Iranian Journal of Child Neurology最新文献

Objectives: Seizure is a common sign in neonates hospitalized in the neonatal intensive care units (NICU) that may lead to morbidity and mortality. Most neonatal seizures are subclinical. Conventional EEG (cEEG) is the gold standard for detecting and monitoring seizures but is not widely available. Amplitude-integrated electroencephalography (aEEG) has been used for over a decade to evaluate infants with seizures. In this study, we tried to determine the efficacy of aEEG as a widely available diagnostic tool in diagnosing seizures.

Materials & methods: All cases with seizures or suspicious seizures were admitted to the NICU of the Children's Medical Center for one year. cEEG and aEEG were performed for these infants. aEEG was recorded for at least six hours with a description of the tracing. Clinical information, outcomes, and questionnaires (patient information) were recorded in detail. The obtained data were analyzed with the SPSS version 24 software.

Results: Eleven out of twenty-five aEEG recordings were abnormal; other patients showed normal aEEGs. The most common clinical and neurological manifestations were seizure (68%) and hypotonia (28%); the mortality rate was 12%. No significant correlation was observed between aEEG findings and gender, age, familial relation, outcome, ultrasound result, type of seizure, and underlying disease.

Conclusion: Studies showed variable sensitivity and specificity values for aEEG. aEEG cannot be recommended as the only way to diagnose and manage seizures in neonates. However, Good accessibility and ease of working with aEEG promote a tendency to use this procedure as a screening tool.

Objectives: Development refers to the progressive enhancement of skills and functional capacity, i.e., qualitative changes in the child's functions. The process of development begins before birth and continues throughout life. The present study aims to evaluate the effectiveness of the "Care for Child Development (CCD)" program on 4-42 months children's developmental skills in orphanages.

Materials and methods: In this study, two orphanages in the capitals of East and West Azerbaijan provinces were selected using the convenience sampling technique, and thirty children were included. Then, they were randomly divided into two intervention and control groups (each group, N=15). Next, after obtaining consent from the head of the orphanages, a group of volunteers from the healthcare center performed the CCD program, considering children's chronological ages (4 to 42 months), for three sessions a week, with each session lasting two hours and it lasted for three months. At the end of the intervention process, the Bayley Scale of Infant and Toddler Development 3^rd version (BSID-III) and the Ages and Stages Questionnaire-II (ASQ) were completed for the two intervention and control groups to compare them in the cognitive, motor, communication, and personal-social domains.

Results: Comparing the two control and intervention groups using the T-test (difference in mean) indicates that except for the domain of cognitive skills (Bayley: P-value = 0.176), there was statistically a significant difference between the two groups in communication (ASQ: P-value = 0.001; Bayley: P-value = 0.003), motor (ASQ: P-value = 0.000; Bayley: P-value = 0.009), and personal-social (ASQ: P-value <0.000) skills.

Conclusion: In the present study, it was concluded that it is required to apply interventions, including standard ones such as the CCD program in environments like orphanages, to enhance the developmental skills of those children living in them.

Objective: The imbalance of phenylalanine (PA) to tyrosine level and decreased dopamine brain level in patients with phenylketonuria (PKU) may have a role in their susceptibility of them to attention-deficit/hyperactivity disorder (ADHD). This study aimed to evaluate the frequency of ADHD in referred patients to PKU Clinic in Yazd, Iran.

Materials & methods: In this cross-sectional analytical study, all patients older than three years with PKU who were referred to the PKU Clinic of Shahid Sadoughi Hospital, Yazd, Iran, in 2018 were evaluated, and ADHD symptoms in them were assessed via parent face-to-face interview. The patients were diagnosed with ADHD if they scored at least of 20 on ADHD diagnostic rating scale via parent interview based on DSM-VI criteria.

Results: Fourteen boys and 21girls with a mean age of 9.55±1.8 years were evaluated. 51.5% of those diagnosed with PKU had ADHD. Accordingly, ADHD was more frequent in girls (77.8% vs. 41% in boys, P=0.03). The mean age of diagnosis of PKU was significantly higher in patients with ADHD (52.54±15.65 months vs. 29.75±9.65 months, P = 0.03). The mean of PA level in the last six months (15.59±5.95 vs. 8.72+5.18, P= 0.005) and mean of the last six PA levels (14.76±4.71 vs. 8.96±3.86, P= 0.03) were significantly higher in ADHD group.

