Iranian Journal of Child Neurology

Introduction

Gratification disorder is a group of self-stimulatory behaviors which tends to form a habit. These normal behaviors are common and have various differential diagnosis including epilepsy. Hence, misdiagnosis may lead to perform unnecessary work-ups and treatments. In this article we have systematically reviewed available treatment options for gratification disorder.

Materials & Methods

We systematically searched Scopus, MEDLINE and Embase for related published articles from the beginning to 12^th May 2021. We followed the search strategy in all electronic databases using these keywords: [“Self-gratification” AND “treatment”]; [“child” AND “masturbation” AND “treatment”]; [“Pediatric” AND “masturbation” AND “treatment”]; [“infantile” AND “masturbation” AND “treatment”]; [“Benign” AND “Infantile” AND “Dyskinesia” AND “treatment”].

Results

The primary search yielded 241 studies; Five studies fulfilled the inclusion criteria and were included in this systematic review. None of the studies provided a good level of evidence. These studies indicate that behavioral therapy, Escitalopram and Aripiprazole can be considered as treatment options.

Conclusion

While pediatricians are familiar with gratification behaviors, their optimal management is overlooked. In addition to parental education and behavioral therapy, Escitalopram and Aripiprazole can be used as treatment options of this issue. There is a need to perform well-designed randomized control trials to obtain ideal evidence of the efficacy of these measures.

Objective: Congenital CMV infection constitutes the main cause of sensory neural hearing loss (SNHL) worldwide. The rate of CMV induced SNHL is not well documented in developing countries like Iran. Therefore, this prospective follow up study was aimed to evaluate this rate among neonates with congenital CMV infection.

Materials & Methods: Infants with congenital CMV infection who were admitted to Neonatal Intensive Care Units and infected infants who were identified in 2 other prospective screening studies in our region were enrolled. Audiological follow up was performed for all the cases. Antiviral therapy was administered for the newborns in case of having severe symptoms.

Results: A total of 22 infants with congenital CMV infection were entered into the study, of which, 8 and 14 had symptomatic and asymptomatic congenital CMV infection, respectively. In total, 3 of 22 infants had SNHL (13.6%; 95% CI: 2.8-39.8), 2 of 8 with symptomatic cCMV infection (25.0%; 95% CI: 3-90) and 1 of 14 with asymptomatic cCMV infection (7.1%; 95% CI: 0.1-39). No association was found between SNHL and risk factors related to CMV in newborns.

In conclusion, the findings of this study revealed that the rate of congenital CMV induced SNHL is high among neonates how born in our region. Severe sequelae of congenital CMV infection indicate the need for screening for CMV infection at birth to reduce the risk of CMV complications and the financial load of treatment imposed on health care and treatment systems in the country.

Abstract

Objectives

Academic difficulties are common in epileptic children. Learning disabilities (LDs) are a reduction in the learning capacity of children or the intellectual ability of adults and this is different from mental retardation or dementia.  

Materials & Methods

Fifty-six patients 35 male (62.5%) and 21 females (37.5%). The participants attended the Neurology outpatient Clinic, Sohag University Hospital between December 2016 to May 2017. Children with chronological age between 7 and 16 years, idiopathic focal and normal mental and motor developmental history were enrolled in this cross-sectional study. The Revised Quick Neurological Screening Test (QNST-R) was used to assess LDs.

 results

 Fifty-six patients with idiopathic focal epilepsy [22 Male (61.1%) and 14 Females (38.9%)]. Learning disabilities were present in 67.9 % of our participants with statistically significant association between LDs in one arm and younger age, earlier age of onset of epilepsy, frequent seizures and seizure semiology particularly of temporal lobe origin, in the other arm. In addition, left epileptic focus on EEG (EEG), prolonged treatment duration with antiepileptic drugs (AED) and polytherapy are significantly correlated to LDs.

Conclusion

Many factors are significantly correlated to the LDs in children with idiopathic focal epilepsy like age of the patient, age of epilepsy onset, seizure semiology, prolonged AED treatment and polytherapy.

Keywords: Learning disabilities · academic underachievement · epileptic focus.

Abstract

Objectives

The aim of this study was comparing of performance of Iranian children on the Bayley Scales of Infant and Toddler Development- third edition with the standardized norms of Iran and United State.

