Iranian Journal of Child Neurology最新文献

Objectives: This study aims to determine the concurrent validity of the Bayley-III and the Bayley screening in Persian-speaking children. Measuring child development with the Bayley-III can be time-consuming and expensive. Accordingly, this research seeks to assess the accuracy of the Bayley screening as a measure of developmental delay for high-risk infants by age group.

Materials & methods: Concurrent validity between raw Bayley screening scores and Bayley-III scores was assessed by administering to 403 1-42 month-old children. The cut score of 1.0 standard deviation below the mean of two tests was calculated using the Bayley-III as the criterion measure. This study used 70% of the sensitivity and specificity cut scores to measure validity. In addition, the study calculated the Pearson and Cohen's kappa correlation for the association between the two measures.

Results: The sensitivity of cognitive, receptive, and expressive communication, fine, and gross motor scales of Bayley screening were 70.7, 81.4, 67.7, 60.7, and 58.1, respectively. Specificity values varied narrower, from 87.8% to 100%. The Cohen's kappa coefficient in all age groups was substantial. The Pearson correlation between two test scores is significant for all scales, although the coefficients are over 0.884. The sensitivity and specificity have no specific trends with children's age, and the best sensitivity concurrence on the two tests was in the 1-12 months old age range.

Conclusion: The study supports the concurrent validity of the Bayley screening, showing an assertive and significant association between Bayley screening and Bayley-III in Persian-speaking children.

Objectives: Computed tomography (CT) scans are one of pediatric emergency departments' most informative and widely used techniques. The research aimed to investigate the frequency of abnormal brain CT scan and determine its correlation with patient complaints in the emergency department.

Materials & methods: A retrospective review was conducted on the records of children who presented with various neurological complaints at the Bahrami Children's Hospital, a referral hospital in Tehran, Iran, over a five-year period (2016-2020). The study included patients who had undergone an emergency brain CT scan within the first 24 hours of their consultation.

Results: Two hundred eligible children, with a mean age of 5.47±3.81 years, were included in this study. They were categorized into seven groups according to their chief complaints. Based on the findings, 113 patients (58.5%) had normal CT scan reports. The remaining CT scan findings, in order of frequency, included imaging results related to the primary diagnosis (19%), incidental findings (12%), and indications of preexisting disorders (10.5%). Seizure was the most common complaint, leading to a brain CT scan (34%). Children presenting with focal neurological symptoms accounted for the highest frequency of CT scan abnormalities.

Conclusion: This study concluded that most children presenting with neurological complaints have either normal or insignificant findings on their brain CT scans. Given the potentially harmful effects of radiation exposure, as well as considerations of healthcare costs and time, it is essential to adhere to precise protocols when conducting these scans.

Objectives: Guillain-Barré syndrome (GBS) involves the peripheral nervous system developed by infections or immune conditions. Cerebrospinal fluid (CSF) analysis and electrodiagnostic tests are essential diagnostic methods for GBS. However, limited data are available on how the findings from these methods relate to each other. This study aimed to evaluate changes in CSF analysis and electrodiagnostic tests in pediatrics with GBS.

Materials & methods: The present study retrospectively evaluated electrodiagnostic tests and CSF changes in pediatrics with GBS who were admitted to Tabriz Hospital, Iran, from 2010 to 2020 due to CBS. Patients' data, including age, gender, CSF analysis, and electrodiagnostic test results, were recorded from the patients' files. Electrodiagnostic data included pace and amplitude of tibial, median, peroneal, sural, ulnar nerves, nerve conduction velocity (NCV), F-wave, and motor unit action potential (MUAP). The significance level was considered less than 0.05.

Results: The mean age of patients was 4.83±2.72 years, and 54.6% were boys. The most common type of GBS involvement was demyelinating type. No gender differences were found between involved nerves unless the ulnar nerve was significantly more involved in girls (P-value: 0.012). The obtained findings indicated no significant relationship between electrodiagnostic tests and CSF protein (P-value: 0.439).

Conclusion: No association was observed between electrodiagnostic results and CSF changes in pediatrics with GBS.

