Iranian Journal of Child Neurology (IJCN)
Volume:8 Issue: 3, Summer 2014

Infantile spasm (IS) is a convulsive disease characterized by brief, symmetric axial muscle contraction (neck, trunk, and/or extremities). IS is a type of seizure that was first described by West in 1841, who witnessed the seizure in his own son. West’s syndrome refers to the classic triad of spasms, characteristic EEG, and neurodevelopmental regression. Most cases involve flexors and extensors,but either of the types may be involved independently.IS, as its name implies, most often occurs during the first year of life with an incidence of approximately 1 per 2000-4000 live births. Most, but not all, patients with this disorder have severe EEG abnormalities; this pattern was originallyreferred to as hypsarrhythmia by Gibbs and Gibbs. Cases with known etiology or signs of brain damage are considered as symptomatic. The Overall prognosis of the disease is poor. Peak onset age of the epileptic syndrome is 3 to 7 months, which mainly occurs before 2 years of age in 93% of patients. Hypsarrhythmia is the EEG hallmark of IS, which comprised a chaotic, bilaterally asynchronous high-voltage polyspike, and slow wave discharges interspersed with multifocal spikes and slow waves.Etiological classification is as follows: 1) Symptomatic: with identifiable prenatal, perinatal, and postnatal causes with developmental delay at the presentation time; 2) Cryptogenic: unknown underlying cause, normal development at the onset of spasms, normal neurological exam and neuroimaging, and no abnormality in the metabolic evaluation; 3) Idiopathic: pure functional cerebral dysfunction with complete recovery, no residual dysfunction, normal neuroimaging and normal etiologic evaluation, and normal neurodevelopment.

Familial hemiplegic migraine (FHM) is an autosomal dominantly inherited subtype of migraine with aura, characterized by transient neurological signs and symptoms. Typical hemiplegic migraine attacks start in the first or second decade of life. Some patients with FHM suffer from daily recurrent attacks since childhood. Results from extensive studies of cellular and animal models have indicated that gene mutations in FHM increase neuronal excitability and reduce the threshold for spreading depression (SD). SD is a transient wave of profound neuronal and glial depolarization that slowly propagates throughout the brain tissue and is characterized by a high amplitude negative DC shift. After induction of SD, S218L mutant mice exhibited neurological signs highly reminiscent of clinical attacks in FHM type 1 patients carrying this mutation. FHM1 with ataxia is attributable to specific mutations that differ from mutations that cause pure FHM1 and have peculiar consequences on cerebellar Cav2.1 currents that lead to profound Purkinje cell dysfunction and neuronal loss with atrophy. SD in juvenile rats produced neuronal injury and death. Hormonal factors involved in FHM affect SD initiation and propagation. The data identify SD as a possible target of treatment of FHM. In addition, FHM is a useful model to explore the mechanisms of more common types of migraine.

Intractable epilepsy is a major difficulty in child neurology, because the numbers of drugs that are available for treatment are limited and new treatments such as diets must be tried. Now there are some diets available for treating patients with intractable epilepsy. The oldest diet is the classic ketogenic diet and one of thenewest diets is the modified Atkins diet. Patients have a harder time accepting the classic ketogenic diet than the Atkins diet, which is easier to accept because the food tastes better. This study compares the efficacy of the ketogenic diet and the Atkins diet for intractable epilepsy in children.This study is a clinical trial survey with sample size of 40 children with refractory epilepsy who were patients at Mofid hospital in Tehran, Iran. Initially, from Jan 2005–Oct 2007, 20 children were treated with the Atkins diet, and then from Oct 2007–March 2010, the other group was treated with the classic ketogenic diet and the results were compared. In this study, response to treatment was greater than a 50% reduction in seizures and at the end of first, second, and third months for the ketogenic diet were 55%, 30%, and 70% and for the Atkins diet were 50%, 65%, and 70%, respectively.The results of this study show that there is no significant difference between the classic Ketogenic diet and the Atkins diet at the end of first, second, and third months and both had similar responses to the treatments.

