Ketogenic therapy in childhood and adolescence: recommendations of the Brazilian experts group.

Ketogenic dietary therapies (KDTs) are a safe and effective treatment for pharmacoresistant epilepsy in children. There are four principal types of KDTs: the classic KD, the modified Atkins diet (MAD), the medium-chain triglyceride (MCT) diet, and the low glycemic index diet (LGID). The International Ketogenic Diet Study Group recommends managing KDTs in children with epilepsy. However, there are no guidelines that address the specific needs of the Brazilian population. Thus, the Brazilian Child Neurology Association elaborated on these recommendations with the goal of stimulating and expanding the use of the KD in Brazil.

As terapias dietéticas cetogênicas (TDC) são um tratamento seguro e eficaz para epilepsia farmacorresistente em crianças. Existem quatro tipos principais de TDCs: a dieta cetogênica (DC) clássica, a dieta de Atkins modificada (DAM), a dieta de triglicerídeos de cadeia média (DTCM) e a dieta de baixo índice glicêmico (DBIG). O Grupo Internacional de Estudos de Dietas Cetogênicas (International Ketogenic Diet Study Group) propõe recomendações para o manejo da DC em crianças com epilepsia. No entanto, faltam diretrizes que contemplem as necessidades específicas da população brasileira. Assim, a Associação Brasileira de Neurologia Infantil elaborou essas recomendações com o objetivo de estimular e expandir o uso da DC no Brasil.

Ketogenic therapy in childhood and adolescence: recommendations of the Brazilian experts group / Terapia cetogênica na infância e adolescência: recomendações do grupo de especialistas brasileiros

Abstract Ketogenic dietary therapies (KDTs) are a safe and effective treatment for pharmacoresistant epilepsy in children. There are four principal types of KDTs: the classic KD, the modified Atkins diet (MAD), the medium-chain triglyceride (MCT) diet, and the low glycemic index diet (LGID). The International Ketogenic Diet Study Group recommends managing KDTs in children with epilepsy. However, there are no guidelines that address the specific needs of the Brazilian population. Thus, the Brazilian Child Neurology Association elaborated on these recommendations with the goal of stimulating and expanding the use of the KD in Brazil.

Resumo As terapias dietéticas cetogênicas (TDC) são um tratamento seguro e eficaz para epilepsia farmacorresistente em crianças. Existem quatro tipos principais de TDCs: a dieta cetogênica (DC) clássica, a dieta de Atkins modificada (DAM), a dieta de triglicerídeos de cadeia média (DTCM) e a dieta de baixo índice glicêmico (DBIG). O Grupo Internacional de Estudos de Dietas Cetogênicas (International Ketogenic Diet Study Group) propõe recomendações para o manejo da DC em crianças com epilepsia. No entanto, faltam diretrizes que contemplem as necessidades específicas da população brasileira. Assim, a Associação Brasileira de Neurologia Infantil elaborou essas recomendações com o objetivo de estimular e expandir o uso da DC no Brasil.

Guillain-Barré syndrome after coronavirus disease 2019 vaccine: A temporal association.

Background: Guillain-Barré syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy, preceded by gastrointestinal or respiratory infections in up to two-thirds of patients. On rare occasions, people develop GBS after vaccination, but no causal association has been proven. In the current coronavirus disease 2019 (COVID-19) pandemic, some cases have been reported associating COVID-19 vaccine with GBS. Case presentation: We report a case of a 62-year-old woman with GBS after the first dose of the Oxford/AstraZeneca vaccine against SARS-CoV-2. The symptoms started 3 weeks after the vaccine, and were characterized by ascending and progressive paresthesia in the upper and lower limbs, followed by loss of strength of the upper limbs and dysphagia for solids. The hypothesis of GBS was confirmed by clinical presentation compatible with albuminocytologic dissociation in cerebrospinal fluid and based on the Brighton criteria level 2. The treatment was a 5-day course of intravenous immunoglobulin with an improvement of symptoms. Conclusions: In the absence of other causes, the diagnosis of GBS was made, with evidence of a clear temporal association with COVID-19 vaccine. However, a cautious position is important when assigning a particular side-effect directly to a vaccine. It is important to emphasize that it is a temporal association only and the benefits of COVID-19 vaccination continue to outweigh the possible consequences.

Postictal Psychosis in Epilepsy: A Clinicogenetic Study.

