Iranian Journal of Child Neurology

Abstract

As one of the most common neurological diseases in children, epilepsy affects 0.9–2% of children. Complex interactions among the etiologies of the epilepsy, interictal discharges, seizures and antiepileptic drugs lead to cognitive impairments in children with epilepsy. Since epilepsy is considered as a network disorder that seizures have widespread impact on many parts of the brain, childhood epilepsy can even affect the normal development of language. About 25% of children with epilepsy do not respond to medications, therefore brain surgery is considered as a treatment option for some of them. Presurgical neuropsychological evaluations including language mapping is recommended to preserve cognitive and language abilities of patients after surgery.  Functional magnetic resonance imaging as a non-invasive technique for presurgical language mapping   has been widely recommended in many epileptic centers. The present study reviewed language representation and presurgical language mapping in children with epilepsy. In addition, mapping language in children with epilepsy helps to localize epileptogenic zone, to predict cognitive outcome of epilepsy surgery, and possible cognitive rehabilitation. This review gathered information about language representation and language mapping in pediatric   epilepsy settings. 

Key Words: pediatric epilepsy, language development, presurgical evaluation, language laterality

Abstract

Neurometabolic disorders are hereditary conditions mainly affect the function of the brain and the nervous system. The prevalence of these disorders is 1 in 1000 live births. Such disorders, at different ages could manifest as sepsis, hypoglycemia, and other neurologic disorders. Having similar manifestations leads to delayed diagnosis of neurometabolic disorders. A number of neurometabolic disorders have known treatments; however, to prevent long‑term complications the key factors are early diagnosis and treatment. Although a large number of neurometabolic diseases have no treatment or cure, the correct and on‑time diagnosis before death is important for parents to have plans for prenatal diagnosis. Different diagnostic procedures could be offered to parents, enzymatic procedures and determining metabolites in plasma, urine, and CSF, and molecular genetic diagnosis. Molecular genetic diagnostic procedures are expensive and could not be offered to all parents. Therefore, we aimed to design algorithms to diagnose neurometabolic disorders according to some frequent and characteristic signs and symptoms. By designing these algorithms and using them properly, we could offer diagnostic enzymatic panels. These enzymatic panels are inexpensive, therefore, could reduce the financial burden on the parents. Also, having an early diagnosis according to these panels could lead to offering more accurate and less expensive molecular genetic tests.

Objective: Determining the predictors of respiratory failure and duration of intubation in children with Guillain-Barre Syndrome (GBS).

Material and methods: Children diagnosed with GBS at Tabriz Children’s Hospital were studied. Factors associated and influencing respiratory failure and duration of intubation were determined using both univariate and multiple analysis.

Results: Three hundred twenty-four children were enrolled in the study, of whom 54.0% were males. Thirty-one (9.6%) patients underwent mechanical ventilation, the patients under 5 years old were more prone to requirement of mechanical ventilation (11.3% vs. 6.9%). The cases who are hospitalized in winter were more likely to need ventilation compared to the cases hospitalized in the spring (OR =7.00   ; 95% CI:1.51 - 32.53 ). Also autonomic involvement (OR=  8.88 ,95% CI :4.03 - 19.58 ,p<0.001) and cranial  nerves involvement (OR =9.88 ,95% CI:3.68 - 26.52, p<0.001) emerged as risk factors for mechanical  ventilation requirement.

Overall, 16.1% of patients with axonal electrophysiologic pattern required mechanical ventilation compared to 7.4% among those with demyelinating type, (OR:2.15, 95% CI: 1.01 - 4.69). In univariate analysis, the only variable which has a correlation with the duration of intubation is axonal electrophysiologic pattern p= 0.028.

Conclusion: Approximately 10% of patients required mechanical ventilation. Season, cranial nerve involvement, autonomic dysfunction, and the electrophysiologic pattern is more important variables for prediction  and duration of respiratory failure and mechanical ventilation.

Objectives:  Previous studies in adults with epilepsy revealed a higher prevalence of metabolic syndrome, which resulted in cerebrovascular and cardiovascular events, however, there is insufficient data about body composition and metabolic syndrome in children, especially in Middle Eastern region. We aim to Investigate metabolic syndrome criteria and body composition in ambulatory children with Epilepsy in Southern Iran.

Material and Methods: 90 children aged 11.4 ± 3.2 years with epilepsy and their age- gender-matched controls were included in this study. Anthropometric data, lipid profile, blood sugar and blood pressure were checked. Body composition was evaluated using Hologic system dual – energy X-ray absorptiometry.

Results: Prevalence of metabolic syndrome in patients (6.7%) was more than the controls (1.1%) (p-value = 0.043). Patients’ fat mass index was more than the controls (P = 0.012), and lean mass+ Bone Mineral Content (BMC) index lower than the controls (P = 0.017). Serum triglycerides in patients using carbamazepine was higher than the others (P = 0.019, Beta = 0.379). Blood pressure was higher in patients using carbamazepine (p = 0.016, Beta = -0.26). Fat mass index was higher in patients using sodium valproate (p = 0.031, Beta = 0.238).

 Conclusion: Our study revealed that children with epilepsy are more prone to metabolic syndrome and higher body fat mass. Therefore, early diagnosis and prevention of metabolic syndrome criteria in patients with epilepsy, performing regular exercise and having a healthy diet should be encouraged in these children.

