Cytokines profile in pure neural leprosy.

Introduction: Pure Neural Leprosy (PNL) is a form of this long time known disease that affects only the peripheral nervous system. Since it is a rare form of the disease, its pathophisiology is still poorly understood. Objective: Describe the cytokines profile in patients with PNL. Methods: 30 Patients diagnosed with PNL in the Souza Araujo Outpatient Clinic and with cytokines evaluated were selected. They were evaluated by neurologists and diagnosed after a nerve biopsy. Serum levels of IL-1 ß, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-ϒ, CXCL-10/IP-10 and TGF-ß were evaluates at the moment of the diagnosis. Results: Neural thickening was a common clinical finding in this groups of patients. Small and medium sensitive fibers signs and symptoms were present in 92% of the patients and motor involvement in 53%. 43% of patients presented neuropathic pain and no one had neuritis TGF-beta, IL-17, CCl-2 and IP-10. CCL-2 levels were associated with demyelinating patters and IP-10 and IL-1o were associated with axonal patterns at NCS. Discussion: PNL patients' cytokine profile appears to be different of other clinical forms of leprosy, with the presence of cytokines described in both tuberculoid and lepromatous leprosy. High levels of CCl-2 may be related to the presence of silent neuritis as well as the presence of IL-10. PNL is unique a form of leprosy, therefore, understanding its immunological profiles essential to better understand the disease itself.

Cytokine Levels in Neural Pain in Leprosy.

Pain is a frequent symptom in leprosy patients. It may be predominantly nociceptive, as in neuritis, or neuropathic, due to injury or nerve dysfunction. The differential diagnosis of these two forms of pain is a challenge in clinical practice, especially because it is quite common for a patient to suffer from both types of pain. A better understanding of cytokine profile may serve as a tool in assessing patients and also help to comprehend pathophysiology of leprosy pain. Patients with leprosy and neural pain (n = 22), neuropathic pain (n = 18), neuritis (nociceptive pain) (n = 4), or no pain (n = 17), further to those with diabetic neuropathy and neuropathic pain (n = 17) were recruited at Souza Araujo Out-Patient Unit (Fiocruz, Rio de Janeiro, RJ, Brazil). Serum levels of IL1ß, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-γ, CXCL-10/IP-10, and TGF-ß were evaluated in the different Groups. Impairment in thermal or pain sensitivity was the most frequent clinical finding (95.5%) in leprosy neuropathy patients with and without pain, but less frequent in Diabetic Group (88.2%). Previous reactional episodes have occurred in patients in the leprosy and Pain Group (p = 0.027) more often. Analysis of cytokine levels have demonstrated that the concentrations of IL-1ß, TNF, TGF-ß, and IL-17 in serum samples of patients having leprosy neuropathy in combination with neuropathic or nociceptive pain were higher when compared to the samples of leprosy neuropathy patients without pain. In addition, these cytokine levels were significantly augmented in leprosy patients with neuropathic pain in relation to those with neuropathic pain due to diabetes. IL-1ß levels are an independent variable associated with both types of pain in patients with leprosy neuropathy. IL-6 concentration was increased in both groups with pain. Moreover, CCL-2/MCP-1 and CXCL-10/IP-10 levels were higher in patients with diabetic neuropathy over those with leprosy neuropathy. In brief, IL-1ß is an independent variable related to neuropathic and nociceptive pain in patients with leprosy, and could be an important biomarker for patient follow-up. IL-6 was higher in both groups with pain (leprosy and diabetic patients), and could be a therapeutic target in pain control.

Catatonia, beyond a psychiatric syndrome.

Although catatonia is a well-known psychiatric syndrome, there are many possible systemic and neurological etiologies. The aim of this case report was to present a case of a patient with cerebral venous sinus thrombosis and infarction in which catatonia was the clinical manifestation of a possible nonconvulsive status epilepticus. To our knowledge, only one such case has been reported in the literature, which had a simplified diagnostic investigation. It is important to correctly recognize the organic cause underlying catatonia in order to treat the patient as soon as possible thereby improving outcome. Therefore, physicians need to update their knowledge on catatonia, recognizing that it can be part of a psychiatric or neurologic condition.

Embora a catatonia seja uma síndrome psiquiátrica bem conhecida, existem várias etiologias possíveis, tanto sistêmicas quanto neurológicas. O objetivo deste relato de caso é apresentar um quadro de trombose venosa central com infarto venoso em que a catatonia foi a manifestação clínica de um possível status não convulsivo. Na concepção dos autores, apenas um caso é encontrado na literatura, porém com uma propedêutica simplificada. É importante o correto reconhecimento das causas orgânicas que podem estar causando a catatonia para que sejam corrigidas assim que possível, melhorando o prognóstico do paciente. Além disso, os médicos precisam atualizar seus conhecimentos sobre a catatonia, uma vez que ela pode ser parte tanto de uma condição psiquiátrica quanto neurológica.

