Pediatric Neurology Unit Research Articles

Clinical and Paraclinical Profiles of Children Living with Epilepsy Admitted to Neuro-Psychopathological Center of Kinshasa

<i>Background</i>: children in Africa are mainly affected by epilepsy. This study describes the clinical and paraclinical profiles of epileptic children followed at the Neuro-Psychopathological Center of the University of Kinshasa (CNPP/Unikin). <i>Method</i>: a descriptive cross-sectional study analyzed the files of patients aged ≤ 15 years old admitted to the pediatric neurology unit of the CNPP/Unikin, from December 2015 to December 2020. The variables of interest were sociodemographic, clinical, and evolutionary. Excel 2010 and SPSS version 20.0 were used to manage the data. <i>Results</i>: 118 files of children (70 boys and 48 girls) were collected; 40.7% were between 11 and 15 years old. The mean (SD) age was 8.2 (4.1) years. Sixty percent of admissions were due to epilepsy. Forty-seven (39.8%) patients had idiopathic epilepsy. The majority (83.1%) of seizures is generalized and tonic-clonic seizure is predominant (64.4%). Comorbidities were dominated by neurodevelopmental disorders (8%). Nutritionally, hypocalcaemia (51.7%), hypomagnesaemia (16%) and hypokalemia (1.7%) were observed. Abnormalities during pregnancy and childbirth (23.7%) were the most common etiological factors. The seizures outcome under treatment was characterized, in general, by regression (64.4%). <i>Conclusion</i>: epilepsy is the leading cause of admission in CNPP/Unikin neuropediatric unit, with increasing rates. Nutritional disorders, abnormalities during pregnancy, and childbirth are the dominant etiological factors in these children living with epilepsy. Therefore, health policies must be deeply reassessed, Sub-Saharan Africa, to improve the management and prevention of both epilepsy and identified etiological factors.

Characteristics of patients with drug-resistant epilepsy in a tertiary hospital

Drug-resistant epilepsy has a cumulative incidence between 14 and 20% of patients with epilepsy. It is associated with more comorbidities and with higher healthcare expenditure and impact on quality of life. A retrospective longitudinal descriptive study was performed covering the period from 01/01/2010 to 02/28/2024. All patients with epilepsy seen in the Pediatric Neurology unit of our center were collected and a review of medical records was carried out to collect the characteristics and evolution during the study period. The classification and definitions used were those established by the International League Against Epilepsy. A total of 325 patients with epilepsy were identified, with a cumulative incidence of DRE of 29%. The most frequent etiology was structural both in all patients with epilepsy (22%), and in drug-resistant epilepsy (36%). A statistically significant association was established between refractoriness and genetic and structural causes, and between having status epilepticus and the development of drug-resistant epilepsy. Seventy-nine percent of patients with drug-resistant epilepsy developed epileptic encephalopathy. The most commonly used antiseizure drug was valproic acid (90%), 19% received a ketogenic diet and 4.2% received epilepsy surgery. In our setting the incidence of drug-resistant epilepsy is high, more so than previously described. Genetic and structural etiologies are associated with a higher incidence. Having suffered status epilepticus is associated with refractoriness. A higher incidence was observed in patients with defined electroclinical syndromes, probably influenced by epileptic and developmental encephalopathies.

The Beneficial Effect of Probiotics as an Adjuvant Treatment in Childhood Drug Resistant Epilepsy

