Showing papers in "Iranian journal of child neurology in 2010"

Serum zinc level in patients with simple febrile seizure

Abstract: 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.

Comparison between the effect of neurodevelopmental treatment and sensory integration therapy on gross motor function in children with cerebral palsy.

Abstract: ObjectiveThis study was planned to compare the effects of neurodevelopmental treatment and sensory integration therapy on gross motor function in children with cerebral palsyMaterials & MethodsTwenty two children with spastic CP were randomly divided into two groups. Sensory integrative therapy was given to the first group (n=11), and neurodevelopmental treatment was given to the second group (n=11). All children were evaluated with GMFM-88. Treatment was scheduled for three - one hour sessions per week for 3 months.ResultsTwenty two children with spastic CP (11 diplegia and 11 quadriplegia) participated in this study. When two groups were compared, a significant difference was found in lying and rolling (P=0.003), sitting (0.009), crawling and kneeling (0.02) and standing ability (P=0.04). But there was no significant difference in walking, running, and jumping abilities between the two groups (0.417). Paired t-tests revealed a significant difference between pre and post test results, with increases in scores of lying and rolling, sitting, crawling and kneeling, standing in sensory integration therapy (SIT) and neurodevelopmental treatment (NDT) approaches.Conclusion Neurodevelopmental treatment and sensory integration therapy improved gross motor function in children with cerebral palsy in four dimensions (lying and rolling, sitting, crawling and kneeling, standing). However, walking, running and jumping did not significantly improve.

Relationship between Iron Deficiency Anemia and Febrile Seizures

Abstract: Objective Febrile seizure is the most common convulsive disorder in childhood. The role of iron in metabolism of neurotransmitters and carrying oxygen to the brain suggests the possibility of a relationship between iron deficiency anemia and febrile seizures. The aim of this study was to investigate the relationship between iron deficiency anemia and febrile seizures. Materials & Methods This case - control study was performed on 132 cases and 88 controls, aged 9 months to 5 years, from July 2007 to June 2009 in Baqyiatallah Hospital. Patients were selected using simple random sampling. The case group included children with first febrile seizure (core temperature over 38.5˚C during seizure) without a central nervous system infection or an acute brain insult. The control group included children suffering from a febrile illness without seizure. Iron deficiency anemia was defined with one of these laboratory indexes: 1) Hemoglobin (Hb) <10.5mg/dl 2) Plasma ferritin <12ng/dl 3) Mean corpuscular volume (MCV) <70 fl. The data collected from patients were analyzed with SPSS.13 software. Results Low plasma ferritin was found in 35 cases (26.5%) compared to 26 controls (29.5%), low Hb level was found in 4 cases (3%) compared to 6 controls (6.8%) and low MCV was found in 5 cases (3.8%) compared to 6 controls (6.8%). There was no significant difference in plasma ferritin , Hb level and MCV indices between the two group. Conclusion Considering the above-mentioned results, there is no relationship between iron deficiency anemia and febrile seizures.

Evaluation of Iron Status in 9-Month to 5-Year-Old Children with Febrile Seizures: A Case-Control Study in the South West of Iran

Abstract: 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.

Motor Development and Postural Control Evaluation of Children With Sensorineural Hearing Loss: A Review of Three Inexpensive Assessment Tools-Pbs, Tgmd-2, And P-Ctsib

Abstract: Objective Sensorineural hearing loss is believed to be the result of a physiologic malfunction in the inner ear or acoustic nerve. Depending on the rapidity of progression and severity, sensorineural hearing loss can be endlessly annoying, frightening and can constitute a permanent after effect. Moreover, there is no surgical procedure that can reverse or lessen the severity of a sensorineural hearing loss. Furthermore, children with sensorineural hearing loss present with additional disabilities in 30 to 40% of the cases. Children with profound sensorineural hearing loss may exhibit abnormalities of vestibular structures, which may lead to impairment of postural control, locomotion and gait. The development of gross motor functions such as head control, sitting and walking are likely to be delayed in these children. Evaluation of motor skills and balance are the core of the pediatrician and physical therapist's expertise and practice. Knowledge of the reliable, valid and inexpensive assessment tools for measuring motor skills and balance are necessary to gauge the progression of the disease and the impact of treatment. In this review, we aim to summarize inexpensive tools such as TGMD-2, PBS, and P-CTSIB.

