Background: Aphasia is a common neurocognitive disorder caused by impaired speech and language, with stroke being the most frequent cause. The neuroanatomical mechanism underlying this condition is not yet fully understood. Case description: This case describes a 74-year-old Caucasian woman admitted with a clinical picture of right total anterior circulation infarct (TACI) and aphasia, scoring 17 on the National Institutes of Health Stroke Scale. Neuroimaging showed a large cortico-subcortical frontotemporoparietal and insular infarct involving the basal ganglia of the right hemisphere and bilateral focal atherosclerotic stenosis on the M1 segment of the middle cerebral artery. There was no left hemispheric lesion or abnormal electric activity on the electroencephalogram. A formal evaluation was compatible with transcortical motor aphasia. The aetiological study revealed atrial fibrillation, and the case was admitted as an ischaemic stroke of undetermined aetiology with two possible causes – intracranial atherosclerotic stenosis or atrial fibrillation. Conclusion: Our patient fulfilled all the formal criteria for crossed aphasia in dextral (CAD): aphasia, a lesion in the right hemisphere coupled with the structural integrity of the left hemisphere, an established preference for right-hand use without a familial history of left-handedness individuals, and an absence of brain damage in childhood. Our patient’s case adds to the evidence that deep structures – alone or in combination with cortical structures – are primarily affected in CAD.

Background Status epilepticus (SE) is a medical condition that bestows substantial morbidity and mortality. Literature is scarce regarding SE in elderly patients, particularly in the context of internal medicine wards. Aim To characterize SE patients admitted to an internal medicine ward, identify potential outcome predictors and differences between young and elderly, as well as convulsive (CSE) and non-convulsive SE (NCSE) patients. Methods We enrolled 135 consecutive patients in an observational, retrospective cohort study. We established elderly patients as more than 64 years old and defined worse prognosis as a modified Rankin Scale (mRS)>4. Results The SE population was 73% elderly, and 75% presented with NCSE, mainly metabolic, idiopathic, or vascular SE. The intra-hospital mortality was 51%, and 62% had an mRS>4 at discharge. NCSE and electroencephalogram (EEG) with paroxysmal activity at discharge were predictive of a worse prognosis. Elderly patients had increased disability at admission, most had NCSE (81%), and the SE etiology differed with more idiopathic and vascular causes. In the elderly, mortality was increased, as was the number of patients with mRS>4 at discharge. NCSE patients had the more neurodegenerative disease (30%) and presented predominantly with vascular and anoxic causes. Morbidity and mortality were also increased in the NCSE group. There was no difference in the antiepileptic drugs used or in the percentage of patients achieving an EEG with no paroxysmal activity between the subpopulations. Conclusion SE in elderly patients should be addressed distinctly. Current approaches based on the strategies used for standard CSE have shown little or no efficacy overall.

Wilson's disease is a rare autosomal recessive condition. A defect on the copper carrier protein ATP7B prevents the excretion of copper, which then accumulates in several organs. The prognosis of Wilson's disease is favourable if the diagnosis is made early. The Leipzig criteria standardized phenotypic classification and diagnostic criteria, thus simplifying the diagnostic approach. A search for ATP7B mutations is not necessary for diagnostic purposes and studies of genotype–phenotype correlation have not produced any conclusive evidence so far. More information is needed to reliably assess the prognosis for each patient. Here we describe a young patient with a combination of two mutational variants: c.3402del and c.3061-12T>A. To our knowledge, this is the first report of this compound heterozygote genotype.

Recent advances in imaging studies and treatment approaches have greatly improved our knowledge about Giant Cell Arteritis (GCA). Previously thought of as a predominantly cranial disease, we now know that GCA is a systemic disease that may involve other medium and large vessel territories. Several imaging studies have shown that between 30 and 70% of patients with GCA present with large-vessel vasculitis. Moreover, a significant proportion of patients present large-vessel disease in the absence of cranial involvement. Extra-cranial disease also poses management challenges as these patients may have a more refractory-relapsing disease course and need additional therapies. Aortic dilation and aneurysms are well-described late complications of GCA involving the large artery territories. In this chapter, we discuss the clinical picture of extra-cranial involvement in GCA, focusing on improved diagnostic protocols and suitable treatment strategies.

