Iranian Journal of Child Neurology

Objective

Status Epilepticus (SE) has been described as a series of major motor seizure without recovery of consciousness between seizures.

SE is a medical emergency that requires prompt diagnosis and appropriate treatment.

In this article we shall conclude history, epidemiology, etiology, risk factors, the

best management as well as the prognosis of the condition.

Objective

Sleep disorders are common in children; they also have a significant impact on the whole family, the parents in particular. Few studies in this field have been performed in Iran; hence the  present study was carried out in order to determine sleep patterns and the  prevalence of sleep disorders in school aged children in Qazvin.

Material and Methods

This cross sectional study was done on 300 students (150 males and 150 females) of elementary schools in Qazvin, selected from cluster random samples. The duration of the study was from November 2006 to February 2007, and data was acquired by means of a standard pediatric sleep questionnaire.

Results

The 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 studied children. Sleep patterns during the school days differed significantly from what was observed on Fridays (p=0.000). In addition, there was a significant difference between males and females in terms of duration of sleep on Fridays (p=0.014).

Conclusion

Considering the high prevalence of sleep disorders in children, their harmful impact on the development of the  nervous system and the child's learning and psychological health as well as on parental competency, and again bearing in mind the low level of awareness among the general population about sleep hygiene, we strongly recommend educational programs via public media and via meetings with the parents in schools.

Objective

This survey evaluates the necessity of biochemical and imaging findings for patients with initial diagnosis of febrile convulsion, and also aims at determining the degree to which results of paraclinical examinations delineate management of patients.

Materials & Methods

Data of 302 patients referred to Mofid Children Hospital during two years (2005-2006) for febrile convulsions (FC), were collected in formatted questionnaires and analyzed.

Results

Abnormal lumbar punctures were reported in 9 patients, 3 of which had bacterial meningitis (1%). Biochemical tests including sodium, potassium, calcium, urea and glucose measurement, were performed for 289 (of 302 patients); of these 289, 9 cases were reported with hyponatremia, one case

with hypokalemia and one case with hypocalcemia. Brain CT was done in 51 cases and the results were abnormal in 4%, included a patient with status epilepticus.

Conclusion

Considering the results obtained from data, we suggest that most of the paraclinical examinations are not needed for Febrile Convulsions (FC), for simple FC, in particular.

 Objective

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.

Results

One hundred and twelve subjects were enrolled during this period. The average annual incidence rate was 2.21 per 100000 population of children aged

Conclusion

 The axonal type of GBS is a relatively common form of childhood GBS occurring in East Azerbaijan.

Objective

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 & Methods

For 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.

Results

Of 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.

Conclusion

Staging 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.

Objective

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 & Methods

This 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' experiences

Results

Female/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.

Conclusion

MS, 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 scattered

neurological deficits that remit, particularly weakness, disturbance of vision and co-ordination, they need to consider the possibility of MS.

Objective

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& Methods

Thirty-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).

Results

In the MP-IVIG group, FG improved at least one grade after 5 days of treatment. 

Conclusion

This study suggests that combined treatment with MP-IVIG in children with GBS does cause rapid improvement in the acute phase, but does not result in any significant difference in the long term outcome.

Objective

The 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 & Methods

The 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.

Results

A 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.

Conclusion

All patients with anorectal malformations (ARM) should be investigated for spinal and vertebral anomalies to improve treatment outcomes in ARM.

Objective

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 in HHE 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.

Objective

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.