Iranian Journal of Child Neurology

Objective

Headache is one of the most common reason that children are referred to the Pediatric Neurology Services. It is said that ten percent of children aged 5 to 15 years have migraine.

Subsequently, it is essential for clinician to have a through, comprehensive and systematic approach to the evaluation and management of the child or adolescent who complains of headache.

This writing aims to explore the symptoms of headache, its epidemiology, classification, appropriate evaluation, differential diagnosis and management.

Headaches are divided into primary and secondary categories. Migraine and tension type headaches are prototype of primary headaches without underlying pathology. On the other hand, the type of headache which stems from organic diseases such as: brain tumor, increased intracranial pressure, systemic disease, drug toxicity, ear-nose and throat problems are considered secondary.

On the whole, the majority of children with primary headache have two patterns of headache. One is a chronic low-grade and the other is an intermittent disabling headache. The cause of the former is either caffeine or analgesic abuse, and the latter is predominantly migraine.

Traditionally, if a child presents himself with chief complain of headache, care taker physician begins with history taking followed by thorough physical and neurological examinations.

In the majority of the cases, this initial process leads to a diagnosis or indicate the need for further testing.

Once the diagnosis is made, a management program can be put into place.

Objective

Phenylketonuria (PKU) is an autosomal recessive metabolic genetic disorder characterized by a deficiency in the hepatic enzyme phenylalanine hydroxylase (PAH) which can cause problems with brain development, leading to progressive mental retardation, brain damage, and seizures. In this study we evaluated the frequency of seizure, EEG abnormality and behavioral disorders.

Materials & Methods 

In this case study, 94 PKU children aged between 1 month and 23 years who were referred to Mofid children Hospital between 2009 and 2010 were enrolled.

Patients were age and sex matched. Statistical tests were used for comparing patients' data.

Results

 The mean age of patients was 8.4 years. Parents were relatives in 80.9% of the cases (76 patients). Of all, 43% (45 patients) had seizure but EEG was abnormal only in 81% of them (35 patients out of 43 patients). Totally, EEG was abnormal in 67% of the cases (63 patients) of whom 44.4% (28 patients of 63 patients) did not have seizure. Therefore, there was a significant relationship between seizure and EEG abnormality. The phenylalanine level ranged from 8mg/dL to 50mg/dL (mean: 18.88 mg/dL) at the time of diagnosis and from 0.4mg/dL to 18mg/dL (mean: 7.37mg/dL) at the time of evaluation. On the other hand,we observed abnormal behaviors in all EEG abnormalities and there was a significant relationship between EEG abnormality and behavioral disorders.

Conclusion

In our study, the prevalence of seizure was less than EEG abnormality and there was a significant relationship between EEG abnormalities and behavioral disorders in patients with Phenylketonuria regardless of seizure.The authors believe that treatment of EEG abnormalities may lead to the correction of behavioral disorders in these patients.

Objective

Epilepsy is one of the most important problems in neurology. The purpose of this study was to evaluate the relationship between dairy products and seizures of the epileptic children based on their mothers' experience.

Materials & Methods

In a descriptive- analytic study, mothers' experience regarding the relationship between dairy products and seizures of their children was evaluated via a questionnaire. This research was done in the pediatric neurology clinic of Shaheed Sadoughi Medical Sciences University- Yazd- Iran in 2007.

Results

One hundred and forty eight mothers with an age range of 17-52 years (mean ± SD: 31.6 ± 6.6 years) were evaluated. Their children were 58.5% boys and 41.5% girls with an age range of 1-18 years (mean ± SD: 6.6 ± 4.2 years).

The most common dairy products which provoked seizure based on mothers' experience, were milk and ice cream. The effect of different kinds of dairy products was not different between males and female children. Mothers who experienced the effect of dried whey (kashk in Persian) on seizure were younger than others. Lack of correlation between milk and ice cream on seizures was reported in educated mothers.

Conclusion

Parent education on the diet of their epileptic children is necessary. On the other hand, extracting of suspicious food ingredients and testing them on animal models, should be done by other researches.

Objective

To determine the role of ketogenic diet in the treatment of intractable epilepsy in children.

Materials & Methods

Sixty six consecutive children (1-16 years old) with intractable epilepsy whose seizure were not neurodegenerative nor febrile in origin were recruited. They received the ketogenic diet and we evaluated its effect on seizure frequency for 3 months. All these children had more than five seizures per week despite adequate therapy with at least 3-4 anticonvulsant medications. Carbohydrates were initially limited to 10 gr/day and fats constituted 75% of the total energy requirement. Response to the diet was categorized as free of seizure, 99%-75%, 50%-75%, 25%-49% and lower than 25% reduction (resistant to therapy).

Results

Fifty five patients (84%) out of 66 children initiating the diet continued it after 1 week. After 3 months, 80% of the patients kept the diet. After one week, one month and 3 months, there was a more than 50% decrease in the frequency of the seizures in 40 (60%), 50 (75%) and 39 (59%) of the patients, respectively. Three patients (4.5%) were seizure-free after 1 week, 12 (18%) were seizure-free after one month and 12 (18%) were seizure-free after three months and a significant relationship was found between seizure reduction and the type of epilepsy (p<0.017).