The effect of the ketogenic diet on the growth and biochemical parameters of the children with resistant epilepsy

Abstract: 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.

Efficacy of The Ketogenic Diet as A Therapy for Intractable Epilepsy in Children

Abstract: 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.

A modified Atkins diet for intractable childhood epilepsy.

Abstract: Objective The aim of the present study was to evaluate the efficacy and tolerability of a modified Atkins diet for intractable childhood epilepsy. Materials & Methods Twenty one children with medically intractable epilepsy were enrolled in the study. Inclusion criteria were at least four seizures per month and a trial of at least three anticonvulsants without becoming seizure-free. The subjects received the diet over a 6-month period. Results Three months after diet initiation, 15 patients (71.4%) remained on the diet and 12 (57.1%) had >50% seizure reduction. Eleven patients (52.4%) completed the 6-month study and 8 (38.1%) chose to remain on the diet afterward. At 6 months, 9 patients (42.8%) had >50% seizure reduction. The diet was more effective in cryptogenic epilepsy (p=0.032). Most complications were transient and successfully managed by careful follow-up and conservative strategies. Conclusion The modified Atkins diet is an effective and well- tolerated therapy for intractable childhood epilepsy.

Evaluation of the nutritional status in children admitted to the neurology ward of Mofid children's hospital.

Abstract: Objective is commonly considered as an important risk factor that can produce a negative influence on the prognosis of patients with chronic neurological diseases. We aimed to evaluate the nutritional status of patients admitted to the neurology ward of Mofid children’s hospital via subjective and objective methods. Materials & Methods 61 children (2-6 years of age) who were consecutively hospitalized at the neurology ward between January and March 2008 underwent objective (weight, height, mid upper arm circumference– MUAC- and triceps skinfold thicknessTSF) and subjective nutritional assessment. Results result showed that 42.6%, 37.7% and 25.9% of patients were consecutively wasted, underweight and stunted. The z- Scores for TSF and MUAC were below -1 in 32.7% and 41.8% of the patients, consecutively. According to subjective Assessments (SGNA), 52.7% were malnourished. The prevalence of malnutrition was not significantly different between sex or diagnosis groups Conclusion is of high prevalence in patients with neurologic diseases. Regular

The efficacy and safety of zonisamide as an add-on drug in the treatment of lennox-gastaut syndrome

Abstract: Objective The Lennox-Gastaut syndrome (LGS: the triad of intractable seizures of various types, a slow spike-wave pattern in EEG and mental retardation) is one of the most difficult epilepsy syndromes to treat. The aim of this study was to evaluate the efficacy and safety of zonisamide (ZNS) as add-on therapy in seizures of children with LGS. Materials & Methods In a quasi- experimental study, seizure frequency and side effects of 40 children with LGS who were referred to the pediatric neurology clinic of Shaheed Sadoughi University of Medical Sciences, Yazd, Iran, between September 2008 and November 2009 and were on ZNS for six months were evaluated. Results Twenty one boys and 19 girls with a mean age of 6.6 3.6years were evaluated. At the end of six months of treatment with ZNS, 25% became seizure free, 25% had > 50% reduction in seizure frequency while 35% did not have a notable change in seizure frequency and 15% experienced an increase in seizure frequency. Drug was effective in 62.5% of the myoclonic and generalized tonic-clonic, 50% of the atonic, 43% of the mixed type and 33.4% of the tonic seizures. Transient side effects were seen in 25% of the patients: drowsiness in 10%, hyperthermia in 5 % and irritability, fatigue, ataxia and anorexia (each one) in 2.5% of the patients. No serious side effects were reported. Conclusion ZNS could be considered as an add-on therapy in the management of intractable epilepsy in LGS.