Diabetes is one of the most common chronic disorders in the world, characterized by chronic hyperglycemia. Among the rare causes of diabetes, Leigh syndrome is a rare genetic mitochondrial disorder with unusual manifestations like neurological deficits in addition to typical diabetes symptoms. This report enlightens others about the unusual presentation of diabetes in a pediatric population. The studied case is a 6-year-old girl with hypothyroidism and diabetes. Post-SARS-CoV-2 infection, she developed progressive lower limb weakness. Magnetic resonance imaging (MRI) and electromyography-nerve conduction velocity (EMG-NCV) revealed brain lesions and polyneuropathy. Genetic testing using whole exome and Sanger sequencing confirmed mitochondrial gene mutations in the MT-NDI location, diagnosing her with Leigh syndrome. Pediatric diabetic patients typically present with Type 1 diabetes mellitus (T1DM) or Type 2 diabetes mellitus (T2DM), but other causes must be considered. Leigh syndrome can manifest with neurological symptoms, requiring clinicians to recognize its diverse presentations for proper management. This case highlights the importance of considering rare etiologies for diabetes to improve the prognosis and quality of life.

Objectives: This randomized clinical trial aims to evaluate and compare phenobarbital and levetiracetam effectiveness and safety in controlling neonatal seizures as first-line therapeutic options.

Materials & methods: Neonates with seizure manifestations presenting to the Bahrami Hospital from March 2020 to March 2022 were enrolled in this study. Patients' data were recorded, including demographic findings, anthropometric measurements, birth characteristics, and laboratory findings. Patients were randomly divided into the phenobarbital and levetiracetam treatment groups. Outcomes defined as adverse effects, response to medication, time to start enteral feeding, and length of hospital stay were compared between the two treatment groups.

Results: The current study comprises 44 neonates divided into phenobarbital and levetiracetam groups. Seizure control among the two groups was not significantly different, with levetiracetam and phenobarbital groups showing seizure control of 68.2% and 59.1%, respectively. Moreover, no significant difference was observed regarding the duration of hospitalization and time to initiate feeding. No adverse effect was reported in either group.

Conclusion: This study showed no significant difference between levetiracetam and phenobarbital for neonatal seizure control.

Objectives: Acute disseminated encephalomyelitis (ADEM) is a rapid-onset inflammatory central nervous system (CNS) disorder in children, causing demyelination, encephalopathy, and neurological deficits, often following infections.

Materials & methods: This 10-year retrospective study evaluated pediatric patients with seronegative acute disseminated encephalomyelitis (ADEM), focusing on clinical, laboratory, and imaging profiles. The various profiles were assessed to determine age- and/or sex-based differences.

Results: The study reviewed 36 patients, with an average age of 6.08 years and predominantly male (61.1%). Clinical presentations included fever, nausea, vomiting, and seizures, with left facial hemiparesis being more common in girls (P-value = 0.023), while abnormal deep tendon reflexes (DTRs) and right-sided pathologies were more common in older patients (P-value < 0.05). Recent laboratory results have revealed differences between peripheral lymphocytes and polymorphonuclear (PMN) cells. Imaging revealed predominantly bilateral lesions, with older patients more likely to show lesions in the right parietal and occipital lobes (P-value = 0.01 and 0.04). Bilateral parietal lobe lesions were significantly correlated with several laboratory findings across the different subgroups. Multivariate logistic regression revealed that these findings were statistically significant in regards to peripheral PMN and lymphocytes in the age category and cerebrospinal fluid (CSF) protein in the gender category (P-value < 0.05). Additionally, girls, particularly those who were older, had significantly higher involvement of the cervical spine (P-value = 0.04 and 0.02).

Conclusion: This study reveals age and sex-related differences in the clinical presentation and imaging findings of seronegative pediatric ADEM, showcasing the various demographic factors in patient profiles.

Objectives: The study aims to compare the diagnostic accuracy of MRI and CT scan features in differentiating medulloblastoma from ependymoma, two similar pediatric brain tumors.