Conclusion

The ketogenic diet should be considered as an alternative therapy for children with intractable seizures. It is more effective than many of the new anticonvulsant medications and is well tolerated by children andtheir families.

Objective

To evaluate the efficacy and common side effects of intermittent clonazepam in febrile  seizures.

Materials & Methods

This study was an experimental trial designed to determine the efficacy of intermittent clonazepam in febrile seizures .Thirty patients with an age range of 6 months to 5 years (60% male, 40% female) were studied. Children with a history of psychomotor delay, abnormal  neurological examination, a history of antiepileptic drug consumption, and afebrile seizures were excluded from the study. Patients received a single dose of prophylactic Clonazepam (0.05 mg/kg/ day) on the first day of febrile illness and twice daily during the course of fever.

An antipyretic medication (Acetaminophen) was advised if fever exceeded 38oC. Patients were followed up for one year after the study inclusion date.

Results

Three patients were excluded from study since they didnot follow the tritment and three patients experienced afebrile seizures. Twenty four patients had 162 febrile episodes during the course of the study and all patients were seizure-free after 1 year.

Conclusion

Clonazepam was 100% effective but lethargy and ataxia were common side effects in patients. Fortunately, their parents continued treatment because they had prior awareness of the  possible side effects of clonazepam. Clonazepam is efficacious as an intermittent therapy for febrile seizures if parents are informed of its side effects.

Objective

To evaluate the serum zinc level of the patients with simple febrile seizure and compare them with febrile children without seizure.

Materials & Methods

This prospective case - control study was performed on 60 patients aged 6 months to 6 years from Apr. 2009 to Jan.2010 in Ghaem, Imam Reza and Dr. Sheikh Hospitals in Mashhad. The serum zinc level was assessed and compared between the cases (30 individuals who suffered from simple febrile seizure) and the controls (30 individuals who had fever without seizure).

Results

Mean serum zinc level was 663.7 µg /l and 758.33  µg /l in the case group and the control group, respectively (P

Conclusion

It was revealed that the serum level of zinc was significantly lower in children with simple febrile seizure in comparison with febrile children without seizure.

Objective

Febrile convulsions are prevalent in children aged between 9 months and 5 years, with an incidence of 2-5%. On the other hand, iron deficiency anemia is the most common hematologic disease of infancy and childhood with a period of incidence that coincides with the time of developing febrile convulsions.

Therefore, it is hypothesized that there is a possible association between these conditions. This study was designed to elucidate this association.

Materials & Methods

Two sex and age matched groups (n=50 in each) of 9-month to 5-year-old febrile children who were admitted to Abuzar Hospital between September 2003 and October 2004 were selected. The first group, or the case group, included children with the first attack of febrile seizure and the second group, or the control group, included febrile children without seizure. Blood samples were collected for measuring complete blood count (CBC) indices, serum Iron,ferritin and total iron binding capacity (TIBC) levels.

Results

Both groups were comparable for age, sex, and the type of febrile illness at admission, except for seizure. There was no significant difference in CBC, Iron and TIBC between two groups but a signicant difference was seen in MCV (Mean Corpuscular Volume), especially in females (P= 0.017). The ferritin level in the case group was significantly lower (30.3 ±16.5 µg/dl) than the control group (84.2 ±28.5 µg /dl) (P= 0.000).

Conclusion

The findings of this study suggested a positive association between iron deficiency and the first febrile seizure in children. Supplemental iron may prevent the recurrence of febrile seizure. Prudently, further studies with larger sample sizes and longer follow-up periods need to be undertaken to substantiate this hypothesis.

Objective

We describe three patients with very severe Spinal Muscular Atrophy (SMA) presented with reduced fetal movement in utero, profound hypotonia, severe weakness and respiratory insufficiency at birth. In all infants, electrodiagnostic studies were compatible with a neurogenic pattern. In genetic studies, all cases had homozygous deletions of exons 7 and 8 of Survival Motor Neuron (SMN) and exon 5 of Neuronal Apoptosis Inhibitory Protein (NAIP) gene. SMA should be considered in the differential diagnosis of reduced fetal movement and respiratory insufficiency at birth.

Objective

Macular Corneal Dystrophy (MCD) is a rare autosomal recessive disorder affecting the stroma of cornea. Most cases of MCD are caused by mutations in CHST6 gene. The aim of this study was to determine mutations in the carbohydrate sulfotransferase 6 gene (CHST6) through genetic analysis of 7 Iranian patients with MCD.

Materials & Methods

We screened the CHST6 gene to determine the range of pathogenic mutations. Genomic DNA was extracted from peripheral blood leukocytes. The coding regions of the CHST6 gene were amplified using three pairs of primers, and directly sequenced in the final step.

Results

Four mutations were found to affect the translated protein and each of them corresponded to a particular disease haplotype that has been previously reported.