OBJECTIVE: Psychoses affecting people with epilepsy increase disease burden and diminish quality of life. We characterized postictal psychosis, which comprises about one quarter of epilepsy-related psychoses, and has unknown causation. METHODS: We conducted a case-control cohort study including patients diagnosed with postictal psychosis, confirmed by psychiatric assessment, with available data regarding epilepsy, treatment, psychiatric history, psychosis profile, and outcomes. After screening 3,288 epilepsy patients, we identified 83 with psychosis; 49 had postictal psychosis. Controls were 98 adults, matched by age and epilepsy type, with no history of psychosis. Logistic regression was used to investigate clinical factors associated with postictal psychosis; univariate associations with a p value < 0.20 were used to build a multivariate model. Polygenic risk scores for schizophrenia were calculated. RESULTS: Cases were more likely to have seizure clustering (odds ratio [OR] = 7.59, p < 0.001), seizures with a recollected aura (OR = 2.49, p = 0.013), and a family history of psychiatric disease (OR = 5.17, p = 0.022). Cases showed predominance of right temporal epileptiform discharges (OR = 4.87, p = 0.007). There was no difference in epilepsy duration, neuroimaging findings, or antiseizure treatment between cases and controls. Polygenic risk scores for schizophrenia in an extended cohort of postictal psychosis cases (n = 58) were significantly higher than in 1,366 epilepsy controls (R2  = 3%, p = 6 × 10-3 ), but not significantly different from 945 independent patients with schizophrenia (R2  = 0.1%, p = 0.775). INTERPRETATION: Postictal psychosis occurs under particular circumstances in people with epilepsy with a heightened genetic predisposition to schizophrenia, illustrating how disease biology (seizures) and trait susceptibility (schizophrenia) may interact to produce particular outcomes (postictal psychosis) in a common disease. ANN NEUROL 2021;90:464-476.

Cutaneous adverse reactions associated with antiseizure medication: clinical characteristics and implications in epilepsy treatment.

OBJECTIVE: To describe the clinical characteristics of cutaneous adverse reactions and cross-sensitivity induced by antiseizure medications and compare the pattern of use of antiseizure medications in patients with epilepsy according to skin rash history. METHODS: We analysed patients with a history of skin rash presenting for up to 12 weeks after initiating antiseizure medication. The history of skin rash was verified by medical charts, interviews, and identification of skin lesions by patients based on illustrative images. The minimum follow-up period was eight months. The control group comprised epilepsy patients with regular antiseizure medication use for at least 12 weeks without skin rash. We included 109 cases and 99 controls. RESULTS: The median (interquartile range) period from the index rash was six years (2-11). Carbamazepine was the trigger medication in 48% of cases and induced skin rashes in all patients with cross-sensitivity and carbamazepine exposure. Stevens-Johnson syndrome, toxic epidermal necrolysis, or drug reactions with eosinophilia and systemic symptoms affected 36% of cases. Carbamazepine- or oxcarbazepine-induced maculopapular exanthema occurred earlier (median: one week) than that induced by other antiseizure medications (median: three weeks) (p=0.006). Cross-sensitivity was more common in patients with at least one episode of Stevens-Johnson syndrome (29%) and Stevens-Johnson/toxic epidermal necrolysis overlap (50%) than in patients with maculopapular exanthema (8%) (p=0.01). Although most cases were mild, the pattern of antiseizure medication use differed from that of controls, with a lower proportion of antiseizure medication typically associated with severe cutaneous adverse reactions (carbamazepine, phenytoin, phenobarbital, primidone, oxcarbazepine, and lamotrigine) (p<0.001). Most cases exposed to high-risk medication, however, did not develop cross-sensitivity. SIGNIFICANCE: Cutaneous adverse reaction history may influence antiseizure medication use. Cross-sensitivity is more common in severe cases and most patients are affected by mild, self-limited skin rashes. Further research should consider the relevance of mild skin rashes in lifelong epilepsy treatment.

Severity and disability related to epilepsy from the perspective of patients and physicians: A cross-cultural adaptation of the GASE and GAD scales.

PURPOSE: To perform a cross-cultural adaptation of the Global Assessment of Severity of Epilepsy (GASE) and Global Assessment of Disability (GAD) scales to Brazilian Portuguese and compare patients' self-rated scores with their attending physicians' ratings. METHODS: We conducted an observational, multicentre, cross-sectional study. Patients followed up in five epilepsy centres in Brazil responded to GASE and GAD questionnaires and to the Hospital Anxiety and Depression Scale and the Adverse Events Profile, both previously validated in Brazil. GASE and GAD scales were also completed by 20 attending physicians providing care to these patients. RESULTS: A total of 138 patients were interviewed, with a mean age of 39.9 ± 13.81 years and a median of 11 (interquartile range, IQR = 7.5-12) years of education. Eighty-five (61.6%) patients were female. Most patients were diagnosed with focal epilepsy (82.6%). Only 5.8% and 3.6% of respondents reported having difficulty understanding the GASE and GAD, respectively. The patients scored a median of 3 (IQR = 2-5) on the self-perceived GASE and 4 (IQR = 2-6) on the GAD. Physician ratings were moderately to highly correlated with patients' self-perceived scores on the GASE and GAD. Linear regression analysis demonstrated that physicians' GASE and GAD scores predicted 37% and 20% of the patients' self-reported GASE and GAD variation, respectively. CONCLUSION: Brazilian Portuguese cross-cultural adaptation of the GASE and GAD was successful. These scales were found to be easy to use by patients and health professionals, and revealed the burden of epilepsy on patients' lives.