ABSTRACT

Objectives: Sensory processing and sleep quality affect children's academic performance and their quality of life. The purpose of this study was to investigate the relationship between sensory processing patterns and sleep quality in primary schoolchildren.

Materials & Methods: In this cross-sectional study, 231 primary school students aged 7 to 12 years old (133 girls and 98 boys, mean age of 8.68±1.51) who were studying in schools in Tehran were randomly selected through cluster sampling. Researchers distributed a questionnaire for children's sleep habits to assess the quality of sleep and a Sensory Profile Questionnaire to assess the sensory processing patterns.

Results: In this study, we found a meaningful moderate relationship between sensory processing patterns and the general scores of sleep habits (p <0.001) and each of the patterns of sensory processing (avoidance, sensitivity, seeking, and registration) had a negative relationship with areas of sleep habits (p =.005). Also, there was a significant difference between children who had more challenges with sleep and children with normal sleep patterns in sensory processing; mean differences in all four sensory quadrants were significant (p <001).

Conclusion: Sensory processing patterns and sleep habits in primary school children have moderate correlation. Occupational therapists should consider the relationship between sensory challenges and sleep habits during their practice decisions with sensory challenges and sleep problems. Better sleep may occur with attention to sensory needs within the sleep routines. When sleep is better, this may lead to improved quality of life for the family and student performance at school.

Objectives:

The current study aimed to assess the need for emergency neuroimaging in children with first CFC.

Materials and methods: This is an analytic cross-sectional study which was conducted on children aged 6 -60 months with first CFS.  Data were gathered by a form which assess age, sex, radiology type, temperature, the duration of fever before convulsion, the duration and the frequency of convulsion, family history of febrile seizure. Data were reported by descriptive statistics (mean, standard deviation, frequency, and percent) and analyzed by Fisher Exact Test in SPSS version 19.

Results: In this study, 111 patients with first CFC and mean age of 21.18±11.83 months enrolled. Regarding the type of CFC, results showed that the highest and lowest frequencies belonged to multiple and multiple focal prolonged FC, respectively.  Upper respiratory infection was the most common diagnosis. 2 unimportant abnormal neuroimaging results were noted.

Conclusion:

In this study results showed that performing emergency neuroimaging in patients with first CFS was not mandatory in the absence of developmental disorders, abnormal neurologic examination, underlying neurological disorder, and head trauma. This result is more important in our country, according to the lack of access to neuroimaging modalities in many hospitals in our country and due to its irradiation risk in childhood and cost consuming.

Objective:

To evaluate the serum level of vitamin D in children aged six to 60 months with febrile seizure and febrile children without the seizure

Materials & Methods

Febrile children aged six to 60 months with or without seizure were studied. Demographic characteristics, serum level of vitamin D, and other laboratory findings were recorded.

Results

Among the 104 children, 51 patients had fever without a seizure and 53 patients had a febrile seizure. The mean subjects’ age was significantly more in the febrile seizure group compared to the without seizure group (16.26 ± 11.87 versus 26.36 ± 14.11 months, p = 0.001). The mean serum level of vitamin D in the with and withouts eizure groups was 41.92 ± 22.42 and 48.41 ± 15.25 microgram perd eciliter, respectively (p = 0.08). There was no significant correlation between serum level of vitamin D and seizure occurrence (p = 0.07).The mean serum sodium and potassium levels, and platelet count were significantly lower in the febrile seizure group compared to the without seizure group (p < 0.05). There were no significant differences between the two groups regarding hemoglobin, blood sugar, creatinine, blood urea nitrogen, calcium, alkaline phosphatase levels, and white blood cell count (p > 0.05).

Conclusion

The serum level of vitamin D in febrile children with or without seizure was normal. The serum level of vitamin D was lower inp atients with the seizure but not statistically significant. More clinical studies are needed to evaluate the relationship between febrile seizurea nd the serum level of vitamin D.

Abstract

Epilepsy in autism is a relatively common phenomenon. However, reflex seizures provoked by multifactorial stimuli are rare occasions in autistic patients. We reported the first case of defecation induced seizure in a 15 years old girl with autism. The patient had been diagnosed with epilepsy within first year after birth, however, seizures induced by bowel movements were found at the present time in her fifteenth. Reflex seizures showed a tonic pattern that changed to mioclonic represented with one-sided neck deflection. EEG pattern showed abnormal polyspikes and wave during defecation, while the patterns were normal between the attacks. The Patient was responsiveness to adrenocorticotropic hormone therapy with reduced frequency of both reflex and generalized seizures. Although seizure is commonly encountered in autism, reflexed seizures induced by defecation has not been previously reported in this condition.

Abstract

Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease which usually presents with skin rashes along with muscle weakness. We report a case of JDM in a 10- year-old girl with no skin manifestations.
She was presented with progressive muscle weakness and fatigue. Further laboratory investigation along with a muscle biopsy confirmed the diagnosis of Adermathopathic Juvenile Dermatomyositis. The patient was treated with intravenous immunoglobulin, corticosteroids, methotrexate, hydroxychloroquine, pamidronate, and Rituximab.
Following treatment, patients symptoms subsided and she gained normal muscular strength over the course of a year.