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Diagnóstico por imagem de arteriopatia cerebral associada a mutação ACTA2: rela...
Rocha, Valéria Cristina do Rosário Rebouças; Vasconcelos, Thais de Maria Frota; Albuquerque, Clarissa Pinto de; Nóbrega, Paulo RibeiroObjectives: To describe the radiological characteristics that allow the recognition of cerebral arteriopathy cssociated with ACTA2 mutation in magnetic resonance imaging. Methodology: Magnetic resonance (MRI) and magnetic resonance angiography of the brain (MRA) images were analyzed. Results: Female patient, 19 years old, with cognitive deficit and occipital headache, evolving with paraparesis on the right and ...
Autoimmune encephalitis in Latin America: a critical review
Vasconcelos, Gabriel de Albuquerque; Barreira, Rodrigo Montenegro; Antoniollo, Karmelita Emanuelle Nogueira Torres; Pinheiro, Alina Maria NuñezAutoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world lite...
Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyon...
Nóbrega, Paulo Ribeiro; Rodrigues, Pedro Gustavo Barros; Pereira, Isabelle de Sousa; Santos, Carolina de Figueiredo; Gerson, GunterBackground: The term “Tolosa-Hunt syndrome” (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of...
Multinodular and vacuolating neuronal tumor of the cerebrum: three case reports
Lessa, Antônio Gláuber Uchôa; Albuquerque, Lucas Alverne Freitas de; Morais, Norma Martins de Menezes; Dias, Daniel Aguiar; Nóbrega, Paulo RibeiroMultinodular and vacuolating neuronal tumor (MVNT) of the cerebrum is a seizure-associated rare lesion that currently appears to be a malformative lesion or hamartoma rather than a low-grade neoplastic lesion. MVNT pathognomonic magnetic resonance imaging (MRI) features allow for diagnostic accuracy in the differential diagnosis. Lesions are usually asymptomatic, nonprogressive and incidentally found requiring ...
Encefalites autoimunes por anticorpos contra superfície neuronal no estado do C...
Nóbrega, Paulo RibeiroINTRODUÇÃO: Encefalite autoimune associada a anticorpos contra antígenos de superfície neuronal é uma doença recentemente descrita, cuja apresentação clínica é variada e que pode apresentar muitos anticorpos implicados no seu desenvolvimento. Alguns casos são de origem paraneoplásica, enquanto em outros são identificados potenciais desencadeantes virais, mas a maioria ainda permanece de etiologia idiopática. Os...
Clinical features and inflammatory markers in autoimmune encephalitis associate...
Nóbrega, Paulo Ribeiro; Pitombeira, Milena Sales; Mendes, Lucas Silvestre; Krueger, Mariana Braatz; Santos, Carolina FigueiredoAcute encephalitis is a debilitating neurological disorder associated with brain inflammation and rapidly progressive encephalopathy. Autoimmune encephalitis (AE) is increasingly recognized as one of the most frequent causes of encephalitis, however signs of inflammation are not always present at the onset which may delay the diagnosis. We retrospectively assessed patients with AE associated with antibodies aga...
Immunoglobulin-responsive chikungunya encephalitis: two case reports
Scott, Stephanie Suzanne de O.; Braga-Neto, Pedro; Pereira, Lícia Pacheco; Nóbrega, Paulo Ribeiro; Gondim, Francisco de Assis AquinoChikungunya virus is an alphavirus transmitted by the mosquito Aedes, mainly Aedes aegypti and Aedes albopictus, that can cause acute illness, mostly self-limited, characterized by fever, maculopapular rash, and disabling polyarthritis/arthralgia, with an incubation period of 1 to 12 days. Chikungunya was largely regarded as a non-fatal and self-limited disease, but recently, serious cases have been reported in...
High phenotypic variability in Gerstmann-Sträussler-Scheinker disease
Smid, Jerusa; Studart Neto, Adalberto; Landemberger, Michele Christine; Machado, Cleiton Fagundes; Nóbrega, Paulo RibeiroGerstmann-Sträussler-Scheinker is a genetic prion disease and the most common mutation is p.Pro102Leu. We report clinical, molecular and neuropathological data of seven individuals, belonging to two unrelated Brazilian kindreds, carrying the p.Pro102Leu. Marked differences among patients were observed regarding age at onset, disease duration and clinical presentation. In the first kindred, two patients had rapi...
Encephalitis associated with the chikungunya epidemic outbreak in Brazil: repor...
Pereira, Licia Pacheco; Villas-Bôas, Rafaela; Scott, Stephanie Suzanne de Oliveira; Nóbrega, Paulo Ribeiro; Sobreira-Neto, Manoel AlvesChikungunya, an alphavirus infection presenting with fever, rash, and polyarthritis, is most often an acute febrile illness. Neurologic complications of chikungunya infection have been reported. Here we report the clinical and neuroimaging data of 2 patients with chikungunya-associated encephalitis during the recent Brazilian epidemic.