Guidelines for the assessment and acceptance of potential brain-dead organ donors. / Diretrizes para avaliação e validação do potencial doador de órgãos em morte encefálica.

Organ transplantation is the only alternative for many patients with terminal diseases. The increasing disproportion between the high demand for organ transplants and the low rate of transplants actually performed is worrisome. Some of the causes of this disproportion are errors in the identification of potential organ donors and in the determination of contraindications by the attending staff. Therefore, the aim of the present document is to provide guidelines for intensive care multi-professional staffs for the recognition, assessment and acceptance of potential organ donors.

Diretrizes para avaliação e validação do potencial doador de órgãos em morte encefálica / Guidelines for the assessment and acceptance of potential brain-dead organ donors

RESUMO O transplante de órgãos é a única alternativa para muitos pacientes portadores de algumas doenças terminais. Ao mesmo tempo, é preocupante a crescente desproporção entre a alta demanda por transplantes de órgãos e o baixo índice de transplantes efetivados. Dentre as diferentes causas que alimentam essa desproporção, estão os equívocos na identificação do potencial doador de órgãos e as contraindicações mal atribuídas pela equipe assistente. Assim, o presente documento pretende fornecer subsídios à equipe multiprofissional da terapia intensiva para o reconhecimento, a avaliação e a validação do potencial doador de órgãos.

ABSTRACT Organ transplantation is the only alternative for many patients with terminal diseases. The increasing disproportion between the high demand for organ transplants and the low rate of transplants actually performed is worrisome. Some of the causes of this disproportion are errors in the identification of potential organ donors and in the determination of contraindications by the attending staff. Therefore, the aim of the present document is to provide guidelines for intensive care multi-professional staffs for the recognition, assessment and acceptance of potential organ donors.

Parakinesia brachialis oscitans during thrombolytic therapy.

The authors describe a case report of a patient with parakinesia brachialis oscitans, which occurred during thrombolytic therapy, in a patient with left-middle cerebral artery occlusion.

Dopamine transporter imaging in clinically unclear cases of parkinsonism and the importance of scans without evidence of dopaminergic deficit (SWEDDs).

UNLABELLED: The clinical diagnosis of Parkinson's disease (PD) is susceptible to misdiagnosis, especially in the earlier stages of the disease. Recently, in vivo imaging techniques assessing the presynaptic dopamine transporter (DAT) have emerged as a useful tool in PD diagnosis, improving its accuracy. OBJECTIVE: It was to illustrate the clinical usefulness of a brain single-photon emission computed tomography (SPECT) DAT ligand, and highlight relevant aspects of scans without evidence of dopaminergic deficit (SWEDDs) in this context. CASES: We described four representative patients with clinically unclear parkinsonian syndromes who underwent [(99m)Tc]-TRODAT-1 SPECT and reviewed the clinical implications. CONCLUSION: DAT-SPECT is an important, cost-effective, technique for the differential diagnosis of parkinsonian syndromes. Additionally, SWEDD cases present clinical and paraclinical peculiarities that may retrospectively identify them as essential/dystonic tremor. The lack of histopathological data limits further conclusions.

Dopamine transporter imaging in clinically unclear cases of parkinsonism and the importance of Scans Without Evidence of Dopaminergic Deficit (SWEDDs) / Neuroimagem do transportador de dopamina em casos de parkinsonismo clinicamente indefinidos e a importância dos SWEDDs (exames sem evidência de déficit dopaminérgico)

The clinical diagnosis of Parkinson's disease (PD) is susceptible to misdiagnosis, especially in the earlier stages of the disease. Recently, in vivo imaging techniques assessing the presynaptic dopamine transporter (DAT) have emerged as a useful tool in PD diagnosis, improving its accuracy. OBJECTIVE: It was to illustrate the clinical usefulness of a brain single-photon emission computed tomography (SPECT) DAT ligand, and highlight relevant aspects of scans without evidence of dopaminergic deficit (SWEDDs) in this context. CASES: We described four representative patients with clinically unclear parkinsonian syndromes who underwent [99mTc]-TRODAT-1 SPECT and reviewed the clinical implications. CONCLUSION: DAT-SPECT is an important, cost-effective, technique for the differential diagnosis of parkinsonian syndromes. Additionally, SWEDD cases present clinical and paraclinical peculiarities that may retrospectively identify them as essential/dystonic tremor. The lack of histopathological data limits further conclusions.

