Optic neuritis in Turkish children and adolescents: A multicenter retrospective study.

BACKGROUND: Various etiologies may underlie optic neuritis, including autoantibody-mediated disorders described in the last decade. We re-examined demographic, clinical, laboratory features and prognostic factors in pediatric patients with autoimmune optic neuritis according to current knowledge. METHODS: Cases of pediatric ON from 27 centers in Türkiye diagnosed between 2009 and 2022 were included for retrospective evaluation. RESULTS: The study included 279 patients, 174 females and 105 males, with a female-to-male ratio of 1.65. The average age at onset was 12.8 ± 3.4 years, and mean follow-up, 2.1 years (range: 1-12.1 years). Patients <10 years old were grouped as "prepubertal" and those ≥10 years old as "others". The diagnoses made at the end of follow-up were multiple sclerosis associated optic neuritis (n = 90, 32.3 %), single isolated optic neuritis (n = 86, 31 %), clinically isolated syndrome (n = 41, 14.7 %), myelin oligodendrocyte glycoprotein antibody associated optic neuritis (n = 22, 7.9 %), and relapsing isolated optic neuritis (n = 18, 6.5 %). Predominant diagnoses were myelin oligodendrocyte glycoprotein antibody associated optic neuritis and acute disseminated encephalomyelitis associated optic neuritis in the prepubertal group and multiple sclerosis associated optic neuritis in the older group. Recurrences were observed in 67 (24 %) patients, including 28 with multiple sclerosis associated optic neuritis, 18 with relapsing isolated optic neuritis, 11 with myelin oligodendrocyte glycoprotein antibody associated optic neuritis, 8 with aquaporin-4 antibody related optic neuritis, and 2 with chronic relapsing inflammatory optic neuropathy. Recurrences were more common among female patients. Findings supporting the diagnosis of multiple sclerosis included age of onset ≥ 10 years (OR=1.24, p = 0.027), the presence of cranial MRI lesions (OR=26.92, p<0.001), and oligoclonal bands (OR=9.7, p = 0.001). Treatment in the acute phase consisted of intravenous pulse methylprednisolone (n = 46, 16.5 %), pulse methylprednisolone with an oral taper (n = 212, 76 %), and combinations of pulse methylprednisolone, plasmapheresis, or intravenous immunoglobulin (n = 21, 7.5 %). Outcome at 12 months was satisfactory, with 247 out of 279 patients (88.5 %) demonstrating complete recovery. Thirty-two patients exhibited incomplete recovery and further combination treatments were applied. Specifically, patients with relapsing isolated optic neuritis and aquaporin-4 antibody related optic neuritis displayed a less favorable prognosis. CONCLUSION: Our results suggest optic neuritis is frequently bilateral in prepubertal and unilateral in peri­ or postpubertal patients. Age of onset 10 or older, presence of oligoclonal bands, and brain MRI findings reliably predict the development of multiple sclerosis. The risk of developing multiple sclerosis increases mostly during the second and third years of follow-up. Relapsing isolated optic neuritis remains a separate group where the pathogenesis and outcome remain unclear. Investigation of predisposing and diagnostic biomarkers and long follow-up could help to define this group.

Effects of levetiracetam treatment on autonomic nervous system functions in pediatric epilepsy patients.

