Iranian Journal of Child Neurology (IJCN)
Volume:15 Issue: 4, Autumn 2021

With the outbreak of the novel pandemic coronavirus disease 2019 worldwide, numerous pediatric rehabilitation clinics have closed to support social and physical distancing, and therapists similar to other individuals are staying at their homes. There is a common concern of parents and caregivers that how and with what quality children’s rehabilitation exercises should proceed. Most children with neurological diseases have problems, such as muscle spasticity, range of motion (ROM) limitation, muscle shortening, balance loss, and mobility and movement impairments. The normalization of muscle tone, preservation of ROM, muscle stretch, and improvement of fine and gross motor skills and balance are essential activities that need to be considered. Therefore, this study aimed to summarize the necessities of a home-based rehabilitation exercise program during the quarantine period.

Subependymal Giant Cell Astrocytomas (SEGAs) are slow-growing glioneuronal tumors typically found around the ventricles of the brain, particularly near the foramen of Monro in 15%-20% of patients with tuberous sclerosis complex (TSC). Surgical resection is the standard treatment for these symptomatic tumors. The mTOR inhibitor everolimus can be regarded as an alternative treatment for SEGAs due to the complications of surgery. The present study primarily aimed to specify the effect of everolimus on SEGA volume change before and after treatment. The secondary objective was to determine the effect of this drug on renal angiomyolipoma (AML), skin lesions, and seizures in TSC patients.

This pre- and post-treatment clinical trial was performed on 14 children (eight females and six males with a mean age of 10 years) previously diagnosed with TSC based on the diagnostic criteria. The subjects received oral everolimus at a dose of 3 mg/m2 for at least six months.

Half of the patients had more than 30% of volume loss in SEGA, and in 28.5% of them, a ≥ 50% reduction in SEGA volume was observed (P=0.01). Moreover, 92.9% of the patients had a ≥ 50% decrease in the frequency of seizures (P=0.000). The response rate in AML and skin lesions was 14.2% and 50%, respectively.

Everolimus significantly reduced the seizure frequency and SEGA volume in the subjects; hence, it can be used as a potential alternative treatment for symptomatic SEGA in TSC patients.

Guillain-Barré Syndrome (GBS) is an acute inflammatory polyneuropathy characterized by a rapid progressive symmetric weakness. The GBS is the most common cause of acute flaccid paralysis (AFP) in most parts of the world. This study was carried out to investigate the epidemiological features of GBS in Iranian children.

The data were extracted using the AFP surveillance system that is a national screening program to detect all cases of AFP aged 0-15 years around the country. National Population Statistics data and AFP demographic data during 2008-2013 intervals were obtained from the relevant authorities in the Ministry of Health in Iran. The GBS cases were then extracted from the aforementioned database. The Chi-square test and Fisher’s exact test were used for statistical analysis.

A total of 1884 cases of GBS were identified in the study period, and the average annual incidence rate was 1.72 per 100,000 individuals. The highest incidence rate was within the range of 0-5 years. There was no statistically significant relationship between the incidence of GBS and the season in the whole country.

High costs of GBStreatment,morbidity and occasional mortality, and number of new cases, which is estimated to be approximately 300 individuals per year, need the particular attention of the health system.

 Guillain-Barré syndrome (GBS) is an immune-mediated disease of the peripheral nervous system which affects all age groups through the world. Although the pathogenesis and optimal treatment of GBS are still not completely established, one of the most common infectious diseases to trigger the syndrome is cytomegalovirus (CMV) infection. GBS following CMV infections is rarely reported in childhood and there is no data on GBS with antecedent CMV infection in children in Iran. The aim of this study was to evaluate the association of CMV infection and GBS in children in Iran.

 Case-control study design was used in 30 cases of Guillain-Barre syndrome, and 30 matched controls. All serum samples were tested for the presence of anti-CMV IgM and IgG antibodies using a commercially available ELISA (EUROIMMUN Medizinische, Germany).CMV viral DNA in the specimen was detected using polymerase chain reaction (PCR), (Cytomegalovirus PCR Detection Kit, CinnaGen company, Iran).

 Anti–CMV immunoglobulin (Ig) G antibodies were detected in GBS patients and healthy controls in 97% and 93% of children, respectively. Anti-CMV IgM antibodies were demonstrated in 33% (n=10) of the healthy controls and 33% (n=10) of the children with GBS. The borderline level of anti-CMV IgM antibodies was found in 23% healthy controls (n=7) and 13% (n=4) in the children with GBS (p=0.57), respectively. None of the specimens from the both controls the cases with GBS was positive for CMV DNA by PCR.