O diagnóstico clínico da doença de Parkinson (DP) pode estar sujeito a erros especialmente nas fases iniciais da doença. Recentemente, técnicas de imagem in vivo que avaliam o transportador de dopamina pré-sináptico (DAT) surgiram como uma ferramenta empregada no diagnóstico da DP, melhorando sua acurácia. OBJETIVO: Foi demonstrar a utilização clínica de um traçador de tomografia computadorizada por emissão de fóton único (SPECT) e destacar importantes aspectos dos casos de SWEDDs (exames sem evidência de déficit dopaminérgico) neste contexto. CASOS: Descrevemos quatro pacientes representativos de síndromes parkinsonianas clinicamente incertas submetidos a [99mTc]-TRODAT-1 SPECT e revisamos suas implicações. CONCLUSÃO: O DAT-SPECT é uma técnica importante e eficiente para o diagnóstico diferencial das síndromes parkinsonianas. Além disso, os casos de SWEDDs apresentam peculiaridades clínicas e paraclínicas que podem retrospectivamente identificá-los como tremor essencial ou tremor distônico. As conclusões têm alcance limitado pela falta de dados histopatológicos.

Neurological diagnoses in the emergency room: differences between younger and older patients.

UNLABELLED: Neurological diseases are prevalent in the emergency room (ER). The aim of this study was to compare the neurological diagnoses between younger and older patients evaluated in the ER of a tertiary care hospital. METHOD: Patients admitted to the ER who required neurological evaluation in the first 24 hours were separated into two groups based on age, ≤50 years old and >50 years old. RESULTS: Cerebrovascular disease (59.6% vs. 21.8%, p<0.01) was most frequent in the >50 years old group. Seizures (8.1% vs. 18.6%, p<0.01) and primary headache (3.7% vs. 11.4%, p<0.01) were most frequent in the ≤50 years old group. CONCLUSION: The current study demonstrated that these three neurological diagnoses represented the majority of the neurological evaluations in the ER. National guidelines for ER teams that treat these prevalent disorders must be included in clinical practice and training.

Neurological diagnoses in the emergency room: differences between younger and older patients / Diagnósticos neurológicos na sala de emergência: diferenças entre pacientes jovens e idosos

Neurological diseases are prevalent in the emergency room (ER). The aim of this study was to compare the neurological diagnoses between younger and older patients evaluated in the ER of a tertiary care hospital. METHOD: Patients admitted to the ER who required neurological evaluation in the first 24 hours were separated into two groups based on age, <;50 years old and >50 years old. RESULTS: Cerebrovascular disease (59.6 percent vs. 21.8 percent, p<0.01) was most frequent in the >50 years old group. Seizures (8.1 percent vs. 18.6 percent, p<0.01) and primary headache (3.7 percent vs. 11.4 percent, p<0.01) were most frequent in the <;50 years old group. CONCLUSION: The current study demonstrated that these three neurological diagnoses represented the majority of the neurological evaluations in the ER. National guidelines for ER teams that treat these prevalent disorders must be included in clinical practice and training.

Doenças neurológicas são prevalentes na sala de emergência (SE). O objetivo deste estudo é comparar a ocorrência de diagnósticos neurológicos entre pacientes jovens e idosos atendidos na SE de um hospital terciário. MÉTODO: Pacientes admitidos na SE que necessitaram avaliação neurológica nas primeiras 24 horas após a admissão foram separados em dois grupos baseados na idade, <;50 anos de idade e >50 anos de idade. RESULTADOS: Doença cerebrovascular foi o diagnóstico mais comum nos pacientes >50 anos (59,6 por cento vs. 21,8 por cento, p<0,01). Convulsões (8,1 por cento vs. 18,6 por cento, p<0,01) e cefaléias primárias (3,7 por cento vs. 114 por cento, p<0,01) foram mais frequentes no grupo <;50 anos. CONCLUSÃO: O presente estudo demonstrou que esses três diagnósticos neurológicos representam a maioria das avaliações neurológicas na SE. Diretrizes nacionais para os profissionais emergencistas que tratam estas doenças devem ser incluídos na prática clínica e no treinamento médico.