Iranian Journal of Child Neurology

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system,with unknown etiology in which both genetic and environmental factors are thought to be involved. The HLA system provides a set of genetic markers which lend themselves to systematic study. The disease also has variable clinical manifestations, ranging from a relapsing-remitting course to a chronic progressive disease.

In this article we review HLA profile and clinical presentation of the disease from current available data in Iran.

Objective

The aim of this study was to identify the best criteria for diagnosis of migraine in our pediatric population.

Materials and Methods

A total of 85 children aged below 15 years who had been referred to the Neurology Clinic of Al-Zahra Hospital with headache as their chief complaint, were enrolled. Validated questionnaires were completed by them. The patients were evaluated using five sets of diagnostic criteria including the Vahlquist, the Prensky, the International Headache Society (IHS), the IHS-Revised (IHS-R), and the Maytal criteria; the sensitivity of each set of criteria was assessed.

Results

Of the 85 children, clinically diagnosed as having migraine, 61 (72%), 73 (86%), 75 (88%), 76 (89.5%), and 76 (89.5%) met the criteria of IHS, Vahlquist, Prensky, IHS-R, and Maytal, respectively. Both the IHS-R and Maytal criteria had the highest, while the IHS criteria had the lowest sensitivity. Fifty-four children (63.5%) were positive for all five sets of criteria. The application of IHS criteria for diagnosing pediatric migraine led to a smaller percentage of children with migraine being identified.

Conclusion

Assessment of the sensitivity of the five sets of criteria for the diagnosis of migraine revealed the inadequacies and limitations of the IHS criteria in the diagnosis of pediatric migraine.

Objective

This study was designed to determine the etiology of status epilepticus (SE) andits relation to mortality.

Materials and Methods

This descriptive study was carried out based on the medical records of 40 patients with diagnosis of SE discharged from pediatric hospital of Bandar Abbas between March 2002 and March 2004. Multivariant analysis was pereformed to determine the prevalence of the disorder and the relation between SE and other factors such as gender, age, response to treatment, and mortality. We classified the etiology according to international league against epilepsy (ILAE) classification and also uses another classification regarding underlying causes such as fever (non-CNS infection), central nervous system infection, hypoxia, and metabolic causes.

Results

Status epilepticus was responsible for 0.3% of all hospital  dmissions during the study period. Based on the ILAE classification, frequencies for acute symptomatic, febrile, progressive encephalopathic, remote, and cryptogenic SE were 42.5%, 32.5%, 10%, 7.5%, and 7.5%, respectively.

The most common underlying causes resulting in SE were fever (45%), metabolic disorders (15%), CNS infection (12.5%), chronic neurologic diseases (7.5%), idiopathic (7.5%), hypoxia (5%), drug withdrawal (2.5%), CNS hemorrhage (2.5%), neurodegenerative disease (2.5%), brain abscess (2.5%), and post DPT (Diphteria,Pertusis,Tetanus) vaccination (2.5%). Mortality rate was 25% (80% in the patients younger than 5 years and 40% in those aged less than 1 year). Occurrence of SE and its mortality was found to be related to age (p< 0.05).

Conclusion

For SE, if seizures continue for more than 5 minutes, treatment must beinitiated. The outcome is determined by etiology, age, seizure duration and management; however, all we can do is enhance the management and increase its effectiveness.

Objective

Behavioral interventions such as relaxation training and biofeedback are some of the most effective interventions for management of migraine headaches. In this study we evaluated the utility of biofeedback-assisted progressive muscle relaxation training (PRT) in the treatment of migraine.

Materials and Methods

In this prospective study, twenty patients (12-18 years old) with a confirmed diagnosis of migraine headache, admitted to the Talie counseling center between March 2005 and December 2005, were evaluated; they were randomly assigned into an experimental group receiving biofeedback-assisted PRT and a control group in waiting list. All patients recorded the intensity of the headache and its frequency and duration before and 12 weeks after the treatment.

Results

The data was analyzed using Independent sample t-test for mean differences.In the biofeedback-assisted progressive muscle relaxation trained group, a statistically and clinically significant decrease was detected in intensity (p

Conclusion

It can be concluded that biofeedback-assisted progressive muscle relaxation training is a successful form of therapy in treatment of migraine.

