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This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.
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Doenças Transmissíveis , Doença de Lyme , Neurologia , Reumatologia , Animais , Humanos , Doença de Lyme/diagnóstico , Doença de Lyme/tratamento farmacológico , Doença de Lyme/prevenção & controle , América do Norte , Estados UnidosRESUMO
This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.
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Doenças Transmissíveis , Doença de Lyme , Neurologia , Reumatologia , Animais , Humanos , Doença de Lyme/diagnóstico , Doença de Lyme/tratamento farmacológico , Doença de Lyme/prevenção & controle , América do Norte , Estados UnidosRESUMO
SS is a chronic, autoimmune condition characterized by lymphocytic infiltration of the exocrine glands and B-cell dysfunction. Current treatment strategies are largely empirical and offer only symptomatic relief for patients. There are no proven treatments that alter disease progression or treat the systemic manifestations of disease. B-cell depletion is used in patients with systemic disease but its overall clinical efficacy has not been demonstrated in two large randomized controlled trials. Studies are now focussing on alternative strategies to target B-cells, including co-stimulation targets, with promising data. It is increasingly clear that clinical trials in SS will require patient stratification and relevant and sensitive outcome measures to identify successful treatment modalities.
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Linfócitos B/efeitos dos fármacos , Linfócitos B/imunologia , Terapia Biológica/métodos , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/imunologia , HumanosRESUMO
Prion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann-Straussler-Scheinker syndrome), and acquired (kuru, variant CJD, and iatrogenic CJD). This article will review the pathophysiology, genetics, clinical presentations, and diagnostic challenges in patients with prion disease. Case discussions, images, and tables will be used to highlight important characteristics of prion disease and prion mimics.
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Síndrome de Creutzfeldt-Jakob/líquido cefalorraquidiano , Síndrome de Creutzfeldt-Jakob/diagnóstico por imagem , Encefalopatia de Wernicke/líquido cefalorraquidiano , Encefalopatia de Wernicke/diagnóstico por imagem , Idoso , Animais , Síndrome de Creutzfeldt-Jakob/psicologia , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Priônicas/líquido cefalorraquidiano , Doenças Priônicas/diagnóstico por imagem , Doenças Priônicas/psicologia , Encefalopatia de Wernicke/psicologiaRESUMO
Chronic meningitis can be a diagnostic dilemma for even the most experienced clinician. Many times, the differential diagnosis is broad and encompasses autoimmune, neoplastic, and infectious etiologies. This review will focus on a general approach to chronic meningitis to simplify the diagnostic challenges many clinicians face. The article will also review the most common etiologies of chronic meningitis in some detail including clinical presentation, diagnostic testing, treatment, and outcomes. By using a case-based approach, we will focus on the key elements of clinical presentation and laboratory analysis that will yield the most rapid and accurate diagnosis in these complicated cases.
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Meningite , Adulto , Idoso , Doença Crônica , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Meningite/etiologia , Pessoa de Meia-Idade , Adulto JovemRESUMO
PURPOSE OF REVIEW: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system. It has been recently associated with selective immunosuppression in patients with multiple sclerosis. This review describes the pathogenesis, clinical presentation, diagnosis, and treatment of natalizumab-associated PML. RECENT FINDINGS: Treatment of multiple sclerosis with natalizumab first involves risk stratifying patients. Clinicians can employ new tools for risk stratification including JC-virus antibody status, prior immunosuppression, and length of natalizumab treatment. These tools can help minimize the risk of developing PML. Identifying patients with natalizumab-associated PML poses a diagnostic challenge for clinicians. Unique clinical features, sensitive laboratory analyses, and advanced MRI techniques have been identified that aid in the diagnosis of natalizumab-associated PML. SUMMARY: There continues to be significant gaps in our understanding of PML pathogenesis and its relationship with therapeutic immunosuppression. There have been advances made in our ability to treat multiple sclerosis, although these have come with the unintended risk of PML. Fortunately, natalizumab-associated PML remains a rare entity compared to multiple sclerosis-associated disability, and the risk may be mitigated with appropriate patient selection, accurate and rapid diagnosis, and aggressive treatment strategies.
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Leucoencefalopatia Multifocal Progressiva/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Humanos , Vírus JC/imunologia , Vírus JC/isolamento & purificação , Leucoencefalopatia Multifocal Progressiva/diagnóstico , Leucoencefalopatia Multifocal Progressiva/etiologia , Leucoencefalopatia Multifocal Progressiva/virologia , Esclerose Múltipla/complicações , Esclerose Múltipla/tratamento farmacológico , Natalizumab , Fatores de Risco , Resultado do TratamentoRESUMO
Early disseminated Lyme disease can involve the peripheral or central nervous system, but with early diagnosis and treatment, prognosis for full recovery is excellent. The typical clinical presentations of neuroborreliosis are highlighted, and an approach to diagnosis and treatment is described.