Materials & Methods

A total of 248 Iranian children at the ages of 1-42 months were participated in this study. The Bayley Scales of Infant and Toddler Development- third edition was administered to evaluate the cognitive, language and motor development of these children.

Results

The scaled scores of the study group were higher based on the reference norms in receptive communication, fine motor, and gross motor subtests. The scaled scores were variable in expressive communication
and cognitive subtests. The differences were significant for receptive communication and fine motor subtests (P<0.05). Using the reference norms instead of Iran norms resulted in under-referral regarding
receptive communication and fine motor subtests. More children scored below 1 and 2 SD using Iran norms in comparison to those reported for using the reference norms.

Conclusion

Iran norms differ significantly from the reference norms over two
subscales. It is recommended to use population-specific norms to identify children with developmental delay and early intervention

Abstract

Objective:

Epilepsy is a common brain disorder that characterizes by the persistent tendency to develop seizures in the neurological cognitive and psychological contents. MRI imaging is a neuroimaging study that helps finding structural epileptogenic lesions. The goal of this study is comparing the MRI findings between patients with intractable and drug-responsive epilepsy.

 Material and methods:

This case-control study was performed from 2007 to 2019. We consecutively enrolled all 1 to 16-year-old patients with intractable epilepsy who referred to shafa neuroscience center (72 patients) as case group and drug responsive patients who referred to pediatric neurology clinic of Baqiyatallah hospital as control group.

Results:

72 (23.5%) patients included in intractable epilepsy group and 200 (76.5%) patients in the drug-responsive group. The average age of total patients were 6.70± 4.13 years and gender distribution of the cases were 126 males and 106 females. Normal brain MRI was seen in 21(29.16%) patients case group and 184 (92.46%) in control group.

Neuronal migration disorder (NMD) was seen in 7 (9.72%) patients in case group and zero patient in control group. Also, the hippocampal abnormality and focal lesions (mass, dysplasia, etc) MRI findings were seen in 10 (13.88%) patients in case group and only 1 (0.05%) case was in the control group.

The corpus callosum agenesis, hydrocephalus, brain malacia, and developmental cyst were seen non-significantly more in the case group.

Conclusion:

MRI findings such as hippocampal abnormalities, focal lesions (mass, dysplasia, etc), NMD, porencephalic cysts, gliosis, and atrophy are seen significantly more in intractable epilepsy patients.

Objectives

The Bayley Scales of Infant and Toddler Development (Bayley III) assess cognitive, language, and motor development of children aged 1–42 months, and have been widely used globally. It is unclear whether or not the reference norms of the Bayley III are acceptable for use in other populations or lead to over or under-estimating development. The purpose of this study was to estimate the distribution of the Bayley scores by age in Persian speaking children and to compare the norms between Persian and reference norms.

Materials & Methods

We constructed Bayley III norms for cognitive, language, and motor scales using 1,674 typically developing children by consecutive sampling from health care centers. First cut-off point was determined, and then the scaled scores, based on Persian speaking and reference norms, were compared. The proportions of children with low scores (scoring less than -1SD and -2 SD) based on the two norms were compared, to identify over or under-referral for developmental delay resulting from reference norms.

Results

Scaled scores based on Persian norms varied across values based on reference norms on all subtests. The mean differences were significant across all five sub-tests (p < .05), with large effect sizes for receptive and expressive communication, fine and gross motor sub-tests of .20, .23, .14, and .25 respectively, and with small effect size for cognition sub-test of .02. Large effect sizes for all age groups were found for cognition, expressive communication, and fine motor sub-tests. For the receptive communication sub-test, effect sizes were generally large, with the exception of four age groups. For the gross motor sub-test, effect sizes were generally large, with the exception of six age groups.

More children scored below 1 and 2 SD using the Persian norms and resulted in under-referral regarding cognitive, receptive and expressive communication, fine and gross motor skills.

Conclusion

The Persian norms differ from the reference norms for all sub-tests and these differences are clinically significant. The use of the reference norms leads to fewer referrals in all sub-scales and leads to fewer diagnoses of children with developmental delay. Population specific norms are required to identify children with low scores for referral and intervention.

Objective: One-third of epilepsy of children is refractory and this study was conducted to evaluate efficacy and adverse events of levetiracetam as an add-on therapy in treatment of refractory epilepsy of children.

