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Extrapyramidal symptoms (EPS) that include akathisia, dystonia, pseudoparkinsonism, and dyskinesia are abnormal movements commonly induced by antipsychotic medications. These symptoms are also associated with specific non-antipsychotic agents. This case report describes a case of a 9-year-old boy on antibiotics treatment that developed EPS. A 9-year-old boy presented to the emergency department of Imam Hossein Children›s Hospital with chief complaints of trismus, difficulty speaking, and tongue protrusion. One week before these presentations, he had been prescribed Tavanex® (levofloxacin) and clindamycin. His symptoms improved after the withdrawal of antibiotics and administering Biperiden, and he was discharged in good condition. On a follow-up visit one week after discharge, no remaining symptoms were present, and he was in good condition. Based on the questions in the Naranjo criteria, levofloxacin receives a score of 7 and is a probable cause of adverse drug reaction (ADR). Clindamycin, with a score of 6, is also a probable cause for this adverse drug reaction, but clinical judgment was in favor of levofloxacin as the culprit. Clinicians should be aware of the potential EPS of levofloxacin at standard doses. Effective management of adverse events is necessary to ensure patient safety and optimal outcomes. [ABSTRACT FROM AUTHOR]

Byline: Reza. Azizkhani, Farhad. Heydari, Mohammadreza. Ghazavi, Maryam. Riahinezhad, Mohammadreza. Habibzadeh, Ali. Bigdeli, Keihan. Golshani, Saeid. Majidinejad, Abolfazl. Mohammadbeigi Background: Pediatric anxiety and restlessness may create issues and difficulties in [...]

Background: Pediatric-onset multiple sclerosis (POMS) is an autoimmune demyelinating disorder of the central nervous system (CNS), affecting individuals younger than 18 years of age. We sought to characterize the epidemiological and clinical features of patients with POMS in Isfahan, Iran, from April 1997 to March 2020. Methods: The medical records of patients with POMS in the databases of Isfahan Department of Public Health and Isfahan Multiple Sclerosis Society (IMSS) were retrospectively reviewed. The 2006 and 2016 Isfahan Province population censuses were used as reference values for assessing the temporal trend of POMS. Results: From April 1997 to March 2020, 509 individuals under18 years of age were diagnosed with POMS in Isfahan. 404 of these patients (79.4%) were girls, and 105 patients (20.6%) were boys (a female to male ratio of 3.85:1). Most of the patients (83%) were monosymptomatic at onset, with optic neuritis and brainstem-cerebellar disorders being the most frequent initial presentations. Mean ± standard deviation (SD) of age at disease diagnosis was 15.8 ± 2.5 years (ranging from 3 to 18, mode = 18). From April 2019 to March 2020, the crude prevalence and the crude incidence rate of the POMS were 5.42 per 100000 and 1.86 per 100000, respectively. Poisson regression analysis revealed a 3.4% increase in the incidence rate of POMS from April 1997 to March 2020 [relative rate:1.034, 95% confidence interval (CI): 1.021-1.048]. Conclusion: The female to male ratio in our cohort was significantly higher than any other studies conducted previously. The high female to male ratio and increasing incidence of the disease suggest increasing regionalization of care. [ABSTRACT FROM AUTHOR]

Objective Carnitine plays a significant role in fatty acid transportation in mitochondria and has been shown to have a prophylactic effect on adult migraine. The aim of this randomized controlled trial was to compare and evaluate the effects of L-carnitine supplementation versus propranolol in the prevention of pediatric migraine. Materials & Methods A total of 60 pediatric patients with episodic migraine were randomly allocated to 2 independent groups to receive either 50 mg/kg/day L-carnitine or 1 mg/kg/day propranolol as a prophylactic drug. Frequency, severity, and duration of migraine attacks and headache disability based on the Pediatric Migraine Disability Assessment Score (PedMIDAS) were studied at the baseline and after 2, 4, and 12 weeks. Results A total of 56 patients were evaluated in the study: 23 girls (41%) and 33 boys (59%) with a mean age of 9.7 ± 2.1 years. Frequency of migraine headaches per month reduced from 11.4 ± 7.1 to 5.34 ± 2.4 in the L-carnitine group and from 10.7 ± 6.2 to 4.96 ± 3.9 in the propranolol group by the end of the study. Headache severity score was also reduced from 19.38 ± 14 to 2.88 ± 7.4 and from 12.92 ± 13 to 0.82 ± 1.3 in the L-carnitine and propranolol groups, respectively. We found a significant decrease in frequency, severity, and duration of headache attacks in both groups (P < 0.01). No significant difference was observed between the efficacies of the 2 drugs. Conclusion This study concluded that L-carnitine supplementation can play a prophylactic role in the management of pediatric migraine. [ABSTRACT FROM AUTHOR]

