No abstract

A ABC (Aberrant Behavior Checklist) visa avaliar a resposta ao tratamento de transtornos comportamentais em portadores de retardo mental. O objetivo deste estudo é descrever a adaptação transcultural parcial da escala ABC para o português do Brasil. Foi realizada avaliação da equivalência conceitual e de itens, foram feitas duas traduções (T1 e T2), suas respectivas retraduções (R1 e R2), avaliação das equivalências referencial e geral, avaliação de especialistas, pré-teste e elaboração da versão final. Em relação à equivalência conceitual e de itens a ABC foi considerada pertinente à nossa cultura. Quanto à equivalência semântica, houve uma boa correspondência entre os itens de R1 e a ABC original, e razoável entre estes e R2. Portanto optou-se por utilizar os itens de T1. Todos os professores compreenderam 94,8% da escala, enquanto todos os parentes entenderam 87,9%. Fica disponível a versão em português do Brasil da escala ABC, respeitando a equivalência conceitual e de itens e semântica.

ABSTRACT.Cognitive impairment includes mild cognitive decline and dementia, such as Alzheimer's disease (AD) and cerebrovascular-related pathologies.Objective:To investigate the profile of AD-related CSF biomarkers in a sample of cognitively impaired and unimpaired older adults with concomitant subcortical cerebrovascular burden.Methods:Seventy-eight older adults attending an outpatient psychogeriatric clinic were enrolled. Diagnoses were based on clinical, neuropsychological, laboratory, and neuroimaging data. Participants were classified into: cognitively normal (controls, n = 30), mild cognitive impairment (MCI, n = 34), and dementia (AD, n = 14). All subjects were submitted to CSF analyses for determination of amyloid-beta (Aβ1-42), total tau (t-tau), phosphorylated tau (p-tau) and Aβ1-42/p-tau ratio according to the Luminex method. MRI was performed in all individuals, and was scored independently by two experts according to Fazekas scale. Statistical analyses were conducted with the aid of general linear model procedures, and the Chi-squared test.Results:T-tau levels were significantly associated with subcortical lesion pattern when Fazekas was considered as a group factor. CSF biomarkers were not associated with MCI, AD, or controls when considered separately. There was a tendency for reduction in CSF Aβ1-42 together with increasing Fazekas scores, but without statistical significance. Comparisons of Aβ1-42 and t-tau with each clinical group or with each neuroimaging pattern did not reach statistical differences. Likewise, Fazekas scores had no impact on CAMCOG scores.Conclusion:We found a significant association between t-tau levels and subcortical lesions when all Fazekas classifications were considered as a single group; comparisons of Fazekas subgroups and CSF biomarkers did not reach significance.

By the end of the human genome sequencing in 2003, it was possible to analyse the content of chromosome 6, which comprises around 6% of the genome (Mungall et al., 2003). Chromosome 6 has an important role in the innate and adaptive immune system since it contains the genes of the major histocompatibility complex (MHC) (6p21.3) (Mungall et al., 2003). The importance of chromosome 6 goes beyond the role of MHC since several genetically complex diseases have

Context: Cerebral amyloid angiopathy (CAA) is characterized by progressive deposition of amyloid-ß fibrils in the walls of small arterioles and capillaries of the leptomeninges and cerebral cortex. A rare subtype of CAA is CAA-related inflammation (CAA-RI), which exhibits marked perivascular or transmural inflammatory infiltration in brain tissue. The major clinical features of CAA-RI are rapidly progressive dementia, behavioral changes, headache, seizures, or stroke-like signs. Conclusive diagnosis requires histopathological confirmation, but validated clinicoradiological criteria for the diagnosis of probable CAA-RI have good sensitivity (82%) and specificity (97%). Treatment with high dose corticosteroids with or without other immunosuppressive therapy is recommended. We report a case of probable CAA-RI that did not respond to corticosteroid therapy but had a surprising improvement with acetylcholinesterase inhibitor. Case report: A 77-year-old illiterate woman presented with a history of subacute onset of seizures and behavioral changes. Her medical history was positive for a hearing loss due to a toxic exposure in childhood, and a cured breast cancer. The neurological examination showed attention impairment, disorientation, and incoherent speech. CSF showed a mildly elevated protein count. Brain MRI met criteria for probable CAA-RI. She had a poor response with high doses of corticosteroids, but after a trial with Donepezil she showed important cognitive and functional improvement. Conclusion: This result attracts attention to the importance of the cholinergic pathway in the etiology and pathological mechanisms of CAA. Randomized Controlled Trials would be required to confirm our hypothesis and to find new therapeutic options for CAA.

Introduction: Foix-Chavany-Marie syndrome, also known as opercular syndrome, consists of voluntary orofacial muscle paralysis due to bilateral lesions in the anterior frontotemporal operculum. Classically, there is an automatic-voluntary dissociation, with impairment of speech and chewing. We present a case of a patient who had a similar presentation to opercular syndrome, secondary to unilateral ischemic lesion in the right frontal operculum. Case report: A 55-year-old, right-handed, black woman with history of arterial hypertension was admitted to the emergency department presenting left-sided weakness and inability to speak, noticed upon waking. When approached, the patient was unable to produce sounds but managed to communicate through articulatory movements of lips and was able to write correctly without signs of aphasia. On examination, there was left central facial paralysis, marked reduction in mouth opening amplitude, inability to perform tongue protrusion without lateral deviation and bilateral reduction in palate elevation. There was also a mild left brachial- predominant hemiparesis (grade IV). A cranial computed tomography scan exhibited an acute ischemic lesion of the right frontal opercular region, without other lesions in the homologous contralateral area. The etiological investigation with electrocardiogram, echocardiogram and carotid doppler ultrasound had no significant abnormalities. Conclusion: We reported a case of a patient presenting with aphonia and bulbar musculature paresis due to a right unilateral frontal opercular lesion, mimicking the opercular syndrome presentation.