Abstract in English:

Abstract Background Most guidelines recommend that people living with dementia and their care partners should be managed in primary care. However, the knowledge and confidence of these teams in managing dementia is low, and training programs are lacking. Objective To identify the training needs of primary care teams by integrating insights from these professionals, as well as dementia patients and their care partners. Methods Qualitative research methods were applied, using focus group interviews with health professionals and individual interviews with people living with dementia and their care partners. A direct qualitative analysis of 15 recorded interviews (3 focus groups and 12 individuals) was performed using the transcribed data. Results Primary care professionals recognize the importance of continuous education on dementia and expressed the need for more knowledge about diagnosis, symptom management, and interpersonal and communication skills. Care partners and dementia patients highlighted the need for a better diagnostic disclosure process, improved continuity of care, and availability of greater postdiagnosis support. Conclusion Our study, novel in Latin America, strongly supported the need for more training in dementia for primary care professionals, as well as for additional content and information not usually included in standard dementia education.

Abstract in English:

Abstract Scientific investigations have highlighted the benefits of cognitive stimulation for cognitive, psychological, and social aspects in older individuals. However, there is a dearth of long-term, methodologically-rigorous studies. The aim of the present study was to describe the methods and the initial characteristics of the participants in a randomized controlled trial on cognitive stimulation. A total of 578 older individuals accepted invitations to participate in the study. Of these respondents, 362 met the eligibility criteria, and 255 were selected and randomized into the training, active control, and passive control groups. During the baseline stage (T0), 48 participants withdrew, resulting in a final T0 sample of 207 participants. The three groups were similar in terms of cognitive performance and sociodemographic and psychosocial variables, but they differed significantly regarding depressive symptoms, with the training group scoring higher. The methods herein described can help guide future research on cognitive stimulation in older adults.

Abstract in English:

Abstract Background The rates of complications after major abdominal surgeries remain high, despite the advances in pre- and postoperative care and surgical techniques. In these cases, neurological disorders mainly include stroke and delirium, with a high increase of morbidity. Ataxia is rarely a consequence of abdominal procedures like, more often being related to long-term complications of bariatric surgery due to chronic vitamin deficiency. Objective To describe seven cases of ataxia following major abdominal surgeries and propose a prophylactic approach. Methods A retrospective case series in which medical records of patients from the Ataxia Unit of Universidade Federal de São Paulo were evaluated from January 2007 to August 2024. We identified seven patients who developed acute cerebellar ataxia after gastrointestinal surgery. Demographic, clinical, laboratory, neuroimaging, and treatment data were extracted. Descriptive statistics was used to summarize findings. Results There were two cases that evolved with neurological improvement, and five remained with severe cerebellar ataxia. Brain imaging showed cerebellar atrophy in three patients and signs of Wernicke encephalopathy in two. Conclusion This case series describes an unusual form of acute ataxia with poor outcomes, possibly related to complications from major abdominal surgery. Early intervention and prophylactic supplementation with vitamins B1 and B12 in patients receiving TPN should be considered to avoid such severe neurological complications.

Abstract in English:

Abstract Background Stroke is influenced by numerous factors, both modifiable and non-modifiable. Among these, gamma-glutamyl transpeptidase (GGT) serves as a prognostic biomarker in cardiovascular diseases and, within this context, in neurological conditions like stroke. Objective To determine whether an association exists between GGT and both ischemic and hemorrhagic strokes. Methods A systematic and comprehensive literature search was conducted across 5 databases, encompassing studies published from their inception to January 28, 2024, following a population, exposure, comparator, outcome, and study (PECOS) framework. Ten primary studies meeting the eligibility criteria were selected. Results Our findings, based on a meta-analysis of the ten studies, indicate an increased risk of ischemic and hemorrhagic strokes in patients with elevated GGT levels, after excluding outliers. The analysis demonstrated a significant association, with a relative risk of 1.42 (95%CI: 1.01–1.99; I2 = 19%) for hemorrhagic stroke and 1.22 (95%CI: 1.10–1.36; I2 = 49%) for ischemic stroke. Conclusion Our study reveals an elevated risk of stroke in patients with high GGT levels, demonstrating a 42% higher likelihood of hemorrhagic stroke and a 22% increased risk of ischemic stroke.

