Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

Federal University of Minas Gerais

Universidade Federal do Paraná

Federal University of Rio de Janeiro

Universidade Federal Fluminense

University of São Paulo

University of Campinas

Universidade Federal de São Paulo

Universidade Santo Amaro (UNISA)

Hospital Israelita Albert Einstein

Santa Casa Medicine School, São Paulo

University of Toronto

Publication of the Academia Brasileira de Neurologia - ABNEURO. Mission: To publish the results of clinical and experimental researches in neurology and related areas.

Arquivos de Neuro-psiquiatria

[Not Available].

Background: 
A treatment-related fluctuation (TRF) in a patient with Guillain-Barré syndrome (GBS) is defined as clinical deterioration within two months of symptom onset following previous stabilization or improvements with treatment.

Objective: 
To investigate the clinical characteristics and factors that could increase the risk of relapse of GBS in patients with and without TRFs.

Methods: 
Retrospective review of medical records of patients (>18 years) with GBS evaluated between January/2006 and July/2019. Demographic and clinical characteristics, ancillary studies, treatment received, and the clinical course of patients with and without TRFs were analyzed.

Results: 
Overall, 124 cases of GBS were included; seven (5.6%) presented TRFs. GBS-TRF cases were triggered more frequently by infectious mononucleosis (28.57 vs. 8.55%; p=0.01). GBS-TRF were initially treated with plasmapheresis more frequently than those without TRF (14.29 vs. 1.70%; p=0.0349). Combined treatment (71.43 vs. 4.27%; p<0.001) and corticosteroids (42.86 vs. 1.71%; p<0.001) were more commonly used in the GBS-TRF group. GBS-TRF patients presented a higher median initial disability score (4 vs. 2; p=0.01).

Conclusions: 
Patients with GBS triggered by infectious mononucleosis and a high degree of initial disability have higher chances of developing TRFs. Although patients with TRF were treated with plasmapheresis more often, the total number was too low to suggest a link between plasma exchange and TRF.

Treatment-related fluctuations in Guillain-Barré syndrome: clinical features and predictors of recurrence

Cerebellar ataxias comprise a heterogeneous group of diseases characterized by motor and non-motor symptoms, which can be acquired, degenerative, or have a genetic cause, such as spinocerebellar ataxias (SCA). Usually, the genetic and neurodegenerative forms of cerebellar ataxias present a progressive and inevitable worsening of the clinical picture so that rehabilitation treatment is fundamental. Rehabilitation treatment includes physical therapy, respiratory therapy, speech, voice and swallowing therapy, occupational therapy, and new technologies, such as the use of exergames. The current treatment of patients with cerebellar ataxias, especially neurodegenerative forms, genetic or not, should include these different forms of rehabilitation, with the main objective of improving the quality of life of patients.
Keywords:
Cerebellar Ataxia; Rehabilitation; Physical Therapy Specialty; Speech Therapy; Occupational Therapy

Rehabilitation in patients with cerebellar ataxias

The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.
Keywords:
Parkinson Disease; Antiparkinson Agents; Deep Brain Stimulation; Rehabilitation

Guidelines for Parkinson’s disease treatment: consensus from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology - motor symptoms

Recent advances in technology have allowed us access to a multitude of datasets pertaining to various dimensions in neurology. Together with the enormous opportunities, we also face challenges related to data quality, ethics and intrinsic difficulties related to the application of data science in healthcare. In this article we will describe the main advances in the field of artificial intelligence and Big Data applied to neurology with a focus on neurosciences based on medical images. Real-World Data (RWD) and analytics related to large volumes of information will be described as well as some of the most relevant scientific initiatives at the time of this writing.

Artificial intelligence and Big Data in neurology

Background: 
Myasthenia gravis is largely a treatable disease, but it can result in significant morbidity and even mortality, which can usually be avoided, or at least mitigated, with timely diagnosis and appropriate treatment of the disease. Objective: this review aims to summarize the main practical aspects of the diagnostic approach, treatment and care of myasthenic patients.

Methods: 
The authors performed a non-systematic critical review summarizing the main practical aspects of myasthenia gravis.

Results: 
Most patients with myasthenia have autoantibodies targeted at acetylcholine receptors or, less commonly, muscle-specific kinase - MuSK. Electrophysiology plays an important role in the diagnosis of neuromuscular junction dysfunction. The central clinical manifestation of myasthenia gravis is fatigable muscle weakness, which can affect eye, bulbar, respiratory, and limb muscles. With rare exceptions, patients have a good response to symptomatic treatment, but corticosteroids and/or immunosuppressants are usually also necessary to obtain good control of the manifestations of the disease.

