Pub Date : 2025-09-01Epub Date: 2025-09-19DOI: 10.1055/s-0045-1811722
Elif Simin Issi
Small fiber neuropathy (SFN) affects thinly myelinated and unmyelinated fibers, often presenting with subtle clinical signs that are undetectable in routine nerve conduction studies. Vitamin B12 deficiency is a known risk factor for SFN, yet early-stage cases frequently remain undiagnosed. Sympathetic skin response (SSR) and cutaneous silent period (CSP) are noninvasive electrophysiological techniques used to assess autonomic and somatic small fiber function.The present study aimed to evaluate the diagnostic utility of SSR and CSP in detecting possible subclinical small-fiber neuropathy (pSFN) in individuals with early-stage vitamin B12 deficiency.The present observational study included 28 patients with vitamin B12 deficiency who had nonspecific complaints, Douleur Neuropathique en 4 Questions (DN4) scores < 4, and normal nerve conduction studies, along with 25 healthy controls. Electrophysiological testing involved SSR recordings from all extremities and CSP measurements from the right median and sural nerves.In the patient group, Median Nerve Cutaneous Silent Period (MN-CSP) and Tibialis Anterior -Sural Cutaneous Silent Period (TA-sural CSP) durations were significantly shorter, while termination and onset latencies were prolonged compared with controls. MN-CSP and TA-sural CSP durations demonstrated high diagnostic accuracy. Sympathetic skin response latencies were significantly prolonged in both hands and feet, indicating autonomic dysfunction. No significant differences were observed in SSR amplitudes.Sympathetic skin response and CSP are valuable tools for detecting possible subclinical SFN in vitamin B12 deficiency. Sympathetic skin response effectively identified autonomic dysfunction, while CSP provided additional diagnostic value for somatic small fiber impairment. Combining SSR and CSP may enhance early detection of pSFN in vitamin B12 deficiency and allow timely intervention.
{"title":"Assessment of possible small fiber Neuropathy in early-stage vitamin B12 deficiency using electrophysiological methods.","authors":"Elif Simin Issi","doi":"10.1055/s-0045-1811722","DOIUrl":"10.1055/s-0045-1811722","url":null,"abstract":"<p><p>Small fiber neuropathy (SFN) affects thinly myelinated and unmyelinated fibers, often presenting with subtle clinical signs that are undetectable in routine nerve conduction studies. Vitamin B12 deficiency is a known risk factor for SFN, yet early-stage cases frequently remain undiagnosed. Sympathetic skin response (SSR) and cutaneous silent period (CSP) are noninvasive electrophysiological techniques used to assess autonomic and somatic small fiber function.The present study aimed to evaluate the diagnostic utility of SSR and CSP in detecting possible subclinical small-fiber neuropathy (pSFN) in individuals with early-stage vitamin B12 deficiency.The present observational study included 28 patients with vitamin B12 deficiency who had nonspecific complaints, Douleur Neuropathique en 4 Questions (DN4) scores < 4, and normal nerve conduction studies, along with 25 healthy controls. Electrophysiological testing involved SSR recordings from all extremities and CSP measurements from the right median and sural nerves.In the patient group, Median Nerve Cutaneous Silent Period (MN-CSP) and Tibialis Anterior -Sural Cutaneous Silent Period (TA-sural CSP) durations were significantly shorter, while termination and onset latencies were prolonged compared with controls. MN-CSP and TA-sural CSP durations demonstrated high diagnostic accuracy. Sympathetic skin response latencies were significantly prolonged in both hands and feet, indicating autonomic dysfunction. No significant differences were observed in SSR amplitudes.Sympathetic skin response and CSP are valuable tools for detecting possible subclinical SFN in vitamin B12 deficiency. Sympathetic skin response effectively identified autonomic dysfunction, while CSP provided additional diagnostic value for somatic small fiber impairment. Combining SSR and CSP may enhance early detection of pSFN in vitamin B12 deficiency and allow timely intervention.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 9","pages":"1-7"},"PeriodicalIF":1.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12449103/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145091133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-09-01Epub Date: 2025-07-28DOI: 10.1055/s-0045-1809332
Pedro Augusto Sampaio Rocha-Filho
Medication-overuse headache affects 1 to 2% of the global population and is often associated with chronic migraine. This condition significantly impacts the lives of patients, as well as their families, and it poses a major economic burden due to lost productivity and medical costs. The present narrative review is part of a controversy session. We argue that reversing the behavior of overusing symptomatic pain medications is important for the treatment of this type of headache. To support this argument, the article reviews and critically analyzes the relevant literature on the subject.