Background: Stuttering is a kind of speech disorder that affects about 1% of total population. As the origin of this disorder is not obviously diagnosed yet, various remedies have been practiced and among them different medicines have been studied, but unfortunately no significant effective drugs have been recognized yet. As stuttering imposes a great social and mental costs to the patients and their families, finding an effective medicine will help significantly. In this study we have focused on the effects of levetiracetam (LEV) treatment on children suffering from stuttering. Methods: In this clinical trial study, 30 children aged > 3 years (median 3.8 years) with stuttering and abnormal sleep electroencephalogram (EEG) were treated by LEV and followed-up for a minimum period of 6 weeks. The starting dose of 20 mg/kg/day was increased at an interval of 1 week by 20 mg/kg/day, if necessary, up to maximum dose of 60 mg/kg/day. Results: Overall LEV was effective in 70% of patients, decreasing stuttering to at least 50%. Three children (10%) became stuttering-free and only in one (3.3%) child an increase in stuttering was observed. There were statistically significant differences for efficacy in the presence of variables such as age groups, seizure, stuttering family history, and EEG data. Conclusions: LEV is an effective drug for treatment of childhood stuttering in those that have abnormal sleep EEG. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Metabolic disorders, which involve many different organs, can be ascribed to enzyme deficiency or dysfunction and manifest with a wide range of clinical symptoms. This study evaluated some of the demographic and clinical findings in pediatric patients affected by organic acidemia. MATERIALS & METHODS: This cross-sectional study was part of a larger study conducted in patients with metabolic disorders during a period of 7 years from 2007 to 2014 in Isfahan Province, Iran. Our study covered a wide range of cases from newborn infants (one-week old) to adolescents (children up to the age of 17 years). This study evaluated patients' demographic information, history of disease, developmental and educational status, clinical and general conditions. Phone and in-person interviews were used to gather information. RESULTS: Out of 5100 patients screened in this study, 392 patients were affected by one of the different metabolic disorders and 167 individuals were diagnosed as organic acidemia. Propionic acidemia/methyl malonic acidemia (PA/MMA) was the most prevalent form of this metabolic disorder. The frequency of consanguinity was 84.7% in the group of patients. The mortality rate was 18.8% in patients with organic academia. CONCLUSION: Each of the metabolic diseases, as a separate entity, is rare; nevertheless, in aggregate they have a somewhat high overall prevalence. These diseases result in mental and developmental disorders in the absence of quick diagnosis and initiation of treatment. Furthermore, more mutations should be identified in societies affected by consanguinity. Further research should also be conducted to determine worthwhile and more-efficient screening methods as well as long term neurological prognosis. [ABSTRACT FROM AUTHOR]

Objective Developmental delay screening is essential in pediatric medicine. The purpose of this study was to estimate the developmental delay in apparently normal children at entry to kindergarten. Materials & Methods In this cross- sectional study conducted in 2013, the developmental status of a sample of children who entered to kindergarten at the age of 4-60 months were evaluated by the Persian version of ages and stages questionnaires (ASQ) in Isfahan county, central Iran. Results Totally 680 children were enrolled, 11.8% of them were suspected to delayed in at least one domain and 1.3% and 1.2% in two and three domains, respectively. Developmental delay was in the following items: 5% in problem solving; 4.9% in fine motor; 3.2% in gross motor, 2.2% and 1.2% in personal -- social and communication domains, respectively. Conclusion Considerable proportions of apparently normal children who are entering kindergarten had developmental delay, which could be detected by evaluation with appropriate screening tools. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to evaluate the effect of the ketogenic diet on the growth parameters of the children with resistant epilepsy. Materials & Methods A total of 36 children with resistant epilepsy who were 2 to 7 year old were put on the ketogenic diet. Their growth and biochemical parameters were studied at the beginning of the study and after 3 months. Results Weight decreased in all patients. Serum levels of hemoglobin, calcium, and blood sugar decreased significantly but remained in the normal range. Creatinine did not change, but BUN showed a significant increase. Conclusion We can lower the complications of ketogenic diets by using more unsaturated fat, more water, and more minerals. [ABSTRACT FROM AUTHOR]

