Arquivos de neuro-psiquiatria最新文献

Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction characterized by fluctuating skeletal muscle weakness. We report a 61-year-old woman presenting with progressive dysphagia as the sole initial symptom for 15 months, leading to diagnostic delay. Esophageal manometry suggested ineffective motility, and electroneuromyography was normal, reinforcing a non-neurological hypothesis. During hospitalization for worsening dysphagia, cervical magnetic resonance imaging (MRI) incidentally revealed an anterior mediastinal nodule consistent with thymoma. Pulse corticosteroid therapy caused abrupt clinical deterioration, prompting neurological evaluation. Pyridostigmine produced marked improvement within 48 hours, and acetylcholine receptor antibody testing confirmed MG (18.7 nmol/L, radioimmunoassay). Robotic thymectomy revealed a type AB thymoma. The patient remains stable on azathioprine 2.5 mg/kg/day, with oncologic follow-up. This case highlights that MG should be considered in persistent unexplained dysphagia, particularly when symptoms worsen after corticosteroid exposure. Early recognition avoids morbidity and unnecessary invasive procedures.

Chorea is a rare manifestation of Wilson's disease (WD), resulting from copper-induced dysfunction of the basal ganglia. We herein report the case of a 22-year-old woman with progressive dysarthria and generalized chorea, later confirmed as WD. After 1 year of penicillamine therapy, she showed marked clinical improvement, with only residual chorea in the right upper limb. A 7-Tesla (7-T) magnetic resonance imaging (MRI) scan revealed predominant involvement of the left basal ganglia and corticospinal tract, including the hyperintense globus pallidus rim sign. This case highlights chorea as a potential initial manifestation of WD and reinforces its reversibility with the appropriate treatment.

Chronic migraine (CM) is often complicated by medication-overuse headache (MOH), worsening disability. Although withdrawal of overused medications is recommended, adherence is poor and relapse is frequent. Monoclonal antibodies (mAbs) targeting the calcitonin gene-related peptide (CGRP) pathway may offer an effective preventive option without requiring discontinuation, but current evidence is limited by few and heterogeneous randomized controlled trials (RCTs).To systematically assess the efficacy and safety of anti-CGRP mAbs in treating CM with MOH (CM + MOH) under a conservative approach.The PubMed, Embase, and Cochrane Central databases were searched for RCTs comparing anti-CGRP mAbs with placebo in adults with CM + MOH. The primary outcome was the mean change in monthly migraine days (MMDs) at 3 months. The secondary outcomes included acute medication use, disability, drug overuse resolution, response rate, and adverse events (AEs). Random-effects models with Sidik-Jonkman estimator and Knapp-Hartung adjustments pooled effect sizes.We included seven RCTs, totalling 3,094 patients. Anti-CGRP mAbs significantly reduced MMDs (mean difference [MD] = -0.35; 95%CI: -0.43 to -0.26) and acute medication use (MD = -0.35; 95%CI: -0.51 to -0.19) compared with placebo. Higher rates, of ≥ 50%, of response (risk ratio [RR] = 1.94; 95%CI: 1.60-2.34) and drug overuse resolution (RR = 1.38; 95%CI: 1.04-1.83) were observed, with no significant increase in AEs (RR = 1.09; 95%CI: 0.85-1.40).Anti-CGRP mAbs were effective and well tolerated in CM + MOH, representing a viable alternative, especially for patients unable to discontinue acute medications. Further research should assess long-term outcomes and subgroup effects.

Amyotrophic lateral sclerosis (ALS) is a rare degenerative disease of motor neurons, predominantly sporadic, with approximately 10% of the cases showing familial inheritance.To characterize the clinical and sociodemographic profile of patients with familial ALS type 8 (fALS8) and compare it with sporadic ALS (sALS).We reviewed the medical records (1997-2022) from a specialized Brazilian center. Patients with a confirmed diagnosis of ALSs were included, and sociodemographic and clinical data were collected.The sample was composed of 89 ALS patients, with a slight female predominance (53%) and a high frequency of fALS8 cases (45%). The fALS8 patients were diagnosed at a younger age, at approximately 50 years, compared to 53 years among the sALS patients (p = 0.043). Lower limb onset predominated in the fALS8 group (87%), while the sALS group showed more heterogeneous presentations, including bulbar onset (14%). The time until the diagnosis was significantly longer in the fALS8 group compared to the sALS group, both from symptom onset (approximately 51 versus 30 months respectively; p < 0.001) and after admission to a specialized center (7 versus 4 months respectively; p = 0.002). Dysphagia and gastrostomy were more frequent in the sALS group compared to the fALS8 group (p = 0.02 and p < 0.01 respectively), and older age at diagnosis was associated with worse functional scores.The fALS8 group presented with distinct clinical and demographic features compared to the sALS group, including younger age at diagnosis, more homogeneous symptom onset, and lower frequency of dysphagia and need for gastrostomy. The diagnosis was more delayed in the fALS8 group, and older age at diagnosis was associated with worse functional status. The current study contributes to the scarce data on fALS8 in South America.

