RESUMO
Since the immunochemical identification of the bullous pemphigoid antigen 230 (BP230) as one of the major target autoantigens of bullous pemphigoid (BP) in 1981, our understanding of this protein has significantly increased. Cloning of its gene, development and characterization of animal models with engineered gene mutations or spontaneous mouse mutations have revealed an unexpected complexity of the gene encoding BP230. The latter, now called dystonin (DST), is composed of at least 100 exons and gives rise to three major isoforms, an epithelial, a neuronal and a muscular isoform, named BPAG1e (corresponding to the original BP230), BPAG1a and BPAG1b, respectively. The various BPAG1 isoforms play a key role in fundamental processes, such as cell adhesion, cytoskeleton organization, and cell migration. Genetic defects of BPAG1 isoforms are the culprits of epidermolysis bullosa and complex, devastating neurological diseases. In this review, we summarize recent advances of our knowledge about several BPAG1 isoforms, their role in various biological processes and in human diseases.
Assuntos
Distonina/metabolismo , Regulação da Expressão Gênica , Penfigoide Bolhoso/genética , Penfigoide Bolhoso/imunologia , Animais , Autoantígenos/imunologia , Adesão Celular , Movimento Celular , Citoesqueleto/metabolismo , Células Epiteliais/metabolismo , Éxons , Perfilação da Expressão Gênica , Homeostase , Humanos , Imuno-Histoquímica , Camundongos , Músculo Esquelético/metabolismo , Músculos/metabolismo , Mutação , Neurônios/metabolismo , Plaquinas/metabolismo , Domínios Proteicos , Isoformas de Proteínas/metabolismoRESUMO
BACKGROUND: To study the 1-year evolution of quantitative dual-task gait parameters in comparison with single-task gait parameters and detailed neuropsychological assessment in patients with multiple sclerosis (MS) treated with natalizumab. METHODS: Walking speed, stride length and stride time during a dual task (walking while forward counting, backward counting, semantic fluency, and phonemic fluency), a single walking task, and a detailed neuropsychological assessment were prospectively measured and assessed twice at the 1-year interval in 9 consecutive patients with MS treated with natalizumab. RESULTS: Dual-task-related gait changes (walking speed, stride length and stride time while performing semantic fluency and walking speed, and stride time while performing phonemic fluency) showed a significant improvement after 1 year of treatment with natalizumab. The single walking task and detailed neuropsychological assessment did not present any modification. CONCLUSIONS: Dual-task-related gait changes using a cognitive task with a specific executive demand represent an interesting marker of disease-modifying therapy in patients with MS.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Função Executiva/efeitos dos fármacos , Fatores Imunológicos/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/psicologia , Desempenho Psicomotor/efeitos dos fármacos , Adulto , Fenômenos Biomecânicos , Cognição/efeitos dos fármacos , Cognição/fisiologia , Função Executiva/fisiologia , Feminino , Seguimentos , Marcha/efeitos dos fármacos , Marcha/fisiologia , Humanos , Masculino , Esclerose Múltipla Recidivante-Remitente/fisiopatologia , Natalizumab , Testes Neuropsicológicos , Estudos Prospectivos , Desempenho Psicomotor/fisiologia , Resultado do Tratamento , Caminhada/fisiologiaRESUMO
A 75-year-old woman with unremarkable medical history, consulted for a 5-month history of involuntary shaking of left upper limb. Clinical examination revealed polyminimyoclonus of the upper limbs with cogwheel-like rigidity, hyperreflexia, bradykinesia, inconstant spastic-like rigidity in the lower limbs and a stiff and cautious gait. These symptoms, together with the memory impairment found on neuropsychological assessment yielded suspicion for a subacute encephalopathy probably due to a non-conventional infectious agent. There was no 14-3-3 protein found in the cerebrospinal fluid and no periodic sharp wave complexes on EEG. These findings made the diagnosis of Creutzfeldt-Jakob disease (CJD) rather unlikely according to the current WHO diagnostic criteria. However, typical isolated cortical hyperintensity of right temporal, parietal and occipital lobes on MRI suggested a probable CJD and prompted cerebral biopsy which confirmed the diagnosis. This article emphasises the need to update the current WHO criteria by including radiological findings.