Comprehensive Assessment of The Relative Frequency of Sleep Disorders in Migraine and Non Migraine in 6- To 14-Year-Old Children

Abstract: ObjectiveHeadaches are common during childhood. In addition, sleep disorders are common problems in children, too. Although it is commonly thought thatheadache and sleep problems are related, there is not enough evidence to confirm this relationship. Finding evident relations between these problemswould help practitioners a lot to make earlier diagnosis and plan treatment modalities for both problems as soon as possible. This study aimed to assess the relative frequency of sleep disorders in migraine and non migraine children.Material & MethodsIn a cross sectional study, 148 children were enrolled in migraine (60) and non migraine (88) groups. They were aged 6 to 14 years. Migraine group consistedof patients who had definite migraine according to IHS (International Headache Society) criteria. Ten sleep problems (snoring, nightmares, sleep walking, sleeptalking, bedtime struggle, bruxism, sweating during sleep, excessive daytime sleepiness, insomnia in early or mid night and sleep apnea) were comparedbetween 2 groups. Fisher exact and chi square tests were used for making comparisons.ResultsWe found relationships between night sleep and daytime headaches. Some of these relationships were easy to explain but for some others, finding complicated explanations are necessary.Our findings showed that bruxism, sleep walking, early and midnight insomnia was significantly higher in migraine children. There was a common etiology forheadache and sleep disorders. Also, parents and migraine children were well familiar with the effect of relaxation on decreasing migraine episodes .Theyprovided appropriate facilities for night sleep for this reason.Conclusionwe found relationships between night sleep and daytime headaches. Some of these relationships were easy to explain but for some others, finding complicated explanations are necessary.

Developmental coordination disorder in children.

Abstract: Objective In this article, a motor skill disorder called developmental coordination disorder (DCD), that is usually first diagnosed during childhood, is explained and discussed In the year 1987, DCD was formally recognized as a distinct disorder in children by the American Psychiatric Association (APA) DCD is a generalized term for the children who have some degrees of impairment in the development of motor coordination and therefore have difficulties with physical skills which significantly interfere with their academic achievements and /or performing everyday activities As they develop, other age-related tasks are also below average Because these impairment & conditions are often associated with emotional distress, they can seriously interfere with the person's everyday life and social relationships Reviews indicate that most of the training rocedures have only a limited effect on the development of general coordination, and that they have no effect at all on academic progress This includes approaches based on assumed underlying deficiencies such as sensory integration deficits and kinesthetic functioning deficits, as well as the more traditional perceptual - motor training One new approach is Cognitive Orientation to daily Occupational Performance (CO-OP), based on problem - solving strategies and guided discovery of the child and task specific strategies The aim of this article was to inform, promote and disseminate more information about some difficulties in applying the diagnostic criteria for DCD Also, a brief review of the researches on the intervention methods is presented

Changing in thyroid function test in children underwent antiepileptic therapy

Abstract: Objective To determine the changes in thyroid function tests in children who underwent antiepileptic therapy in Shahid Beheshti Hospital, Kashan, in 2008. Materials & Methods This analytical-observational study was carried out in a cohort fashion without an external control group (self controlled) on 45 children with new onset epilepsy who had not been previously treated with antiepileptic medications. Three subjects were excluded from the study because of presenting clinical symptoms of hypothyroidism. Plasma levels of TSH, T3, FT3, T4 and FT4 hormones were measured and compared at baseline and 3 and 6 months after treatment. Results The results of Mann-Whitney statistical analysis suggested that the increase in the plasma level of TSH was significant only in the Sodium Valproate group. The plasma level of T3 significantly decreased 3 and 6 months after treatment in the Phenobarbital group while the plasma level of FT3 significantly decreased only in the Sodium Valproate group. The decrease in T4 plasma level was significant in all groups (Carbamazepine group, Sodium Valproate group and Phenobarbital group) 3 and 6 months after the onset of treatment but the decreasing in FT4 plasma level was only significant in the Carbamazepine group 6 months after the commencement of treatment. Conclusion Phenobarbital had the least effect on thyroid hormones. Considering the effect of such medications on thyroid function tests, it seems necessary to check the plasma levels of hormones periodically after beginning the treatment.