Abstract Background Epilepsy is the most frequent chronic neurologic condition in children and every year >4.9 million people develop new-onset epilepsy. The foundation of treatment in epilepsy is drug therapy with anti- seizure medications (ASM). However, about one-third develop drug resistant epilepsy (DRE) due to inability to achieve seizure control despite the use of appropriate ASM. Recent studies indicate that gut dysfunction/dysbiosis is presumably involved in the pathogenesis of and susceptibility to epilepsy. The reconstruction of the intestinal microbiome through probiotic intervention has shown beneficial effects on DRE. Aim of the Work The aim of this study is to study safety and effectiveness of probiotics in controlling epileptic seizures in DRE patients by decreasing frequency and severity of seizures. Patients and Methods This study is a prospective study – Single arm Clinical Trial (Pilot study) conducted on 35 DRE patients at the neurology outpatient clinic, Pediatric Neurology Unit, Ain Shams University, from January to August, 2023, of which 14 (40%) dropped out due to socioeconomic reasons and non-compliance while 21 (60%) patients completed this clinical trial. All the results were calculated based on the 21 patient who completed the study. Patients were supplemented with probiotics for 4 months and assessed pre and post probiotics intake by the following tools: seizure assessment by Chalfont seizure severity scale, a blood sample taken to measure serum sCD14 as an inflammatory marker, an EEG was done and QOLIE-31 was used to asses quality of life. Probiotics (spores of antibiotic resistant Bacillus clausii; Enterogermina 2 Billion @ SANOFI) were used with the following doses daily for 4 months (as per SANOFI’s dose prescribtion): ages 1 year to 2 years: 1 vial per day, ages 2 years to 18 years: 2 vials per day. Results Our study included 21 participants; the median age at the time of the study was 11 years ranging from 2.5 to 15 years of age and the median age of onset of seizure was 7 ranging from 6 months to 12 years. The majority of participants were found to be female 15 (71.4%) and the rest were male 6 (28.4%). This clinical trial shows that probiotics could be a useful supplement in the treatment of DRE where 42.9% of our patients achieved the Therapeutic goal of reduction (≥50%) in the number of seizures & improvement in seizure severity (p-value < 0.001) and frequency (p-value < 0.005). Serum sCD14 levels were withdrawn as an inflammatory marker to understand the potential cause of the improvement in epileptic seizures and their levels were outstandingly improved between pre and post probiotics intake with p-value < 0.001. We also found that quality of life was significantly improved as measured by questionnaire QOLIE31 between pre and post probiotics intake in our patients in all its aspects with total score <0.001. We compared between our 2 main diagnoses (The GDD and Genetic epilepsy) and found that there was higher quality of life in the genetic group than the global developmental delay group with p-value < 0.001 mostly attributing this to the fact that most of the genetic patients had a higher starting point than their counterpart in their developmental history and other aspects. Our patients were also sub-divided into 2 groups based on the effectiveness of the probiotics in improving seizures frequency 50% or more and it was found that the effective group had a lower median age (7 years) than the non-effective group (12.5 years) delineating that younger age responds better to probiotics or management in general. Conclusion This study revealed that probiotics decrease the frequency and severity of seizures and improve the quality of life of drug resistant epilepsy patients. Since this is a pilot study and there are deficient data in this area, more studies are needed to evaluate the applicability, safety and the mechanism of action of probiotics.

Characterizing the clinical, radiological and serological features of children diagnosed with neuromyelitis optica spectrum disorder at pediatric neurology unit

Background. Neuromyelitis optica spectrum disorder is a rare autoimmune disease with a chronic inflammatory demyelinating characteristics that affects the central nervous. The aim of the study is to characterize the clinical, serological and radiological features of neuromyelitis optica disease in a sample of children from Iraq. Methods. A cross-sectional descriptive study has been carried out in the period from August 2019 to September 2020, in pediatric neurology ward in Children Welfare Teaching Hospital, Baghdad. In 12 retrospectively gathered cases, the medical registration of the ward was assessed. A total of 13 patients, aged 3-16 years were included. Results. Among the patients, girls represented 61.5% (8), and the females: males ration was (1.6:1). The patients' ages ranged from 3 to 16 years (mean 9.3 ± 4.02 years). All patients younger than 6 years were AQP-ve, in contrast to 15.3% in those older than 6 years. Negative AQP patients equally distributed between males and females. While females predominated in those with AQP positive status (5,71.4%). Weakness of the limbs and sphincteric disturbance were the first two common clinical features in both AQP positive and negative patients. The three most common brain sites insulted were deep white matter (10, 76.9%), periaqueductal area (5, 38.4%), and brainstem (4, 30.7%). All patients showed long spinal lesions, affecting predominantly the cervico-thoracic (61.53%). Conclusion. The demographic characteristics of the present cohort were comparative to that reported in the literature. Transverse myelitis phenotype was the most common and consistent one. Vomiting was more common in AQP +ve patients.

Fall prevention in a pediatric unit: a best practice implementation project.