Assuntos
Córtex Cerebral , Síndrome de Creutzfeldt-Jakob/diagnóstico , Proteínas 14-3-3/líquido cefalorraquidiano , Idoso , Biópsia , Córtex Cerebral/patologia , Síndrome de Creutzfeldt-Jakob/patologia , Eletroencefalografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Sensibilidade e Especificidade , Organização Mundial da SaúdeRESUMO
BACKGROUND: The envelope protein from multiple sclerosis (MS) associated retroviral element (MSRV), a member of the Human Endogenous Retroviral family 'W' (HERV-W), induces dysimmunity and inflammation. OBJECTIVE: The objective of this study was to confirm and specify the association between HERV-W/MSRV envelope (Env) expression and MS. METHODS: 103 MS, 199 healthy controls (HC) and controls with other neurological diseases (28), chronic infections (30) or autoimmunity (30) were analysed with an immunoassay detecting Env in serum. Env RNA or DNA copy numbers in peripheral blood mononuclear cells (PBMC) were determined by a quantitative polymerase chain reaction (PCR). Env was detected by immunohistology in the brains of patients with MS with three specific monoclonals. RESULTS: Env antigen was detected in a serum of 73% of patients with MS with similar prevalence in all clinical forms, and not in chronic infection, systemic lupus, most other neurological diseases and healthy donors (p<0.01). Cases with chronic inflammatory demyelinating polyneuropathy (5/8) and rare HC (4/103) were positive. RNA expression in PBMC and DNA copy numbers were significantly elevated in patients with MS versus HC (p<0.001). In patients with MS, DNA copy numbers were significantly increased in chronic progressive MS (secondary progressive MS vs relapsing-remitting MS (RRMS) p<0.001; primary progressive MS vs RRMS -<0.02). Env protein was evidenced in macrophages within MS brain lesions with particular concentrations around vascular elements. CONCLUSION: The association between MS disease and the MSRV-type HERV-W element now appears quite strong, as evidenced ex-vivo from serum and PBMC with post-mortem confirmation in brain lesions. Chronic progressive MS, RRMS and clinically isolated syndrome show different ELISA (Enzyme-Linked Immunosorbent Assay) and/or PCR profiles suggestive of an increase with disease evolution, and amplicon sequencing confirms the association with particular HERV-W elements.
Assuntos
Encéfalo/virologia , Retrovirus Endógenos , Esclerose Múltipla/virologia , Proteínas do Envelope Viral/metabolismo , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/sangue , Reação em Cadeia da Polimerase em Tempo Real , Proteínas do Envelope Viral/análiseRESUMO
Immune-mediated diseases of the CNS, such as multiple sclerosis and its animal model, experimental autoimmune encephalitis (EAE), are characterized by the activation of antigen-presenting cells and the infiltration of autoreactive lymphocytes within the CNS, leading to demyelination, axonal damage, and neurological deficits. Hepatocyte growth factor (HGF) is a pleiotropic factor known for both neuronal and oligodendrocytic protective properties. Here, we assess the effect of a selective overexpression of HGF by neurons in the CNS of C57BL/6 mice carrying an HGF transgene (HGF-Tg mice). EAE induced either by immunization with myelin oligodendrocyte glycoprotein peptide or by adoptive transfer of T cells was inhibited in HGF-Tg mice. Notably, the level of inflammatory cells infiltrating the CNS decreased, except for CD25(+)Foxp3(+) regulatory T (T(reg)) cells, which increased. A strong T-helper cell type 2 cytokine bias was observed: IFN-gamma and IL-12p70 decreased in the spinal cord of HGF-Tg mice, whereas IL-4 and IL-10 increased. Antigen-specific response assays showed that HGF is a potent immunomodulatory factor that inhibits dendritic cell (DC) function along with differentiation of IL-10-producing T(reg) cells, a decrease in IL-17-producing T cells, and down-regulation of surface markers of T-cell activation. These effects were reversed fully when DC were pretreated with anti-cMet (HGF receptor) antibodies. Our results suggest that, by combining both potentially neuroprotective and immunomodulatory effects, HGF is a promising candidate for the development of new treatments for immune-mediated demyelinating diseases associated with neurodegeneration such as multiple sclerosis.