Conclusion: The prevalence of ADHD in phenylketonuria patients in the present study was much more than in other studies. Late diagnosis of PKU and long-term high PA blood and brain level might be associated with increased neonatal screening. Hence, regular follow-up and continuous evaluation of patients with PKU for ADHD symptoms should be performed.

Objectives: Multiple sclerosis (MS) is among the most prevalent chronic immune-mediated inflammatory diseases. If MS onset is under 18, it is defined as pediatric-onset MS (POMS). This study aimed to determine the clinical and epidemiological aspects of POMS.

Materials & methods: This population-based study was conducted in East-Azerbaijan (EA) province and concerned POMS patients. The data concerning almost all of the POMS patients of the province was gathered from the only MS registry center in the university hospital of the Tabriz University of Medical Sciences by the end of 2017. The diagnosis of patients was based on McDonald's criteria.

Results: Out of 2976 total cases of MS, eighty-five (2.85%) were POMS. The overall regional prevalence of POMS was 11.67 per 100,000 (95% CI:9.43-11.43). Sixty-seven cases were female (prevalence: 18.94 per 100,000 [95% CI:14.91-24.07], and eighteen were male (prevalence: 4.80 per 100,000 [95% CI:3.03-7.62]. The crude regional incidence in 2017 was 1.37/100,000 (95% CI:0.74-2.55). The mean age of onset was 15.81±1.33 years, with a minimum age of 12. 71.76% of the patients were diagnosed in the 16- or 17-years old age group. 7.05% had a positive family history, and 87.5% of the patients diagnosed the disease promptly. The most common first clinical presentations were blurred vision (43.75%), sensory (28.12%), cerebellar (15.62%), and brainstem (9.37%) symptoms.

Conclusion: POMS is not a rare condition, and it mainly affects females. POMS prevalence increases significantly after age 15 years old, and the first manifestation of the disease is usually blurred vision.

Objectives: Premature birth causes some permanent or temporary abnormalities in the hearing system of the newborn. Inadequate development of the central auditory nervous system and balance, as well as the delay in the formation of the nerve myelin, can be the cause of many hearing disorders, including permanent or temporary auditory neuropathy spectrum disorder (ANSD). The present study aims to identify and understand developmental delay disorder in the hearing system of infants and investigate the possibility of temporary auditory neuropathy in infants.

Materials & methods: In this comparative analytical study, twenty premature infants were randomly selected for hearing tests using auditory brainstem response and transient otoacoustic emissions at the time of discharge and three months after the first evaluation. The different components of these tests were analyzed and compared before and after developing the auditory system.

Results: The OAEs test showed a signal-to-noise ratio above six dB with appropriate amplitudes in all infants. The grand average waveform of the ABR showed a significant difference between the amplitudes of waves III and V before and after maturation in both ears (p<0.05). In addition, the absolute latency of waves, specifically III and V, showed a significant difference between the two assessment times (0.05).

Conclusions: The present study confirmed the occurrence of temporary ANSD or delayed maturation in premature infants following the lack of complete growth and myelination of auditory nerve fibers. There is a need to determine the hearing status of premature infants by frequent examinations and prevent any unnecessary prescription of amplifications.

Objectives: This study aimed to investigate the relationship between fine motor skills and reading components and compare the fine motor skills of normal children and children with dyslexia.

Materials & methods: In this study, 47 children with dyslexia children and 47 normal children in the first to the third grade of elementary school in the age range of seven to nine years were examined with the Bruininks-Oseretsky Test of Motor Proficiency and NEMA reading test. Data were analyzed using Kolmogorov-Smirnov, Shapiro-Wilk, U Mann-Whitney methods, multiple linear regression, and Spearman correlation coefficient.

Results: The results showed children with dyslexia were significantly weaker in fine motor skills than normal children (p <0.001). In addition, a relationship existed between the subtest of response speed and reading accuracy in normal children, but it was in normal children. A significant relationship was found between visual-motor control subtests and Upper-limb speed and dexterity with reading accuracy and speed. None of the motor subtests were related to reading comprehension. In children with dyslexia, no association was found between motor subtests and reading components.

Conclusion: Seemingly, fine motor skills can be used as an essential factor along with other effective factors in improving the reading skills of children with reading disabilities.