Objective

Considering the controversial results in present day literature regarding the relationship between febrile seizures and anemia and the high rate of such seizures in children, this study was conducted to evaluate the association between pediatric febrile seizures and anemia.

Material and Methods

In this case-control study, conducted in 2003, 60 children with febrile seizure(cases) and 60 febrile children without seizure(controls) were evaluated in the Kashan Shahid Beheshti hospital; all patients were matched for age, sex, type of feeding, and use of supplemental iron. Thirty-six (60%) and 39 (65%) of the patients in case and control groups respectively were male, and the remaining female. Levels of hemoglobin, hematocrit, and red blood cell indices were determined in all children and Chi-square and Fisher exact tests were used to analyze data.

Results

Of the case group, 13.3% (6 male, 2 female) and of controls, 20% (9 male, 3 female) of children had anemia (p= 0.327), the condition being more common in male children aged over 6 months. Febrile seizures were found to occur mostly between the ages of 6 to 24 months.

Conclusion

The risk of febrile seizure occurrence in anemic children seems to be less than that in children who do not suffer from the condition.

Objective

Epilepsy occurs in 12 % to 90 % of children with cerebral palsy (CP). However its clinical course is not well defined. This investigation was undertaken to study and determine the characteristics and prevalence of epilepsy in children with cerebral palsy.

Materials & Methods

Of 133 children with cerebral palsy, seen between 1998 and 2001, in the pediatric neurology clinic of the Imam Reza hospital, fifty-three had epilepsy.

During the same period, a group of 70 epileptic children with normal neurodevelopmental status was studied as the controls.

Results

Patients with spastic quadriplegia were the most commonly affected with epilepsy. When compared with the control group, children with CP had a higher incidence of epilepsy with onset within the first year of age (52.8% vs. 18.5%), history of neonatal seizures (20.7% vs. 4.2%), and poly therapy (73.6% vs. 29.1%) respectively.

Conclusion

Epilepsy is common in children with CP and can be predicted if seizures occur in the first year of life ,in the neonatal period and based on the need for polytherapy.

Objective

Mitochondrial DNA (mtDNA) is considered a candidate modifier factor for neuro-degenerative disorders. The most common type of ataxia is Friedreich's ataxia (FA). The aim of this study was to investigate different parts of mtDNA in 20 Iranian FA patients and 80 age-matched controls by polymerase chain reaction (PCR) and automated DNA sequencing methods to find any probable point mutations involved in the pathogenesis of FA.

Materials and Methods

We identified 13 nucleotide substitutions including A3505G, T3335C, G3421A, G8251A, A8563G, A8563G, G8584A, T8614C, T8598C, C8684T, A8701G, G8994A and A9024G.

Results

Twelve of 13 nucleotide substitutions had already been reported as polymorphism. One of the nucleotide substitutions (A9024G) had not been reported before. The A9024G nucleotide substitution does not change its amino acid. The controls were also investigated for this polymorphism which was found in two of them (2.5%).

Conclusion

None of the mutations found in this study can affect the clinical manifestations of FA. This survey also provides evidence that the mtDNA A9024G allele is a new nonpathogenic polymorphism. We suggest follow-up studies for this polymorphism in different populations.

Mirror movement is an interesting but often overlooked neurological soft sign;these movements are described as simultaneous contralateral, involuntary, identical movements that accompany voluntary movements. This neurologic problem is very rarely seen in children; in familial cases there is a positive history of these movements in parents, diminishing with time. Here, we have presented the case of an 11-year old girl with mirror movements in her upper limbs which interfered with her hand writing. Her neurological examination revealed normal results. In this report, we have tried to explain some of the pathophysiologic mechanisms related to these abnormal movements.

Objective

Facial paralysis in children is very often idiopathic and isolated facial nerve palsy, resulting from leukemic infiltration is a rare occurrence. Here we present the case of a 14 year-old boy with acute lymphobastic leukemia, who first presented with isolated right side peripheral facial nerve paralysis and was initially diagnosed with Bell's palsy.

Conclusion

The presence of Bell's palsy in young children requires a complete evaluation, keeping in mind the possibility of leptomeningeal disease.