Intellectual disability (ID) is a leading cause for referral to genetic services, with the most severe cases typically attributed to single genetic defects. This study aimed to evaluate the diagnostic yield of cost-effective proband-only exome sequencing for individuals diagnosed with ID within the Iranian population for the first time where a high rate of parental consanguinity exists. A total of 99 unrelated patients with ID were investigated by exome sequencing during 8 years. As a result, 43 pathogenic/likely pathogenic variants were identified in 40 patients, indicating a molecular diagnostic rate of 40.4% (40/99). The inclusion of five chromosomal copy number variations in the subsequent analysis increased the diagnostic rate of proband-only exome sequencing to 45.4% (45/99). Additionally, parental testing revealed five de novo variants. This contributed to a total diagnostic rate of 50.5% (50/99). In our study, proband-only exome sequencing achieved a remarkable diagnostic rate, identifying nearly half of the ID cases. This rate of diagnosis could be primarily attributed to prevalent consanguineous marriage in the Iranian population and the rare identification of de novo variants. With the ongoing advancements in neurogenetics, proband-only exome sequencing demonstrates significant potential as a future cost-effective diagnostic approach in Iran., (© 2024 Wiley Periodicals LLC.)

Objective: This study aimed to assess the efficacy and safety of topical timolol in treating facial angiofibromas (FAs) in pediatric patients with tuberous sclerosis complex (TSC)., Methods: A prospective clinical trial was conducted involving 15 children diagnosed with TSC and presenting with FAs. The participants were administered topical timolol gel 0.5% twice daily. Prior to the intervention, the severity of FAs in each patient was evaluated using the FA severity index (FASI), which assessed erythema, size, and extent of lesions. Clinical response was assessed at weeks 2 and 4 during the intervention period as well as 1 month after discontinuation of treatment., Findings: Four weeks after discontinuing topical timolol 0.5%, statistically significant reductions were observed in the mean FASI score, erythema, size, and extent of lesions ( P < 0.0001, P < 0.0001, P = 0.012, P = 0.008, respectively). FASI scores at 4 and 12 weeks postintervention, as well as 4 weeks after treatment cessation, demonstrated a significant decrease compared to baseline ( P < 0.001). Erythema and extension scores also exhibited a significant decrease 1 month after treatment cessation compared to baseline ( P < 0.05), while the mean size of lesions before and after the intervention did not show a statistically significant difference ( P = 0.004)., Conclusion: Topical timolol 0.5% represents a cost-effective and readily available treatment option for pediatric patients with FAs associated with tuberous sclerosis., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Journal of Research in Pharmacy Practice.)