Materials & methods: This retrospective cross-sectional study was conducted on all pediatric patients with posterior fossa tumors admitted to teaching hospitals affiliated with Isfahan University of Medical Sciences from 2017 to 2022. Forty-three patients with posterior fossa tumors were identified, and seven patients were excluded due to diagnoses other than medulloblastoma or ependymoma. Tumor morphology on MRI, tumor density on CT scan, and apparent diffusion coefficient (ADC) values were assessed to differentiate medulloblastoma from ependymoma.

Results: Histopathologic diagnosis was medulloblastoma in 21 patients (60%) and ependymoma in 14 patients (40%). Mean ADC values in medulloblastoma and ependymoma cases were 0.67±0.19 (range= 0.50-1.25) and 1.22±0.29 (range=0.67-1.72), showing a significant statistical difference between the two groups (p-value=0.000). The ADC cut-off point of 0.9825 was associated with 90% sensitivity and 92.9% specificity for differentiation of ependymoma from medulloblastoma.

Conclusion: While tumor morphology on MRI and other studied parameters are unreliable for differentiating medulloblastoma and ependymoma, ADC values may provide a potential diagnostic tool. Further studies are needed to confirm the utility of DWI and other advanced MRI techniques in differentiating these tumors.

Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare, nonhereditary, nonprogressive congenital neurocutaneous syndrome with underlying ectodermal dysgenesis. The classic triad of this syndrome is central nervous system (CNS), ocular, and cutaneous involvement as unilateral lipomatous lesions of the scalp, neck, and face with ipsilateral brain anomalies and ipsilateral ocular choristoma. Herein, this study reports a case of a 2-year-old boy presented with status epilepticus for the first time. Intraspinal lipoma, arachnoid cyst, cerebral hemiatrophy, asymmetric hydrocephaly, choristoma, and corneal clouding were noted. This case fulfilled Moog's clinical criteria for diagnosis of Haberland syndrome. Additionally, this study introduces linear and whorled nevoid hypermelanosis and cerebral periventricular white matter hyperintensity as novel manifestations of this syndrome.

Objectives: Cerebral Palsy (CP) is a group of movement disorders. A recently proposed occupational therapy approach to reduce spasticity and improve balance and postural control is Hippotherapy Simulation (HS). The present study attempts to investigate how HS impacts balance, postural control, and spasticity of adductor muscles in children with spastic bilateral CP.

Materials & methods: Thirty-one children with bilateral spastic CP, five to nine years old, were selected via availability sampling and divided into the intervention group (n=16) and the control (n=15) through stratified block randomization. Assessment tools, including Pediatric Balance Scale (PBS), Trunk Control Measurement Scale (TCMS), Modified Ashworth Scale (MAS), Gross Motor Function Measure (GMFM), Pediatric Evaluation of Disability Inventory (PEDI), and goniometry, were completed by an assessor unaware of the children's grouping.

Results: After the intervention, the average total scores of all variables in the HS group significantly exceeded those in the control group (P<0.01). However, no significant differences were observed between the groups at follow-up, underlining the necessity of providing long-term or recurrent interventions to maintain improvements in gross motor function. This approach also constructively influenced postural control and mitigated the spasticity of adductor thigh muscles.

Conclusion: This study showed the multimodal effect of simulated hippotherapy combined with occupational therapy routine exercises on the physical performance of children with bilateral spastic CP.

Ataxiatelangiectasia (A-T) is an infrequent genetic neurodegenerative disorder inherited autosomal recessively. It is mainly characterized by early-onset progressive cerebellar ataxia and dilated capillaries in the oculocutaneous regions especially conjunctivae so-called telangiectasia. A-T is a multisystem disorder and requires multi-disciplinary approach to management. Diagnosis is difficult in some cases because presentation is not in the same manner and showing a phenotypic spectrum. In atypical cases serum immunoglobulins and alfa fetoprotein are normal and telangiectasia is absent. We present a 5.5-year-old boy with progressive cerebellar ataxia and history of repeated sino-pulmonary infections that was homozygote for ataxia-telangiectasia mutated gene and had a giant arachnoid cyst in left hemisphere. It is important to keep in mind those cases with ataxia and repeated sino-pulmonary infections may be ataxia telangectasia patients. Genetic study is helpful and confirms the diagnosis by showing ataxia-telangiectasia mutated gene.