Recurrence of Febrile Seizures in Yazd Shahid Sadoughi Hospital

Abstract: Objective Febrile seizure is the most common problem in pediatric neurology that occurs in 3- 4 % of children. The purpose of this study was to determine febrile seizure recurrence frequency and to evaluate its risk factors. Materials & Methods In a descriptive prospective study, 139 children (6 months to 6 years) with first febrile seizure were admitted to Yazd Shaheed Sadoughi Hospital between March 2004 and August 2005 and were followed up for at least 15 months for febrile seizure recurrence. Results Seventy six boys and 63 girls with a mean age of 2.03 ± 1.21 years were followed up for 25.1±5.5 months. About 30% of them had complex febrile seizures and 37.4% had febrile seizure recurrence with a mean recurrence time of 6.7 ± 5.9 months. About 65% of the children younger than one year and 30% of those older than one year had febrile seizure recurrence. (P value= 0.0001) Recurrence of seizure was seen in 63% of those who had seizure within an hour from the onset of fever and in 33% of those who had seizure after one hour from the onset of fever. (P value = 0.005) Seizures in children younger than one year old and seizures occurring in association with a fever lasting less than an hour were risk factors of febrile seizure recurrence. Conclusion Febrile seizure is more disturbing in children younger than one year old. Antipyretic usage was not effective in preventing seizure recurrence but may reduce discomfort and is reassuring.

Comparison the Effect of Simultaneous Sensory Stimulation and Current Occupational Therapy Approaches on Motor Development of the Infants with Down Syndrome

Abstract: Objective characteristics in Down syndrome result in generalized muscular hypotonia, developmental delays and sensory integration deficits. The aim of this study was to compare the effects of simultaneous sensory stimulations and current occupational therapy approaches on motor functions development of infants with Down syndrome. Materials & Methods Eighteen infants with Down syndrome, aged 6 -18 months, were evaluated in two groups: intervention group (simultaneous sensory stimulation and occupational therapy) and control group (occupational therapy alone). They attended the program 3 times a week for 6 months and each session lasted 45 minutes. Motor functions were assessed before, during, and after intervention in the two groups, using GMFM test. Resultsmotor function increased in both groups according to the GMFM test (P = 0.000). Comparison of the changes showed that although the mean difference of this variable was higher in the intervention group, the difference was not statistically significant (P = 0.576). Mean motor deficit reduced in both groups during the period of the study, which was statistically significant (P < 0.05). Comparison of the difference in mean motor deficit in the first and last evaluations showed that this difference was more in the intervention group but statistically insignificant (P = 0.617) Conclusion use of simultaneous sensory stimulations can improve the quality of motor skills in Down syndrome infants. It is suggested that it may be used as an early intervention in association with other methods in the rehabilitation of these patients. However, more studies in this regard are warranted.

The Effect of Anticonvulsant Drugs (Phenobarbital and Valproic Acid) on the Serum Level of Cholesterol, Triglyceride, Lipoprotein and Liver Enzymes in Convulsive Children

Abstract: Objective on the effect of various antiepileptic drugs on serum lipids show contradictory results. We aimed to find the effect of Phenobarbital and Sodium Valproate monotherapy on serum lipid profile and liver function tests in epileptic children. Materials & Methods This cohort study was conducted in Amirkola Children Hospital. One hundred and ten children with epilepsy were included in this study. Children with hepatic or renal disease, those receiving medications which could alter liver function tests or serum lipid profile were excluded from the study. Patients were allocated into two groups. The first group, including 63 patients, received Phenobarbital and the second group, including 47 patients, received Sodium Valproate, both in divided doses. A venous blood sample was collected after overnight fasting to evaluate serum triglyceride, total cholesterol, LDL, HDL, and liver function tests. Data was analyzed with SPSS version 17. Results receiving Phenobarbital, total cholesterol, LDL, HDL, ALP, SGOT and SGPT increased significantly after treatment, but TG level showed no significant changes. In children receiving Sodium Valproate, HDL, ALP, SGOT, SGPT significantly increased after treatment but there were no statistically significant changes in total cholesterol, LDL and TG. In our study, the plasma levels of LPa elevated significantly after treatment with Phenobarbital and Sodium Valproate (P Value=0.0001). This increase was more significant in patients receiving Sodium Valproate. Conclusion results suggested a need for monitoring serum total cholesterol, HDL, LDL, and TG levels in patients receiving Phenobarbital and Valproic Acid.

Intractable epilepsy in children

Abstract: Objective Epilepsy is a common disorder affecting approximately 1% of the population. It is estimated that about 20- 30% of the patients become refractory to proper medical therapies. Such cases are often termed intractable. Intractable epilepsy (IE) is a serious condition in children, leading to significant impairment in quality of life, as well as behavioral and psychiatric problems. In this review, we tried to define intractability, mention the causes of intractable epilepsy and its predictive factors in children, and outline the management and various treatments of intractable epilepsy.