BACKGROUND: This study investigated the effects of levetiracetam (LEV) treatment on cardiac rhythm and heart rate variability. METHODS: The study included two groups of patients diagnosed with non-lesional epilepsy who had not yet been treated and who presented to the outpatient pediatric neurology clinic at Van Training and Research Hospital, Van, Turkey, between 2019 and 2020. The heart rate variability (HRV) of 47 patients in the first group, before and at the 3rd month of treatment, and intravenous (IV) LEV loading in 13 patients in the second group was evaluated by Holter electrocardiography (ECG). RESULTS: It was determined that the values of triangular index, standard deviation of the RR intervals over a 24-hour period (SDNN), standard deviation of all 5-minute mean RR intervals (SDANN), mean of standard deviations of all normal RR intervals (SDNNI), the percentage of RR intervals with >50-millisecond variation (PNN50), and the square root of mean squared differences of successive RR intervals (RMSSD). HRV of 47 patients under LEV treatment significantly increased in the 3rd month of treatment compared to baseline (p < 0.05). No difference was found in HRV between the intravenous loading and the control group (p > 0.05). CONCLUSIONS: Our study suggests that the sympathovagal balance before treatment in the patient group is in favor of the sympathetic nervous system and that the sympathovagal imbalance improves after treatment. Our results show that LEV monotherapy and loading have no negative effect on HRV and potential cardiac arrhythmia risk in children with epilepsy.

Pediatric-Onset Chronic Inflammatory Demyelinating Polyneuropathy: A Multicenter Study.

BACKGROUND: To evaluate the clinical features, demographic features, and treatment modalities of pediatric-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Turkey. METHODS: The clinical data of patients between January 2010 and December 2021 were reviewed retrospectively. The patients were evaluated according to the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society Guideline on the management of CIDP (2021). In addition, patients with typical CIDP were divided into two groups according to the first-line treatment modalities (group 1: IVIg only, group 2: IVIg + steroid). The patients were further divided into two separate groups based on their magnetic resonance imaging (MRI) characteristics. RESULTS: A total of 43 patients, 22 (51.2%) males and 21 (48.8%) females, were included in the study. There was a significant difference between pretreatment and post-treatment modified Rankin scale (mRS) scores (P < 0.05) of all patients. First-line treatments include intravenous immunoglobulin (IVIg) (n = 19, 44.2%), IVIg + steroids (n = 20, 46.5%), steroids (n = 1, 2.3%), IVIg + steroids + plasmapheresis (n = 1, 2.3%), and IVIg + plasmapheresis (n = 1, 2.3%). Alternative agent therapy consisted of azathioprine (n = 5), rituximab (n = 1), and azathioprine + mycophenolate mofetil + methotrexate (n = 1). There was no difference between the pretreatment and post-treatment mRS scores of groups 1 and 2 (P > 0.05); however, a significant decrease was found in the mRS scores of both groups with treatment (P < 0.05). The patients with abnormal MRI had significantly higher pretreatment mRS scores compared with the group with normal MRI (P < 0.05). CONCLUSIONS: This multicenter study demonstrated that first-line immunotherapy modalities (IVIg vs IVIg + steroids) had equal efficacy for the treatment of patients with CIDP. We also determined that MRI features might be associated with profound clinical features, but did not affect treatment response.

[Severe Encephalitis with Human Herpes Virus-7 (HHV-7) Associated Transient Splenial Lesion in an Immunocompetent Child: A Case Report]. / Immün Sistemi Saglam Çocukta Insan Herpes Virüs-7 (HHV-7) Iliskili Geçici Splenial Lezyon ile Birlikte Agir Ensefalit: Olgu Sunumu.

Encephalitis is the inflammation of the brain parenchyma accompanied by mental or behavioral neurological dysfunction, sensory or motor deficits, speech or movement disorders, and seizure. Encephalitis is an acute, life-threatening emergency that requires prompt recognition and a systematic approach for appropriate management. Human herpes virus (HHV-7) is one of the causative agents of encephalitis. In this report, a three years and six months old girl admitted to the hospital with the complaints of fever, cough, gushing vomiting, and altered consciousness, with fever, neck stiffness and blurred consciousness in her physical examination, and positive HHV-7 DNA polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF) was presented. The CSF biochemistry of the patient was normal, and lymphocytic pleocytosis was detected in the CSF. Electroencephalography of the case revealed a cerebral dysfunction and hyperexcitability due to background activity abnormalities, and a cytotoxic transient lesion of the splenium in cranial magnetic resonance imaging. A 14-day foscarnet treatment was given to the patient after she progressed under empirical acyclovir treatment and HHV-7 was found to be the causative agent in the CSF. The patient was cured with the treatment and was followed up on an outpatient basis without any sequelae. In general, HHV-7 is estimated to be a common cause of pediatric acute encephalitis cases. It has been observed in the literature that almost all of the HHV-7-associated encephalitis cases occur after the age of six years, suggesting that HHV-7 causes neurological disease in children as a late infection. This case was three years and six months old and it was thought that she had encephalitis during primary infection. With this case report, we contributed to the literature by presenting a case of encephalitis in an immunocompetent pediatric patient with a transient splenial lesion associated with HHV-7, which progressed with empirical acyclovir treatment and responded to foscarnet treatment.