Assuntos
Células Dendríticas/imunologia , Encefalomielite Autoimune Experimental/imunologia , Fator de Crescimento de Hepatócito/imunologia , Tolerância Imunológica , Linfócitos T Reguladores/imunologia , Animais , Apresentação de Antígeno , Proliferação de Células , Citocinas/biossíntese , Citocinas/imunologia , Encefalomielite Autoimune Experimental/genética , Fatores de Transcrição Forkhead/imunologia , Fator de Crescimento de Hepatócito/genética , Subunidade alfa de Receptor de Interleucina-2/imunologia , Ativação Linfocitária , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Baço/citologia , Baço/imunologia , Linfócitos T Reguladores/citologia , Células Th2/imunologiaRESUMO
Bacterial and viral infections have long been implicated in pathogenesis and progression of multiple sclerosis (MS). Incidence and severity of its animal model experimental autoimmune encephalomyelitis (EAE) can be enhanced by concomitant administration of pertussis toxin (PTx), the major virulence factor of Bordetella pertussis. Its adjuvant effect at the time of immunization with myelin antigen is attributed to an unspecific activation and facilitated migration of immune cells across the blood brain barrier into the central nervous system (CNS). In order to evaluate whether recurring exposure to bacterial antigen may have a differential effect on development of CNS autoimmunity, we repetitively administered PTx prior to immunization. Mice weekly injected with PTx were largely protected from subsequent EAE induction which was reflected by a decreased proliferation and pro-inflammatory differentiation of myelin-reactive T cells. Splenocytes isolated from EAE-resistant mice predominantly produced IL-10 upon re-stimulation with PTx, while non-specific immune responses were unchanged. Longitudinal analyses revealed that repetitive exposure of mice to PTx gradually elevated serum levels for TGF-ß and IL-10 which was associated with an expansion of peripheral CD4(+)CD25(+)FoxP3(+) regulatory T cells (Treg). Increased frequency of Treg persisted upon immunization and thereafter. Collectively, these data suggest a scenario in which repetitive PTx treatment protects mice from development of CNS autoimmune disease through upregulation of regulatory cytokines and expansion of CD4(+)CD25(+)FoxP3(+) Treg. Besides its therapeutic implication, this finding suggests that encounter of the immune system with microbial products may not only be part of CNS autoimmune disease pathogenesis but also of its regulation.
Assuntos
Doenças Autoimunes/prevenção & controle , Doenças do Sistema Nervoso Central/prevenção & controle , Toxina Pertussis/química , Linfócitos T/imunologia , Animais , Linfócitos T CD4-Positivos/citologia , Proliferação de Células , Feminino , Fatores de Transcrição Forkhead/metabolismo , Interleucina-10/metabolismo , Subunidade alfa de Receptor de Interleucina-2/metabolismo , Camundongos , Camundongos Endogâmicos C57BL , Toxina Pertussis/metabolismo , Linfócitos T Reguladores/metabolismo , Fator de Crescimento Transformador beta/metabolismoRESUMO
SUMMARY: This study presents a method to record and analyze multichannel visual-evoked potential (VEP) and somatosensory-evoked potential (SEP) in an objective, automatic, and quantitative manner. The intention of this study was to assess their diagnostic value in multiple sclerosis (MS). A 256-channel VEP and SEP were recorded in 44 healthy subjects, 26 patients with MS, and 20 patients with other neurologic diseases. Topographic pattern recognition methods were applied and a normative database was established. Z-score statistics allowed identifying the number of subjects with significant abnormal values in each group. These values were compared with conventional single-channel waveform analysis. The diagnostic value of the new measures for MS reached a sensitivity of 72% and a specificity of 100% for the VEP, which was significantly higher than the conventional analysis. For the SEP, the specificity was also high (93%) but the sensitivity remained low as in the conventional analysis (30%). The quantitative topographic analysis of multichannel VEP revealed high-diagnostic sensitivity and specificity for MS. Moreover, the method reliably identified the most dominant VEP and SEP components in the healthy subject group. The results indicate that objective topographic analysis of multichannel recordings increase the value of VEP as surrogate marker for MS.
