Investıgatıon of the relatıon between oxıdatıve stress and carnıtıne levels ın epıleptıc chıldren treated wıth valproıc acıd or carbamazepıne

Valproik asit ve karbamazepin değişik epilepsi tiplerinin tedavisinde yaygın olarak kullanılan antiepileptik ilaçlardır. Valproik asit veya karbamazepin kullanan bazı çocuk hastalarda oksidatif stres geliştiği ve antioksidan düzeylerinin azaldığı gösterilmiştir. Oksidatif stres; reaktif oksijen ürünlerinin aşırı birikmesi ya da antioksidanların yetersizliğine bağlı gelişir. Serbest oksijen radikalleri hücresel yapı ve fonksiyonlarının harabiyetine ve sonuçta hücre ölümüne yol açar. Artmış oksidatif stres ilaç yan etkilerinin oluşumuna katkıda bulunabilir. Valproik asit veya karbamazepin kullanımı sonucu oksidatif stresdeki artışın mekanizması henüz net anlaşılamamıştır. Özellikle Valproik asit de daha sık olmak üzere her iki ilacı kullanan hastalarda kan karnitin düzeyinde azalma saptanmıştır. L-karnitinin henüz mekanizması bilinmeyen antioksidan ve antiperoksidatif etkileri tanımlanmıştır. Bu çalışmada, valproik asit veya karbamazepin kullanan epilepsili çocuk hastalarda oksidatif stres gelişiminde kan karnitin düzeyindeki azalmanın rolünü ayrıca obezite ile oksidatif stres artışı arasında ilişkinin araştırılmasını amaçladık. Çalışmaya VPA kullanan 45 hasta, karbamazepin kullanan 25 hasta ve 30 sağlıklı çocuk olmak üzere 100 çocuk alındı. Serbest karnitin, TAS, tiyol, TOS ve OSI düzeyleri değerlendirildi. VPA kullanan hastaların tiyol düzeyi karbamazepin kullananlara ve kontrol grubuna göre daha düşük bulundu. Serbest karnitin düzeyi ile tiyol arasında ve ağırlık artışı ile tiyol arasında herhangi bir korelasyon saptanmadı.Valproic acid and carbamazepine are antiepileptic drugs that are widely used to treat different types of epilepsy. Development of oxidative stress and decreased antioxidant levels have been reported in some patients receiving valproic acid or carbamazepine. Excess of reactive oxygen species production and/or deficiency of antioxidants result in oxidative stress. Free oxygen radicals disrupt cellular structure and functions, ultimately leading to cell death. Increased oxidative stress may contribute to the pathogenesis of side effects of these drugs. The exact mechanism of increased oxidative stress due to valproic acid or carbamazepine has not been resolved yet. Both drugs, in particular valproic acid are associated with decreased blood carnitine levels. The antioxidant and antiperoxidative effects of L-carnitine have been described, but the specific mechanism is still unclear In this study, we aimed to investigate the role of decreased carnitine levels in the pathogenesis of oxidative stress in epileptic children treated with valproic acid or carbamazepine. We also aimed to test whether weight gain could be related to increased oxidative stress in these patients. Forty- five patients treated with valproic acid, 25 patients treated with carbamazepine and 30 healthy children were included in this study. Free carnitine, TAS, thiol, TOS and OSI levels were measured and analyzed. We found significiantly decreased thiol level in VPA group compared to CBZ and control groups. There was no correlation between free carnitine and thiol levels or weight gain and thiol level either

Subacute sclerosing panencephalitis in a child with recurrent febrile seizures.

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis

Re-examining the characteristics of pediatric multiple sclerosis in the era of antibody-associated demyelinating syndromes.

Background: The discovery of anti-myelin oligodendrocyte glycoprotein (MOG)-IgG and anti-aquaporin 4 (AQP4)-IgG and the observation on certain patients previously diagnosed with multiple sclerosis (MS) actually have an antibody-mediated disease mandated re-evaluation of pediatric MS series. Aim: To describe the characteristics of recent pediatric MS cases by age groups and compare with the cohort established before 2015. Method: Data of pediatric MS patients diagnosed between 2015 and 2021 were collected from 44 pediatric neurology centers across Turkiye. Clinical and paraclinical features were compared between patients with dis-ease onset before 12 years (earlier onset) and >= 12 years (later onset) as well as between our current (2015-2021) and previous (< 2015) cohorts. Results: A total of 634 children (456 girls) were enrolled, 89 (14%) were of earlier onset. The earlier-onset group had lower female/male ratio, more frequent initial diagnosis of acute disseminated encephalomyelitis (ADEM), more frequent brainstem symptoms, longer interval between the first two attacks, less frequent spinal cord involvement on magnetic resonance imaging (MRI), and lower prevalence of cerebrospinal fluid (CSF)-restricted oligoclonal bands (OCBs). The earlier-onset group was less likely to respond to initial disease-modifying treatments. Compared to our previous cohort, the current series had fewer patients with onset < 12 years, initial presentation with ADEM-like features, brainstem or cerebellar symptoms, seizures, and spinal lesions on MRI. The female/male ratio, the frequency of sensorial symptoms, and CSF-restricted OCBs were higher than reported in our previous cohort. Conclusion: Pediatric MS starting before 12 years was less common than reported previously, likely due to exclusion of patients with antibody-mediated diseases. The results underline the importance of antibody testing and indicate pediatric MS may be a more homogeneous disorder and more similar to adult-onset MS than previously thought