{"title":"Calcitonin gene-related peptide monoclonal antibodies and medication-overuse headache: stopping excessive pain medication is still necessary.","authors":"Pedro Augusto Sampaio Rocha-Filho","doi":"10.1055/s-0045-1809332","DOIUrl":"10.1055/s-0045-1809332","url":null,"abstract":"<p><p>Medication-overuse headache affects 1 to 2% of the global population and is often associated with chronic migraine. This condition significantly impacts the lives of patients, as well as their families, and it poses a major economic burden due to lost productivity and medical costs. The present narrative review is part of a controversy session. We argue that reversing the behavior of overusing symptomatic pain medications is important for the treatment of this type of headache. To support this argument, the article reviews and critically analyzes the relevant literature on the subject.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 9","pages":"1-4"},"PeriodicalIF":1.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12303620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144726972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-09-01Epub Date: 2025-09-08DOI: 10.1055/s-0045-1811622
Gustavo Maximiano-Alves, Eder Leandro da Silva Dantas, Maria Elena Crespo-Lopez, José Luiz Martins do Nascimento
Mercury intoxication poses a significant challenge and growing threat to public health, particularly in the Amazon region. Despite a known history of neurological damage, as evidenced by Japan's Minamata disease, mercury intoxication remains underdiagnosed in Brazil. This review underscores the need for increased clinical awareness among neurologists, as mercury exposure has been linked to over 250 neurological symptoms, including cognitive impairment, cerebellar ataxia, peripheral neuropathy, and psychiatric disturbances. The Indigenous and riverside populations in the Amazon present a high prevalence of cognitive and motor deficits, tremors, and sensory disturbances, which are associated with mercury body burdens. Diagnosis relies on a combination of clinical suspicion, environmental exposure history, and biomonitoring through hair and urine analyses. Given the widespread environmental contamination and potential long-term health consequences, neurologists must be vigilant in recognizing and managing mercury-related neurotoxicity, particularly in vulnerable Brazilian populations.
{"title":"A Brazilian Minamata disease? Neurologists must be aware of mercury exposure and intoxication.","authors":"Gustavo Maximiano-Alves, Eder Leandro da Silva Dantas, Maria Elena Crespo-Lopez, José Luiz Martins do Nascimento","doi":"10.1055/s-0045-1811622","DOIUrl":"10.1055/s-0045-1811622","url":null,"abstract":"<p><p>Mercury intoxication poses a significant challenge and growing threat to public health, particularly in the Amazon region. Despite a known history of neurological damage, as evidenced by Japan's Minamata disease, mercury intoxication remains underdiagnosed in Brazil. This review underscores the need for increased clinical awareness among neurologists, as mercury exposure has been linked to over 250 neurological symptoms, including cognitive impairment, cerebellar ataxia, peripheral neuropathy, and psychiatric disturbances. The Indigenous and riverside populations in the Amazon present a high prevalence of cognitive and motor deficits, tremors, and sensory disturbances, which are associated with mercury body burdens. Diagnosis relies on a combination of clinical suspicion, environmental exposure history, and biomonitoring through hair and urine analyses. Given the widespread environmental contamination and potential long-term health consequences, neurologists must be vigilant in recognizing and managing mercury-related neurotoxicity, particularly in vulnerable Brazilian populations.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 9","pages":"1-10"},"PeriodicalIF":1.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12417040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145022796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-09-01Epub Date: 2025-08-31DOI: 10.1055/s-0045-1811233
Renan Barros Domingues, Luísa Aires Domingues, Victor Rebelo Procaci, José Luiz Pedroso
The present review article explores the neuroscience of musical perception, examining the roles of specific brain regions in decoding and interpreting music. Musical perception engages multiple cortical and subcortical areas that work in an integrated manner to process musical elements such as melody, harmony, and rhythm. The paper reviews the current knowledge about the brain circuits involved, as well as pathological conditions that result in abnormalities of musical perception. In addition, the relationship between musical perception and neurological conditions such as epilepsy and Alzheimer's disease is explored. The present review is based on findings from structural and functional neuroimaging studies, neuropsychology, neurophysiology, and clinical research, aiming to show how the brain transforms music sounds into meaningful experiences and addressing pathological conditions in which this complex process may be affected, either in isolation or in association with other forms of neurological impairment.