Materials & Methods: In a quasi- experimental study, seizures frequency and side effects of 314 children aged 1-14 years with refractory epilepsy who were referred to Pediatric Neurology Clinic of Shahid Sadoughi Medical Sciences University, Yazd, Iran and treated with levetiracetam for six months, were evaluated.

Results: 142 girls and 172 boys with mean age of 6.78±2.12 years were evaluated. At the end of six months of treatment with levetiracetam, 20% became seizure free, 28% had more than 50% decrease in seizure frequency, 38% did not have a notable change in seizure frequency and 14% experienced an increase in seizure frequency. Good response (stopping of all seizures or more than 50% reduction in seizure frequency) was seen in 51% of mixed type, 61% of myoclonic seizures, 64% of generalized tonic clonic seizures, 69 % of partial seizures, 100 % of tonic seizures and in 40 % of atonic seizures. Levetiracetam was significantly more effective in partial seizures, idiopathic epilepsies, children with normal developmental status and normal brain MRI.

Twelve children discontinued the treatment due to severe drowsiness, restlessness and exacerbation of seizures. Transient and mild side effects were seen in 9%(N=28) including somnolence, anorexia, fatigue, headache, ataxia and diplopia.

Conclusion: Levetiracetam could be considered as an efficient and safe adjunct therapy in treatment of refractory epilepsy in children.

Abstract

Objectives

Deformation of the skull by external forces in the absence of synostosis has been defined as positional plagiocephaly. The aim of this investigation was to determine the risk factors of positional plagiocephaly (PP) in healthy Iranian infants.

Materials & Methods

This case-control study was performed on 300 healthy Iranian infants aged 8-12 weeks who referred to pediatric neurology clinic at Shahid Beheshti Hospital of Kashan. Plagiocephaly evaluations were done by using Argenta’s scale.

Results

Based on multivariate logistic regression analysis, there was significant association between PP and male gender (OR=2.26; P=0.002), head circumference (OR=1.22; P=0.006), multiple pregnancy (OR=2.55; P=0.03), abnormal presentation in uterine (OR=2.18; P=0.02), primiparity (OR=2.43; P=0.003), and supine sleep position (OR=2.97; P<0.001). But type of delivery, firmness of headrest, oligohydramnios, and prolonged labor were not correlated with PP.

Conclusions

The current investigation supports the idea that head circumference, male gender, primiparity, multiple pregnancy, supine sleep position, and abnormal presentation in uterine are correlated with a greater incidence of PP. Further investigations should be undertaken to fully understand PP and its related risk factors.

Objectives

Many studies have suggested that Cochlear Implant (CI) users vary in terms of speech recognition in noise. Studies in this field attribute this variety partly to subcortical auditory processing. Since study on speech-Auditory Brainstem Response (speech-ABR) provides good information about speech processing, so this work was designed to compare speech-ABR components between two groups of CI users with good and poor speech recognition in noise scores.

Materials & Methods

The present study was conducted on two groups of CI users aged 8-10 years old. The first group (CI-good) consisted of 15 children prelingual CI users who had good speech recognition in noise performance. The second group (CI-poor) matched with the first group, but they had poor speech recognition in noise performance. The speech-ABR test in a sound-field presentation was performed for all the participants.  

Results 

The speech-ABR response showed more delay in C, D, E, F, O latencies in CI-poor than CI-good users (P <0.05), meanwhile no significant difference was observed in initial wave (V(t= -0.293, p= 0.771 and A(t= -1.051, p= 0.307). Analysis in spectral-domain showed a weaker representation of fundamental frequency as well as the first formant and high-frequency component of speech stimuli in the CI-poor users.

Conclusions

Abstract

Objectives

Post-Diphtheritic Paralysis (PDP), one of the most severe complications of Diphtheria, is caused by exotoxin of Corynebacterium diphtheria. Since there has been a resurgence of diphtheria in India in the recent years attributed to a number of epidemiological factors, this study was planned.