Conclusion: 
Knowledge of the peculiar aspects of their clinical and electrophysiological presentations is important for the diagnosis. Likewise, specific treatment and response time to each drug are crucial for proper care.

Myasthenia gravis in clinical practice

Background Sleep deficits caused by the overuse of digital technology is observed among medical students. Due to the coronavirus disease 2019 (COVID-19) pandemic, an emergency remote teaching method was put into practice, which may have resulted in changes in the sleep-wake cycle. The balance between the influences of external and internal synchronizers can be affected by sudden alterations in daily life, including changes in nightly habits and sleep quality, which can lead to increased levels of anxiety and reduced functional performance, for example.
Objective To understand the relationship between the use of digital technology, changes in the circadian cycle, and academic performance during the pandemic.
Methods The present is an analytical, cross-sectional, observational study in which a sample of 123 medical students filled out an online questionnaire on self-perception regarding sleep quality and academic performance before and during the pandemic.
Results Assessing changes in sleep quality and productivity, the study revealed that 100% of the students made continuous use of screens before bedtime. Thus, during the period of social distancing and remote classes, 77.2% of the students reported “poor” or “very poor academic performance, which was probably related to the fact that 65.9% of these students were unable to maintain their productivity due to daytime sleepiness.
Conclusions The prolonged use of screens was associated with poor sleep quality and changes in academic performance, with significant psychological impact. Thus, it is worth emphasizing the importance of sleep hygiene in light of the new forms of teaching implemented during the COVID-19 pandemic.

Digital technology and its impacts on the sleep quality and academic performance during the pandemic

Background In 1839, the Escola de Farmácia de Ouro Preto pioneered the teaching of pharmaceutical sciences in Brazil. At the end of the 19th century, the Escola de Farmácia possessed a French collection of anatomical models, some made of wax and paper-mâché . The models were a critical part of teaching anatomy, particularly in an era of paradigm changes about how the human brain works.
Objective The present study aimed to anatomically describe the brain models through a comparative analysis with the current anatomical description.
Methods Comparative analysis of the brain models with modern anatomical descriptions.
Results In the individual analysis of the wax models, we verified excellent anatomical accuracy of the cortical and subcortical regions. Our results identified internal structures, like the basal ganglia and white matter. Compared with modern anatomical books and websites, the wax brain models have high scientific quality.
Conclusion The models of the present study gave students hands-on experience of human anatomy in the 19 th century. Nowadays, the models are part of the memory of Universidade Federal de Ouro Preto and Museu da Pharmacia de Ouro Preto . The collection of wax models shows the appreciation of neuroanatomy teaching at the turn of the century concomitant with advances in neurology and anatomy around the world.

Anatomical description of the brain wax models of Museu da Pharmacia de Ouro Preto

Background Reliable instruments that lead to early diagnosis for CP are extremely important so that these children are referred for early stimulation, benefiting their development.
Objective To perform a cross-cultural adaptation and reliability assessment of a Brazilian version of the Hammersmith Neonatal Neurological Examination (HNNE), expanded and summarized.
Methods A methodological, cross-sectional, nonexperimental quantitative analysis was conducted in two phases as follows: cultural adaptation of the HNNE, expanded and summarized, and reliability assessment of the Brazilian version of the HNNE. Phase one was developed in five stages (initial translation, synthesis of the translation, a committee of experts, backtranslation, and submission to the author), with the semantic questions, content, and face validity being evaluated. Phase two included 143 newborns and we analyzed the internal consistency, stability, and equivalence (intra- and interexaminer) of the instrument. Internal consistency was calculated using Cronbach's alpha, and intra- and interexaminer reliability and reproducibility assessed through test-retest were calculated using the intraclass correlation coefficient
Results Although internal consistency, assessed using Cronbach's alpha, showed unsatisfactory results, the results of inter-and intraexaminer equivalence showed a high agreement between the evaluations in all domains. The test-retest also showed excellent agreement between the domains.
Conclusions The Brazilian HNNE expanded and summarized versions can be considered to be adapted and reliable for the neurological assessment of Brazilian newborns to identify changes in neurological development and early referral to the stimulation or early rehabilitation units and as a promising option to be used in the context of primary care in Brazil.

