Iranian Journal of Child Neurology (IJCN)
Volume:9 Issue: 2, Spring 2015

Various epileptic syndromes may present in adolescence and Juvenile Myoclonic Epilepsy (JME) is known to be the most common idiopathic generalized epileptic syndrome presenting itself with different types of seizure activity. The exact etiology of JME is still unknown, but hypoxia, storage disease, toxic-metabolic disorders, drug reactions, and neurodegenerative disorders have been revealed to cause disease manifestation. Previous research shows that JME includes 5–10% of all cases diagnosed with epilepsy. It is estimated to include 18% of idiopathic generalized epilepsies. Females are at higher risk of developing this condition. Levatiracetam (LEV) is an anti-epileptic drug that has become one of the most used drugs for the management of epileptic syndromes. It has less drug interactions, milder side effects, and broad-spectrum efficacy to make it an ideal drug to control seizures. Different mechanisms of actions have made LEV a novel anti-epileptic drug. This new medication can be used as a mono- or add-on therapy to previous anti-epileptic drugs. One of the clinically valuable pharmacological aspects of LEV is that it can be started at a high therapeutic dosage and is well tolerated. The median starting dosage varied according to patients underlying disease, age, and disease severity. We have also discussed the effect of LEV on other epileptic syndromes, which showed promising results in both adults and children. In childhood epilepsy, there is evidence proving that a higher rate of behavioral disturbances with neurological disorders can beimproved by LEV therapy. Finally, our review showed the beneficial effects of LEV on seizure-control in different epileptic syndromes especially as a monotherapy.

Hypoxic insults are implicated in the spectrum of fetal disorders, including cerebral palsy (CP). In view of the major contribution of intrapartum risk factors and prematurity to subsequent neurological morbidity and mortality in humans, this study aimed to clarify the pathophysiology of brain injury, especially periventricular white matter damage (WMD), that occur in utero to the immature and near-term fetal CNS.An evaluation of the resulting neurological and behavioural phenotype in the newborn was performed by utilising a battery of neurobehavioural tests, including the Morris water-maze and the open-field test, followed by cerebral MRI and histopathology.This study used a murine model to examine the deleterious effects of WMD brought about by cerebral hypoxia-ischemia (HI) and the characteristic features of CP in mice. Murine models have proven themselves valuable in the area of experimental neuroscience. Hypoxia-treated mice were observed to demonstrate a significant neurofunctional deficit compared with sham mice on two behavioral measures. Indeed, different brain regions, including the sensorimotor cortex, the striatum, and the hippocampus were noticeably damaged after HI insult, as determined by both MRI and histopathology. These results, albeit qualitative in nature, appear to support the pre-existing finding that the long-term neurofunctional outcome in animal subjects with CP is strongly associated with the anatomical extent and pattern of cerebral damage as determined by both delayed neuroimaging and histopathology.

Bell’s palsy is a rapid onset, usually, unilateral paralysis of the facial nerve that causes significant changes in an individual’s life such as a decline in personal, social, and educational performance. This study compared efficacy of combined prednisolone and acyclovir therapy with prednisolone alone.This study is a randomized controlled trial conducted on 43 Children (2–18 years old) with Bell’s palsy. The first group of 23 patients was treated with prednisolone and the remaining patients were treated with a combination of prednisolone and acyclovir. The required data were extracted, using an informational form based on the House-Brackmann Scale, which grades facial nerve paralysis. The data were analyzed with Mann-Whitney test using SPSS version 16.The mean age of the first and second group were 8.65 ± 5.07 and 8.35 ± 4.92 years, respectively, (p=0.84). Sixty one percent and 39% of patients in the first group, and 45% and 55% of patients in the second group were male and female, respectively. No significant differences exist between the groups in terms of age and gender. The rate of complete recovery was 65.2% in group I and 90% in the group II (p=0.04).The results of this study showed that the combined prednisolone and acyclovir therapy of patients with Bell’s palsy is far more effective than treatment with prednisolone alone. Actually, age and gender had no impact on the rate of recovery.

Brainstem auditory evoked potentials (BAEPs) have long been utilized in the investigation of auditory modulation and, more specifically, auditory brainstem functions in individuals with autism. Although most investigators have reported significant abnormalities, no single BAEPs pattern has yet been identified. The present study further delineates the BAEPs deficits among subjects with autism. BAEPs were recorded in 43 male patients, aged 35–104 months, who underwent standard evaluations after receiving a diagnosis of autism. The control group consisted of 43 age-matched typically developing boys. The study took place in a tertiary neurodevelopmental center over a period of two years. The mean values of all absolute and/or interpeak latencies were longer in patients when compared to controls, albeit the differences were not significant for any of the parameters. Prolonged or shortened absolute/interpeak latencies (control group mean ± 2.5SD) were unilaterally or bilaterally identified in 33% of patients, compared to 9% of controls. The most frequent findings included prolongation of absolute latencies I, V and III, followed by shortening of interpeak latency I-V. In addition, abnormalities (either shortening or prolongation) of absolute latencies I and V, as well as interpeak latency I-V, were significantly more common among patients. Taken together, BAEPs in 23% of patients were indicative of a clinically abnormal response in 32% of patients. As can be easily concluded, BAEPs abnormalities characterize only a subset of subjects with autism, who may be important to identify clinically. The latter individuals may benefit from targeted intervention to utilize brainstem plasticity.