Introdução: A pericardite aguda é uma síndrome frequente caracterizada pela inflamação do pericárdio e usualmente atribuído a etiologia viral/idiopática. Apesar dos avanços no tratamento, existe informação limitada em relação a etiologia e prognóstico. O objetivo do nosso trabalho foi identificar a prevalência de pericardite aguda com etiologia especifica e avaliar marcadores clínicos de prognóstico. Material e Métodos: Estudo retrospetivo de doentes hospitalizados por pericardite aguda entre 2012-2016. A população foi caracterizada quanto a etiologia, apresentação clínica, tratamento e prognóstico. Para avaliação de prognóstico foram avaliadas recorrência de pericardite, pericardite constritiva e mortalidade global um ano após alta hospitalar. Resultados: Foram incluídos 94 doentes com idade mediana 46 anos (IIQ 32-61), 65% eram do sexo masculino. A etiologia idiopática foi responsável por 68% dos casos. Etio- logia especifica foi identificada em 32% dos doentes, sendo mais frequente doença autoimune (12%) e neoplasia (5%). A pericardite idiopática foi mais associada a miopericardite (p = 0,049), enquanto a etiologia específica se associou a derrame pericárdico (p = 0,001) e a tamponamento pericárdico (p = 0,027). A recorrência de pericardite ocorreu em 13% dos doentes. O tratamento com corticosteroides em doentes com etiologia definida não se associou com aumento de recorrência (p = 0,220). A mortalidade global a um ano foi de 9%. A etiologia definida de pericardite aguda revelou-se o único preditor independente de mortalidade na análise multivariada (OR 40,3; 95% CI 1,9 – 137,2; p = 0,016). Conclusão: Cerca de um terço dos doentes hospitalizados por pericardite aguda têm uma causa específica identificável de pericardite e estes doentes apresentam risco aumentado de mortalidade.

Human fascioliasis is a trematode parasitic disease with worldwide distribution. Natural reservoirs include several ruminants, mainly sheep, goats and cattle.1 Humans are accidental hosts and acquire infection by ingestion of metacercaria present in aquatic plants.1,2 This food-borne zoonosis has claimed little attention in clinical practice. However, increasing reports of the disease in travel medicine and the recognition of endemic areas in many countries have contributed to the increasing importance of Fasciola infection.3 Despite that, it continues to be misdiagnosed as the clinical picture may easily be attributed to other diseases. A 76-year-old man from Cape Verde had a history of chronic obstructive pulmonary disease (COPD) and arterial hypertension. He has been living in Portugal for the last 30 years. The last travel to Cape Verde was in 2014. This patient presented to the emergency department on November 2015 with a 4-month history of dyspnea,...

Introduction: Acute pericarditis is a common inflammatory condition of the pericardium usually assumed to be viral or idiopathic. Despite recent treatment improvements, information is scarce regarding etiology and prognosis. Our aim was to determine the incidence of pericarditis with a known etiology and assess clinical prognostic predictors. Material and Methods: A clinical retrospective analysis of hospitalized patients with acute pericarditis was conducted from 2012 to 2016. Population was characterized according to etiology, clinical presentation, treatment and prognosis. Outcomes of interest, evaluated at one year after hospital discharge, were pericarditis recurrence, hospitalization, constriction and overall mortality. Results: A total of 94 patients were enrolled, median age 46 years (inter-quartile range 32-61), 65% were male. Idiopathic etiology was responsible for 68% of cases. A specific etiology was found in the remaining 32% of patients, being the most frequent autoimmune disease (12%) and malignancy (5%). Idiopathic pericarditis was associated with myopericarditis (p = 0.049) and a known etiology with pericardial effusion (p = 0.001) and cardiac tamponade (p = 0.027). Recurrence of pericarditis was found in 13% of patients. Corticosteroid treatment in patients with an identified etiology was not associated with an increase in recurrence (p = 0.220). Overall 1-year mortality was 9%. A defined etiology was the only independent predictor of mortality at multivariate analysis (OR 40.3; 95% CI 1.9 – 137.2; p = 0.016). Conclusion: Up to one third of hospitalized patients with acute pericarditis have an identified cause of pericarditis and these patients are at increased risk of mortality.

A 40-year-old man presented to the emergency department with dyspnoea and fatigue after bupropion and popper consumption. Clinical examination was remarkable for central cyanosis not responding to supplementary oxygen. Arterial blood gas analysis showed a methaemoglobin value of 30.3%. Methaemoglobinemia was diagnosed and the patient was treated with methylene blue. However, during methylene blue administration, the patient developed a generalized tonic-clonic seizure that was successfully managed with diazepam. Combined intoxications can be a critical problem in the emergency department. Early recognition and treatment of poisoning are key for good patient outcome.LEARNING POINTS:Distinguishing toxidromes is critical for adequate treatment of patients with drug intoxication; the most common side effect of bupropion consumption is dose-dependent seizures.The diagnosis of methaemoglobinemia requires a high index of suspicion, particularly in a patient presenting with central cyanosis not responding to supplementary oxygen.Treatment with methylene blue is recommended when the percentage of methaemoglobin is above 30% or when the patient has symptoms related to methaemoglobinemia.