A Study on the Risk Factors for Obstetrical Brachial Plexus Palsy

Abstract: Objective Considerable medical and legal debates have surrounded the prognosis and outcome of obstetrical brachial plexus injuries and obstetricians are often considered responsible for the injury. In this study, we assessed the factors related to the outcome of brachial plexus palsy. Material & Methods During 24 months, 21 neonates with obstetrical brachial plexus injuries were enrolled. Electrophysiology studies were done at the age of three weeks. They received physiotherapy and occupational therapy. They were examined every 3 months for one year and limbs function was assessed according to Mallet scores; also, maternal and neonatal factors were collected by a questionnaire. Results There were 10 boys and 11 girls. Of all, 76.2% had Erb's palsy, 19% had total brachial palsy and 4.8% had klumpke paralysis. Risk factors including primiparity, high birth weight, shoulder dystocia, and prolonged second stage of labor were assessed. Electrophysiology studies showed neuropraxia in 52.4% and axonal injuries in 42.9% of the patients. At the end of the first year, 81% of the patients had functioned recovery around grade III or IV of Mallet scores. There were only significant relationships between functional improvement and neurophysiologic findings. Conclusion Outcome of obstetrical brachial injuries has a close relationship to neurophysiologic study results than other risk factors.

Febrile seizure in thalassemic patients.

Abstract: Objective Febrile seizure is the most common seizure disorder in children. Its pathophysiology is not fully understood yet; however, some risk factors have been cited for it. Iron is one of these influential elements and is involved in the metabolism of some neurotransmitters which are reduced in irondeficiency anemia and also increases the sensitivity of neural cells during a febrile episode. The present study aimed to determine the rate of febrile seizure in thalassemic patients and to compare it with the corresponding rate in the normal population. Materials & Methods This descriptive cross-sectional study was conducted on 766 patients with thalassemia major. They were all older than 6 months and were referred to Dastghaib Cooly's Clinic, affiliated to Shiraz University of Medical Sciences, from Oct 2006 to May 2007, and 766 normal and healthy children as the control group. Questionnaires containing demographic data and past history of febrile seizure, age of febrile seizure, number of episodes, hospitalization, and related family history were prepared and filled through interviewing the parents. Results Febrile seizure was detected in 7 cases of the patient group (0.9%) versus 18 cases (2.3%) of the control group. The frequency of febrile seizure in the controls was 2.5 times more than that in the thalassemia group, which was statistically significant (P < 0.05). Conclusion This study showed a lower rate of febrile convulsion in thalassemic patients compared to the control group. Accordingly, it could be suggested that high iron storage is a protective factor against febrile convulsion.

Rabson-Mendenhall Syndrome: A Case Report

Abstract: Rabson-Mendenhall syndrome is a rare genetic disorder characterized by growth retardation, dysmorphisms, lack of subcutaneous fat, acanthosis nigricans, enlarged genitalia, hirsutism, dysplastic dentition, coarse facial features, paradoxical fasting hypoglycemia, postprandial hyperglycemia, extreme hyperinsulinemia and pineal hyperplasia. Herein, we described a 10- year-old girl with physical features of the Rabson-Mendenhall syndrome that was presented with polyuria. To our knowledge, this is the first report of the Rabson-Mendenhall syndrome from Iran.

Risk Factor Profiles of Adverse Neuromotor Outcome in Infants

Abstract: Objective Assessment of risk predictors for adverse neurodevelopmental outcome at 1 year of age in term and near-term infants. Material & Methods This case-control study was a representative sample of infants from different health-care centers of north and east of Tehran. The association between risk factors and delayed motor development (developmental quotient below 70 indicating a significant delay) was analyzed using correlating risk factors; including the perinatal and neonatal data to the developmental status. The case group consisted of 143 infants whose DQ score was less than 70 and the control group consisted of 140 infants who had a DQ score of more than 70. Results Neonatal seizures, Apgar score less than 3 after 5 minutes of birth (OR = 2.87 [95% CI; 1.68, 4.92]), low birth weight (OR = 5.86 [95% CI; 3.07, 11.18]), preterm delivery (OR =6.17 [95% CI; 3.04, 12.52]), Premature rupture of membranes (PROM)>24 hours (OR = 6.18[95% CI; 2.07, 18.51]) and hyperbilirubinemia leading to phototherapy or exchange transfusion (OR =3.75 [95% CI; 2.12, 6.65]) were associated with an increased risk for neuromotor delay on developmental examination at 1 year. Conclusion This study identified distinct risk factors for an adverse outcome in infants. In this environment, perinatal risk predictors are most important.