Evaluation of developmental profiles of children with hydrocephalus / Evaluación de perfiles de desarrollo de niños con hidrocefalia

Objective The objective of this study was to compare the developmental characteristics of children with hydrocephalus with those of healthy children. Material and methods A total of 109 children aged between 2 and 46 months were included in the study, 54 patients diagnosed with hydrocephalus and 55 healthy children were evaluated with demographic data forms and Denver Developmental Screening Test II. Results The mean personal–social (p<0.001), fine motor-adaptive (p<0.001), language (p<0.001), and gross motor subscale scores were significantly lower in children with hydrocephalus than in the control group. Personal–social (p=0.002) and gross motor (p=0.029) subscale scores were significantly lower in children with obstructive hydrocephalus than communicating hydrocephalus. There was a significant negative correlation between language scores and ages of the children with hydrocephalus (r=−0.350, p=0.009). It was found that children with obstructive hydrocephalus carry a 6.7 folds higher risk of experiencing problems in terms of personal–social development compared to those with communicating hydrocephalus (p=0.011). Conclusion We found that patients with hydrocephalus were developmentally retarded compared to the healthy control subjects. Retardation was the most prominent in the obstructive group. Our results showed that neurodevelopmental follow-up should be carried-out regularly in pediatric patients with hydrocephalus, and early intervention should be started in necessary cases. (AU)

Objetivo El objetivo de este estudio fue comparar las características del desarrollo de niños con hidrocefalia con las de los niños sanos. Materiales y métodos Se incluyeron en este estudio un total de 109 niños con edades entre dos y 46 meses. Del total, 54 pacientes diagnosticados con hidrocefalia y 55 niños sanos fueron evaluados con formularios de datos geográficos y test screening de desarrollo Denver II. Resultados El promedio de los puntajes de las subescalas: desarrollo personal y social (p<0,001), motricidad fina-adaptativa (p=0,001), lengua (p=0,001) y motricidad gruesa fueron significativamente menores en niños con hidrocefalia que en el grupo control. Los puntajes de las subescalas desarrollo personal y social (p=0,002) y motricidad gruesa (p=0,029) resultaron significativamente menor en niños con hidrocefalia obstructiva que en hidrocefalia comunicante. Hubo una correlación negativa significativa entre los puntajes de lengua y las edades de los niños con hidrocefalia. (r=−0,350, p=0,009). Se encontró que los niños con hidrocefalia obstructiva portaban 6,7 veces un mayor riesgo de experimentar problemas en términos de desarrollo personal-social comparado con aquellos con hidrocefalia comunicante (p=0,011). Conclusión Encontramos que pacientes con hidrocefalia presentaron un retraso madurativo en comparación con los sujetos de control sanos. El retraso madurativo fue lo más prominente en el grupo obstructivo. Nuestros resultados mostraron que debería realizarse un seguimiento de neurodesarrollo regularmente en pacientes pediátricos con hidrocefalia y una intervención temprana debería comenzar en los casos que lo requieran. (AU)

The neurodevelopmental profile of healthy children with premature anterior fontanel closure.