Abstract in English:

Abstract Background The main neurologic manifestation of definitive human T-lymphotropic virus 1 (HTLV-1)-associated myelopathy (HAM) is spastic paraparesis, but it only occurs in 5% of the patients. In contrast, about 40% of HTLV-1-infected subjects present symptoms of urologic dysfunction, including nocturia, urgency, and incontinence, which may progress to an inability to void urine. As these patients do not present motor dysfunction, they are classified as probable HAM. Atrophy of the thoracic spinal cord (SC) is the main abnormality found on magnetic resonance image (MRI) scans of patients with definitive HAM, but damage to the SC has not been reported in patients with probable HAM. Objective To determine if, through an evaluation of the metrics of conventional MRI, we can detect a decrease in the area of the SC in patients with probable HAM. Methods Infection by HTLV-1 was herein diagnosed by a Western blot, and the MRI scan was performed using a 1.5-T scanner. Atrophy was considered when the SC area was less than 25% of the intrathecal area. Results We observed a progressive reduction in all segments of the SC area among HTLV-1 carriers, patients with probable and definitive HAM. Significantly, 48.3% of patients with probable HAM presented atrophy of the lumbar area. Conclusion Using MRI metrics, the present study shows the atrophy of lumbar segments of the SC area in patients who present urinary symptoms associated with HTLV-1 but without motor dysfunction.

Abstract in English:

Abstract Background Juvenile systemic lupus erythematosus (jSLE) often involves the central nervous system, with headache being the most common symptom. Objective To describe the frequency, characteristics, and impact on quality of life of headaches in jSLE patients. Methods We conducted a cross-sectional study with jSLE patients under the age of 19 years through chart reviews and questionnaires. The participants underwent clinical and neurological exams, assessments of disease activity and damage, and evaluations of headache characteristics. Quality-of-life impacts were measured using the Brazilian Portuguese version of the Pediatric Quality of Life Inventory (PedsQL), and cognitive function was assessed with the Mini-Mental State Examination. Results Out of 34 patients enrolled, 17 presented with headaches (which were more prevalent in female subjects). The age of the patients at the time of the evaluation ranged from 8 to 18 years, and the mean age at headache onset was of 11.3 years. Most participants with headaches reported having episodic headaches (76.5%), no perimenstrual crises (73.3%), and a family history of migraines (88.2%). Additionally, most patients reported physical activity (35.3%) and stress (58.8%) as triggers. Patients with headaches had significantly lower PedsQL scores compared with those without headaches, both in health and activities (61.76 versus 73.71 respectively; p = 0.04) and feelings (42.65 versus 60.35 respectively; p = 0.049). Conclusion Headaches were more prevalent in female jSLE patients, and the most subjects experienced migraines as the primary cause of headache. Patients with headaches presented lower scores on quality-of-life questionnaires. These findings highlight the need for a comprehensive approach to headache management in jSLE to enhance patient well-being.

Abstract in English:

Abstract Neurocutaneous syndromes are a group of genetically and phenotypically diverse disorders that primarily affect the skin, central and peripheral nervous systems, and eyes. Classifying neurocutaneous syndromes based on genetic mechanisms often proves impractical in routine clinical settings. This review proposes a practical classification of neurocutaneous syndromes based on their neurological manifestations, including neoplastic lesions, epilepsy, vascular abnormalities, and ataxia. In this narrative review, we examined original articles and reviews that explore neurocutaneous syndromes, published between January 2000 and July 2024. The figures are part of a personal collection of the authors. Early recognition of dermatological and neurological hallmarks can guide diagnosis and prompt timely evaluation and treatment. Therefore, a thorough understanding of neurocutaneous syndromes highlights the importance of integrated diagnostic strategies that combine neurological and dermatological assessments.

Abstract in English:

Abstract There are many conditions associated with primary headaches, including mood (depression), anxiety, and sleep disorders, which are highly prevalent in the general population and in tertiary headache centers. We call this set of symptoms migraine, anxiety, mood, and sleep (MAMS). The presence of one or more of these symptoms can alter the clinical course and represent a practical challenge. In the present study, we aimed to describe a practical approach to treat these symptoms frequently associated with headaches. Diagnostic aspects and secondary causes were addressed as well as the shared mechanisms and cause and effect relationship. Finally, we commented on the therapeutic approach used to treat these symptoms.

Abstract in English:

Abstract Tuberculosis (TB), a chronic infection caused by the Mycobacterium tuberculosis complex, has an increased risk of reactivation in conditions that affect the immune system, such as MS, and its treatment with disease-modifying drugs (DMDs). The present is a retrospective study of 2,036 patients diagnosed with MS followed at the Department of Neurology and Neurosurgery of Escola Paulista de Medicina, Universidade Federal de São Paulo, from February 1994 to September 2023. Of that total, 6 were included in this case series, taking different DMDs: fingolimod (n = 2), interferon beta 1a (n = 2), glatiramer acetate (n = 1) and cyclophosphamide (n = 1). In our study, two patients experienced worsening disability during tuberculosis treatment, while three others had increased disability after completing treatment. We reinforce the importance of screening all patients eligible for DMD treatment, especially the highly effective modern ones, and the importance of developing research-based guidelines for screening infectious diseases among patients with MS.