Post-operative neurodevelopmental findings in syndromic and non-syndromic craniosynostosis

Abstract: Objective To evaluate the developmental situation of children that undergo operation because of syndromic and non-syndromic craniosynostosis Materials & Methods In this prospective study, 24 children (4 to 16 months of age) who underwent neurosurgeryical intervention because of non-syndromic (79%) and syndromic (21%) craniosynostosis were recruited For psychological evaluation, the Bayley Scales of Infant Development-Second Edition (BSID-II) was applied one month before and one year after surgical correction The scale consists of three parts, Mental Developmental Index (MDI), Psychomotor Developmental Index (PDI), and Behavior Rating Scale The MDI and PDI yield age-standard scores (100 ± SD) Results Mean baseline BSID-II scores revealed a mild delay in mental and motor scores (MDI: 843±21; PDI: 805±42) in non-syndromic craniosynostosis and a moderate delay in mental and motor scores (MDI: 703±31; PDI: 643±17) in syndromic craniosynostosis Mean postoperative BSID-II score revealed improved motor scores (MDI: 913±01; PDI: 913±02) in non-syndromic craniosynostosis and mean postoperative BSID-II score revealed a mild delay in mental scores and no change in PDI Among children with syndromic craniosynostosis, mean BSID-II score indicated mild baseline deficits in both mental and motor scores post operatively Conclusion Mental development and prognosis was better in non-syndromic craniosynostosis comparing syndromic craniosynostosis Surgery is effective in neurodevelopmental growth in non syndromic types of craniosynostosis but in syndromic type, remodeling surgery does not significantly affect neurodevelopmental outcome Keywords : Craniosynostosis, BSID-II, Neurodevelopmental

Childhood Headache Syndromes (Part II)

Abstract: 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.

Assessment of Cognitive and Motor Development in 150 Children with Refractory Epilepsy

Abstract: Objective Neuropsychological impairment is an important co-morbidity of chronic epilepsy. The aim of this study was to determine the state of the cognitive and motor development of patients with refractory epilepsy. Materials & Methods We studied 150 consecutive children with epilepsy who were referred to Mofid Children Hospital, a third level public referral University Hospital in Tehran, Iran, from October 2007 to October 2008. Refractory epilepsy was defined as therapeutic failure of three antiepileptic drugs which were used appropriately. Data regarding sex, age, age at which the first seizure occurred, microcphaly, muscle tonicity, EEG findings, kind of treatment for controlling seizures and cognitive and motor development delay were collected from medical records. Development delay was defined as delay in acquiring cognitive ability and motor skills for age according to the Denver Scale II. Results Of 150 patients 72% were younger than 2 years old and 56.7% were male. About 35.3% were microcephalic while 76% had normal muscular tonicity.Only 2.7% had normal EEGs. About 37.3% showed a good response to anticonvulsive drugs and became seizure free, 13.3% showed a relative response to anticonvulsants but 49.3% did not respond. In the present study, 68% had cognitive developmental delay and 60.7% suffering motor delay. There was a significant difference in response to treatment between patients with cognitive and motor development delay. Conclusion Cognitive developmental delay was more frequent in patients with refractory epilepsy, suggesting that early cognitive screening and introduvtion of rehabilitation programs are necessary for patients with refractory epilepsy .

Hereditary Spastic Paraplegia: From Gene to Clinic

Abstract: Objective Hereditary Spastic Paraplegia (HSP) is a degenerative disease of genetic origin affecting the corticospinal tracts in the spinal cord. There are three forms of inheritance: Autosomal dominant HSP, Autosomal rececive HSP and X-linked HSP. This disease is characterized by progressive spasticity of leg muscles with varying degrees of stiffness and weakness of other muscle groups. In this review, we will discuss the latest findings on the pathophysiology of axonal degeneration and all the responsible genetic defects in HSP.