Catatonia, beyond a psychiatric syndrome / Catatonia, além de uma síndrome psiquiátrica

ABSTRACT Although catatonia is a well-known psychiatric syndrome, there are many possible systemic and neurological etiologies. The aim of this case report was to present a case of a patient with cerebral venous sinus thrombosis and infarction in which catatonia was the clinical manifestation of a possible nonconvulsive status epilepticus. To our knowledge, only one such case has been reported in the literature, which had a simplified diagnostic investigation. It is important to correctly recognize the organic cause underlying catatonia in order to treat the patient as soon as possible thereby improving outcome. Therefore, physicians need to update their knowledge on catatonia, recognizing that it can be part of a psychiatric or neurologic condition.

RESUMO Embora a catatonia seja uma síndrome psiquiátrica bem conhecida, existem várias etiologias possíveis, tanto sistêmicas quanto neurológicas. O objetivo deste relato de caso é apresentar um quadro de trombose venosa central com infarto venoso em que a catatonia foi a manifestação clínica de um possível status não convulsivo. Na concepção dos autores, apenas um caso é encontrado na literatura, porém com uma propedêutica simplificada. É importante o correto reconhecimento das causas orgânicas que podem estar causando a catatonia para que sejam corrigidas assim que possível, melhorando o prognóstico do paciente. Além disso, os médicos precisam atualizar seus conhecimentos sobre a catatonia, uma vez que ela pode ser parte tanto de uma condição psiquiátrica quanto neurológica.

Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system / Encefalite límbica auto-imune: manifestação do lúpus eritematoso sistémico no sistema nervoso central

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Encefalite Límbica Autoimune (EL) associada a lúpus eritematoso sistêmico (LES) é uma entidade rara, com poucos relatos na literatura até o momento. Em geral, EL associada com LES tem uma resposta satisfatória ao tratamento imunossupressor, mas a patogênese desta associação é pouco compreendida e pode incluir um componente de autoimunidade. Descrevemos em um estudo de caso o diagnóstico e o tratamento empregado na encefalite límbica ocorrida no contexto de uma paciente com LES ativo e história pregressa de doenças neoplásicas (adenocarcinoma endometrial em 2004 e carcinoma papilar urotelial em 2011 ambos com o tratamento curativo), a qual foi seguida durante um ano. Discutimos uma possível associação de encefalite límbica e todos os antecedentes neoplásicos e imunomediados desta paciente. Neste caso em particular, a autoimunidade é o fator mais relevante relacionado com a encefalite límbica devido a uma triagem neoplásica negativa. Além disso, houve uma grande resposta com a imunossupressão, o que não é visto na encefalite límbica paraneoplásica, mais relacionada com uma má resposta. Neste caso, a associação de EL com LES é possível, uma vez que testes laboratoriais confirmaram a atividade lúpica, bem como a paciente apresentava envolvimento de outros sistemas (como hematológico) neste interim. No entanto, a presença de outros anticorpos de superfície da membrana é possível em busca de diferentes etiologias.

Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system.

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Encefalite Límbica Autoimune (EL) associada a lúpus eritematoso sistêmico (LES) é uma entidade rara, com poucos relatos na literatura até o momento. Em geral, EL associada com LES tem uma resposta satisfatória ao tratamento imunossupressor, mas a patogênese desta associação é pouco compreendida e pode incluir um componente de autoimunidade. Descrevemos em um estudo de caso o diagnóstico e o tratamento empregado na encefalite límbica ocorrida no contexto de uma paciente com LES ativo e história pregressa de doenças neoplásicas (adenocarcinoma endometrial em 2004 e carcinoma papilar urotelial em 2011 ambos com o tratamento curativo), a qual foi seguida durante um ano. Discutimos uma possível associação de encefalite límbica e todos os antecedentes neoplásicos e imunomediados desta paciente. Neste caso em particular, a autoimunidade é o fator mais relevante relacionado com a encefalite límbica devido a uma triagem neoplásica negativa. Além disso, houve uma grande resposta com a imunossupressão, o que não é visto na encefalite límbica paraneoplásica, mais relacionada com uma má resposta. Neste caso, a associação de EL com LES é possível, uma vez que testes laboratoriais confirmaram a atividade lúpica, bem como a paciente apresentava envolvimento de outros sistemas (como hematológico) neste interim. No entanto, a presença de outros anticorpos de superfície da membrana é possível em busca de diferentes etiologias.