Materials & Methods

Thirty-five children with Post-Diphtheritic paralysis (PDP) were studied in a tertiary care hospital in Southern India. Neurological complications occurred in 38.5% of 91 patients of faucial diphtheria. Thirteen (37.1%) children were unimmunized, 12 (34.3%) were partially immunized, two (5.7%) were completely immunized and unknown status in eight (22.6%). Isolated bulbar palsy in 20 (57.1%) and bulbar palsy followed by limb weakness was seen in 15(42.9%) of patients. The first symptoms of PDP occurred 5-34 days after the onset of local diphtheria infection. Eleven (31.4%) out of 35 patients had received anti-toxin between days 5-7 of illness. Ventilation dependent respiratory failure occurred in three (8.6%) patients with PDP. Nine patients (25.7%) had evidence of co-existent myocarditis while myocarditis with renal failure was seen in two (5.7%) patients. Four (11.4%) patients died, three from severe cardiomyopathy and one from aspiration. Demyelinating neuropathy was noted in 64%.  Bulbar palsy recovered by 4-7weeks, while limb symptoms improved by 6-17weeks.

Conclusion

Post-Diphtheritic paralysis should be considered in any child presenting with bulbar palsy/ quadriparesis following previous history of fever/ sore throat. Awareness and availability with timely administration of ADS within 48 hours is important to reduce PDP, as antitoxin seems ineffective if administered after the second day of diphtheritic symptoms.

Objective

Autism spectrum disorder is a heterogeneous neuropsychiatric group of pervasive development disorder, which is mostly diagnosed through the intricate behavioral phenotype. According to strong genetic involvement, detecting the chromosome regions and the key genes linked to autism can help to elucidate its etiology. The present study aims to investigate the value of cytogenetic analysis in syndromic autism as well as to find an association between autism and chromosome abnormalities.

Materials & Methods

Thirty-six autism patients from 30 families, diagnosed clinically with DSM-5 criteria, were recruited. The syndromic patients who had additional clinical features involving development delay, attention deficit, hyperactivity disorder, seizure, language, and intellectual impairment were selected due to elevating the detection rate. Cytogenetics analysis was performed using GTG banding on the patients' cultured fibroblasts. Moreover, array-comparative genomic hybridization was also performed for a patient with a de novo and novel variant.  

Results

Karyotype analysis in 36 syndromic autism patients detected chromosomal abnormalities in two (5.6%) families, including 46,XY,dup(15)(q11.1q11.2) and 46,XX,ins(7)(q11.1q21.3)dn. In the latter, array-comparative genomic hybridization detected three abnormalities on chromosome 7, including deletion and insertion on both arms; 46,XX,del(7)(q21.11q21.3),dup(7)(p11.2p14.1p12.3)dn.

Conclusion

We reported a novel and de novo cytogenetic abnormality on chromosome 7 in an Iranian patient diagnosed with syndromic autism. However, the detection rate in syndromic autism was low which implies that it cannot be utilized as the only diagnostic procedure.

Abstract

Objectives

Spinal cord tumors are a rare diagnosis in children, mostly presented with unspecific symptoms which may pose a problem due to their possible malignancy and further complications. Yet there is little data regarding spinal cord lesions in our country.
The aim of this study is to present a series of 37 cases of primary spinal tumors treated at the same institution and briefly review their pathology, symptoms and site of occurrence.

Materials & Methods

 37 cases of spinal cord tumors and masses were selected from March 2007 to 2017, excluding spinal dysraphism. Data regarding age, sex, clinical presentation, location of the mass, and pathology were retrospectively collected.

Results

Mean age at diagnosis was 5 years and 8 months (standard deviation: 4.1 years). 21 were male and 16 were female (male-to-female ratio: 1.31). Pathological findings were 9 Neuroepitheliomas (6 Neuroblastoma, 2 Ganglioneuroma, 1 Ganglioneuroblastoma/Ganglioneuroma), 4 Ependymomas, 3 Primitive Neuroectodermal Tumors, 3 Glial tumors, 4 Neurodevelopmental tumors, 3 Lymphomas, 1 Hemangiopericytoma and 1 Neurofibroma. 26 patients had Motor symptoms (74.2%), 14 had pain (40%), 6 showed sensory symptoms (16.6%) and 4 had urinary symptoms (11.4%). The most common location of occurrence was the lumbosacral region.

 conclusion

While differing in pathological composition and location of tumors in comparison to other papers, our study presents possible presentations and/or expected pathologies in pediatric spinal cord tumors.