Migraine in children and adolescents

Abstract: Objective Headache is a frequent symptom in children and adolescents. Migraine is one of the most common types of primary headache disorders in children that at times can be extremely disabling. Many clinical features of migraine in children differ from that starting in adulthood. This review discusses the epidemiology,clinical features, management, and prognosis of migraine headache in children and adolescents.

Very severe spinal muscular atrophy (type 0): a report of three cases

Abstract: Objective 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.

Relationship between Celiac Disease and Refractory Idiopathic Epilepsy in Children

Abstract: Objective Epilepsy occurs with a yearly incidence of 40 per 100,000 children, of which more than 25% are resistant to drug therapy. Epilepsy may occur in autoimmune diseases like lupus, celiac disease and myasthenia gravis. In this study, the relationship between celiac disease and refractory epilepsy was evaluated in children with idiopathic epilepsy. Material & Methods Hundred-fifty-five children (mean age, 6.7±3.3 years) with idiopathic and cryptogenic epilepsy referred to the neurology clinic were studied in two groups; drug controlled epilepsy (control, 82 patients) and refractory epilepsy groups (case, 73 patients). Both groups underwent serological tissue transglutaminase antibody measurement by ELISA. In seropositive cases, small intestine biopsy was conducted. Data analysis was performed using student's t test and 2 test. Results Seven (0.04%) patients had celiac disease based on a positive tissue transglutaminase antibody and three patients (0.01%) based on a positive biopsy. Three patients (2.4%) with drug controlled epilepsy (control group) and five with refractory epilepsy (case group) had seropositive celiac disease (p=0.255). In the biopsy survey of six seropositive patients, one patient (1.2%) in the drug controlled epilepsy and two patients (2.7%) in the refractory epilepsy group had positive biopsy for celiac disease (p = 0.604). One seropositive patient did not cooperate for biopsy. Conclusion If the relationship between celiac disease and epilepsy, especially in cases of symptomatic or oligosymptomatic celiac is proved, using gluten free diet increases the ability to control epilepsy particularly in refractory cases. We suggest celiac disease survey is not required in patients with idiopathic epilepsy.

Oral versus nasal vasopressin in the treatment of nocturnal enuresis in 5- to 12-year-old children.

Abstract: Objective Nocturnal enuresis is a common childhood problem and has various treatments. This study was carried out to compare oral and nasal vasopressin in the treatment of nocturnal enuresis in 5- to 12-year-old children who were referred to the Shahid Beheshti Clinic in 2008. Materials & Methods This study included 100 children (62 males and 38 females) with nocturnal enuresis. One group (50 patients) received 20 mcg nasal vasopressin which increased up to 40 mcg, depending on the patients' response. The other group (50 patients) received 0.2 mg oral vasopressin which increased up to 0.4 mg. The patients were followed up for one month after response to the last dose of drug. Data were recorded in prepared forms and analyzed using Chi-Square and Fisher Test. Results The success rate with oral and nasal method was 80% and 92%, respectively (P=0.08). Only 2% of the children had complications during the treatment; one child treated orally developed gastroenteritis and another child treated with the nasal method developed convulsions (P=1). Sixteen percent of the children treated with the oral method and 28% of the children treated with the nasal method had recurrence (P=0.148). Conclusion Oral and nasal forms of vasopressin have equal therapeutic effects. However, oral form of the treatment has fewer serious side effects and is easier to use. Therefore, the use of oral medicine is recommended.

Proteus Syndrome: A Case Report

Abstract: Objective Proteus Syndrome is an extremely rare, sporadic and progressive disorder We describe a four-month-old male baby with central nervous system manifestations in this article Clinical presentation A four-month-old boy was admitted into our hospital with three tonic - clonic generalized seizure attacks which started from the day before admission Each seizure attack lasted less than 10 minutes and the baby was well between attacks No fever was detected On physical examination, abnormal facial features, macrocrania, a wide nasal bridge, overgrowth of the right lower limb, macrodactyly in the third left toe, epidermal nevus on the left side of the abdomen and skin vascular abnormalities were detected His developmental status was normal Brain Magnetic Resonance Imaging (MRI) revealed left sided hemihypertrophy, ventricle enlargement and macrocrania on the same side