Herpes simplex encephalitis (HSE), caused by herpes simplex virus type 1 (HSV-1), is the most common cause of severe sporadic encephalitis worldwide. HSE is occasionally accompanied by the recurrence of clinical symptoms that usually occur a few weeks following the initial infection. According to recent studies, the recurrence can be due to a secondary autoimmune mechanism rather than the virus invasion. One of the most common etiologies for autoimmunity is Anti-N-Methyl-D-Aspartate receptor encephalitis. This disorder is a treatable autoimmune encephalitis manifesting as movement disorder or neuropsychological involvement.

Case presentation: The article pertains to the presentation of an 18-month-old infant with a primary diagnosis of herpetic encephalitis who was re-admitted to the hospital shortly after discharge with restlessness, speech disorder, and abnormal movements. The movements were predominantly choreiform and disappeared during sleep. Brain MRI revealed abnormal predominance in the left temporoparietal regions with encephalomalacic changes in some areas in favor of sequella of previous encephalitis in addition to recent right temporal involvement of sequella of previous encephalitis. The polymerase chain reaction test of cerebrospinal fluid for herpes simplex infection was negative. Therefore, the possibility of autoimmune encephalitis was raised. More laboratory examinations revealed that the Anti-N-Methyl-D-Aspartate receptor antibody level was significantly elevated in cerebrospinal fluid. Thus, the diagnosis of Anti-N-Methyl-D-Aspartate receptor encephalitis was established.

Conclusion: Relapsing symptoms after herpes simplex virus encephalitis, especially with movement disorders, should raise a high clinical suspicion of Anti-N-Methyl-D-Aspartate receptor encephalitis in children. Therefore, clinicians should be cautious of its occurrence in infants; despite its rarity in that age group.

Various reports of neurological manifestations of SARS-COV-2 infection after the virus outbreak are available, including anosmia, seizures, acute flaccid myelitis, Guillain-Barré syndrome (GBS), and encephalitis. Most of the literature has focused on the respiratory manifestation of SARS-CoV-2 infection in adults, but recent evidence showed that it is not confined to the respiratory tract. This report is about a rare variant of GBS acute motor axonal neuropathy (AMAN) in a child due to COVID-19 infection An 11 years old boy was referred to the hospital with a history of three-day lasting mild fever, and gastroenteritis, two weeks before starting symptoms. He was presented with progressive ascending weakness, paresthesia, and areflexia in four limbs four days ago. Nasopharyngeal swab polymerase chain reaction (PCR) was positive for SARS-CoV-2. The electrodiagnostic finding was compatible with acute generalized axonal motor neuropathy, and imaging revealed thoracolumbar syrinx and nerve root enhancement in lumbosacral MRI. Other lab tests were normal. GBS and its variant are one of the manifestations of SARS-CoV-2 in children. Children with an unexplained neurological process should be tested for SARS-CoV-2.

Autoimmune encephalitis is an inflammatory condition caused by different factors, including viral infections, diagnosed after ruling out other causes of encephalitis. The current study reported novel autoimmune encephalitis in an 11-year-old girl who presented with seizures, cognitive dysfunction, and neurological impairments. During the admission, the researchers observed high levels of anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in the cerebrospinal fluid (CSF). Besides, she had positive anti-COVID-19IgG. Therefore, the diagnosis of COVID-19-induced autoimmune encephalitis was specific. The patient received anti-epileptic, anti-viral drugs, IVIG, and rituximab and was discharged with remission. The case diagnosis was made by anti-NMDAR antibodies, which highlights the importance of this diagnostic tool. Similar cases have been reported earlier, but the point of this case was her younger age compared to the previous cases and her developing neurological deficit before COVID-19 presentations.

Objectives: The present study aimed to evaluate the serum proteome of women with obsessive-compulsive disorder (OCD)/bipolar disorder (BP) compared to pure OCD subjects and healthy controls.

Materials & methods: Serum proteome of women with OCD/BP, pure OCD individuals, and healthy controls were subjected to 2DE-based proteomics accompanied with MALDI-TOF-TOF mass spectrometry. Further evaluation of the identified protein spots with the significance of p<0.05 and fold≥1.5 was done by applying protein interaction mapping via Cytoscape v. 5.3.1 and its plugins.

Results: The results indicate that vitamin D binding protein (GC) and haptoglobin spots (HP) significantly changed expression in OCD and OCD/BP with different expression patterns. These identified spots may contribute to OCD/BP and act as differentially recognized biomarkers comparing pure OCD and OCD/BP.

Conclusion: The Findings imply that these proteins in the serum of the patients could be potential distinguishable biomarkers in clinical usage after related validation experiments. Therefore, this study provides a preliminary evaluation to understand OCD/BP proteome behavior better.