Iranian Journal of Child Neurology (IJCN)
Volume:2 Issue: 4, Autumn 2008

ObjectiveStatus Epilepticus (SE) has been described as a series of major motor seizurewithout recovery of consciousness between seizures.SE is a medical emergency that requires prompt diagnosis and appropriatetreatment.In this article we shall conclude history, epidemiology, etiology, risk factors, thebest management as well as the prognosis of the condition.

ObjectiveSleep disorders are common in children; they also have a significant impacton the whole family, the parents in particular. Few studies in this field havebeen performed in Iran; hence the present study was carried out in order todetermine sleep patterns and the prevalence of sleep disorders in school agedchildren in Qazvin.Material and MethodsThis cross sectional study was done on 300 students (150 males and 150 females)of elementary schools in Qazvin, selected from cluster random samples. Theduration of the study was from November 2006 to February 2007, and data wasacquired by means of a standard pediatric sleep questionnaire.ResultsThe prevalence of sleep disorder among subjects was 44.3% (47.3% in males,and 41.3% in females), and the disorder was severe in 21% of the studiedchildren. Sleep patterns during the school days differed significantly from whatwas observed on Fridays (p=0.000). In addition, there was a significant differencebetween males and females in terms of duration of sleep on Fridays (p=0.014).ConclusionConsidering the high prevalence of sleep disorders in children, their harmfulimpact on the development of the nervous system and the child’s learning andpsychological health as well as on parental competency, and again bearing inmind the low level of awareness among the general population about sleephygiene, we strongly recommend educational programs via public media andvia meetings with the parents in schools.

ObjectiveThis survey evaluates the necessity of biochemical and imaging findingsfor patients with initial diagnosis of febrile convulsion, and also aimsat determining the degree to which results of paraclinical examinationsdelineate management of patients.Materials & MethodsData of 302 patients referred to Mofid Children Hospital during twoyears (2005-2006) for febrile convulsions (FC), were collected in formattedquestionnaires and analyzed.ResultsAbnormal lumbar punctures were reported in 9 patients, 3 of which hadbacterial meningitis (1%). Biochemical tests including sodium, potassium,calcium, urea and glucose measurement, were performed for 289 (of 302patients); of these 289, 9 cases were reported with hyponatremia, one casewith hypokalemia and one case with hypocalcemia. Brain CT was donein 51 cases and the results were abnormal in 4%, included a patient withstatus epilepticus.ConclusionConsidering the results obtained from data, we suggest that most of theparaclinical examinations are not needed for Febrile Convulsions (FC), forsimple FC, in particular.

This study aims at determining the epidemiologic, presenting symptoms, clinical course and electrophysiologic features of childhood Guillain-Barre Syndrome (GBS) in the East Azarbaijan province over a period of five years.Materials & Methods All the patients, aged< 15 years, referred/admitted to Tabriz Children Hospital with GBS between January 2001 and December 2005 were investigated.ResultsOne hundred and twelve subjects were enrolled during this period. The average annual incidence rate was 2.21 per 100000 population of children aged

According to current data available, neuroblastoma is the most frequent extracranial solid tumor in infants and children; because of its relationship to the primitive sympathetic ganglia, it may progress or regress spontaneously to more malignant or benign forms of tumors, respectively. It is also an important cause of the Opsoclonus Myoclonus Ataxia Syndrome (OMAS), or the "dancing eyes"syndrome. The risk factor of neuroblastoma includes patient''s age at diagnosis; degree of tumor spread, and selected biologic variables such as serum LDH, urinary and serum catecholamines such as VMA and HVA, ploidy and MYCN copy numbers. So, detection of risk factors and risk directed therapy are the mainstay of patient management.Materials & MethodsFor this study the records of 43 out of 46 patients, aged less than 14 years, admitted over 8 years (1996-2004), with the confirmed diagnosis of neuroblastoma or ganglioneuroblastoma were evaluated for full course of therapy and follow up.ResultsOf the patient group, 60% were male and 40% female. The most frequent clinical stage was stage 3 (34.7%), followed with stage 4 (32.6%) and 2 (26%).Less than 2% of patients presented with pure neurologic symptoms and these responded well to treatment. Relapse was seen more frequently in stage 4 patients and less in stage 4S. The OPEC protocol, started in 81% of patients, achieved a 54% response; on the other hand, in 15%, N6 was used, with the response rate of 40%. Patient data was analyzed and interpreted using SPSS software to reveal which clinical and biologic factors improve neuroblastoma outcome.ConclusionStaging and patients'' age at the time of diagnosis are the most important clinical factors to predict outcome, while primary tumor site and some biologic findings such as urinary VMA and serum LDH levels have a less important value.