Assuntos
Encéfalo/fisiopatologia , Potenciais Somatossensoriais Evocados , Potenciais Evocados Visuais , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/fisiopatologia , Adolescente , Adulto , Bases de Dados como Assunto , Eletroencefalografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Padrões de Referência , Sensibilidade e Especificidade , Processamento de Sinais Assistido por Computador , Adulto JovemRESUMO
Mechanisms of action as well as cellular targets of glatiramer acetate (GA) in multiple sclerosis (MS) are still not entirely understood. IL-1beta is present in CNS-infiltrating macrophages and microglial cells and is an important mediator of inflammation in experimental autoimmune encephalitis (EAE), the MS animal model. A natural inhibitor of IL-1beta, the secreted form of IL-1 receptor antagonist (sIL-1Ra) improves EAE disease course. In this study we examined the effects of GA on the IL-1 system. In vivo, GA treatment enhanced sIL-1Ra blood levels in both EAE mice and patients with MS, whereas IL-1beta levels remained undetectable. In vitro, GA per se induced the transcription and production of sIL-1Ra in isolated human monocytes. Furthermore, in T cell contact-activated monocytes, a mechanism relevant to chronic inflammation, GA strongly diminished the expression of IL-1beta and enhanced that of sIL-1Ra. This contrasts with the effect of GA in monocytes activated upon acute inflammatory conditions. Indeed, in LPS-activated monocytes, IL-1beta and sIL-1Ra production were increased in the presence of GA. These results demonstrate that, in chronic inflammatory conditions, GA enhances circulating sIL-1Ra levels and directly affects monocytes by triggering a bias toward a less inflammatory profile, increasing sIL-1Ra while diminishing IL-1beta production. This study sheds light on a mechanism that is likely to participate in the therapeutic effects of GA in MS.
Assuntos
Proteína Antagonista do Receptor de Interleucina 1/efeitos dos fármacos , Interleucina-1beta/efeitos dos fármacos , Monócitos/imunologia , Esclerose Múltipla/tratamento farmacológico , Peptídeos/farmacologia , Animais , Encefalomielite Autoimune Experimental/tratamento farmacológico , Acetato de Glatiramer , Humanos , Imunossupressores/farmacologia , Inflamação/tratamento farmacológico , Proteína Antagonista do Receptor de Interleucina 1/sangue , Camundongos , Esclerose Múltipla/imunologia , Transcrição GênicaRESUMO
Inflammatory neuropathies include those neuropathies in which the diagnosis, outcome and type of treatment are badly known, the reason of this review. They are expressed as diffuse (such as CIDP and ganglionopathies), multifocal (vasculitic neuropathy) or focal (MMN; plexopathies; immune reconstitution inflammatory syndrome). These forms of neuropathies are important to be known because the beneficial therapeutic possibilities of immunosuppression.
Assuntos
Mononeuropatias , Neurite (Inflamação) , Polirradiculoneuropatia , Humanos , Mononeuropatias/diagnóstico , Mononeuropatias/terapia , Neurite (Inflamação)/diagnóstico , Neurite (Inflamação)/terapia , Polirradiculoneuropatia/diagnóstico , Polirradiculoneuropatia/terapiaRESUMO
OBJECTIVES: multiple sclerosis (MS) is an inflammatory disease of unknown origin affecting the central nervous system. Magnetic resonance imaging (MRI) plays an increasingly important role in its diagnosis and further monitoring of disease progress. METHODS: the typical MRI appearance of MS on conventional MRI sequences and current diagnostic criteria for MS are discussed. Advanced imaging techniques are reviewed with respect to application in MS. Finally, the atypical variants of MS are briefly reviewed. CONCLUSIONS: although MRI is not intended and will not replace clinical assessment in MS, the recognized MRI criteria may aid in establishing an earlier and more accurate diagnosis of MS in the context of a clinical suspicion or clinically isolated syndrome. In addition, MRI might contribute to rule out differential diagnoses for MS. Moreover, MRI may be used to monitor the evolution of MS and in pharmaceutical trials. Advanced imaging techniques might, in the future, further characterize MS lesion subtypes and potentially guide tailored therapy.
