Publications by authors named "Adelia Henriques Souza"

Background:  Epilepsies are among the most prevalent chronic neurological diseases, usually beginning in childhood. About 30% of children with epilepsies develop seizures that are difficult to control with medication. Recurrent epileptic seizures hinder diet intake, impairing the nutritional status.

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Background: Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent.

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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.

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Article Synopsis
  • - Neuronal ceroid lipofuscinosis type 2 (CLN2) is a serious genetic disorder in children, rapidly progressing and often leading to death by age 10, prompting a need for earlier diagnosis due to available treatment options.
  • - A group of Brazilian child neurologists developed a consensus on managing CLN2, focusing on identifying symptoms like language delay and epilepsy in young children, while acknowledging limited access to necessary diagnostic tests.
  • - Cerliponase α, an enzyme replacement therapy approved in Brazil since 2018, offers hope by slowing disease progression and improving patients' quality of life, highlighting the importance of early diagnosis and comprehensive management.
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Background: Zika virus (ZIKV) is a recently emerged arbovirus, which infection during pregnancy is associated with a series of congenital malformations, collectively denominated Congenital Zika Syndrome (CZS). Following infection, ZIKV RNA has a median duration period of 10 days in plasma and up to 6 months in semen in immunocompetent adult individuals. Moreover, ZIKV is able to replicate and persist in fetal brains and placentas, consequently, infection is associated with pregnancy loss, albeit the pathogenic mechanisms are still unknown.

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Article Synopsis
  • Zika virus (ZIKV) was first identified in Brazil in 2015, leading to a rise in cases of Guillain-Barre Syndrome (GBS), prompting a study of six neurological cases linked to ZIKV in Pernambuco, Brazil.
  • All six cases had ZIKV detected in their serum or cerebrospinal fluid, categorized as four GBS cases, one acute disseminated encephalomyelitis (ADEM), and one encephalitis.
  • The study emphasizes the importance of considering ZIKV in neurological diagnoses in affected regions, suggesting that diagnostic tests like RT-PCR and viral isolation should be used even after the initial phase of the virus due to prolonged viremia in patients.
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Epilepsy is a potentially devastating brain disorder characterized by a predisposition to spontaneous epileptic seizures. In patients with medically refractory epilepsy, new non-pharmacological therapeutic approaches may be considered. In this scenario, palliative surgery such as vagus nerve stimulation (VNS) or deep brain stimulation (DBS) may be indicated in a subset of patients.

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The use of cannabidiol in some neurological conditions was allowed by Conselho Regional de Medicina de São Paulo and by Agência Nacional de Vigilância Sanitária (ANVISA). Specialists on behalf of Academia Brasileira de Neurologia prepared a critical statement about use of cannabidiol and other cannabis derivatives in neurological diseases.

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  • The study investigates early onset multiple sclerosis (MS) in Brazilian patients diagnosed before age 18, highlighting the challenges in diagnosis and management.
  • Data was collected retrospectively from 20 MS units across 11 states, resulting in 117 cases, with a mean onset age of 13.7 years and an average disease duration of 10 years.
  • Overall, while some cases were severe, most patients with pediatric MS in Brazil exhibited relatively mild symptoms, with low disability levels and an average relapse rate of one episode every 2.5 years.
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We describe magnetic resonance image findings of 26 children (16 boys; average age, 9.4 years) with schistosomal myelopathy. All children lived in Pernambuco State, Brazil, an area of endemic mansoni schistosomiasis.

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We evaluated the efficacy of vigabatrin (VGB) as a first drug to be used as monotherapy for West syndrome (WS), its side effects and correlations with the electroencephalogram (EEG). The sample consisted of 13 infants examined between October 2001 and September 2002 at IMIP ambulatory patients office or private clinic. Administration of vigabatrin was around 118 mg/kg/day.

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OBJECTIVE: To observe the wide variety of reported prognosis after a first unprovoked convulsion and of risk factors that are associated with recurrence, and a uniform conduct. SOURCES: Systematic review of Bireme. SUMMARY OF THE FINDINGS: Recurrence rates differ from a first seizure study because of different inclusion criteria.

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