Cultural adaptation and reliability assessment of the Hammersmith neonatal neurological examination for Brazilian newborns at risk of cerebral palsy

Therapeutic hypothermia initiated after 6 hours of age and benefits in the treatment of hypoxic ischemic encephalopathy

Cuevas Medek exercises a neurological rehabilitation for children, the effect in developmental hip dysplasia in a patient with cerebral palsy: case study

Jean-Martin Charcot, widely regarded as a leading founder of modern neurology, made substantial contributions to the understanding and characterization of numerous medical conditions. His initial focus was on internal medicine, later expanding to include neuropathology, general neurology, and eventually emerging fields such as neuropsychology and neuropsychiatry. Furthermore, Charcot's intellectual pursuits extended beyond medicine, encompassing research in art history, medical iconography, sociology, religious studies, and the arts, solidifying his status as a polymath.

Resumo
Jean-Martin Charcot, amplamente considerado como um proeminente fundador da neurologia moderna, fez contribuições substanciais para a compreensão e a caracterização de várias condições médicas. Seu foco inicial era a medicina interna, expandindo-se posteriormente para incluir a neuropatologia, a neurologia geral e, por fim, campos emergentes como a neuropsicologia e a neuropsiquiatria. Além disso, as buscas intelectuais de Charcot foram além da medicina, abrangendo pesquisas em história da arte, iconografia médica, sociologia, estudos religiosos e artes, solidificando seu status de polímata.

Jean-Martin Charcot: the polymath

Background: The diagnosis of Alzheimer's disease (AD) can bring financial and emotional consequences to patients and caregivers. Whether or not the diagnosis should be disclosed to patients is a matter of debate amongst physicians and can be influenced by culture and experience.
Objective: To investigate the current practice of physicians who attend and treat patients with dementia in Brazil regarding the disclosure of dementia diagnosis and compare the practice with what has been performed 15 years ago in the country.
Methods: Data were evaluated using an electronic questionnaire. The questions used to carry out this research were similar to the questions of the study carried out 15 years ago 9. The form was sent to the Brazilian Academy of Neurology, the Brazilian Association of Geriatrics and Gerontology, and the Brazilian Association of Psychiatry, which forwarded it to their members. Analyses were conducted through non-parametric statistical tests, with a post-hoc assessment.
Results: 397 physicians responded to the survey, of which 231 are neurologists, 124 geriatricians, 29 psychiatrists and 13 from other specialties. The mean age was 45.2 years. The majority (66.7%) of the physicians reveal the diagnosis of AD always or usually. The youngest group of neurologists were more likely to disclose the diagnosis than the oldest group with a significant difference between them. In comparison to the 2008 Brazilian study, the percentage of physicians who always or usually disclose the diagnosis has risen by 22%. On the other hand, 12.3% of the physicians rarely or never disclose the diagnosis, in comparison to 25,3% in 2008. The main reasons for not disclosing the diagnosis concern the patients' mental health.
Conclusion: Advances in dementia knowledge and biomarkers availability probably explain the increase in the rate of disclosure. The main challenge is to reconcile the autonomy of affected individuals, mental health issues after the diagnosis and the family member's opinion.

Diagnostic disclosure of Alzheimer's disease in Brazil: a national survey of specialized physicians

Background Hereditary or familial spastic paraplegias (SPG) comprise a group of genetically and phenotypically heterogeneous diseases characterized by progressive degeneration of the corticospinal tracts. The complicated forms evolve with other various neurological signs and symptoms, including movement disorders and ataxia.
Objective To summarize the clinical descriptions of SPG that manifest with movement disorders or ataxias to assist the clinician in the task of diagnosing these diseases.
Methods We conducted a narrative review of the literature, including case reports, case series, review articles and observational studies published in English until December 2022.
Results Juvenile or early-onset parkinsonism with variable levodopa-responsiveness have been reported, mainly in SPG7 and SPG11. Dystonia can be observed in patients with SPG7, SPG11, SPG22, SPG26, SPG35, SPG48, SPG49, SPG58, SPG64 and SPG76. Tremor is not a frequent finding in patients with SPG, but it is described in different types of SPG, including SPG7, SPG9, SPG11, SPG15, and SPG76. Myoclonus is rarely described in SPG, affecting patients with SPG4, SPG7, SPG35, SPG48, and SPOAN (spastic paraplegia, optic atrophy, and neuropathy). SPG4, SPG6, SPG10, SPG27, SPG30 and SPG31 may rarely present with ataxia with cerebellar atrophy. And autosomal recessive SPG such as SPG7 and SPG11 can also present with ataxia.
Conclusion Patients with SPG may present with different forms of movement disorders such as parkinsonism, dystonia, tremor, myoclonus and ataxia. The specific movement disorder in the clinical manifestation of a patient with SPG may be a clinical clue for the diagnosis.

Movement disorders in hereditary spastic paraplegias

Background Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder.
Objective To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center.
Methods We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD.
Results Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI.
Conclusion Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

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