Assuntos
Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico , Humanos , Imageamento por Ressonância Magnética/métodos , Imageamento por Ressonância Magnética/tendênciasRESUMO
"What are you doing today? I'm suffering." So expressed himself Alphonse Daudet the French writer. The pain he had gave him the spirit when he wrote a text entitled "La Doulou" a word from the South of France meaning "The pain". Speaking about pain requires listening to suffering patients. Many of them can be good observers and describe pain deeply and subtly. This paper covers the clinical approach of pain in MS patients. Can pain be the first sign of MS, are they frequent, what are their specific properties, how do we classify them, how do we recognize them to alleviate the burden of pain. These are the daily questions we ask as neurologists, while running neurological examination and listening to each patient. Gathered information will hopefully help you to find an appropriate answer for each patient.
Assuntos
Esclerose Múltipla/complicações , Dor/etiologia , Humanos , Esclerose Múltipla/diagnóstico , Dor/classificaçãoRESUMO
BACKGROUND: As results from an increasing number of clinical trials with disease-modifying drugs (DMDs) in multiple sclerosis (MS) become available, the challenge for the treating neurologist is how to decide on the appropriate therapy for an individual patient. OBJECTIVE: An International Working Group for Treatment Optimization in MS met to consider how the principles of evidence-based medicine (EBM) should be used to assess the current best evidence regarding the treatment of MS. This report summarizes the outcome from the workshop at which this topic was addressed. RESULTS: Class I evidence from head-to-head studies provides the best tool for direct comparisons of DMDs. However, other EBM approaches to data analysis from placebo-controlled trials can be used to help determine the benefits and risks of a particular DMD relative to placebo by calculating the number needed to treat to achieve a positive outcome, such as avoiding a relapse, and the number needed to harm to produce an additional adverse event, such as having a therapy-related dropout. This provides a structured basis for comparisons between DMDs. CONCLUSION: While such comparisons have their limitations, particularly when drugs with substantially different side-effect profiles are to be compared, they can provide useful information to guide treatment decisions.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Medicina Baseada em Evidências , Interferon beta/uso terapêutico , Mitoxantrona/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Anticorpos Monoclonais Humanizados , Humanos , Interferon beta-1a , NatalizumabRESUMO
BACKGROUND: Central nervous system involvement in primary Sjogren's syndrome is a matter of controversy, and its diagnosis remains difficult. METHODS: We report 3 patients with primary Sjogren's syndrome and central nervous system involvement in whom we assessed intrathecal immunoglobulin G synthesis and the presence of cerebrospinal fluid anti-SSA and anti-SSB autoantibodies. RESULTS: We found intrathecal immunoglobulin G synthesis and presence of cerebrospinal fluid anti-SSA autoantibodies in all patients, with demonstration for the first time of specific anti-SSA autoantibody intrathecal synthesis in 2 patients. CONCLUSION: We suggest that cerebrospinal fluid anti-SSA autoantibodies could serve as a biomarker for Sjogren's-syndrome-related central nervous system involvement.
Assuntos
Autoanticorpos/líquido cefalorraquidiano , Sistema Nervoso Central/fisiopatologia , Transtornos dos Movimentos/etiologia , Síndrome de Sjogren/imunologia , Anticorpos Antinucleares/sangue , Anticorpos Antinucleares/líquido cefalorraquidiano , Autoanticorpos/sangue , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunoglobulina G/líquido cefalorraquidiano , Pessoa de Meia-Idade , Síndrome de Sjogren/líquido cefalorraquidiano , Síndrome de Sjogren/patologia , Síndrome de Sjogren/fisiopatologiaRESUMO
The etiology of multiple sclerosis (MS) is incompletely understood, and evidence suggests there may be more than one underlying cause in this disorder. Furthermore, this complex and heterogeneous autoimmune disease shows a high degree of clinical variability between patients. Therefore, in the absence of a single therapeutic target for MS, it is difficult to apply conventional drug design strategies in the search for new treatments. We review the potential mechanisms of action of several effective therapies for MS that are currently available or in development. The effects of each treatment are described in terms of their actions on key processes in a five-step model of MS pathogenesis. Conventional immunosuppressants targeting intracellular ligands (e.g. mitoxantrone) have broad cytotoxic effects on B cells, T cells, and macrophages. This suppresses the pathogenic immune response in MS with high efficacy but is also associated with high toxicity, limiting the long-term use of these agents. Monoclonal antibodies (e.g. natalizumab and alemtuzumab) are a new generation of immunosuppressants that act on immune-cell surface ligands. These agents have narrower immunosuppressive actions and different safety profiles compared with conventional immunosuppressants. Immunomodulators (interferon-beta and glatiramer acetate), which shift the immune balance toward an anti-inflammatory response, are at the frontline of treatments for MS. Immunomodulators have targeted actions on the immune system, but affect a greater number of immunopathogenic processes than monoclonal antibodies. Given the inherent heterogeneity of MS, such treatments, which act at many levels of the disease, may achieve the best clinical results. Using our understanding of the interplay between mechanism of action and clinical effects in MS therapies may help us to better design and select new treatments for the future.