 These data demonstrated the presence of anti-CMV antibodies in a majority of both patients with GBS and controls and no relation between CMV infection and GBS was observed. However, further studies with larges sample sizes are highly recommended.

Children with developmental coordination disorder (DCD) are physically characterized by poor motor coordination and are at particular risk of losing their motor control. Quiet eye training (QET), with the latest techniques as an uncomplicated approach recently entered the field of rehabilitation research, has drawn the researchers’ attention. Therefore, the objective of this study was to examine the effects of QET on the performance of bimanual coordination in children with DCD.

Children with DCD (n=20; aged 8-9 years) were randomly divided into two groups, including QET and traditional training (TT). The participants performed bimanual in-phase and anti-phase movements with their wrists at three speed levels ranging from slow to fast. Bimanual coordination accuracy was assessed at the baseline, after 4 weeks at study completion, and at the retention test.

Bimanual coordination improved over time from the baseline to study completion. The results showed that there was a significant difference between the pretest and posttest in the QET group (P=0.001), and bimanual coordination accuracy in the posttest significantly increased, compared to that reported for the pretest. Moreover, there was a significant difference between the pretest and posttest in bimanual coordination accuracy in the TT group (P=0.01), and the posttest accuracy significantly increased (F=2.32); however, the increase was less than that of the QET group .

The obtained results indicated that the performance of the in-phase and anti-phase coordination modes was strongly influenced by QET. Furthermore, it was concluded that a successful performance of a bimanual linear task mainly depends on the availability of visual feedback.

Epilepsy is a chronic disease of the nervous system, which remarkably affects children’s performance and behaviors. Epileptic children are at greater risk of cognitive and behavioral disorders compared to healthy children. In this regard, a variety of factors associated with this disease may affect the patients’ families.

The present study was a randomized controlled clinical trial, which aimed to evaluate the effect of family empowerment on the quality of life in epileptic children referred to the concerned centers (the Bessat Clinic affiliated to the Kerman University of Medical Sciences and Shiraz’s Imam Reza Clinic). The participants, who were 80 parents with epileptic children meeting the inclusion criteria, were assigned into two experimental and control groups. In this study, the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE) was used to collect the required data from the parents.

The studied children’s age ranged from 4-8 years. These results revealed a statistically significant difference between the two groups regarding the level of changes in all dimensions, indicating that the experimental group’s mean score of quality of life in different dimensions increased (P <0.05 in all dimensions).

The implementation of the family-centered care plan by care providers, based on patient-family support relationships, the detection of their strengths and weaknesses, prioritization of the provided services, and effective interaction with the health team would increase the family and staff’s satisfaction, reduce the costs, and improve the outcome of the disease.

Epilepsy is a relatively common disease in childhood. In some patients, despite clinical control of seizures, the electroencephalogram (EEG) is clearly abnormal. In some studies, the adverse effects of epileptic discharges on cognition, learning, behavior, and seizure recurrence in children have been identified. In this study, the effect of Levetiracetam on epileptic discharges in the interictal phase of EEG in children with epilepsy was investigated.

In this clinical trial, 59 children aged 2 to 15 years with epilepsy, whose clinical seizures were controlled by sodium valproate in the last six months, but they still had epileptic discharges in the EEG, were randomly divided into two groups. Intervention group (24 people), whom Levetiracetam was added to sodium valproate, and control group, whom treatment with sodium valproate (35 people) alone was continued, then the patients were followed for one year.

The frequency of normalization of epileptic discharges in the intervention and control group was 66.7% and 57.1% (P = 0.500), respectively. The mean time for normalization of epileptic discharges in the interictal phase was 14.55%±14.31% months in the intervention group and 12.50%±20.18% months in the control group (P = 0.033).

In children with controlled seizures whose EEG was still abnormal, both sodium valproate therapy alone and sodium valproate combined with Levetiracetam were effective in reducing the severity of epileptic discharges. But addition of Levetiracetam to sodium valproate normalizes EEG more rapidly.

We aimed to investigate the risk factors predicting the development of intractable epilepsy in children with cerebral palsy (CP), with an emphasis on perinatal characteristics, seizure semiology, imaging, and EEG findings.

Following a descriptive, retrospective, case-control design, 106 children with CP and epilepsy from 2015 to 2020 were studied (46 children with CP and intractable epilepsy and 60 with CP and controlled epilepsy). Data were retrieved from medical records of participants (i.e., demographics, clinical characteristics, perinatal history, etiology of seizure and CP, seizure semiology, intellectual functions, therapeutic options, brain imaging, and EEG findings).