Inpatient falls account for 6% to 24% of pediatric safety incidents and can lead to increased length of hospital stay, increased cost of care, and decreased satisfaction with care. A review of a pediatric hematology, oncology, neurology, and rehabilitation unit in a hospital in the southern United States revealed an average of one to two falls monthly. This project aimed to promote evidence-based practices (EBPs) regarding fall prevention in the pediatric unit. This project used the JBI Evidence Implementation Framework to promote EBPs regarding pediatric fall prevention. A baseline audit was conducted to identify gaps between evidence and current practice. Barriers to EBPs were identified, strategies were implemented to promote EBPs, and a follow-up audit was conducted to measure the effectiveness of the improvement strategies. The baseline audit revealed 100% compliance with all criteria except for Criterion 1 (using a validated assessment tool), which scored 0%, and Criterion 5 (communication between nurses at shift handover), which scored 69%. In the follow-up audit, Criterion 1 remained at 0%, but Criterion 5 rose to 82%. This project identified areas of non-compliance with best practice recommendations for the prevention and assessment of pediatric fall risk. Interventions were implemented, with positive results. Further investigation is needed to assess the long-term effectiveness of the interventions. http://links.lww.com/IJEBH/A196.

Severe and rare neurological manifestations following COVID-19 infection in children: A Malaysian tertiary centre experience

IntroductionSince the emergence of COVID-19, we have experienced potent variants and sub-variants of the virus with non-specific neurological manifestations. We observed a surge of the Omicron variant of COVID-19 patients with neurological manifestations where less cases of multisystem inflammatory syndrome in children (MIS-C) were reported. This article describes our experience of children with severe and rare neurological manifestations following COVID-19 infection. MethodsThis is a retrospective observational case series of patients under 18 years old who fulfilled the WHO COVID-19 case definition and were referred to our paediatric neurology unit at Hospital Tunku Azizah Kuala Lumpur. Their demographic data, neurological symptoms, laboratory and supporting investigations, neuroimaging, treatment and outcomes were collected and analysed. ResultsThere were eleven patients with neurological manifestations who fulfilled the WHO COVID-19 case definition. Nine patients presented with seizures and/or encephalopathy, one patient with eye opsoclonus and another patient with persistent limbs myokymia. Based on the history, clinical, electrophysiological and radiological findings, two of them had febrile infection-related epilepsy syndrome, two had acute disseminated encephalomyelitis, two had acute necrotising encephalopathy of childhood, one each had hemiconvulsion-hemiplegia-epilepsy syndrome, acute encephalopathy with bilateral striatal necrosis, hemi-acute encephalopathy with biphasic seizures and reduced diffusion, infection-associated opsoclonus and myokymia. ConclusionsThis case series highlighted a wide spectrum of neurological manifestations of COVID-19 infection. Early recognition and prompt investigations are important to provide appropriate interventions. It is essential that these investigations should take place in a timely fashion and COVID-19 quarantine period should not hinder the confirmation of various presenting clinical syndromes.

A solved puzzle: Familial adult myoclonus epilepsy is a new expansion repeats disorder.

EpilepsiaAccepted Articles SPECIAL ISSUE ARTICLEOpen Access A solved puzzle: Familial Adult Myoclonus Epilepsy is a new expansion repeats disorder Antonietta Coppola, Corresponding Author Antonietta Coppola antonietta.coppola1@gmail.com orcid.org/0000-0002-4845-4293 Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy Correspondence Antonietta Coppola, MD PhD, Department of Neuroscience, Reproductive Sciences and Odontostomatology, Federico II University of Naples, Via Pansini 5, 80131 Napli Italy. Email: antonietta.coppola1@gmail.comSearch for more papers by this authorLeonilda Bilo, Leonilda Bilo Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Naples, ItalySearch for more papers by this authorPasquale Striano, Pasquale Striano orcid.org/0000-0002-6065-1476 Pediatric Neurology and Muscular Diseases Unit, "G. Gaslini" Institute, Genova Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genova, ItalySearch for more papers by this author Antonietta Coppola, Corresponding Author Antonietta Coppola antonietta.coppola1@gmail.com orcid.org/0000-0002-4845-4293 Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy Correspondence Antonietta Coppola, MD PhD, Department of Neuroscience, Reproductive Sciences and Odontostomatology, Federico II University of Naples, Via Pansini 5, 80131 Napli Italy. Email: antonietta.coppola1@gmail.comSearch for more papers by this authorLeonilda Bilo, Leonilda Bilo Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University, Naples, ItalySearch for more papers by this authorPasquale Striano, Pasquale Striano orcid.org/0000-0002-6065-1476 Pediatric Neurology and Muscular Diseases Unit, "G. Gaslini" Institute, Genova Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genova, ItalySearch for more papers by this author First published: 13 February 2023 https://doi.org/10.1111/epi.17545 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/epi.17545. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future. RelatedInformation

Primary headache with onset in childhood and adolescence: natural history and prognostic factors in a Portuguese population.

