MS is a chronic inflammatory disorder of the CNS and is assumed to be the most frequent cause of neurological disability in young adults. This disorder consists in inflammation, demyelination and variable levels of axonal loss. The etiology is still unknown but it is presumed to involve interaction between genetic and environmental factors that triggers an autoimmune attack, resulting in damaged myelin and axons. Clinically, most of the patients experience a relapsing-remitting phase, characterized by relapses followed by recovery. The majority of them, late on enter in a progressive phase called secondary progressive MS. The remaining patients pursue a progressive course that is called primary progressive MS. There is also clinically isolated syndrome corresponding to a first episode of neurologic symptoms in the CNS, and people who experience it may or may not develop MS. In terms of therapeutic options, disease-modifying treatments are approved specially to treat relapsing remitting form of the disease. GBS is also an inflammatory demyelinating disease of the PNS and it is the most frequent cause of acute flaccid paralysis. This autoimmune disorder is, in most cases, preceded by viral or bacterial infections, such as Campylobacter jejuni or Influenza virus, that are capable of triggering an abnormal immune responses directed against components of the peripheral nerves by molecular mimicry. Clinically, the most frequent forms of GBS is acute inflammatory demyelinating polyradiculoneuropathy and acute motor axonal neuropathy, but there is also acute motor-sensory axonal neuropathy and Miller-Fischer syndrome. Patients with GBS commonly have respiratory insufficiency and autonomic dysfunction as associated complications. The treatment of this syndrome is composed by a multidisciplinary approach that includes general medical care and immunotherapies. The priorities for MS and GBS investigation include establishment of biomarkers and an improved knowledge of the immunopathogenesis, to go towards personalized medicine.
Description
Trabalho Final de Mestrado Integrado, Ciências Farmacêuticas, Universidade de Lisboa, Faculdade de Farmácia, 2019
Keywords
Esclerose Múltipla Síndrome Guillain-Barré Inflamação Desmielinização Neurodegeneração. Mestrado Integrado - 2019