ABSTRACT Primary progressive aphasia is a clinical syndrome caused by neurodegeneration of areas and neural networks involved in language, usually in the left hemisphere. The term “crossed aphasia” denotes an acquired language dysfunction caused by a lesion in the ipsilateral hemisphere to the dominant hand. Objective: To describe a case of crossed aphasia in a 60-year-old left-handed patient with a non-fluent variant of primary progressive aphasia diagnosis (age of onset=52), evidenced by a left asymmetry on brain SPECT scan. Methods: Clinical and family history, the Edinburgh Handedness Inventory, Measurement of Functional Activities in Older Adults in the Community, the “Mini-Mental State Examination”, the Trail Making Test, the Tower of London, and the Neuropsychological assessment for dementia, and neuroimaging studies were carried out. Results: Neuropsychological assessment showed severe cognitive impairment, especially regarding language. The magnetic resonance imaging showed important signs of cortico-subcortical atrophy, with predominance in the frontal and temporal lobes. The single-photon emission computed tomography scan showed moderate to severe hypoperfusion in the left cerebral hemisphere, including the hippocampus. Conclusion: We described a clinical case of crossed aphasia in a left-handed woman with a non-fluent variant of primary progressive aphasia with asymmetry on brain SPECT, mainly on the left, followed up for seven years.

RESUMO A afasia progressiva primária é uma síndrome clínica causada por uma neurodegeneração de áreas e redes neurais envolvidas na linguagem, geralmente no hemisfério esquerdo. O termo “afasia cruzada” denota uma disfunção adquirida de linguagem causada por uma lesão no hemisfério ipsilateral da mão dominante. Objetivo: Relatamos um caso de afasia cruzada em uma paciente de 60 anos, canhota, com um quadro clínico de afasia progressiva primária variante não fluente (idade de início=52), evidenciada por assimetria no SPECT cerebral à esquerda. Métodos: Foram realizados para o diagnóstico do caso: história clínica e familiar, o Inventário de Dominância de Edinburgh, a Escala de Atividades Funcionais de Pfeffer, o Miniexame do Estado Mental, o Teste das Trilhas, o Teste da Torre de Londres, a Avaliação Neuropsicológica Adequada às Demências e exames de neuroimagem. Resultados: A avaliação neuropsicológica mostrou comprometimento cognitivo severo, principalmente sobre a linguagem; a ressonância magnética do crânio mostrou sinais de involução córtico-subcortical, com predominância nos lobos frontal e temporal e a cintilografia cerebral por emissão de fóton único mostrou hipoperfusão moderada a severa no hemisfério cerebral esquerdo, incluindo o hipocampo. Conclusão: Registramos um caso clínico de afasia cruzada em uma paciente canhota com afasia progressiva primária variante não fluente com assimetria no SPECT cerebral, principalmente à esquerda, seguida há sete anos.

RESUMO. Relatamos o caso de um paciente do sexo masculino, 61 anos, com anacusia, síndrome cerebelar, mioclonia e sinais frontais. A ressonância magnética cerebral mostrou hiperintensidade estriada bilateral do fluid-attenuated inversion recovery (FLAIR) e difusão restrita no diffusion-weighted imaging (DWI) e áreas hipointensas correspondendo ao coeficiente de difusão aparente no córtex cerebral. A autópsia revelou imuno-histoquímica positiva para a proteína PrPSc. A doença de Creutzfeldt-Jakob que se apresenta com perda auditiva é incomum.

ABSTRACT. We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted diffusion in the diffusion-weighted imaging and hypointense areas corresponding to the apparent diffusion coefficient in the cerebral cortex. The autopsy revealed positive immunohistochemistry for the PrPSc protein. Creutzfeldt–Jakob disease presenting with hearing loss is unusual.

ABSTRACT Objective: To evaluate the reasons for request of bone mineral density (BMD) evaluation and correlate the BMD results with previous fractures, risk factors for osteoporosis, and clinical characteristics in patients with obesity. Subjects and methods: Cross-sectional, retrospective, single-site study including adult patients with body mass index (BMI) ≥ 30 kg/m2 and BMD evaluation between January 2015 and May 2016 selected from a BMD database. Data on demographic characteristics, lifestyle habits, comorbidities, medications, risk factors, previous fractures, and indications for BMD evaluation were collected from the participants’ medical records. Results: The study included 619 patients (89.9% women, mean BMI 34.79 ± 4.05 kg/m2). In all, 382 (61.7%), 166 (26.8%), and 71 (11.5%) patients had class 1, 2, and 3 obesity, respectively. The most frequent (29.9%) reason for BMD evaluation was for osteoporosis monitoring. In all, 69.4% of the patients had low BMD. Multivariate analysis showed that age, calcium supplementation, and previous osteoporosis or osteopenia were associated with low BMD, while age, vitamin D supplementation, use of proton pump inhibitors (PPIs), and low BMD were associated with previous fractures (p < 0.05 for all). Conclusions: Among patients with obesity identified from a tertiary hospital database, those with low bone mass and risk factors traditionally associated with fractures had an increased history of fractures. Patients with greater BMI had better bone mass and fewer fractures. These findings indicate that the association between reduced weight, risk factors for osteoporosis, and fractures remained despite the presence of obesity in our population.