{"title":"The neuroscience of music perception: a narrative review.","authors":"Renan Barros Domingues, Luísa Aires Domingues, Victor Rebelo Procaci, José Luiz Pedroso","doi":"10.1055/s-0045-1811233","DOIUrl":"10.1055/s-0045-1811233","url":null,"abstract":"<p><p>The present review article explores the neuroscience of musical perception, examining the roles of specific brain regions in decoding and interpreting music. Musical perception engages multiple cortical and subcortical areas that work in an integrated manner to process musical elements such as melody, harmony, and rhythm. The paper reviews the current knowledge about the brain circuits involved, as well as pathological conditions that result in abnormalities of musical perception. In addition, the relationship between musical perception and neurological conditions such as epilepsy and Alzheimer's disease is explored. The present review is based on findings from structural and functional neuroimaging studies, neuropsychology, neurophysiology, and clinical research, aiming to show how the brain transforms music sounds into meaningful experiences and addressing pathological conditions in which this complex process may be affected, either in isolation or in association with other forms of neurological impairment.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 9","pages":"1-11"},"PeriodicalIF":1.6,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12399327/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940671","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-01Epub Date: 2025-08-31DOI: 10.1055/s-0045-1810408
Pedro Braga-Neto, Maria Wanessa Barbosa Dos Santos, Stephanie Suzanne de Oliveira Scott, Renato Puppi Munhoz, Luiz Eduardo Novis, José Luiz Pedroso, Orlando Graziani Povoas Barsottini
Several studies describe a strong association between structural and functional abnormalities of the cerebellum and psychiatric disorders. It is possible to find investigations especially in cases of schizophrenia, bipolar disorder, depression, anxiety disorders, attention-deficit/hyperactivity disorder (ADHD), and autism spectrum disorder (ASD). The involvement of the cerebellum in these conditions is also supported by clinical, functional, and structural imaging studies. The present narrative review aims to discuss and highlight the role of the cerebellum in these disorders, gathering evidence on the possible locations and connections of the affected cerebellar areas and their implications in the cognitive, emotional, and behavioral domains.
{"title":"The cerebellum and psychiatric disorders: unraveling its role in mental health.","authors":"Pedro Braga-Neto, Maria Wanessa Barbosa Dos Santos, Stephanie Suzanne de Oliveira Scott, Renato Puppi Munhoz, Luiz Eduardo Novis, José Luiz Pedroso, Orlando Graziani Povoas Barsottini","doi":"10.1055/s-0045-1810408","DOIUrl":"10.1055/s-0045-1810408","url":null,"abstract":"<p><p>Several studies describe a strong association between structural and functional abnormalities of the cerebellum and psychiatric disorders. It is possible to find investigations especially in cases of schizophrenia, bipolar disorder, depression, anxiety disorders, attention-deficit/hyperactivity disorder (ADHD), and autism spectrum disorder (ASD). The involvement of the cerebellum in these conditions is also supported by clinical, functional, and structural imaging studies. The present narrative review aims to discuss and highlight the role of the cerebellum in these disorders, gathering evidence on the possible locations and connections of the affected cerebellar areas and their implications in the cognitive, emotional, and behavioral domains.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-8"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12399322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
For the purposes of the present study, we define vertigo as the sensation of rotation of the environment or of the person moving. The demand for care for this complaint has been increasing; therefore, it is important to know the profile of patients treated with this complaint, the causes, the comorbidities, the associated factors, and the response of these individuals to rehabilitation.To analyze the epidemiological profile of patients reporting vertigo and their outcome after physiotherapeutic rehabilitation.We conducted a descriptive cross-sectional study of secondary data analysis. The sample was collected through a census in a specialized clinic in the south of the state of Santa Catarina, Brazil. Individuals were evaluated through a questionnaire developed by the authors, which covered the patient's demographic and clinical data. The statistical analysis was performed using the IBM SPSS Statistics for Windows (IBM Corp.) software, version 23.0.A total of 200 medical records were evaluated, 5 of which were excluded due to non-adherence to treatment, totaling a sample of 195 patients. Their mean age was of 51.18 ± 16.84 years, 62.6% were female subjects, and benign paroxysmal positional vertigo (BPPV) was the most prevalent cause (55.9%), with associated symptoms such as tinnitus (30.8%) and depression and/or anxiety (29.2%). Of the 195 patients, 193 responded positively to the vestibular rehabilitation treatment, and the vertigo of 58% improved after 1 session.The population older than 50 years of age is the most affected by vertigo, especially women, with BPPV as the main cause and associated symptoms such as tinnitus, depression, and anxiety. Most patients improved after the first vestibular rehabilitation session.