Seizures are the most common pediatric neurologic problem. Research of the association between iron deficiency and seizures has shown conflicting results.This study evaluates iron status of children with a first seizure attack (febrile seizure (FS) or first unprovoked afebrile seizure (FUS) and healthy control group. In a cross sectional case control study, iron status of 6–60 month year old admitted children with first seizure to Shahid Sadoughi Hospital from August 2011–December 2012 were evaluated and compared with healthy control children that were referred to primary health care center of Azadshar, Yazd, Iran.150 children were compared in three equal (FS, afebrile seizure, and control) groups.Hemoglobin levels in FUS (11.39 ± 1.07 g/dl) and FS (11.46 ± 1.18 g/dl) were lower than the control group (11.9 ± 0.89 g/dl) group.Serum iron levels in FS (38.52 ± 11.38 μg/dL) and FUS (42.68 ± 14.76 μg/dL) were lower than the control group (54.32 ± 13.46 μg/dL).Serum ferritin level in FUS (46.21 ± 27.63 ng/mL) and FS (48.91 ±22.96 ng/mL) was lower than the control group (75.13 ± 35.57 ng/mL).Iron deficiency (48% in FS, 44% in FUS and 28% in control group) and iron deficiency anemia (26% in FUS, 22% in FS, and 10% in healthy children) was more frequent in children with seizures.Iron status should be evaluated in children with a first attack of febrile or afebrile seizures.

Recognition of risk factors for febrile seizures (FS) and epilepsy is essential. Studies regarding the role of melatonin in these convulsive disorders are limited.This study determines the relationship between serum melatonin levels and FS and epilepsy in children.A population of 111 children with simple FS, complex FS, and epilepsy (37 children per group, respectively) were included as case groups. In addition, 37 febrile children without seizures comprised the control group. Serum melatonin levels were measured and compared between all groups.The serum melatonin levels in the simple, complex FSs, and epilepsy groups were 2, 2.4, and 2 pg/ml, respectively. The serum melatonin level in the control group was 2.1pg/ml.Moreover, there were no significant differences observed while comparing the case groups.The present study reveals that there is no association between serum melatonin level and simple or complex FS and epilepsy. It appears that melatonin plays no significant role in these convulsive disorders.

Febrile seizures (FSs) are the most common neurological disorder observed in the pediatric age group. The present study provides information about epidemiological and clinical characteristics as well as risk factors associated with FS among Iranian children.On the computerized literature valid databases, the FS prevalence and 95% confidence intervals were calculated using a random effects model. A metaregression analysis was introduced to explore heterogeneity between studies. Data manipulation and statistical analyses were performed using Stata10.The important viral or bacterial infection causes of FSs were; recent upper respiratory infection 42.3% (95% CI: 37.2%–47.4%), gastroenteritis21.5% (95% CI: 13.6%–29.4%), and otitis media infections15.2% (95% CI: 9.8%- 20.7%) respectively. The pooled prevalence rate of FS among other childhood convulsions was 47.9% (95% CI: 38.8–59.9%). The meta–regression analysis showed that the sample size does not significantly affect heterogeneity for the factor ‘prevalence FS’.Almost half of all childhood convulsions among Iranian children are associated with Febrile seizure.

To determine the prevalence of associated behavioral problems and prognosis with Benign Childhood Epilepsy with CentroTemporal Spikes (BCECTS).Descriptive, Cross Sectional study that was conducted from October 2009 to April 2013 in the Department of Pediatric Neurology, the Children’s Hospital Taif, KSA.This study was conducted after approval from the Ethics Committee of the Children’s Hospital Taif, Saudi Arabia. Thirty-two patients from the age of 3 to 10 years old were recruited from the pediatric neurology clinic over a period of 4 years. All the patients were selected based on history, EEGs, and neuropsychological and neurological examinations.EEGs were performed for all the patients while in awake and sleep states. Those who had centrotemporal discharges were included in the study. All the patients also underwent a brain MRI. Only two patients had mild cortical atrophy but developmentally they were normal.In our study, prevalence of BRE is 32/430 (7.44%). Among the 32 cases, 24 were male and eight were female. Six cases out of 32 indicated a family history of BRE. Twenty-eight cases had unilateral right sided centrotemporal discharges and four had bilateral discharges. It is possible that for BECTS, a high number of seizures might play an important role in the development of mild cognitive impairment and/or behavior disturbances.