Detection of seizure, its etiology, and clinical types is important for guiding therapy. This study was designed to evaluate the etiology and clinical evaluations of neonatal seizures in Kashan, Iran.The data of 100 hospitalized neonates with a complaint of seizures in Kashan City, from January 2006 to January 2011 were evaluated. The pediatric neurologist made the final diagnosis. The gestational age, neonate admission age, type of delivery, and laboratory and radiological investigations were reviewed from the medical records. The relation of seizure etiology and other variables were compared using the Chi-square test. All the statistical analyses were performed using SPSS (ver 11.5).A total of 100 neonates were hospitalized with a diagnosis of seizures. The overall incidence rate of seizures was 2.6 per 1,000 live births. A total of 59% of seizures happened in the first three days of life. The etiologies of seizures were hypoxicischemic encephalopathy (HIE) (36%), hyponatremia (12%), hypoglycemia (11%), intracranial hemorrhage (11%), infections (10%), hypocalcemia (8%), metabolic disorders (7%), the structural anomalies (5%), and hypomagnesaemia (4%). In 23% of neonates, no specific etiology was found and 23% had multiple etiologies. In 45% of neonates, the EEG was not recorded. The type of the seizures were focal-clonic (26%), tonic (25%), multifocal clonic (34%), subtle (11%), and myoclonic (4%). The types of the seizure were unrelated to the paraclinical findings.Neonatal seizures are common and HIE was the main cause of seizures in this study. The clinical evaluation of neonatal seizures needs improvement.

Neurodevelopmental treatments are an advanced therapeutic approach practiced by experienced occupational therapists for the rehabilitation of children with cerebral palsy. The primary challenge in children with cerebral palsy is gross motor dysfunction. We studied the effects of neurodevelopmental therapy on gross motor function in children with cerebral palsy. In a quasi-experimental design، 28 children with cerebral palsy were randomly divided into two groups. Neurodevelopmental therapy was given to a first group (n=15) with a mean age of 4. 9 years; and a second group with a mean age 4. 4 years (n=13) who were the control group. All children were evaluated with the Gross Motor Function Measure. Treatments were scheduled for three - one-hour sessions per week for 3 months. We obtained statistically significant differences in the values between the baseline and post treatment in two groups. The groups were significantly different in laying and rolling (P=0. 000)، sitting (0. 002)، crawling and kneeling (0. 004)، and standing abilities (P=0. 005). However، there were no significant differences in walking، running، and jumping abilities between the two groups (0. 090). We concluded that the neurodevelopmental treatment improved gross motor function in children with cerebral palsy in four dimensions (laying and rolling، sitting، crawling and kneeling، and standing). However، walking، running، and jumping did not improve significantly.

The procedure for weaning from mechanical ventilation in many patients is a difficult and long process and increases the time of mechanical ventilation. There are numerous ways to achieve weaning. One common method is the use of CPAP. Considering the lower price of a humidifier, blender, and T-piece compared with CPAP and in light of the limited number of studies in this field the current study purposed to compare these two procedures.Fifty-one patients with neurological disorders who were under mechanical ventilation and ready to wean were allocated randomly into two groups: the CPAP group and the humidifier, blender, and T-piece group. Duration of hospital and PICU stay, number of days under mechanical ventilation, frequency of re-intubation, and mortality rate among patients were documented.The patients were 33 males and 18 females (64.7% and 35.3%, respectively) with an average age of 22.5 ± 4.5 months.The main indication for intubation was impending respiratory failure.Hospital stay was 22±15 and 21±13 days for the humidifier and CPAP groups, respectively.PICU stay was 13±11 and 21±13 days for the humidifier and CPAP groups, respectively. Re-intubation rates were 17.2% and 45.5% for the humidifier and CPAP groups, respectively.Mortality rates were 3.4% and 22.5% for the humidifier and CPAP groups, respectively.Considering no statistically significant difference between the two groups, using the humidifier, blender, and T-piece is recommended.

Epilepsy is a serious, potentially life-shortening brain disorder that occurs in patients of all ages and races. A total of 2–4% of people have experienced seizures at least once in their lifetime. Although treatment usually begins after a seizure, it is an important question whether the first cases of seizure do need to be treated by antiepileptic drugs. In this manner, we compare the recurrence rates of epilepsy in first seizure patients treated with sodium valproic acid as an antiepileptic drug versus a placebo.In a randomized clinical trial study, 101 first seizure patients were randomly divided into two groups: one group was treated with antiepileptic drugs (sodium valproate 200mg, three times a day) and the other group was given a placebo.The recurrence rate of seizures was evaluated and compared between the groups after 6 months of follow up.Eight recurrence cases were detected. All recurrence cases came from the placebo group, with four patients suffering an additional seizure after four months and between 4-6 month follow up. A comparison of recurrence rate detected a statistically significant difference between the drug group and placebo group.Our data shows that the recurrences occurred only in the placebo group with the difference between the recurrence rates in the placebo versus drug-treated was significant. Our results suggest that drug therapy for people after their first seizure attack might reduce the probability of seizure recurrence.