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Doenças dos Nervos Cranianos , Doença de Lyme , Meningite , Polirradiculopatia , Doenças dos Nervos Cranianos/diagnóstico , Doenças dos Nervos Cranianos/etiologia , Humanos , Doença de Lyme/diagnóstico , Doença de Lyme/tratamento farmacológicoRESUMO
Human herpesvirus-6 (HHV-6) was initially isolated in 1986 from patients with lymphoproliferative disorders (Ablashi et al. 1987). Since that time, two genetically distinct variants were sequenced, HHV-6A and HHV-6B (Ablashi and Balachandran 1991). Both variants have been linked with neurologic disease (Crawford et al. 2007). HHV-6 encephalitis has been well described in literature, typically presenting with confusion, coma, seizure, and headache. The majority of HHV-6 encephalitis has been limited to post-transplant recipients (Singh and Paterson 2000). Encephalitis due to HHV-6 infection has been reported in two liver transplant recipients (Massih and Razonable 2009 and Montejo et al. 2002). Although there has been in vitro studies regarding the potential resistance patterns for HHV-6 virus, there has been only one clinic case report supporting these findings (Isegawa et al. 2009). We describe the first case of ganciclovir-resistant HHV-6 encephalitis in a post-liver transplant patient.
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Encefalite Viral/tratamento farmacológico , Foscarnet/uso terapêutico , Infecções por Herpesviridae/tratamento farmacológico , Transplante de Fígado/efeitos adversos , Idoso , Antivirais/administração & dosagem , DNA Viral/análise , Farmacorresistência Viral , Encefalite Viral/etiologia , Encefalite Viral/virologia , Foscarnet/administração & dosagem , Ganciclovir/administração & dosagem , Ganciclovir/efeitos adversos , Infecções por Herpesviridae/complicações , Infecções por Herpesviridae/virologia , Herpesvirus Humano 6/efeitos dos fármacos , Herpesvirus Humano 6/fisiologia , Humanos , Masculino , Reação em Cadeia da PolimeraseRESUMO
Pregnancy is associated with impaired T-cell-mediated immunity putting the woman at risk for infectious diseases not typically seen in immunocompetent individuals. When maternal infection is severe, and when a primary herpesvirus infection is acquired during pregnancy, there is a risk of fetal loss, maternal death, neonatal death, or congenital abnormalities from intrauterine infection. In this review, the authors emphasize specific neurologic infectious diseases that occur in pregnancy, the safety of the antimicrobial therapy of these diseases during pregnancy, as well as the congenital and neonatal sequelae of infection.
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Complicações Infecciosas na Gravidez/diagnóstico , Complicações Infecciosas na Gravidez/tratamento farmacológico , Adulto , Anti-Infecciosos/efeitos adversos , Anti-Infecciosos/uso terapêutico , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/tratamento farmacológico , Feminino , Doenças Fetais/diagnóstico , Doenças Fetais/prevenção & controle , Humanos , Gravidez , Diagnóstico Pré-Natal/métodos , Efeitos Tardios da Exposição Pré-Natal/diagnóstico , Efeitos Tardios da Exposição Pré-Natal/prevenção & controleRESUMO
This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.
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Doença de Lyme/diagnóstico , Doença de Lyme/terapia , Guias de Prática Clínica como Assunto/normas , Sociedades Médicas/normas , Humanos , Doença de Lyme/prevenção & controle , Estados UnidosRESUMO
We present a 26-year-old female with HbSC disease who presented to the emergency department multiple times with pain and shortness of breath, eventually developing unresponsiveness and a brief episode of pulseless electrical activity. She was admitted to the intensive care unit with multisystem organ failure and found to have diffuse ischemic strokes. Infectious workup revealed disseminated anaplasmosis and babesiosis, which had likely caused sickle cell crisis, atypical hemolytic-uremic syndrome, and ischemic brain injury. She was started on eculizumab therapy as well as antimicrobial therapy with doxycycline, clindamycin, and atovaquone. The patient was given tracheostomy and a percutaneous feeding tube. Unfortunately, she did not have significant neurologic recovery after prolonged hospital stay and was discharged to a skilled nursing facility with significant neurologic burden.
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Hypertrophic pachymeningitis (HP) is characterized by inflammation of the dura mater. It has been described in the setting of numerous systemic inflammatory diseases including immunoglobulin G4 (IgG4)-related disease as well as granulomatosis with polyangiitis (GPA). In this case report, we describe a 48-year-old man presenting with headache who was found to have HP and had systemic features of both GPA and IgG4-related disease as well as seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and IgG4. He was treated with prednisone and rituximab with improvement in his symptoms. Co-occurrence of IgG4 and ANCA against myeloperoxidase has been reported in other cases of HP. The overlap between IgG4 and ANCA has also been described in other systemic manifestations of the diseases. These reports suggest a clinical overlap between ANCA and IgG4-related disease, and the case presented herein suggests an overlap between GPA and IgG4-related disease.
