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The objective of this study was to compare the accuracy of ultrasound (US) with that of magnetic resonance imaging (MRI) in identifying muscle abnormalities in patients with inclusion body myositis (IBM). Twelve patients with IBM underwent muscle US and MRI on the same day. Twelve muscle groups were analyzed per patient. On US, a visual grading system was used to detect whether the muscles were affected. On MRI, muscle atrophy, fat infiltration and edema patterns were analyzed. The inter- and intra-reader reproducibility was similar for US and MRI in the evaluation of muscle abnormalities. All patients with muscle abnormalities identified on US presented with fat infiltration on MRI, which was the most common abnormality identified on MRI. Most importantly, the accuracy of US compared with that of MRI for the detection of muscle abnormalities in patients with IBM was 86.8 (κ coefficient = 0.632), with a sensitivity of 84% and specificity of 100%. In conclusion all patients with muscle abnormalities identified on US presented with fat infiltration on MRI, and the marked increase in echo intensity observed in the muscles of IBM patients was related mostly to fatty replacement. Most importantly, US exhibited significant accuracy compared with MRI.

Creutzfeldt-Jakob Disease (CJD) is the prototypical cause of rapidly progressive dementia (RPD). Nonetheless, efforts to exclude reversible causes of RPD that mimic prion disease are imperative. The recent expanding characterization of neurological syndromes associated with antibodies directed against neuronal cell surface or sympathic antigens, namely autoimmune encephalitis is shifting paradigms in neurology. Such antigens are well known proteins and receptors involved in synaptic transmission. Their dysfunction results in neuropsychiatric symptoms, psychosis, seizures, movement disorders and RPD. Faciobrachial dystonic seizure (FBDS) is a novel characterized type of seizure, specific for anti-LGI1 encephalitis.In order to improve clinical recognition we report the cases of two Brazilian patients who presented with characteristic FDBS (illustrated by videos) and anti-LGI1 encephalitis.We have included all patients with FBDS and confirmed anti-LGI1 encephalitis and video records of FDBS in two tertiary Brazilian centers: Department of Neurology of Hospital das Clínicas, Sao Paulo University, Sao Paulo, Brazil and Hospital Geral de Fortaleza, Fortaleza, Brazil between January 1, 2011 and December 31, 2015.Both patients presented with clinical features of limbic encephalitis associated with FBDS, hyponatremia and normal CSF. None of them presented with tumor and both showed a good response after immunotherapy.FBDSs may be confounded with myoclonus and occurs simultaneously with rapid cognitive decline. Unawareness of FDBS may induce to misdiagnosing a treatable cause of RPD as CJD.A doença de Creutzfeldt-Jakob (DCJ) é o protótipo de demência rapidamente progressiva (DRP). No entanto, é imperativo que sejam excluídas causas reversíveis de DRPs que possam simular doença priônica. A recente caracterização de síndromes neurológicas associadas a anticorpos direcionados contra antígenos de superfície neuronal ou sinapse, assim denominadas de encefalites autoimunes, está mudando paradigmas em neurologia. Esses antígenos estão envolvidos na transmissão sináptica, sendo que as disfunções destes podem resultar em sintomas neuropsiquiátricos, psicose, crises epilépticas, distúrbios do movimento e DRP. A crise distônica faciobraquial (CDFB) é um tipo de crise recentemente caracterizada e específica da encefalite anti-LGI1.Para promover um melhor reconhecimento da doença relatamos os casos de 2 pacientes brasileiros que apresentaram CDFBs (ilustradas com vídeos) associadas à encefalite anti-LGI1.Foram incluídos todos os pacientes com CDFBs e encefalite anti-LGI1 confirmados em 2 centros brasileiros terciários: Departamento de Neurologia do Hospital das Clínicas da Universidade de São Paulo, São Paulo, Brasil e o Hospital Geral de Fortaleza entre 01 de janeiro de 2011 e 31 de dezembro de 2015.Ambos os casos apresentaram quadro clinico típico de encefalite límbica associada a CDFBs e exame do LCR sem alterações. Nenhum caso associou-se à presença de neoplasia e ambos apresentaram boa resposta à imunoterapia.A CDFB podem ser confundidas com mioclonias e ocorrer simultaneamente com rápido declínio cognitivo, o seu não reconhecimento pode induzir ao diagnóstico errôneo de uma causa potencialmente tratável de DRP como sendo DCJ.