Assuntos
Esclerose Múltipla/tratamento farmacológico , Adjuvantes Imunológicos/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados , Acetato de Glatiramer , Humanos , Imunossupressores/uso terapêutico , Interferon beta/uso terapêutico , Ligantes , Mitoxantrona/uso terapêutico , Esclerose Múltipla/imunologia , Natalizumab , Peptídeos/uso terapêuticoRESUMO
A variety of viral, bacterial, fungal and spirochete infections may invade both the central and peripheral nervous system. The neurological hallmarks of these infectious diseases are very diverse and resulting in meningitis, meningoradiculitis, or as encephalomyelitis from the very start. Accordingly, patients may present with the cardinal signs of meningitis alone, or in association with focal neurological deficit, or with encephalopathy. This variability is related to the physiopathological mechanisms depending on the nature of the infectious agent. Thus the disease processes are due to the cytopathogenic action of these agents on the one hand and on the collateral damage induced by the immunological defence reaction of the patient on the other hand. In any case, serological and molecular analysis of blood and CSF samples are mandatory in order to diagnose correctly the underlying agent and to install prompt treatment that should be adapted to the new coming results. In this paper we will show examples of the clinical presentation of the most frequently encountered infectious diseases, the diagnostic approach and treatment.
Assuntos
Infecções do Sistema Nervoso Central/diagnóstico , Adulto , Idoso , Encefalomielite Aguda Disseminada/diagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Meningites Bacterianas/diagnóstico , Meningite Viral/diagnóstico , Meningoencefalite/diagnóstico , Meningoencefalite/virologia , Mielite/diagnóstico , Mielite/virologiaRESUMO
The authors conducted a prospective and descriptive pilot study in 14 healthy medical students, investigating whether a psychologically stressful event (final examination) may modify serum tumor necrosis factor alpha (TNF-alpha) levels. There was a dramatic and sustained decrease of phytohemagglutinin (PHA)-induced TNF-alpha several weeks before and the day of the examination, followed by a significant increase of TNF-alpha starting the next day. Examination-induced stress was confirmed by both elevated urinary cortisol concentration and significant increase in stress scale scores. Extending these results to patients suffering from multiple sclerosis (MS) leads to the hypothesis that psychological stress may influence the course of MS by substantially altering TNF-alpha levels, and suggests the need for further studies in MS patients exposed to stressful conditions.
Assuntos
Antineoplásicos/análise , Esclerose Múltipla/patologia , Esclerose Múltipla/psicologia , Estresse Psicológico , Fator de Necrose Tumoral alfa/análise , Adulto , Feminino , Humanos , Hidrocortisona/urina , Masculino , Estudos Prospectivos , RecidivaAssuntos
Adjuvantes Imunológicos/administração & dosagem , Adjuvantes Imunológicos/farmacologia , Interferon beta/administração & dosagem , Interferon beta/farmacologia , Esclerose Múltipla/tratamento farmacológico , Ensaios Clínicos como Assunto , Relação Dose-Resposta a Droga , Esquema de Medicação , Glicosilação , Humanos , Injeções Intramusculares , Injeções Subcutâneas , Interferon beta-1a , Esclerose Múltipla/patologia , Recidiva , Resultado do TratamentoRESUMO
A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) presented with an isolated unilateral adduction deficit and ptosis. Investigations were negative until the onset of limb weakness and fatigue 2 years later. At that time, electroneuromyography, cerebrospinal fluid examination, and magnetic resonance imaging confirmed the diagnosis of CIDP. Thus, ophthalmic signs can precede extremity and bulbar signs with a long latency in CIDP.