Joubert syndrome is a rare type of autosomal recessive genetic disorder that the incidence of it has been estimated to be in 80000 -100000 live births. Brain magnetic resonance image (MRI) with molar tooth sign is sufficient for a definite diagnosis. Immediately after birth, the prognosis depends on the severity and extent of the respiratory disorder. In this case a 5-year-old boy with joubert syndrome was brought by his parents due to inability to chewing due to dental caries and toothache. According to the patient’s clinical condition and inability to cooperate his dental procedures were performed under anesthesia at the hospital.  These patients are sensitive to the respiratory effects of anesthetics like opiate and nitrous oxide, therefore, they should not be used. In this patient we used sevoflurane gas to induce general anesthesia.  Advanced dental caries are observed in the reported cases of this syndrome, which may be attributed to dental hypoplasia and the inability to observe dental and oral hygiene. Thus, the patients’ parents must be given necessary instructions on the observance of oro-dental hygiene and regular follow-ups should be done for dental check-ups and preventive measures.

The behavioral patterns of children with autism are thought to be comprised of interconnected parameters. Acknowledging the value of lighting parameter, a less-studied aspect of the physical environment, this study illustrates how such parameter could contribute to increasing attention to visual stimuli. This paper outlines the establishment of new behavioral patterns following a novel approach using blacklight conditions, which highlighted objects in the foreground of the blackened environment, to modify the visual environment surrounding a child with autism. The child was first attracted to objects in the environment under blacklight conditions. This paper offers healthcare practitioners a useful perspective that has the potential to address a very important aspect of how a child with autism developed interaction with the objects following an environmental modification.

Abstract

Posterior reversible encephalopathy syndrome has a broad spectrum in clinical presentation and radiologic features. Diagnosis of posterior reversible encephalopathy syndrome (PRES) based on reversible clinical manifestations and sequential neuroimaging. Atypical MRI features were defined as hemorrhage, restricted diffusion or contrast enhancement of lesions, involvements of temporal and frontal lobes, brain stem, basal ganglia, corpus callosum, cerebellum, and spine. Atypical PRES with or without spinal cord involvement is a rare presentation, especially in children. Up to 2020, just 5 cases of posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) have been published in pediatrics.

Case report

 Here we describe the youngest patient that has been reported, with the diagnosis of PRES-SCI. All of 6 cases had high signal intensities on T2 weighted images in the brain stem and cervical cord that in fallow up the brain and spinal MRI, had been completely resolved. All the patients had hypertension in the base of renal disease except one girl with chemotherapy-induced hypertension. Headache, altered mental status, seizure, and visual impairment were the most common symptoms respectively. Facial palsy was a remarkable warning sign in some patients before admission.

 PRES-SCI is rare in pediatrics but since it is reversible, prompt diagnosis and management can change the prognosis in these children.

Keywords: Posterior reversible encephalopathy syndrome, Spinal cord, Hypertension, Pediatrics

Guillain-Barre syndrome is an acute ascending paralysis accompanied with autonomic symptoms like abdominal pain. Here we present a 13-year-old boy with lower extremities pain and sensory disturbances with presumptive diagnosis of Guillain-Barre syndrome who experienced severe progression of disease after receiving general anesthesia due to an appendectomy. Whether the progression was due to the natural history of GBS, immunosuppression induced by surgical stress, or usage of anesthetic medications remained unclear.

Desmoplastic infantile ganglioglioma (DIG) has a favorable prognosis; and is classified as a benign infantile brain tumor; it is more common in children under 2 years of age. In this report, we introduce one 5.5 months-old infant who was referred with infantile spasm and was diagnosed with a brain tumor. EEG showed modified hypsarrhythmia. The patient underwent ACTH treatment and was asked for a brain MRI. MRI of the patient’s brain showed large heterogeneous masses in the right hemisphere with shifting to other side. The patient underwent surgery. The extra-axial mass was completely resected, and the diagnosis of DIG grade I confirmed with the pathology. At present, the patient is only on phenobarbital, and the seizures do not recur, and the general condition is good; also, the growth of the patient’s head and development is normal for age. We found 13 reported cases during the search, of which 9 were boys and 4 were girls. The mean age of tumor diagnosis from the onset of symptoms was between 2 weeks to two months. Of 13 patients, in 8 patients, the origin of the mass was the right hemisphere. The most common tumors observed were glioma (4 patients), Hamartoma hypothalamus (3 patients). Except for three patients who died, the remaining patients found complete recovery with complete control of seizures after the mass's surgical resection.