A 76-year-old woman presented with a three-month history of faciobrachial dystonic seizures associated with cognitive impairment and hyponatremia. Cerebrospinal fluid analysis was unremarkable. A whole-body CT scan demonstrated no malignancies. Brain MRI revealed involvement of the mesial right temporal lobe, right striatum and globus pallidus and bilateral supratentorial white matter (). Leucine-rich glioma inactivated protein 1 (LGI-1) antibodies were detected in the serum. The patient received immunosuppressive treatment, with a partial response. LGI-1 encephalitis is an immune-mediated encephalitis characterized by [...]

OBJECTIVES: Low cardiac output syndrome (LCOS) is a serious complication after cardiac surgery and is associated with significant morbidity and mortality. The aim of this study is to identify risk factors for LCOS in patients undergoing coronary artery bypass grafting (CABG) in the Division of Cardiovascular Surgery of Pronto Socorro Cardiológico de Pernambuco - PROCAPE (Recife, PE, Brazil). METHODS: A historical prospective study comprising 605 consecutive patients operated between May 2007 and December 2010. We evaluated 12 preoperative and 7 intraoperative variables. We applied univariate and multivariate logistic regression analysis. RESULTS: The incidence of LCOS was 14.7% (n = 89), with a lethality rate of 52.8% (n = 47). In multivariate analysis by logistic regression, four variables remained as independent risk factors: age > 60 years (OR 2.00, 95% CI 1.20 to 6.14, P = 0.009), on-pump CABG (OR 2.16, 95% CI 1.40 to 7.08, P = 0.006), emergency surgery (OR 4.71, 95% CI 1.34 to 26.55, P = 0.028), incomplete revascularization (OR 2.62, 95% CI 1.32 to 5.86, P = 0.003), and ejection fraction 60 years of off-pump CABG, emergency surgery, incomplete CABG and ejection fraction 60 anos (OR 2.00, IC 95% 1,20 a 6,14, P = 0,009), CRM com circulação extracorpórea (OR 2,16, IC 95% 1,40 a 7,08, P = 0,006), cirurgia de emergência (OR 4,71, IC 95% 1,34 a 26,55, P = 0,028), CRM incompleta (OR 2,62, IC 95% 1,32 a 5,86, P = 0,003) e fração de ejeção < 50% (OR 1,87, IC 95% 1,17 a 3,98, P = 0,007). CONCLUSÕES: Este estudo identificou os seguintes fatores de risco independentes para SBDC após CRM: idade > 60 anos, CRM com CEC, cirurgia de emergência, CRM incompleta e fração de ejeção < 50%.

A 66-year-old Brazilian woman presented with fever, malaise, and myalgia, followed 10 days later by left hemiparesis and altered mental status. Brain MRI revealed bilateral intracerebral hemorrhages (figure, A and B) and CSF showed mild lymphocytic pleocytosis. Serologic testing for dengue fever was positive. Brain biopsy demonstrated nonspecific inflammatory changes and areas of recent hemorrhage and necrosis (figure, C). After 1 month, partial clinical and radiologic improvement occurred (figure, D). Expanded dengue syndrome1 refers to atypical manifestations of dengue such as intracerebral hemorrhage, which is a rare presentation usually reported a week after fever onset, possibly related to release of cytokines with vasogenic activity.2

We report the case of a patient with insidious onset and slowly progressive cognitive impairment, behavioral symptoms, temporal lobe seizures and delusional thoughts typical of delusional misidentification syndromes. Clinical presentation along with extensive diagnostic work-up revealed limbic encephalitis secondary to diffuse large B-cell lymphoma. The patient underwent immunotherapy with high-dose corticosteroid but no significant improvement was observed. No specific treatment for lymphoma was performed because the patient died of septic shock following a nosocomial respiratory infection. Delusional misidentification syndromes are an unusual and unique form of cognitive impairment in which a patient consistently misidentifies persons, places, objects, or events. Capgras syndrome is the most common subtype of this disorder, being defined by the recurrent and transient belief that someone close has been substituted by an imposter. These entities are generally associated with neurodegenerative diseases and psychiatric disturbances. Rare reports of associations between misidentification syndromes and autoimmune diseases such as multiple sclerosis have been published, but no papers address a correlation with limbic encephalitis or lymphoma.

Spine magnetic resonance imaging with dynamic flexion sequences for evaluation of mini-polymyoclonus