BACKGROUND: We aimed to assess the neurodevelopmental status of healthy children with premature anterior fontanel closure. METHODS: This retrospective observational study was conducted on 40 (20 M, 20 F) children admitted to Mersin University Pediatric Neurology Outpatient Clinic between 2015-2020 with complaints of premature fontanel closure. Patients with dysmorphic features, microcephaly, craniosynostosis, hypoxic-ischemic sequelae, infections, metabolic disorders, intracranial hemorrhage, epilepsy, endocrine problems, additional congenital anomalies, intrauterine growth retardation (IUGR), prematurity, and postmaturity were excluded. The Denver II and Bayley III tests were applied to all patients and controls. RESULTS: The Denver II identified retardations in gross motor skills (p = 0.015) and personal-social skills (p = 0.042) and Bayley III in cognitive (p = 0.030) and motor skills (p = 0.007) in the study group. None of the participants in the study group had neurodevelopmental retardation, according to the Bayley III normal standards. DISCUSSION: Our results suggest that children with premature fontanel closure may develop motor retardation. These children should, therefore, be closely monitored for neurodevelopmental aspects.

Evaluation of developmental profiles of children with hydrocephalus.

OBJECTIVE: The objective of this study was to compare the developmental characteristics of children with hydrocephalus with those of healthy children. MATERIAL AND METHODS: A total of 109 children aged between 2 and 46 months were included in the study, 54 patients diagnosed with hydrocephalus and 55 healthy children were evaluated with demographic data forms and Denver Developmental Screening Test II. RESULTS: The mean personal-social (p<0.001), fine motor-adaptive (p<0.001), language (p<0.001), and gross motor subscale scores were significantly lower in children with hydrocephalus than in the control group. Personal-social (p=0.002) and gross motor (p=0.029) subscale scores were significantly lower in children with obstructive hydrocephalus than communicating hydrocephalus. There was a significant negative correlation between language scores and ages of the children with hydrocephalus (r=-0.350, p=0.009). It was found that children with obstructive hydrocephalus carry a 6.7 folds higher risk of experiencing problems in terms of personal-social development compared to those with communicating hydrocephalus (p=0.011). CONCLUSION: We found that patients with hydrocephalus were developmentally retarded compared to the healthy control subjects. Retardation was the most prominent in the obstructive group. Our results showed that neurodevelopmental follow-up should be carried-out regularly in pediatric patients with hydrocephalus, and early intervention should be started in necessary cases.

Re-examining the characteristics of pediatric multiple sclerosis in the era of antibody-associated demyelinating syndromes.

BACKGROUND: The discovery of anti-myelin oligodendrocyte glycoprotein (MOG)-IgG and anti-aquaporin 4 (AQP4)-IgG and the observation on certain patients previously diagnosed with multiple sclerosis (MS) actually have an antibody-mediated disease mandated re-evaluation of pediatric MS series. AIM: To describe the characteristics of recent pediatric MS cases by age groups and compare with the cohort established before 2015. METHOD: Data of pediatric MS patients diagnosed between 2015 and 2021 were collected from 44 pediatric neurology centers across Türkiye. Clinical and paraclinical features were compared between patients with disease onset before 12 years (earlier onset) and ≥12 years (later onset) as well as between our current (2015-2021) and previous (<2015) cohorts. RESULTS: A total of 634 children (456 girls) were enrolled, 89 (14%) were of earlier onset. The earlier-onset group had lower female/male ratio, more frequent initial diagnosis of acute disseminated encephalomyelitis (ADEM), more frequent brainstem symptoms, longer interval between the first two attacks, less frequent spinal cord involvement on magnetic resonance imaging (MRI), and lower prevalence of cerebrospinal fluid (CSF)-restricted oligoclonal bands (OCBs). The earlier-onset group was less likely to respond to initial disease-modifying treatments. Compared to our previous cohort, the current series had fewer patients with onset <12 years, initial presentation with ADEM-like features, brainstem or cerebellar symptoms, seizures, and spinal lesions on MRI. The female/male ratio, the frequency of sensorial symptoms, and CSF-restricted OCBs were higher than reported in our previous cohort. CONCLUSION: Pediatric MS starting before 12 years was less common than reported previously, likely due to exclusion of patients with antibody-mediated diseases. The results underline the importance of antibody testing and indicate pediatric MS may be a more homogeneous disorder and more similar to adult-onset MS than previously thought.