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Chronic meningitis is defined as cerebrospinal fluid pleocytosis that persists for at least 4 weeks without spontaneous resolution. The differential diagnosis of chronic meningitis is broad, encompassing 4 main categories, including infectious, autoimmune, neoplastic, and idiopathic. Up to one-third of cases have no discernible cause, making chronic meningitis a diagnostic dilemma for many clinicians. This article suggests a diagnostic approach to chronic meningitis using clinical history, key examination findings, selective advanced testing, and neuroimaging. Case presentations demonstrate application of a diagnostic algorithm, followed by a brief discussion on common infectious pathogens, autoimmune conditions, and neoplastic disease with selected treatment regimens.
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Meningite/diagnóstico , Doença Crônica , Diagnóstico Diferencial , HumanosRESUMO
Hereditary spastic paraplegias (HSP) are a rare heterogeneous group of inherited neurodegenerative diseases characterized by progressive lower extremity spasticity and weakness. Mutations of the kinesin family member 5A (KIF5A) gene lead to a spectrum of phenotypes ranging from spastic paraplegia type 10 to Charcot-Marie Tooth Disease type 2. We report the second known case of a mutation in the KIF5A gene at c.610C>T presenting with HSP plus an axonal sensorimotor neuropathy.
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PURPOSE OF REVIEW: This article reviews the spectrum of neurologic disease associated with human herpesvirus infections. RECENT FINDINGS: As more patients are becoming therapeutically immunosuppressed, human herpesvirus infections are increasingly common. Historically, infections with human herpesviruses were described as temporal lobe encephalitis caused by herpes simplex virus type 1 or type 2. More recently, however, additional pathogens, such as varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus 6 have been identified to cause serious neurologic infections. As literature emerges, clinical presentations of herpesvirus infections have taken on many new forms, becoming heterogeneous and involving nearly every location along the neuraxis. Advanced diagnostic methods are now available for each specific pathogen in the herpesvirus family. As data emerge on viral resistance to conventional therapies, newer antiviral medications must be considered. SUMMARY: Infections from the herpesvirus family can have devastating neurologic outcomes without prompt and appropriate treatment. Clinical recognition of symptoms and appropriate advanced testing are necessary to correctly identify the infectious etiology. Knowledge of secondary neurologic complications of disease is equally important to prevent additional morbidity and mortality. This article discusses infections of the central and peripheral nervous systems caused by herpes simplex virus type 1 and type 2, varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus 6. The pathophysiology, epidemiology, clinical presentations of disease, diagnostic investigations, imaging characteristics, and treatment for each infectious etiology are discussed in detail.
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Infecções por Herpesviridae/complicações , Doenças do Sistema Nervoso/etiologia , Doenças do Sistema Nervoso/virologia , Idoso de 80 Anos ou mais , Infecções por Herpesviridae/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Doenças do Sistema Nervoso/diagnóstico por imagemRESUMO
Necrotizing and granulomatous meningoencephalitis are common central nervous system diseases known to affect canines. To date, necrotizing granulomatous meningoencephalitis has yet to be described in humans. Current studies of presumed pathogenesis and possible treatment options have only been described in canines. This is a case report of a 55-year-old female patient who was diagnosed with necrotizing granulomatous meningoencephalitis in the setting of new-onset neurological symptoms without any infectious or malignant source.
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We report the case of a 63-year-old male diagnosed with chronic autoimmune mediated meningoencephalitis and periodic cyclical fever syndrome. After 2 years of failed conventional treatment and recurrent hospitalizations, anakinra treatment (an interleukin-1 [IL-1] receptor antagonist) was trialed which resulted in full recovery. As evidenced by this case, anakinra can be used to treat chronic autoimmune meningitis, which can often be refractory to treatment.
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Chronic meningitis and ventriculitis are defined as inflammatory pleocytoses in the cerebrospinal fluid (CSF) and ependyma that persists for at least 1 month without spontaneous resolution. Because the CSF communicates directly with the posterior compartments of the eye, fungal infections in the brain often cause secondary ophthalmologic complications. We report a 23-year-old male who presented to the emergency room with progressive severe headaches associated with insidious monocular vision loss. After extensive workup and a multidisciplinary team effort, the patient was diagnosed with ventriculitis and endogenous endopthalmitis. The etiology is suspected to be due to brown heroin use with secondary disseminated Candida albicans.
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Creutzfeldt-Jakob disease (CJD) is characterized by rapidly progressing dementia with death usually occurring within 6 months. There is no verified disease-specific pre-mortem diagnostic test besides brain biopsy. We describe a 66 y old previously high functioning male who presented with a 5 month history of rapidly progressive dementia. Neurological examination revealed a score of 19/30 on MOCA testing. An extensive workup into various causes of dementia including electroencephalography and imaging studies was unremarkable. The cerebrospinal fluid was sent to National Prion Disease Center and it revealed elevated RT-QuIC levels with negative 14-3-3 and T tau proteins. Based on literature review, our case is one of few living subjects with elevated RT-QuIC levels and negative 14-3-3 and tau proteins.