Headaches are the most frequent neurological disorder in the pediatric population, with great impact on quality of life. This study aims to characterize a cohort of patients followed at a pediatric neurology unit between January 1st 2013 and December 31st, 2021. We reviewed medical records and selected patients with primary headaches and a minimum follow-up of 12 months. A total of 226 patients were included, 54.4% female, with an average age at headache onset of 9 ± 3.5 (3.1-16.5) years; 63.5% were prepubertal. A positive family history of headache was identified in 76.6% of cases and triggers in 63.6%. At first clinical assessment, 45.1% were classified as migraine without aura, 10.6% as migraine with aura, 3.5% tension-type, 8% mixed (tension and migraine), 1.3% other type and 31.4% were unclassifiable. The patients had a median follow-up of 2.4 (1.8-3.3) years. The diagnosis of tension-type headaches remained stable in 75% of the patients and resolved in 25%; 13% of the patients with migraine without aura changed into another type of headache and 17.4% resolved; 44.4% of the patients with migraine with aura turned into another type of headache and 11.1% resolved. Of the variables studied, only duration of headache episode had a significant association with headache remission, with odds ratio 0.16 (p = 0.03; 95% confidence interval: 0.032-0.84). Our study shows that headache type in pediatric population changes over time, especially in those with migraine with aura. The duration of each headache episode was presented as a predictor of headache remission over time.

Pattern of Neurological Disorders among Children Presenting at the Neurology Unit of Tertiary Hospital in Awka

Background: Many children in Nigeria suffer from detrimental, debilitating and lifelong neurologic disorders, many of which are highly preventable using simple, cost-effective interventions. Objective: To examine the pattern of neurological disorders among children presenting at the neurology unit of Chukwuemeka Odumegwu Ojukwu University Teaching Hospital Amaku, Awka, Nigeria. Methods: A retrospective review of the hospital records of children who presented at the Paediatric Neurology Unit between 1st March 2020 and 31st March 2022 was carried out. Data were abstracted using a proforma and analyzed using SPSS Version 21. Results: A total of 138 children aged 0 to 15 years were seen in the unit during the period under review. 115 (83.35%) of these children were diagnosed with chronic neurological disorders. Those with chronic neurological disorders had a male:female ratio of 1.9:1, and the majority (65%) of them were below 5 years of age. The most common presenting complaints were delayed milestones (43.4%), seizures (23.8%), and speech disorders (17.2%). The most typical diagnosis was cerebral palsy (CP) (34.7%), seizure disorder (29.8%) and attention deficit hyperactivity disorder (8.9%). Perinatal asphyxia (47.7%), neonatal jaundice (17.0%) and central nervous system infections (CNS) infections (12.5%) were identified as the major risk factors responsible for these neurologic disorders. Conclusion: Cerebral palsy and seizure disorders constitute the major neurological disorders among children seen in our institution. Efforts should be intensified at reducing the incidence and impact of perinatal asphyxia, neonatal jaundice and CNS infections, identified as the major culprits, to curb the menace of these debilitating lifelong neurologic sequelae.

Lennox Gastaut Syndrome – A strategic shift in diagnosis over time?