We established a model of the most important risk factors that can predict intractable epilepsy in children with CP. The model included the additive effect of a poor Apgar score at 5 minutes, the presence of neonatal seizures, focal epilepsy, and focal slowing on the EEG background (Area under the receiver operating characteristic of 0.810).

The findings can be used to identify intractable epilepsy in children who suffer from CP with further support by offering early therapeutic interventions intended to reduce the burden of refractory seizures.

Medium-chain acyl-coA dehydrogenase deficiency (MCADD) is an autosomal recessive disorder of fatty acid β- oxidation, which is inherited in an autosomal recessive manner. The enzyme plays a role in hepatic Ketogenesis, which is a significant source of energy during prolonged fasting. There is no metabolic screening program except for phenylketonuria (PKU) and hypothyroidism in Iran, and such screening is exclusively implemented in the case of babies with unprovoked seizures and hypoglycemia and previous unexplained sibling deaths. In this paper, we report a case of a seven-year-old boy who presented with afebrile serial seizures leading to coma and death. in this regard, metabolic screening tests were used to determine the exact cause of encephalopathy and the final diagnosis.

To analyze the efficacy and safety of Brivaracetam in pediatric patients with epileptic encephalopathy or unresponsive focal epilepsy.

This retrospective study included eight pediatric patients with EE or unresponsive focal epilepsy. Inclusion criteria: (1) ≤14 years, (2) history of refractory epilepsy, (3) at least one month of continuous therapy with BRV, and (4) at least six months of follow-up. Exclusion criteria: (1) variation of concomitant antiepileptic drugs during the previous and/or subsequent four weeks of the BRV introduction, (2) levetiracetam in therapy, (3) epilepsy secondary to the progressive cerebral disease, tumor, or any other progressive neurodegenerative diseases, and (4) a status epilepticus a month before screening or during the baseline period. The efficacy of BRV was defined as ≥50% of seizure frequency reduction at the end of the follow-up, compared to baseline.

All patients showed ≥50% seizure frequency reduction, of whom 37.5% were seizure-free, 25% had a frequency reduction of ≥75%, and 37.5% had frequency reduction of ≥ 50%. All patients with an epilepsy onset >12 months and epilepsy duration of ≤6 years were seizure-free. The maximum effect was achieved at 2 mg/kg/day, and focal seizures revealed a better response than epileptic encephalopathy. A remarkably positive effect of the Brivaracetam was noticed in patients with encephalopathy regarding the status epilepticus during sleep; however, no relevant side-effects were noted.

Brivaracetam was an effective and well-tolerated treatment in pediatric patients with epileptic encephalopathy or unresponsive focal epilepsy, especially for the epilepsy onset >12 months and the epilepsy duration ≤6 years. The total effect was not dose-dependent. Brivaracetam could represent an indication of encephalopathy regarding the status epilepticus during sleep.

Imerslund-Gräsbeck syndrome is a rare condition caused by vitamin B12 deficiency and proteinuria. In this article, we reported the case of a 10-year-old girl with imbalance and urinary incontinence. The case had cerebellar ataxia as the primary manifestation. The disequilibrium had progressed gradually within three weeks and was consistent with the symptoms of cerebellar involvement and urinary incontinence. Brain and cervico-thoraco-lumbar magnetic resonance imaging were normal. The patient had elevated lactate dehydrogenase (LDH=4775 IU/L), in addition to macrocytic anemia, on laboratory examinations; thus, the possibility of malignancy was raised. Then, bone marrow aspiration was performed, showing hypercellular marrow with megaloblastic changes. This finding proved megaloblastic anemia. Regarding the low prevalence of vitamin B12 deficiency in healthy individuals, extensive studies were performed to find out the cause. The serum level of vitamin B12 was found to be lower than the normal range. Although urinalysis revealed significant proteinuria, further nephrological investigations did not indicate any abnormalities. No evidence of serious problems was observed in the gastrointestinal tract study, and metabolic studies were normal. Finally, based on the obtained data, Imerslund-Gräsbeck syndrome was recognized. Patient was treated by vitamin B12 injection, leading to improved balance, and in one-month follow-up, she was able to walk independently, and the cerebellar symptoms had greatly disappeared; however, proteinuria persisted.

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease diagnosed with the presentation of café-au-lait macules, skinfold freckling, iris Lisch nodules, neurofibromas, osseous lesion, and optic gliomas. Mediastinal mass as the first presentation of NF1 is very rare, with a frequency of about 2.7%. Here, we present a rare case of NF1 in a 3-year-old boy admitted with respiratory distress and superior vena cava syndrome.