Evaluation of micronutrient levels in children with cerebral palsy.

BACKGROUND: Many studies evaluating the nutritional status of children with cerebral palsy (CP) have focused on energy requirements and protein intake. The present work aimed to assess nutritional status and micronutrient levels of children with (CP). METHODS: This multicenter, cross-sectional and observational study was conducted in 10 different cities in Turkey. Data were available for 398 participants. Anthropometric measurements, feeding mode, nutritional status, and micronutrient levels were evaluated. RESULTS: The study was conducted with 398 participants (303 patients and 95 healthy controls). Statistical analysis showed that according to the Gomez Classification, weight-for-age (WFA) revealed malnutrition in 92.6% of children with CP, based on Centers for Disease Control and Prevention percentiles. Measurements of micronutrient levels showed that zinc levels were low in patients, whereas vitamin A levels were low in controls. Phosphorous and manganese levels were significantly lower in malnourished children than in typical children. The results revealed that children consuming enteral nutrition solutions had higher selenium and lower zinc levels than non-consumers. CONCLUSIONS: Malnutrition is not only a protein- or calorie-based problem; micronutrient deficiencies might cause severe health problems. Children with chronic neurological disabilities must be carefully evaluated for these issues. Therefore, nutritional interventions should be adapted to nutrition.

Developmental evaluation in children experiencing febrile convulsions.

BACKGROUND: The objective of this study was to determine the effect of febrile convulsion (FC) on neuromotor development. METHODS: Data of 325 patients, who were followed up at our outpatient clinic and diagnosed with FC between January 2012 and December 2018, were retrospectively evaluated. Of these patients, 203 underwent the Denver Developmental Screening Test II (DDST II) and were included in the study as the patient group and 100 healthy children as the control group. RESULTS: Of the study group, 84 (41.4%) were girls and 119 (58.6%) were boys (B/G: 1.4). Of all patients, 163 (80.3%) were diagnosed with simple FC, 22 (10.8%) with complicated FC, and 18 (8.9%) with FC+. There was no significant relationship found between FC subtypes and gender, family history of FC, family history of epilepsy, iron (Fe) deficiency, and Fe deficiency anemia. DDST II subtest points were significantly lower in all developmental areas in the patient group when compared to the controls (p < 0.001), while suspected and abnormal test results were higher in all developmental areas in the patient group compared to the controls (p=0.01). It was also determined that the language points were lower as the age of first seizure increased (r=- 0.319, p < 0.01). CONCLUSIONS: Although FC is known to usually having a good prognosis, the low DDST II test results measured in this study indicated that the FC may pose a developmental risk and patients with FC should be followed up in terms of developmental features. Because of the retrospective nature of the study, there was no `preconvulsion` developmental evaluation. This is a major limitation of our study.

Views and experiences of family physicians about Syrian refugee patients in Turkey: a qualitative research.

AIM: The aim of this study was to describe the attitudes, views and solution proposals of family physicians (FPs) about primary healthcare problems of Syrian refugee patients. This study would be the very first study for Turkey that evaluates the attitudes, views and solution proposals of FPs about primary healthcare problems of Syrian refugee patients. BACKGROUND: Following the anti-regime demonstrations that started in March 2011, the developments in Syria created one of the biggest humanitarian crises in the world and the largest number of asylum seekers continue to be hosted in Turkey. There are some studies evaluating asylum seekers' access to healthcare services in Europe, and the common result is that refugees have free access to primary healthcare services in most countries; however, they face many obstacles when accessing primary healthcare services. While there are studies in the literature evaluating the situation of access to primary healthcare services from the perspective of asylum seekers; there are few studies evaluating the opinions/views of FPs. METHODS: A qualitative methodology informed by the grounded theory was used to guide the research. A total of 20 FPs were interviewed face to face through semi-structured interviews, using 12 questions about their lived experience and views caring of refugee population. Interviews were analysed thematically. FINDING: The following themes were revealed: Benefiting from Primary Health Care Services, Benefiting from Rights, Differences Between the Approach/Attitudes of Turkish Citizens and Refugees, Barriers to Healthcare Delivery, Training Needs of Physicians, Solution proposals. FPs reported that there is a need for support in primary care and a need for training them and refugees in this regard and they specified refugee healthcare centres are the best healthcare centres for refugees; however, the number of these and provided services should be increased.