BackgroundLennox Gastaut Syndrome (LGS) is an epilepsy syndrome presenting in childhood, classically characterised by a triad of cognitive or developmental impairment, multiple seizure types and EEG features of slow-spike waves (SWW), with or without paroxysmal fast activity (PFA) in sleep. There is increasing scientific opinion in favour of a less rigid approach to LGS diagnosis and this clinical audit attempts to shed light on how the LGS diagnostic criteria used may have changed over time, in a large tertiary paediatric neurology unit (Great Ormond Street Hospital (GOSH), London, UK). MethodsElectronic patient records were reviewed for patients with a diagnosis of LGS made at GOSH within two time periods, 2014-2017 and 2018-2021. Patient demographics, aetiology, clinical features, EEG features, investigation results and medications were reviewed. Findings were compared against the International League Against Epilepsy (ILAE) Diagnostic Manual criteria for LGS diagnosis (the classical triad plus PFA in sleep). Existing GOSH data regarding total number of new epilepsy referrals to GOSH and ICD10 codes (including all epilepsy and epilepsy syndromes) per year were also used to review LGS diagnoses as proportions of new epilepsy referrals and total number of epilepsy diagnoses. Results5 LGS diagnoses were made in 2014-2017 compared to 40 diagnoses made in 2018-2021. There was a steep increase in the number of LGS diagnoses and LGS diagnoses as a proportion of total epilepsy diagnoses in the last 4 years, coinciding with the licensing of cannabidiol for management of LGS in the UK in 2018. There was a much less marked increase in LGS diagnoses as a proportion of epilepsy referrals from 2018 (and an as yet unexplained high proportion in 2014). The 2014-17 cohort fit a more classical LGS diagnostic criteria of the triad plus presence of PFA on sleep EEG (100% in 2014-17 vs 68% in 2018-21), with a more classical preceding history of infantile spasms (80% in 2014-17 vs 23% in 2018-21). Common seizure types were similar in both groups and a male preponderance was also noted in both groups. Cannabidiol use in LGS patients has also increased over time, all prescribed after 2018. ConclusionsDespite the limitations of the small numbers of patients in each group, the results of this clinical audit are in keeping with changing clinical trends in favour of a broader LGS diagnostic criteria. The combination of this less rigid diagnostic criteria, the steep increase in diagnosis seen from 2018 onwards (coinciding with UK cannabidiol licencing for LGS in 2018) and the fact that most of the LGS patients in both groups are currently prescribed cannabidiol, may reflect a strategic shift in attitude towards LGS diagnosis or an increase in referrals for LGS, possibly to help facilitate access to novel treatments.

Childhood Absence Epilepsy Associated With Concomitant Centrotemporal Spikes.

ObjectivesThe coexistence of generalized epileptiform discharges of 3Hz spike-and-wave complexes, which are the hallmark of childhood absence epilepsy (CAE), and centrotemporal spikes, which are characteristic of benign epilepsy with centrotemporal spikes (BECTs) in the same or subsequent EEGs appears to be very rare. Only a few published reports have shown a possible concomitance of CAE and BECTs electrographic changes. The study aimed to analyze electrographic and clinical features of patients with CAE who had concomitant or subsequent EEG features of BECTs.MethodDuring a five-year analysis period (2014-2018), 277 children with BECTs and 93 children with CAE were diagnosed and treated at the pediatric neurology unit of Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. Nine patients were identified to have overlapping EEG findings of both epileptic syndromes. We then analyzed the nine children's clinical features, outcomes, and EEG findings in detail.ResultsThe clinical onset of all our patients aged 5-14 years (six boys, three girls) was characterized by the absence of seizures, either typical (seven children) or atypical (two children). Six out of nine patients presented with concomitant electrographic features of both syndromes, whereas three patients experienced the EEG pattern of two syndromes at different times. All nine children were treated with valproate as the first-line medication, with reasonable seizure control. However, three patients required a second add-on medication. Despite good seizure control, six of our patients had poor school performance and five children had comorbid conditions such as ADHD and learning disability.ConclusionThe coexistence of CAE and BECTS is described in the literature albeit rare. This overlap is mostly in electrographic features with or without the clinical features seen in both syndromes.

Can flunarizine be used routinely as the first option for childhood headache treatment?: Flunarizine and childhood headache.