In the field of rare diseases, progress in molecular diagnostics led to the recognition that variants linked to autosomal-dominant neurodegenerative diseases of later onset can, in the context of biallelic inheritance, cause devastating neurodevelopmental disorders and infantile or childhood-onset neurodegeneration. TOR1A-associated arthrogryposis multiplex congenita 5 (AMC5) is a rare neurodevelopmental disorder arising from biallelic variants in TOR1A, a gene that in the heterozygous state is associated with torsion dystonia-1 (DYT1 or DYT-TOR1A), an early-onset dystonia with reduced penetrance. While 15 individuals with AMC5-TOR1A have been reported (less than 10 in detail), a systematic investigation of the full disease-associated spectrum has not been conducted. Here, we assess the clinical, radiological and molecular characteristics of 57 individuals from 40 families with biallelic variants in TOR1A. Median age at last follow-up was 3 years (0-24 years). Most individuals presented with severe congenital flexion contractures (95%) and variable developmental delay (79%). Motor symptoms were reported in 79% and included lower limb spasticity and pyramidal signs, as well as gait disturbances. Facial dysmorphism was an integral part of the phenotype, with key features being a broad/full nasal tip, narrowing of the forehead and full cheeks. Analysis of disease-associated manifestations delineated a phenotypic spectrum ranging from normal cognition and mild gait disturbance to congenital arthrogryposis, global developmental delay, intellectual disability, absent speech and inability to walk. In a subset, the presentation was consistent with foetal akinesia deformation sequence with severe intrauterine abnormalities. Survival was 71%, with higher mortality in males. Death occurred at a median age of 1.2 months (1 week-9 years), due to respiratory failure, cardiac arrest or sepsis. Analysis of brain MRI studies identified non-specific neuroimaging features, including a hypoplastic corpus callosum (72%), foci of signal abnormality in the subcortical and periventricular white matter (55%), diffuse white matter volume loss (45%), mega cisterna magna (36%) and arachnoid cysts (27%). The molecular spectrum included 22 distinct variants, defining a mutational hotspot in the C-terminal domain of the Torsin-1A protein. Genotype-phenotype analysis revealed an association of missense variants in the 3-helix bundle domain to an attenuated phenotype, while missense variants near the Walker A/B motif as well as biallelic truncating variants were linked to early death. In summary, this systematic cross-sectional analysis of a large cohort of individuals with biallelic TOR1A variants across a wide age-range delineates the clinical and genetic spectrum of TOR1A-related autosomal-recessive disease and highlights potential predictors for disease severity and survival., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Background: An electroencephalogram (EEG) is a test that measures the brain's electrical activity. Here we decided to evaluate and compare the effectiveness of two drugs, hydrate, and nasal dexmedetomidine, in creating sedation during EEG in children., Methods: This clinical trial was performed in 2020-2022 on 65 children that were candidates for sedation for EEG with the Iranian Registry of Clinical Trials (IRCT) code IRCT20210614051574N8 (https://www.irct.ir/trial/61860). Pediatrics were randomized into two groups. Children in the first group received intranasal dexmedetomidine at a dose of 2-3 µg/kg 10 minutes before the procedure. The second group received 5% chloral hydrate syrup at a dose of 50-100 mg/kg orally 10 minutes before the procedure. For each patient, sleep onset latency and sleep duration were also measured. It should be noted that the patient's level of consciousness and sleepiness were checked by AVPU (alert, verbal, pain, unresponsive) criteria., Results: There was no significant difference between the two groups regarding the mean sleep onset latency (P = 0.59), sleep duration (P = 0.12), heart rate (P = 0.30), respiratory rate (P = 0.26), and SPO2 (P = 0.27). Analysis of covariance by adjusting for age and sex in both groups showed that the mean sleep duration (P = 0.04) and heart rate (P = 0.03) in the oral chloral hydrate group were significantly higher than in the nasal dexmedetomidine group. But the mean of other variables was not significantly different between the two groups (P>0.05)., Conclusion: The mean sleep duration and heart rate were significantly lower in the intranasal dexmedetomidine group compared to the oral chloral hydrate group., Competing Interests: None., (IJPPP Copyright © 2022.)

Background: Febrile seizure is the most common type of seizures among children, which is a terrible and frightening experience for parents who are concerned about its recurrence. The aim of this study was to evaluate the effect of diazepam on preventing the recurrence of febrile seizure following acellular pertussis vaccination., Materials and Methods: In this clinical trial, 121 children with a history of febrile seizure that required the pertussis vaccination were enrolled and divided into two groups; the first group was treated with oral diazepam for 48 h after vaccine injection and the control group received antipyretics only if fever occurred after the vaccination and used rectal diazepam for controlling seizure if a seizure occurred. The incidence of fever and seizure after the injection of the vaccine and incidence of febrile seizure were compared., Results: Nearly, 85.7% in the oral diazepam group and 87.9% in the rectal diazepam group had fever after receiving the pertussis vaccine, but the incidence of fever was not significantly different between the groups. Seven children (12.06%) in the rectal diazepam group had a seizure after pertussis vaccination, and none of the children in the oral diazepam group had a seizure after receiving the vaccine at 18 months of age. This difference was significant., Conclusion: Prophylaxis with diazepam administration in children with a history of febrile seizure can prevent recurrence of febrile seizure after pertussis vaccination., Competing Interests: There are no conflicts of interest.

Objective: Developmental delay screening is essential in pediatric medicine. The purpose of this study was to estimate the developmental delay in apparently normal children at entry to kindergarten., Materials & Methods: In this cross- sectional study conducted in 2013, the developmental status of a sample of children who entered to kindergarten at the age of 4-60 months were evaluated by the Persian version of ages and stages questionnaires (ASQ) in Isfahan county, central Iran., Results: Totally 680 children were enrolled, 11.8% of them were suspected to delayed in at least one domain and 1.3% and 1.2% in two and three domains, respectively. Developmental delay was in the following items: 5% in problem solving; 4.9% in fine motor; 3.2% in gross motor, 2.2% and 1.2% in personal - social and communication domains, respectively., Conclusion: Considerable proportions of apparently normal children who are entering kindergarten had developmental delay, which could be detected by evaluation with appropriate screening tools.