Long-term cognitive outcomes of prematurely born infants: A longitudinal follow-up of Denver II, Bayley III and WISC-IV Tests.

BACKGROUND: Prematurity constitutes a risk factor for developmental delay in infancy and childhood. This study aims to: (i) determine long-term cognitive outcomes in prematurely delivered children and compare them with term-delivered children using the WISC-IV and Stroop tests; (ii) examine the relation between Denver II, Bayley III and WISC-IV, Stroop tests. METHODS: The study group consisted of children born prematurely who had been tested with Denver II and Bayley III in their first 2 years, and had been evaluated with WISC-IV and Stroop tests under follow up, 6-10 years later. RESULTS: The study group (n = 60, 25 F, 35 M) was 8.0 ± 2.4 (6-10.7) years old when given WISC-IV and Stroop tests. Gestational age in the study group was 34-37 weeks in 25%, 30-33 weeks in 48.3%, and <29 weeks in 26.7%. On WISC-IV, the verbal comprehension index, perceptual reasoning index, working memory index, and full-scale IQ scores were lower in the study group than the control group (P < 0.05). The study group took longer to complete the Stroop test (P < 0.05). Lower socioeconomic status (P = 0.005) and parental education level (P = 0.000) were associated with lower verbal comprehension index scores. Denver II and Bayley III test results were related to WISC-IV results (P < 0.05) but not to the Stroop test results (P > 0.05). CONCLUSIONS: Our results showed prematurity negatively influences the results of WISC-IV and Stroop tests at school age. Denver II and Bayley III tests applied at age 2 years likely predict WISC-IV results.

Posterior reversible encephalopathy syndrome (PRES) due to acute hypertension in children: 12 years single-center experience.

The objective of the study is to evaluate the clinical and neuroradiological findings, the risk factors for recurrence and the prognosis in patients with posterior reversible encephalopathy syndrome developed secondary to acute hypertension in children. The study was conducted between 2008 and 2019 at Mersin University Faculty of Medicine. A total of 49 episodes were evaluated retrospectively in 38 patients with PRES secondary to acute hypertension. The demographic data, etiology, and clinical and neuroradiological findings were recorded. Twenty-one (55.3%) patients were female; the mean age was 11.8 years. The etiology of acute hypertension in 29 (76.3%) patients was end-stage renal disease (ESRD). The most common clinical findings were seizure (81.6%) and altered consciousness (79.6%). Status epilepticus developed in eight (16.3%) episodes. MRI lesions were atypical in 33 episodes (67.3%). In eight (21%) patients, PRES recurred. Irreversible brain damage was detected after PRES in three (7.8%) patients. C-reactive protein and erythrocyte sedimentation rate were elevated in 82.2% and 71.4% of the episodes, respectively. A statistically significant relationship was found between the recurrence, the duration of hospitalization at the PICU, SE and the occurrence of irreversible lesion (p = 0.013, p = 0.015, p = 0.001 respectively). Also, there were statistically significant relationships between recurrence and ESRD; epilepsy and recurrences; SE and irreversible brain damage (p = 0.02, p = 0.012, p = 0.025 respectively). Although PRES is usually known to have a good prognosis, the mortality and morbidity rates may increase in the long-term follow-up as in our study. In this study, the etiology, the presence of status epilepticus, PICU history, atypical MRI lesions and increased inflammatory markers were found to be important for the prognosis in PRES.