Multiple sclerosis (MS) is an inflammatory demyelinating disease of central nervous system (CNS) that is increasingly being recognized as a disease affecting children. However, the clinical features of childhood MS at onset have been rarely reported from Asia.Materials & MethodsThis report presents a retrospective chart review of 26 patients with MS (20 females and 6 males), with an onset age of MS of less than 16 years, in the south of Iran between March 2001and November 2007; it documents researchers'' experiencesResultsFemale/male ratio was 3:1. Mean age in females was higher than males (13 vs.12.16).The disease was highly variable in onset presentation; the most common initial symptoms were limb weakness, disequilibrium, and diplopia. Three patients had a positive family history of MS in their first degree relatives. VEP was abnormal in 9 of 19(47%). MRI demonstrated multiple plaques in the brain in 24(92%) cases. Relapse remitting MS was a dominant pattern noticed in 23(88%) cases.ConclusionMS, in childhood, is not as rare as commonly believed; although its diagnosis is essentially a clinical one, paraclinical investigations are of great value in the identification of demyelinating disorders in childhood. The disease, as it occurs in children, does not appear to differ clinically from the disease as observed in adults. If pediatricians should confront a child showing evidence of scatteredneurological deficits that remit, particularly weakness, disturbance of vision and co-ordination, they need to consider the possibility of MS.

Guillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis in children, its pathogenesis most probably involving an autoimmune response to Schwann cell or peripheral nerve myelin antigens. Steroid regimes improve demyelinating diseases such as chronic GBS. We assessed the benefit of high dose methylprednisolone (MP) combined with Intravenous immunoglobulin (MP-IVIG) and compared the effects with those of IVIG per se in children with GBS.Materials& MethodsThirty-six children, aged between 1-12 years were randomized to receive IV MP 20mg/kg/day combined with IVIG 400 mg/kg/day (MP-IVIG) or IVIG per se at same dose for 5 days. All patients were diagnosed by standard clinical criteria and entered the trial within less than 2 weeks of the onset of neurological symptoms. All patients were too weak to walk. Functional grade (FG) was at 3 or more (able to walk with support).ResultsIn the MP-IVIG group, FG improved at least one grade after 5 days of treatment (P

ObjectiveThe associations between imperforate anus and spinal and vertebral abnormalities and neurologic deficits are well recognized; these neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction.Materials & MethodsThe medical records of 105 patients with imperforate anus, operated on by us, were retrospectively reviewed from 1996 to 2005. Patient''s sex, anorectal type lesion and vertebral or spinal anomalies were determined by ultrasound, excretory urography, voiding cystouretherography (VCUG) and lumbosacral x-ray.ResultsA hundred and five cases, consisting 48 (45.7%) boys and 57 (54.3%) girls, with anorectal malformations were studied; 70 patients were in high and intermediate type level, and totally 25 patients (35.7%) with spinal and vertebral anomalies were found in this group.ConclusionAll patients with anorectal malformations (ARM) should be investigated for spinal and vertebral anomalies to improve treatment outcomes in ARM

Hemiconvulsion-Hemiplegia-Epilepsy syndrome (HHE) is an initial phase of unilateral or predominantly unilateral convulsive seizures usually of long duration, with a second phase of hemiplegia (usually permanent), immediately following the hemiconvulsions;and then a third stage, characterized by the appearance of partial epileptic seizures. The causes of the initial convulsions inHHE syndrome are multiple but in many patients no cause in obvious. Neuroimaging studies demonstrate an acquired atrophy, that often is preceded by swelling and edema of the hemisphere. Here we report a case of 18 monthold boy, with an initial phase of repeated episodes of partial seizures, followed by hemiparesis and epileptic seizures, with one episode of generalized convulsive status epilepticus. Minor head trauma was the trigger factor of the initial attack. Brain neuroimaging showed generalized atrophy, that was more prominent in the left hemisphere.

Too much restriction of dietary proteins can cause severe protein malnutrition,which can occur in adjusting the diet for some kinds of aminoacidopathies, urea cycle disorder and organic academia. This report presents the case of a 1.5-year-old boy with history of phenylketonuria with a three weeks history of erythematous scaly plaques and edema of his extremities; he had a history of similar skin manifestations three months earlier that resolved spontaneously. The patient had been on very restricted phenylalanine diet. Diagnosed with Kwashiorkor, a phenylalanine level of 0.4 mg/dl, the child was hospitalized and put on a special diet and given the appropriate antibiotic; after a few days of treatment his condition improved. We underscore the importance of education for those considering prescription of diet restriction and emphasize the regulation of balanced diet in such patients.