Homocystinuria is an inborn error of amino acid metabolism caused by cystathionine beta-synthase deficiency that affects methionine metabolism. The clinical features are heterogeneous ranging from mental retardation, ectopia lentis, and osteoporosis to vascular events such as deep vein thrombosis, sagital sinus thrombosis, and myocardial infarction. Cerebral sinovenous thrombosis (CVST) is an unusual disorder in children and requires prompt and accurate management. Some causal factors for thedevelopment of CVST differ between children and adults. The majority of cases with CSVT are found to have an underlying cause for thrombosis like dehydration, infections, prothrombotic and hematologic disorders, malignancy and trauma.Although homocystinuria is usually associated with ischemic strokes, CVST as initial clinical presentation of homocystinuria is rare in children.In this article, we presented a 10-year old boy with seizure, hemiparesis, and ataxia due to CSVT caused by homocystinuria.

Biotin is a coenzyme composed of four carboxylases. It presents in amino acid catabolism, fatty acid synthesis, and gluconeogenesis. Biotinidase recycles the vitamin biotin. A biotinidase deficiency is a neurocutaneous disorder with autosomal recessive inheritance. The symptoms can be successfully treated or prevented by administering pharmacological doses of biotin. Although, according to neonatal prenatal medicine (2011), a biotinidase deficiency does not manifest during the neonatal period. In this study, we report on a case of biotinidase deficiency in the first week of birth.Case Report:A 3100 g term boy was born via cesarean section. After 3 days, he was referred to the 17th Shahrivar Hospital with the chief complaint of tachypnea and grunting.Laboratory results revealed that liver and renal function tests, serum electrolytes, and blood indexes except ammonia were all normal. Within few days after the administration of oral biotin, the patient showed dramatic improvement and was discharged. However, within 4 months he was admitted two other times with the complaints of diarrhea and pneumonia. Unfortunately, he expired after 4 months.According to our results, it seems that clinicians should accurately assess suspicious patients and even assess infants for biotinidase deficiency.

Baclofen is a drug for many diseases for all ages, but it is hazardous in patients with renal failure. This article talks about a case of baclofen overdose in a child with renal failure.A 6-year-old boy admitted to the emergency department with a loss of consciousness, hypotonia, and areflexia following administration of 20 mg baclofen (1mg/kg/daily) in total dose for his voiding dysfunction. His laboratory tests showed advanced renal failure. After withholding the medication and supportive therapy, he recovered completely after two days. After arousal, he complained of insomnia, strange sensations on the skin, intentional tremors, and ataxia. He left the hospital in good condition in three days.Renal function control before baclofen administration is mandatory especially in high-risk groups. A total dose of 1mg/kg lead to encephalopathy in children with advanced renal failure, with subtle persistent complaints persist are often overlooked for a while.

Approximately 5–10% of preschool age children are considered developmentally disabled. Brain Magnetic Resonance Imaging (MRI) plays a key role in the diagnostic evaluation in these children. Many congenital or acquired brain anomalies are revealed with MRIs. Although the majority of these abnormalities are sporadic but patients with subcortical band heterotopia or double cortex syndrome have sex-linked inheritance. We are going to present the first case in Iran from Ahvaz city, which was presented with status epilepticus associated with developmental delay and finally diagnosed as double cortex syndrome, because band heterotopia cases especially for continuous or generalized form is rare.A 4.5-year-old developmentally delayed girl was admitted for generalized tonic clonic seizure attack of 1 hr, upward gaze, locked mouth, and urinary incontinence (status epilepticus) in the child neurology ward. She had a history of recurrent seizures that started as febrile seizures since she was 12 months of age and had frequent admissions for having recurrent seizure attacks. She was the only child of consanguineous parents with negative family history of any neurologic problems. She was a product of uneventful term pregnancy, vaginal delivery with a low Apgar score at birth who was admitted for six days in the neonatal ward for hypotonia and cyanosis. At 4.5 years of age, she had HC: 45cm (<3%) Length: 102 cm (25–75%), and BW: 18kg (75%).She was able to sit, walk with support, speak a few words, and communicate with others. A physical exam was unremarkable. Lab data including CBC, blood biochemical, and urinalysis results were all within normal limits, but the electroencephalography (EEG) revealed generalized poly spike-wave discharges. A brain MRI showed corpus callosal dysplasia, generalized band heterotopia, and polymicrogyria. She was discharged home with oral valproate and regular outpatient follow-ups.In the diagnostic evaluation of developmentally delayed and epileptic children, a brain MRI is strongly recommended for accurate diagnosis of anomalies such as neuronal migration disorders (band heterotopia) and others, because appropriate therapeutic management, prognosis, prevention, and geneticcounseling for prenatal diagnosis are dependent on definite diagnosis of the proband case.