Prevalence of allergies is different around the world. Allergic rhinitis is a common chronic disease in children.Intelligence quotient (IQ) is an indicator of efficacy and many factors including chronic diseases may affect it. This study compares the IQs of children diagnosed with persistent or perennial allergic rhinitis with healthy children.This was a comparative study that was conducted from June 2011–May 2013 in an academic referral clinic. In this study, 90 patients aged 6- to 14-yearsold who were diagnosed with persistent or perennial allergic rhinitis and were compared to 90 age and gender match healthy patients from their respective families. The Wechsler Intelligence Scale for hildren was used to divide and calculate overall IQ, verbal IQ, and practical IQ. The t-test and chi square were used to analyze quantitative variables and qualitative variables, respectively. In this study, out of total 180 children, 90 (50%) in the case group and 90 children (50%), the control group participated for IQ comparison. One hundred (57%) were male and 80 (43%) were female. The overall IQ for allergic rhinitis patients and healthy patients was 109.2 and 107.5, respectively. This difference was not considered significant. Furthermore, there was no significant difference between the IQ scores of males and females.Although allergic rhinitis is a chronic disease and effects quality of life, there were no identifiable negative effects on IQ.

Trace elements such as zinc and copper have physiological effects on neuronal excitability that may play a role in the etiology of intractable epilepsy. This topic has been rarely discussed in Iranian epileptic patients.This study with the analysis of serum zinc and copper levels of children and adolescents with intractable and controlled epilepsy may identifies the potential role of these two trace elements in the development of epilepsy and intractabilityto antiepileptic drug treatment. Seventy patients between the ages of 6 months to 15 years that referred to Children’s Medical Center with the diagnosis of epilepsy, either controlled or intractable to treatment enrolled in the study. After informed parental consent the levels of serum zinc and copper were measured with atomic absorptionspectrophotometer and analyzed with SPSS version 11.35 patients were enrolled in each group of intractable (IE) and controlled epilepsy (CE). 71.45% of the IE and 25.72% of the CE group had zinc deficiency that was statistically significant. 48.58% of the IE and 45.72 of the CE group were copper deficient, which was not statistically significant.Our findings showed significant low serum zinc levels of patients with intractable epilepsy in comparison with controlled epilepsy group. We recommend that serum zinc level may play a role in the etiology of epilepsy and intractable epilepsy therefore its measurement and prescription may be regarded in the treatment of intractable epilepsy.

GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay–Sachs and Sandhoff disease). These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creatinghexosaminidases A، B، and AB. Patients who were diagnosed withGM2-Gangliosidosis in the Neurology Department of Mofid Children’s Hospital in Tehran، Iran from October 2009 to February 2014were included in our study. The disorder was confirmed by neurometabolic and enzyme level detection of hexosaminidases A، B، and ABin reference to Wagnester Laboratory in Germany. We assessed age، gender، past medical history، developmental status، clinical manifestations، and neuroimaging findings of 9 patients with Sandhoff disease and 9 with Tay Sachs disease. 83% of our patients were the offspring of consanguineous marriages. All of them had a developmental disorder as a chief complaint. 38%of patients had a history of developmental delay or regression and 22% hadseizures. The patients with Sandhoff and Tay Sachs disease were followed for approximately 5 years and the follow-up showed all patients were bedridden or had expired due to refractory seizures، pneumonia aspiration، or swallowingdisorders. Neuro-imaging findings included bilateral thalamic involvement، brain atrophy، and hypo myelination in near half of our patients (48%). According to the results of this study، we suggest that cherry-red spots، hyperacusis، refractory seizures، and relative parents in children with developmental delay and/or regression should be considered for assessment of GM2-Gangliosidosis disease.

The most common radiological abnormality seen in young Indian patients with epilepsy is single small enhancing (ring/disc) computed tomographic (CT) lesions. The two most common differential diagnosis of this lesion in clinical practice include neurocysticercosis (NCC) and tuberculomas. They have similar clinical and neuroimaging features. Few researchers believe that in poor and developing countries (where both tuberculosis and NCC are common) that it is difficult to differentiate between tuberculomas and a single cysticercal granulomas.We report a case of a 6-year-old female patient who presented with complex partial seizures. The patient’s neuroimaging showed a single ring-enhancing lesion in the brain that was not differentiated between NCC and Tuberculoma.Finally, Magnetic resonance spectroscopy (MRS) was suggestive of tuberculoma that was successfully treated with antituberculosis therapy.This report highlights diagnostic difficulties with conventional investigations in single ring enhancing lesions in the brain and role of MRS in a diagnosis. MRS is helpful in differentiating these two conditions.