为了本研究的目的,我们将眩晕定义为环境旋转或人移动的感觉。对这一疾病的护理需求一直在增加;因此,重要的是要了解治疗这种疾病的患者的概况,原因,合并症,相关因素,以及这些个体对康复的反应。目的分析眩晕患者的流行病学特征及物理治疗康复后的预后。我们对二手数据分析进行了描述性横断面研究。样本是在巴西圣卡塔琳娜州南部的一家专门诊所通过人口普查收集的。通过作者开发的调查问卷对个体进行评估,调查问卷涵盖了患者的人口统计学和临床数据。统计分析使用IBM SPSS Statistics for Windows (IBM Corp.)软件,版本23.0进行。总共评估了200份医疗记录,其中5份因不坚持治疗而被排除,共计195名患者。他们的平均年龄为51.18±16.84岁,女性占62.6%,良性阵发性体位性眩晕(BPPV)是最常见的病因(55.9%),伴有耳鸣(30.8%)、抑郁和/或焦虑(29.2%)等症状。195例患者中,193例对前庭康复治疗有积极反应,58%的眩晕在1次治疗后得到改善。50岁以上的人群受眩晕的影响最大,尤其是女性,BPPV是眩晕的主要原因,伴有耳鸣、抑郁和焦虑等相关症状。大多数患者在第一次前庭康复后得到改善。
{"title":"Profile and postrehabilitation outcome of patients with vertigo in a clinic in Southern Santa Catarina.","authors":"Emanuela Hannoff Pilon, Vitor Benincá-Fernandes, Letícia Fernandes, Karina Rossa, Tatiana Pizzolotto Bruch","doi":"10.1055/s-0045-1811175","DOIUrl":"10.1055/s-0045-1811175","url":null,"abstract":"<p><p>For the purposes of the present study, we define <i>vertigo</i> as the sensation of rotation of the environment or of the person moving. The demand for care for this complaint has been increasing; therefore, it is important to know the profile of patients treated with this complaint, the causes, the comorbidities, the associated factors, and the response of these individuals to rehabilitation.To analyze the epidemiological profile of patients reporting vertigo and their outcome after physiotherapeutic rehabilitation.We conducted a descriptive cross-sectional study of secondary data analysis. The sample was collected through a census in a specialized clinic in the south of the state of Santa Catarina, Brazil. Individuals were evaluated through a questionnaire developed by the authors, which covered the patient's demographic and clinical data. The statistical analysis was performed using the IBM SPSS Statistics for Windows (IBM Corp.) software, version 23.0.A total of 200 medical records were evaluated, 5 of which were excluded due to non-adherence to treatment, totaling a sample of 195 patients. Their mean age was of 51.18 ± 16.84 years, 62.6% were female subjects, and benign paroxysmal positional vertigo (BPPV) was the most prevalent cause (55.9%), with associated symptoms such as tinnitus (30.8%) and depression and/or anxiety (29.2%). Of the 195 patients, 193 responded positively to the vestibular rehabilitation treatment, and the vertigo of 58% improved after 1 session.The population older than 50 years of age is the most affected by vertigo, especially women, with BPPV as the main cause and associated symptoms such as tinnitus, depression, and anxiety. Most patients improved after the first vestibular rehabilitation session.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-5"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12399323/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-01Epub Date: 2025-08-20DOI: 10.1055/s-0045-1811172
Luciana de Paula Souza, Danielle Caldas Bufara, Tallulah Spina Tensini, Sergio Antonio Antoniuk, Gustavo L Franklin, Ana Chrystina de Souza Crippa
West syndrome is an epileptic encephalopathy for which combination therapies with adrenocorticotropic hormone and vigabatrin have emerged as new treatment options.To evaluate the clinical and electroencephalographic remission rates, tolerability, and relapse rates in patients with West syndrome who failed primary treatment and underwent sequential therapy with vigabatrin and adrenocorticotropic hormone.We included 39 patients with West syndrome from 2 specialized centers, aged 2 to 120 months. The patients were treated with intramuscular tetracosactide depot added to vigabatrin and were prospectively followed up for ≥ 1 year. The outcomes were clinical, and electroencephalographic remission rates at 7 and 30 days and 1 year following combined therapy initiation, progression to other epilepsy types, therapy tolerability, and relapse rates were recorded.Of the original sample, 71% of the subjects were boys, and 87% had a known etiology. The clinical and electroencephalographic remission rates were 46.1%, 94.8% (p = 0.001), and 74.1% (p = 0.01) at 7 and 30 days, and 1 year after the initiation of combined therapy, respectively. At the 1-year follow-up, adverse effects were observed in 86.0% and the relapse rate was 21.6%. After a median follow-up of 21 months, 73.6% of the patients developed epilepsy.Combined therapy demonstrated a favorable efficacy profile in achieving clinical and electroencephalographic remission but was associated with significant seizure relapse rates in the medium term. Thus, it represents a feasible option for patients in whom initial treatment has failed.