Abstract in English:

Abstract Background Parkinson's disease (PD) is the second most prevalent neurodegenerative disorder worldwide. Levodopa has been considered the best treatment option. However, deep brain stimulation (DBS) use has increased over time, mostly when levodopa-related complications arise. Objective To review the current evidence regarding economic evaluations assessing costs and benefits comparing pharmacological versus surgical treatment among subjects with PD. Methods We searched three databases (PubMed, Embase, and Google Scholar) for studies comparing levodopa treatment and DBS among subjects with PD in terms of costs and benefits from therapy. Results Out of the 107 studies identified, 14 met the inclusion criteria. Most of the published studies were from Europe. Incremental cost-effectiveness ratios have shown variable results, from -€979 to €6,729 per change of 1 point in the score on part III of the Unified Parkinson's Disease Rating Scale (UPDRS III), while incremental cost-utility ratios depict values as low as €6,700 and as high as $704,906.03 per quality-adjusted life-years (QALY). Conclusion We observed a higher cost during the 1st year of DBS implantation due to the surgical procedure itself, subsequently, there was a trend for a lower cost over the following years, with no loss of benefit. Overall, the studies showed DBS as a cost-effective measure at 5-years after implantation.

Abstract in English:

Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired autoimmune disorder that leads to progressive motor and sensory impairment, resulting in significant morbidity. While the incidence rates vary, CIDP remains a challenging condition requiring a standardized and optimized approach to diagnosis and management. In Brazil, a middle-income country with substantial regional disparities in healthcare access, the availability of specialized neuromuscular centers is uneven, creating obstacles to timely and effective treatment. To address these challenges, the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN, in Portuguese) has developed national recommendations for the diagnosis, management, and treatment of CIDP, tailored to the country’s healthcare resources. This consensus outlines standardized diagnostic criteria that incorporate electrophysiological and imaging findings, and it emphasizes key differential diagnoses to enhance diagnostic accuracy. The recommendations were developed through expert panel discussions and a non-systematic review of the literature. The recommended treatment strategies include first-line therapies such as corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, with guidance on escalation and titration of immunosuppressive therapy in refractory cases. By emphasizing early intervention to prevent axonal degeneration and disability, these guidelines aim to improve clinical outcomes and support public health policies within the Brazilian National Health System (Sistema Único de Saúde, SUS, in Portuguese), ensuring equitable and effective CIDP management across the country.

Abstract in English:

Abstract Deep brain stimulation (DBS) has been widely accepted as a powerful tool capable of suppressing tremor by modulating the neuronal circuitry, with long-term adaptability and a profile of low adverse effects. It has been the primary treatment for refractory tremor for decades, with sustained long-term efficacy. Recently, magnetic resonance-guided high-frequency focused ultrasound (HIFU) has emerged as an alternative, prompting comparisons between these approaches. Deep brain stimulation offers long-lasting tremor control in Parkinson's disease (PD) and essential tremor (ET). In addition, it enables us to advance our understanding of brain circuits by integrating neuroimaging, electrophysiology, and connectomics data to map the best stimulation spots. Technologies such as adaptive and directional DBS enable real-time adjustments and greater precision, optimizing results and minimizing adverse effects. Although HIFU shows promising results, it remains an ablative and non-adjustable therapy, contrasting with DBS's dynamic and customizable advances.

Abstract in English:

Abstract High-intensity focused ultrasound (HIFU) has emerged as a minimally invasive and incision-free alternative for managing tremors associated with essential tremor (ET) and Parkinson's disease (PD). Approved by the United States Food and Drug Administration (FDA) for unilateral and staged bilateral thalamotomy, HIFU also addresses cardinal PD symptoms such as rigidity and bradykinesia through pallidotomy. Tremor improvement rates range from 50 to 75% for ET and 60 to 90% for tremor-dominant PD, with long-term efficacy sustained up to 5 years posttreatment, including 73% tremor improvement in a recent controlled multicenter study. Unlike deep brain stimulation (DBS), HIFU eliminates hardware-related complications, such as infections and intracerebral hemorrhage, and minimizes postprocedural maintenance. Adverse events are primarily mild and transient, including temporary paresthesia and imbalance. Real-time magnetic resonance imaging (MRI) guidance enhances targeting precision, enabling patients to resume daily activities within 24 hours. These attributes make HIFU a durable and effective treatment option.

Abstract in English:

Abstract Variant transthyretin amyloidosis with polyneuropathy (ATTRv-PN) and cardiomyopathy (ATTRv-CM), formerly known as familial amyloidotic polyneuropathy (FAP), is a severe, progressive disorder caused by mutations in the transthyretin (TTR) gene. Historically, FAP was considered a neglected disease due to its rarity and the limited understanding of its pathophysiology, which led to minimal research funding and few therapeutic options. The present article explores the transformative role of Centro de Paramiloidose Antônio Rodrigues de Mello (CEPARM), established in 1984, in elevating the status of FAP through significant advancements in research and treatment. Although CEPARM was not the sole catalyst for this shift, its contributions in liver transplantation, the development of pharmacological therapies, and holistic patient care have substantially improved the recognition and management of FAP. The article also examines CEPARM's impact on patient care, the ongoing challenges, and ethical considerations within the field.