The prevalence of headache in childhood increases due to environmental factors. Various risk factors in children whose playgrounds are restricted outside and therefore remain inactive. So diagnosis and treatment can be challenging. The aim of this study was to evaluate the experience of flunarizine in childhood headache with a focus on efficacy and success. We conducted a retrospective observational study of 185 pediatric patients at the tertiary pediatric emergency and pediatric neurology unit between May 2018 and May 2020. Patients with headache for >15 days of a month for at least 3 months were included in the study, whether or not receiving treatment. Also, all patients who had an adequate follow-up period were included in the study. All patients were evaluated by history, physical-neurological examination, blood tests, blood pressure, eye examination, and cranial magnetic resonance imaging. All data were evaluated statistically. Ninety-eight (53%) of 185 cases were female and 87 (47%) were male. Average age was 11.4 years (min-max, 4-17). There was family history in 51.3% of the cases. The most frequent applicants were in the autumn season (43%), when schools were opened. Organic causes were hypertension in 1 case, brain tumor in 1 case, and papilledema due to idiopathic intracranial hypertension in 2 cases. The other cases were asked to make a 1-month pain chart and grading according to the visual analog scale. In this process, it was stated that painkillers could be used if needed. At the end of the first month, these patients were reevaluated. Flunarizine treatment was initiated in 95 patients who had to use painkillers for >4 times and who described ≥6 pain score according to the visual analog scale. The treatment was discontinued due to sleepiness and weakness in 2 patients. At the end of the third month, a 50% reduction in headache was observed in 82 cases (86.3%). We used flunarizine as the first choice in all patients and we achieved a high rate of treatment success. Flunarizine can be considered as an alternative option for headache management in terms of low side effects, easy accessibility, and compliance with treatment.

Nutritional Status among Children with Cerebral Palsy attended at Medical University Hospital of Bangladesh

Background: Feeding difficulty among children presented with cerebral palsy (CP) is a common problem which ultimately leads to malnutrition.
 Objective: The purpose of the present study was to assess the nutritional aspect in children with cerebral palsy.
 Methodology: This cross-sectional study was done in the Pediatric Neurology Unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. The children of cerebral palsy aged 1 to 10 years was enrolled in this study. Perinatal, developmental and feeding history was taken meticulously. Nutritional status was assessed by Welcome, Waterlow and WHO classification and on the basis of MUAC and BMI. Neurological assessment was done thoroughly and severity grading was done according to WHO manual 1987.
 Results: Majority (70.0%) of children were in the age group of 1 to 5 years. Most of the children were male (58.0%), born at term (90.0%) and had history of perinatal asphyxia (82.0%). Among enrolled children, 60.0% took adequate calorie. About 32.0% cases and 26.0% patient had difficulty in chewing and swallowing respectively whereas 54.0% faced drooling. Spastic quadriplegia (48.0%) was the most frequent type of Cerebral Palsy followed by mixed type (22.0%). According to Welcome classification, 32.0% cases had undernutrition and 6.0% cases had marasmus. Among children with marasmus, 66.7% cases had quadriplegia. Undernutrition was most frequent in quadriplegic and mixed type of cerebral palsy.
 Conclusion: Malnutrition is a common problem among patients with cerebral palsy. However severe malnutrition is less.
 Journal of Current and Advance Medical Research, January 2022;9(1):42-47

Osteomalacic Myopathy in Children and Adolescents with Vitamin-D Deficiency.

Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents. To describe a cohort of children and adolescents with osteomalacic myopathy. Pediatric neurology unit of a tertiary care hospital. Charts of children and adolescents with osteomalacic myopathy were retrospectively reviewed for demographics, clinical presentation, laboratory investigations, and treatment response. Diagnosis of vitamin-D deficiency was made on the basis of a combination of clinical, biochemical, and radiographic findings. Response to treatment with vitamin-D confirmed vitamin-D deficiency as the cause of myopathic symptoms. Twenty-six children-15 girls and 11 boys aged between 20 months and 19 years-with osteomalacic myopathy were identified. Fifteen (58%) children were between 10 years and 19 years of age. Twenty-one (81%) children presented with myopathic symptoms of progressive walking difficulty, with eventual loss of ambulation in six. Four children came to attention through hypocalcemic seizures. One nonambulatory child with cerebral palsy presented with loss of previously attained ability to roll over and sit. All children had proximal muscle weakness on examination. Fifteen (58%) children had clinical signs of rickets. All the children who underwent biochemical (n = 24) and radiographic (n = 16) investigations had results consistent with vitamin-D deficiency. Only in one child, the diagnosis of osteomalacic myopathy was made on the basis of clinical findings. Response to vitamin D was uniformly good. Vitamin-D deficiency should be considered in the differential diagnosis of proximal myopathy in children and adolescents.