{"title":"Adrenocorticotropic hormone combined with vigabatrin as a second-line therapy for West syndrome.","authors":"Luciana de Paula Souza, Danielle Caldas Bufara, Tallulah Spina Tensini, Sergio Antonio Antoniuk, Gustavo L Franklin, Ana Chrystina de Souza Crippa","doi":"10.1055/s-0045-1811172","DOIUrl":"10.1055/s-0045-1811172","url":null,"abstract":"<p><p>West syndrome is an epileptic encephalopathy for which combination therapies with adrenocorticotropic hormone and vigabatrin have emerged as new treatment options.To evaluate the clinical and electroencephalographic remission rates, tolerability, and relapse rates in patients with West syndrome who failed primary treatment and underwent sequential therapy with vigabatrin and adrenocorticotropic hormone.We included 39 patients with West syndrome from 2 specialized centers, aged 2 to 120 months. The patients were treated with intramuscular tetracosactide depot added to vigabatrin and were prospectively followed up for ≥ 1 year. The outcomes were clinical, and electroencephalographic remission rates at 7 and 30 days and 1 year following combined therapy initiation, progression to other epilepsy types, therapy tolerability, and relapse rates were recorded.Of the original sample, 71% of the subjects were boys, and 87% had a known etiology. The clinical and electroencephalographic remission rates were 46.1%, 94.8% (<i>p</i> = 0.001), and 74.1% (<i>p</i> = 0.01) at 7 and 30 days, and 1 year after the initiation of combined therapy, respectively. At the 1-year follow-up, adverse effects were observed in 86.0% and the relapse rate was 21.6%. After a median follow-up of 21 months, 73.6% of the patients developed epilepsy.Combined therapy demonstrated a favorable efficacy profile in achieving clinical and electroencephalographic remission but was associated with significant seizure relapse rates in the medium term. Thus, it represents a feasible option for patients in whom initial treatment has failed.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-7"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12367372/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-01Epub Date: 2025-08-31DOI: 10.1055/s-0045-1811173
Elizabeth Honorato de Farias, Anderson Matheus Pereira da Silva, Karlos Daniell Araújo Dos Santos, Altair Pereira de Melo Neto, Aishwarya Koppanatham, Júlia Cappi Aguiar Moraes Souza, Niels Pacheco-Barrios, Gabrielle de Souza Rocha, Gustavo Sousa Noleto
Although it is known that the most commonly used therapies for West syndrome (WS) are intramuscular adrenocorticotropic hormone (ACTH) and oral prednisolone, there is still controversy in the literature regarding the equivalence of their effects.We aimed to present an updated review comparing the therapeutic and adverse effects of ACTH therapy versus corticosteroids in children with West syndrome (WS).The PubMed, EMBASE, and Cochrane Central databases were searched. The outcomes of interest selected were spasm cessation on day 14 of therapy, cessation of hypsarrhythmia and adverse effects such as weight gain, infection, irritability, and hypertension. Studies were reviewed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and a meta-analysis was performed. We compared the results using the risk ratio (RR) and odds ratio (OR). for the binary outcomes, with 95% confidence intervals (CI) and a random-effects model. Statistical analysis was performed using RevMan 5.1.7.Compared with corticoids, ACTH was associated with a significant increase in weight (RR: 1.41; 95% CI: 1.01-1.97; p = 0.04). There was no significant difference in cessation of spasms on day 14 (OR: 0.91; 95% CI: 0.47-1.47; p = 0.79), hypsarrhythmia (OR: 0.97; 95% CI: 0.22-4.34), irritability (RR: 0.78; 95% CI: 0.30-1.99), hypertension (RR: 0.64; 95% CI: 0.35-1.15; p = 0.14), and infection (RR: 0.69; 95% CI: 0.19-2.50; p = 0.57).This study provides robust evidence regarding the safety and efficacy of ACTH or corticoids in children with WS. However, the significant heterogeneity between studies restricts the analysis, emphasizing the need for additional research to assess the best WS treatment option.