Cognitive impairment is common in multiple sclerosis (MS), and visual memory impairments are increasingly considered clinically important, yet still underappreciated.To compare cognitive performance assessed by the Rey Complex Figure Test (RCFT) and Symbol Digit Modalities Test (SDMT) in MS patients and healthy controls, and to examine correlations between these measures and physical disability indices.A total of 177 participants were assessed using RCFT subtests and SDMT, of whom 95 had relapsing-remitting MS (RRMS) and 82 were healthy controls. Group comparisons and correlation analyses were performed.The MS patients scored significantly lower on all RCFT subtests and SDMT. Moderate correlations were found between SDMT and RCFT 0 to 2, but not RCFT 3.Both RCFT and SDMT may serve as potentially complementary measures of cognitive domains relevant to MS. Incorporating both recall and recognition-based measures, along with processing speed assessments, can provide a broader evaluation of cognitive strengths and weaknesses in MS, helping clinicians better characterize patients' profiles.

Stroke remains the second leading cause of death worldwide and the leading cause in Brazil. The implementation of a stroke center (SC) has played a crucial role in improving outcomes, increasing both survival rates and functional recovery.To examine the experience of Hospital Universitário de Brasília of Universidade de Brasília's (HUB-UnB) SC, focusing on its performance indicators.The present observational, retrospective, and analytical study evaluated 19 patients diagnosed with acute ischemic stroke (AIS) who received intravenous thrombolysis with alteplase at the HUB-UnB SC between July 2021 and December 2024. Performance indicators were compared with national and international data.The door-to-CT time was less than 25 minutes in 16 patients (84%), and the door-to-needle time was less than 60 minutes in 12 patients (63%). The mean National Institutes of Health Stroke Scale score significantly decreased from 12.4 at admission to 1.6 at discharge. Consequently, 76% of patients achieved a modified Rankin Scale score of 0 to 2. Complication rates were low, including 5.3% hemorrhagic transformation and 10.5% in-hospital mortality. No statistically significant differences were observed when comparing performance indicators with national and international benchmarks.The implementation of the HUB-UnB's SC proved feasible and effective in providing specialized care for AIS within a university hospital of the Unified Health System (Sistema Único de Saúde, SUS, in Portuguese). Despite operating only during weekday business hours, outcomes were comparable to those of high-income countries. Expansion to 24-hour operation, infrastructure improvements, recruitment of additional healthcare professionals, and greater integration with the SUS remain key challenges.

Stroke remains one of the leading causes of morbidity and mortality worldwide, requiring rapid and accurate recognition, especially in the context of prehospital care.To construct, validate, and produce simulated scenarios for teaching and learning about the care of stroke victims in prehospital settings.The present is a methodological, descriptive, and quantitative study involving the development of two simulated scenario scripts based on best simulation practices and Bloom's taxonomy. Validation was conducted by 11 expert judges selected according to Fehring's criteria, using an adapted instrument and the Content Validity Index (CVI), with a value of 0.90 or higher considered valid.All evaluated items of the scripts achieved full agreement among the judges, with a mean CVI of 1.0. The scenarios incorporate both typical and atypical signs and symptoms of stroke and are applicable for training prehospital care teams.The validated simulated scenarios have the potential to enhance the training of prehospital care professionals, promoting the early recognition of stroke across different regional contexts in Brazil.

The recent approval of monoclonal antibodies for the treatment of Alzheimer's disease represents a significant advancement in neurology. This accomplishment coincides with a worrisome trend: the increasing hegemony of large corporate healthcare organizations and commercial laboratory corporations in the supply of these new medications. This editorial examines how corporate influence undermines the traditional physician-patient relationship, diminishes neurologist autonomy, and signals a broader incursion into neurological practice by nonspecialists. The current situation in Brazil, compared with the United States and Europe, including the United Kingdom, is discussed, and strategies are proposed to maintain the integrity of neurological therapy amid growing corporate influence in the medical field.

A 25-year-old man presented with subacute progressive anxiety, cognitive deficits, hypernatremia, and left hemiparesis. Magnetic resonance imaging demonstrated multifocal lesions in bilateral basal ganglia (globus pallidus and putamen), fornices, pineal region, along with Wallerian degeneration of the right corticospinal tract with right brainstem atrophy. Serum B-hCG, alpha-fetoprotein, alkaline phosphatase, and placental alkaline phosphatase were normal. Cerebrospinal fluid studies were unremarkable. Brain biopsy revealed intracranial germinoma. The case herein presented highlights a rare presentation of an uncommon neuro-oncological condition that often demonstrates excellent treatment response but is often diagnosed late due to its non-specific presenting symptoms.