Clinical and laboratory features of children with tremor: a single-center experience.

Tremor is an involuntary, rhythmic, oscillatory movement of body parts around a central point or plane which arises from contraction of antagonist muscles. Evaluation of pediatric patients with tremor can be challenging due to limited population-based studies in children. The aim of this study is to evaluate the demographic, clinical and laboratory features of childhood tremor, retrospectively. Patients under the age of 18years presenting with tremor (n = 111) to the Pediatric Neurology Unit of Kecioren Research and Training Hospital between January 2014 and December 2019 were included in the study. Patients with neuromuscular disease, vertebral pathology or incomplete data in hospital records were not included. Also, benign tremor causes (jitteriness, shuddering attack, etc.) seen in the neonatal and infancy period were excluded from the study as the number of patients was insufficient. Demographic data, type and duration of tremor, accompanying symptoms, chronic diseases and medications, family history, physical and neurological examination, laboratory findings, neuroimaging findings were retrospectively analyzed and recorded. A total of 111 children (59 girls and 52 boys) were included in our study and the female to male ratio was 1.1. The mean age at tremor onset and age at admission to hospital were 13.2 ± 2.8years (range 4-17years), and 14.8 ± 2.0years (range 6-17years), respectively. The most common type of tremor was essential tremor (62.2%), followed by enhanced physiologic (18.9%). None of the patients had acute metabolic disorder. Diagnostic tests revealed the etiology in 12 patients. These were vitamin B12 deficiency in 11 patients and multiple sclerosis in one patient. Drug-induced and task-specific tremors were determined in 4 patients each. It was determined that in patients with a positive family history, tremor appeared or was noticed at a younger age. Most of the cases with tremor can be diagnosed accurately by a detailed medical history, physical and neurological examination. Essential tremor is the most common type of tremor in children. Laboratory tests and imaging methods have limited additional yield in elucidating the etiology. Early recognition of tremor and related signs and symptoms in childhood is important for the detection and treatment of the possible underlying cause.

Health Related Quality of Life in Egyptian Children and Adolescents with Epilepsy

Background: Childhood epilepsy is one of the most significant and prevalent neurological condition in the developing years. The aim of the study was to explore the current status of health related quality of life (HR-QOL) in Egyptian children and adolescents with epilepsy using (WHOQOL-BREF) questionnaire. Patients and Methods: This case control study was carried out in Pediatric Neurology Unit and Outpatient Clinic in Department of Pediatrics at Zagazig University Children's Hospital on 110 children, divided into 2 groups, case group included 55 children with epilepsy and control group included 55 apparently healthy children without epilepsy of matched age, sex and social class. All the children were subjected to history taking, clinical examination and assessment of QOL. Results: There was a significant decrease in all domains and total score of WHOQOL-Bref questionnaire in the case group. There was a significant –ve correlation between age and psychological domain score, also between number of school failure and both psychological and environmental domain. Disease duration and treatment duration were negatively significantly correlated with all QOL domains. A significant +ve correlation was found between social class score and both general and environmental domain. There was a significant decrease in general and environmental domain score between cases with generalized and partial seizers compared to cases with absent seizers. Conclusion: Diminished QOL is common in children with epilepsy than other children. Patients with epilepsy had lower mean scores of all domains of QOL, especially those with frequent fits, those with long duration, and in patients with generalized fits.

Effectiveness of Training Program Regarding Gullian Baree Syndrome Maneuver on Pediatric Nurses’ Knowledge and Practices

Background: Guillain-Barre syndrome is an acquired inflammatory polyneuropathy characterized byrapidly progressive symmetrical flaccid limb weakness and a reflexia.Design. A quasi-experimental design was used to guide this study. The study included a sample of 30 nurseswho work at pediatric intensive care and neurology unit in Assuit University Children Hospital.Data were collected using a questionnaire and an observation checklist, used in pre-post testing to measurethe effectiveness of the training program among nurses.Results: The study revealed deficiency in pre-intervention nurses’ knowledge and practices. Statisticallysignificant improvements on knowledge and practices were demonstrated at the post-intervention time forthe studied nurses. Statistically significant relations were shown between knowledge and practices amongstudied nurses.Conclusion: Implementation of the training program significantly improved nurses’ knowledge and practice.More experienced and aged nurses had better knowledge.