虽然已知最常用的治疗西氏综合征(WS)的方法是肌内促肾上腺皮质激素(ACTH)和口服强的松龙,但文献中对其效果的等效性仍存在争议。我们的目的是发表一篇最新的综述,比较ACTH治疗与皮质类固醇治疗对儿童West综合征(WS)的治疗和不良反应。检索PubMed、EMBASE和Cochrane Central数据库。研究结果包括治疗第14天痉挛停止、低心律失常停止以及体重增加、感染、易怒和高血压等不良反应的停止。根据系统评价和荟萃分析(PRISMA)指南的首选报告项目对研究进行审查,并进行荟萃分析。我们使用风险比(RR)和优势比(OR)对结果进行比较。对于二元结果,采用95%置信区间(CI)和随机效应模型。采用RevMan 5.1.7软件进行统计分析。与皮质激素相比,ACTH与体重显著增加相关(RR: 1.41; 95% CI: 1.01-1.97; p = 0.04)。在第14天痉挛停止(OR: 0.91; 95% CI: 0.47-1.47; p = 0.79)、心律失常(OR: 0.97; 95% CI: 0.22-4.34)、烦躁(RR: 0.78; 95% CI: 0.30-1.99)、高血压(RR: 0.64; 95% CI: 0.35-1.15; p = 0.14)和感染(RR: 0.69; 95% CI: 0.19-2.50; p = 0.57)方面,两组间无显著差异。本研究为WS患儿ACTH或皮质激素的安全性和有效性提供了强有力的证据。然而,研究之间的显著异质性限制了分析,强调需要进一步的研究来评估WS的最佳治疗方案。
{"title":"Corticotherapy versus adrenocorticotropic hormone for treating West syndrome: a systematic review and meta-analysis.","authors":"Elizabeth Honorato de Farias, Anderson Matheus Pereira da Silva, Karlos Daniell Araújo Dos Santos, Altair Pereira de Melo Neto, Aishwarya Koppanatham, Júlia Cappi Aguiar Moraes Souza, Niels Pacheco-Barrios, Gabrielle de Souza Rocha, Gustavo Sousa Noleto","doi":"10.1055/s-0045-1811173","DOIUrl":"10.1055/s-0045-1811173","url":null,"abstract":"<p><p>Although it is known that the most commonly used therapies for West syndrome (WS) are intramuscular adrenocorticotropic hormone (ACTH) and oral prednisolone, there is still controversy in the literature regarding the equivalence of their effects.We aimed to present an updated review comparing the therapeutic and adverse effects of ACTH therapy versus corticosteroids in children with West syndrome (WS).The PubMed, EMBASE, and Cochrane Central databases were searched. The outcomes of interest selected were spasm cessation on day 14 of therapy, cessation of hypsarrhythmia and adverse effects such as weight gain, infection, irritability, and hypertension. Studies were reviewed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and a meta-analysis was performed. We compared the results using the risk ratio (RR) and odds ratio (OR). for the binary outcomes, with 95% confidence intervals (CI) and a random-effects model. Statistical analysis was performed using RevMan 5.1.7.Compared with corticoids, ACTH was associated with a significant increase in weight (RR: 1.41; 95% CI: 1.01-1.97; <i>p</i> = 0.04). There was no significant difference in cessation of spasms on day 14 (OR: 0.91; 95% CI: 0.47-1.47; <i>p</i> = 0.79), hypsarrhythmia (OR: 0.97; 95% CI: 0.22-4.34), irritability (RR: 0.78; 95% CI: 0.30-1.99), hypertension (RR: 0.64; 95% CI: 0.35-1.15; <i>p</i> = 0.14), and infection (RR: 0.69; 95% CI: 0.19-2.50; <i>p</i> = 0.57).This study provides robust evidence regarding the safety and efficacy of ACTH or corticoids in children with WS. However, the significant heterogeneity between studies restricts the analysis, emphasizing the need for additional research to assess the best WS treatment option.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-8"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12399325/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-01Epub Date: 2025-08-25DOI: 10.1055/s-0045-1810624
Francisco de Assis Aquino Gondim
{"title":"On the natural history of Charcot-Marie-Tooth disease in children and adolescents.","authors":"Francisco de Assis Aquino Gondim","doi":"10.1055/s-0045-1810624","DOIUrl":"10.1055/s-0045-1810624","url":null,"abstract":"","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-2"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12377939/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144940645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-08-01Epub Date: 2025-09-08DOI: 10.1055/s-0045-1811235
Lécio Figueira Pinto, Guilherme Simone Mendonça, Carlos Alberto Mantovani Guerreiro
