Charles Bonnet Syndrome (CBS) is a rare cause of complex visual hallucinations (VH) in individuals with preserved cognitive status. We report a case of an elderly patient with VH, diplopia and headaches for 1 week, with preserved visual acuity and without any strength or focal neurological findings. Computed tomography revealed a subacute ischaemic stroke with temporal and occipital involvement. The patient was admitted to the Department of Internal Medicine where neurological, ophthalmological and psychiatric primary disease were ruled out, as well as dementia. CBS was assumed as a result of a subacute ischaemic stroke. Valproic acid was initiated to treat the symptoms, which resolved the VH. After 3 months of follow up, the patient remained completely recovered without any dementia signs. CBS is a benign disease, usually controlled without pharmacological therapy and not associated with a psychiatric disease; nevertheless, its association with dementia is not clear.
Objectives: Although threatening there is not enough awareness for ocular tuberculosis, hence we intend to study and characterize the ocular tuberculosis observed on a Portuguese specialized pulmonology diagnostic center. Materials and Methodologies: Retrospective study of individuals diagnosed with ocular tuberculosis and followed up from 1st January 2016 until 31th December 2018. Results: We studied 38 patients with presumed ocular tuberculosis, with mean age 53,315,7 years old, whose 55,3% were females. Only one patient had known immunosuppression, seven patients had history of previous tuberculosis and only two patients reported a known risk contact. None had extraocular disease. All patients had at least one positive immunologic test, either tuberculin skin test (63,2%) or Interferon Gamma Release Assay test (86,8%). Most patients presented bilateral ocular tuberculosis (44,7%). The standard four-drug regimen was the treatment of choice and corticosteroids were administered to 55,3% patients with no differences in treatment outcomes. The mean length of treatment was 8,6 months and among the patients who completed treatment, 72,4% presented clinical improvement or remission of the ocular manifestations. Conclusions: Ocular tuberculosis, despite a rare condition, carries a huge burden in health care centers. Delay in starting proper treatment can result in permanent blindness and impairment of life's quality. This condition is probably underdiagnosed and, to our knowledge, there are no recent studies characterizing the latest trend of ocular tuberculosis in Portugal.
Objective: To evaluate the effectiveness of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and to characterize its safety profile in cystic fibrosis (CF) patients in a real-world clinical setting. Methods: This was a prospective observational study carried out in a CF referral center in Portugal involving adult CF patients who started treatment with ELX/TEZ/IVA. Clinical characteristics of the patients were collected, and effectiveness and safety data were evaluated. Results: Of the 56 patients followed in the center at the time of the study, 28 were eligible for ELX/TEZ/IVA treatment in accordance with the Portuguese National Authority for Medicines and Health Products at the time of the study. Of these, 24 met the follow-up time requirement to be included in the clinical effectiveness analysis. The mean follow-up time was 167.3 ± 96.4 days. Adverse events were generally mild and self-limited. Significant improvements in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations were observed. No significant differences in outcomes between F508del homozygous and heterozygous patients were found. The effectiveness of this new CFTR modulator combination also applied to patients with advanced lung disease. Conclusions: Treatment with ELX/TEZ/IVA showed effective improvement in real-world clinical practice, namely in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations, with no safety concerns.
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