Ocular defect in children with cerebral palsy and its correlation with the types of cerebral palsy

Background: Cerebral palsy (CP) describes a group of permanent disorder of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbance that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy, and secondary musculoskeletal problem. However, no known study has been found on this aspect. Objective of this study was to evaluate the ocular defect in children with cerebral palsy and to correlate with the types of CP.Methods: Study was done in the Paediatric Neurology unit of BSMMU from January 2012 to July 2012. One hundred thirty children with cerebral palsy were studied. The patients were randomly selected who full filled the inclusion criteria and ophthalmological examination was done.Results: Sixty four (64%) of CP patients had pathological finding and 36% percent had normal finding. Most of the abnormalities were squint (strabismus) (40%) and refractive error (36.9%). Most of the ocular defects were found in spastic cerebral palsy, mainly in spastic quadriplegia (34.6%) and spastic diplegia (29.2%).Conclusions: Ocular defect like squint and refractive error common associations of cerebral palsy. Spastic quadriplegic and diplegic children had more ocular defects.

Exploring the Psychological Risk Factors in Children with Epilepsy

There are many factors that aggravate the clinical picture of children diagnosed with epilepsy. Through this study we will explore risk factors related to disease characteristics, cognitive impairments, intelligence and behavioral problems in children with epilepsy. Methods: Based on the medical data of children diagnosed with epilepsy being treated at the Pediatric Neurology Unit, University Hospital of Pristina, and the “Hope” Psychological Outpatient Center in Kosovo, about 100 epileptic children, 55 female and 45 male, aged between 6 and 11 years of school age were administered the Montreal Cognitive Assessment scale (MoCA), Raven’s Standard Progressive Matrices (IQ), and Child Behavior Checklist (CBCL) tests. Results: Children participating in the study reveals a predomination of the generalized epilepsy, which continue to be treated with anti-epileptics for more than 3 years. From them 67% did not show the presence of another co-neurological deficit. Neurological tests show evidences of moderate EEG changes in some children and MRI registrations in 65% of children are unchanged and continue to be treated with 1 or 2 antiepileptic drugs for being under control. Intelligence, cognitive and behavioral problems in cases where they were associated with a number of neuropsychological characteristics increase the risk of a disease aggravation and compromise their overall development. Discussion: We can imply that based on the severity of these concomitant factors of the epileptic conditions, they will constitute a high-risk factor for cognitive problems, low intelligence and the emergence of a number of internalizing and externalizing problems of the child with epilepsy. Conclusions: As a risk factor that increases the level of difficulty of epileptic children in some contexts, including general functioning, school, family and social context, the presence of neurobiological and neuropsychological factors such as issues in internalizing and externalizing behaviors, problems in the cognitive field and the IQ are seen, which is also expected to affect the overall development of children’s quality of life.

The role of clinical signs in the diagnosis of papilledema: development of an algorithm.

The aim of this study was not only to emphasize the role of clinical signs as well as ophthalmologic evaluation for accurate and differential diagnosis of papilledema (PE), but also to present an instructive algorithm that would help to eliminate unnecessary examinations and treatments. The files of 43 patients (ages 0-18) diagnosed with PE were retrospectively reviewed. The study included 25 patients from our pediatric neurology outpatient clinic, who were thought to have PE, and 18 patients, who were referred from the external centers to our hospital with a pre-diagnosis of PE. Of the 43 patients, 28 had PE, 8 had pseudopapilledema (PPE), and 7 had optic nerve pathologies (ONP). For patients who applied directly to our pediatric neurology unit, a margin of error of 8% was detected based on only a simple ophthalmologic examination and an evaluation of clinical findings. For the patients who were forwarded to our pediatric neurology unit from the external centers without examining any clinical findings and with no details, the margin of error was 72%. For patients with suspected PE, advanced ophthalmologic opinion is a necessary requirement before invasive radiological examinations are used. When the ophthalmologic evaluation is properly elaborated, the distinction can be made more clearly by using noninvasive methods. In order to determine the gold standard in terms of the methods used in the evaluation of patients who are not clinically diagnosed, new prospective studies with more patients should be planned.