Clobazam (CLB) has been an established treatment for epilepsy since the 1970s, with a broad spectrum. It is frequently used as add on therapy for refractory patients. Furthermore, it is different from classic benzodiazepines (BZD) for containing nitrogen atoms in 1 and 5 positions of B ring (other are 1.4-BZD). This explains why CLB has a better tolerability and a lower chance of causing sedation, being an excellent option for epilepsy treatment compared with other BZDs. Evidence argues against the development of CLB tolerance in most patients. Antiseizure medication shortages have been reported by many countries, including the one studied here. Shortages make treatment harder, increase the need for extra clinical appointments, for orientation and medication changes, increase medication errors, decrease adherence, and cause insecurity. A literature review showed scarce evidence of alternatives, with a wide variation in dosage equivalence. A survey of specialists revealed that switch appropriateness was deemed inadequate by the majority, due to risk of seizure worsening and side effects. Clonazepam and nitrazepam were the most used BZDs, but there was great variation for clonazepam dosages (0.25-2 mg, commonly 1 per 10 mg of CLB). Better consensus was obtained for nitrazepam (5 per 10 mg of CLB). Gradual tapering of CLB, with concomitant increase of clonazepam or nitrazepam under close supervision, is advised. It is important to assess tolerability and the need for increased dosage. As CLB is an essential tool in the epilepsy armamentarium, shortages pose great risk to the patients. Governments and society must create mechanisms to prevent shortages of critical and unique medications.
{"title":"Which are the options and dosages for clobazam shortages on epilepsy treatment? Review of literature and survey of specialists.","authors":"Lécio Figueira Pinto, Guilherme Simone Mendonça, Carlos Alberto Mantovani Guerreiro","doi":"10.1055/s-0045-1811235","DOIUrl":"10.1055/s-0045-1811235","url":null,"abstract":"<p><p>Clobazam (CLB) has been an established treatment for epilepsy since the 1970s, with a broad spectrum. It is frequently used as add on therapy for refractory patients. Furthermore, it is different from classic benzodiazepines (BZD) for containing nitrogen atoms in 1 and 5 positions of B ring (other are 1.4-BZD). This explains why CLB has a better tolerability and a lower chance of causing sedation, being an excellent option for epilepsy treatment compared with other BZDs. Evidence argues against the development of CLB tolerance in most patients. Antiseizure medication shortages have been reported by many countries, including the one studied here. Shortages make treatment harder, increase the need for extra clinical appointments, for orientation and medication changes, increase medication errors, decrease adherence, and cause insecurity. A literature review showed scarce evidence of alternatives, with a wide variation in dosage equivalence. A survey of specialists revealed that switch appropriateness was deemed inadequate by the majority, due to risk of seizure worsening and side effects. Clonazepam and nitrazepam were the most used BZDs, but there was great variation for clonazepam dosages (0.25-2 mg, commonly 1 per 10 mg of CLB). Better consensus was obtained for nitrazepam (5 per 10 mg of CLB). Gradual tapering of CLB, with concomitant increase of clonazepam or nitrazepam under close supervision, is advised. It is important to assess tolerability and the need for increased dosage. As CLB is an essential tool in the epilepsy armamentarium, shortages pose great risk to the patients. Governments and society must create mechanisms to prevent shortages of critical and unique medications.</p>","PeriodicalId":8694,"journal":{"name":"Arquivos de neuro-psiquiatria","volume":"83 8","pages":"1-6"},"PeriodicalIF":1.6,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12417036/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145022780","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}