RESUMO CONTEXTO: No tratamento de doenças inflamatórias intestinais (DII) são usados imunossupressores e agentes biológicos, o que aumenta o risco de infecções pela alteração da imunidade celular e humoral. A prevenção de algumas dessas infecções pode ser feita pela vacinação, entretanto pacientes com DII apresentam baixas taxas de cobertura vacinal. OBJETIVO: Analisar a situação vacinal de pacientes com DII e comparar com a recomendação vigente na literatura para verificar se os esquemas de imunização estão sendo corretamente aplicados nessa população. MÉTODOS: Estudo transversal analítico com 239 pacientes com DII em acompanhamento no Serviço de Gastroenterologia do Hospital de Clínicas da Universidade Federal do Paraná, os quais foram submetidos a um questionário sobre dados demográficos relevantes e sobre a situação vacinal. RESULTADOS: A taxa de pacientes que declarou não ter conhecimento de sua situação vacinal é alta - entre 34,3% (dTpa) e 52% (meningocócica) - com exceção das vacinas influenza, hepatite B e HPV. As vacinas com maior taxa de pacientes que declararam ter recebido a vacina são influenza (72,4%), BCG (55,3%), hepatite B (48,3%), tríplice viral (43,8%) e DTPa (43%). As vacinas com menor taxa de pacientes que declararam ter recebido a vacina são Haemophilus influenza b (0,8%), herpes zoster (2,1%) e HPV (3,4%). Pacientes que fazem ou já fizeram tratamento com agentes biológicos têm melhor cobertura vacinal das vacinas para influenza (81%) e PP23V (25,9%), além de maior conhecimento sobre o estado vacinal para essas vacinas. CONCLUSÃO: Apesar de se tratar de um serviço especializado ligado a um hospital universitário, a cobertura vacinal e o conhecimento dos pacientes sobre as vacinas estão abaixo do desejado. A recuperação do histórico vacinal e a recomendação das vacinas necessárias devem ser realizadas logo após o diagnóstico de DII, independentemente do uso de agentes biológicos. Esses achados indicam a necessidade da criação e monitoramento constante da aplicação de um protocolo pela equipe multidisciplinar de serviços especializados em DII.

ABSTRACT BACKGROUND: Treatment for inflammatory bowel disease (IBD) includes a variety of immunosuppressants and biological agents, which increase the risk of infections due to altered cellular and humoral immunity. Prevention of these infections can be done through vaccination, however, patients with IBD are usually under-immunized. OBJECTIVE: Analyze the immunization status of patients with IBD and confront it with the current recommendations to verify if the immunization guidelines are being followed correctly. METHODS: Analytical cross-sectional study including 239 IBD patients being regularly followed in the Gastroenterology Service from Hospital de Clínicas da Universidade Federal do Paraná, which were subjected to a survey about their relevant demographic data and immunization status. RESULTS: The amount of patients that declared being unaware of their immunization status is high - between 34.3% (Tdap) and 52% (meningococcal) - excepting IIV, hepatitis B and HPV. The vaccines with the largest rates of patients declaring to have taken it are inactivated influenza vaccine (72.4%), BCG (55.3%), hepatitis B (48.3%), measles, mumps and rubella vaccine (43.8%) and DTaP (43%). The vaccines with the lowest rates of patients declaring to have taken it are Haemophilus influenza type b (0.8%), herpes zoster (2.1%) and HPV (3.4%). Patients that are being treated or have been treated with biological therapy have the largest immunization coverage for inactivated influenza vaccine (81%) and PPSV23 (25.9%), also they have the largest awareness rates for those vaccines. CONCLUSION: Although being a specialized service linked to a university hospital, vaccination coverage and patients’ awareness rates proved to be below the desirable level. Vaccination and recovery of the immunization history is recommended immediately after the diagnosis of IBD, regardless of the use of biological agents. Those findings support the need of implementing hospital guidelines and constantly verifying its application by the multidisciplinary team in specialized services in IBD.