Magnetic resonance imaging patterns of subacute sclerosing panencephalitis may mimic metabolic disorders: clinical, electroencephalographic and imaging features of six cases.

Subacute sclerosing panencephalitis is a rare, devastating neurodegenerative encephalitis whose diagnosis and therapy are still in question. Atypical clinical presentation and heterogeneity of neuroimaging findings that have been initially confused with metabolic disorders have hampered early diagnosis. To describe a series of patients with subacute sclerosing panencephalitis with imaging findings mimicking metabolic disorders. A total of six patients with subacute sclerosing panencephalitis were diagnosed from January 2012 to December 2016 in whom a metabolic disorder was suspected on initial clinical and MRI findings. Detailed laboratory investigation was performed in all patients. All patients presented with atypical neurologic manifestations, including dystonia, syncopal attacks, involuntary limb movements, meaningless speech and ataxia. Magnetic resonance imaging abnormalities included bilateral putaminal, bilateral posterior periventricular white matter and diffuse or splenial corpus callosum involvement which are particularly unusual in SSPE and mostly observed in metabolic disorders. All patients had elevated cerebrospinal fluid Ig G measles antibodies. The diagnosis of subacute sclerosing panencephalitis through clinical and imaging features can be considerably challenging. It is crucial to differentiate it from metabolic disorders, since the management and clinical outcome are different.

Efficacy of levetiracetam in the treatment of Sydenham chorea.

BACKGROUND: To study the effect of levetiracetam in treating Sydenham chorea. METHODS: We retrospectively collected the data of 140 patients diagnosed with Sydenham chorea in the pediatric neurology and pediatric cardiology outpatient clinics of Van Training and Research Hospital between January 2010 and December 2018. RESULTS: There were 140 patients, 102 (70%) of whom were girls, with mean age of onset 11.8 ± 2.7 years. Symptomatic treatment was initiated in all patients at the time of diagnosis; this medication was changed during follow up in 15 patients. The most frequently prescribed drugs were haloperidol and sodium (Na) valproate, and the most frequently discontinued one was haloperidol, due to side effects. The second-choice drug was most often levetiracetam. Clinical response often began within the first 2 weeks, with Na valproate (P = 0.002), within 4 weeks with carbamazepine (P = 0.037) but 1-6 months with haloperidol (P = 0.018) and levetiracetam (P = 0.008). Time to full remission was similar with Na valproate, carbamazepine, haloperidol, and levetiracetam (P = 0.276). Our study indicated that levetiracetam was as effective as the other commonly used drugs in the symptomatic treatment of Sydenham chorea. CONCLUSION: Levetiracetam might be an option in the treatment of Sydenham chorea because of its acceptable effect and safety profile. This observation needs further support with evidence obtained through controlled and blinded trials.

Radiologic analysis of the location, shape and size of the external aperture of the carotid canal in children.

OBJECTIVES: This retrospective computed tomography (CT) study was aimed to assess the growth dynamic of the external aperture of the carotid canal (EACC) in children aged between 1 and 20 years. METHODS: Two hundred patients (sex 100 females/100 males, average age 10.50 ± 5.77 years) with good head CT image quality were included in this study. CT images of the patients were used to obtain data related to the location, shape and dimension of EACC. RESULTS: EACC shapes were identified as oval shaped, round shaped, and tear-drop shaped in 58.3% (233 sides), 24% (96 sides) and 17.8% (71 sides), respectively. EACC length, disEACC-MSP (distance between EACC and midsagittal plane), and EACC width did not change from the prepubescence period; while, the disEACC-SC (distance between EACC and supramastoid crest) seemed to reach adult size in the postpubescence period. Linear functions for EACC length and width were calculated as: y = 5.453 + 0.091 × years, and y = 5.398 + 0.059 × years, respectively. CONCLUSION: The regression equations of the measured parameters representing the growth dynamic of EACC in children can be helpful to estimate its size, location and angulation, which suggest that the dimension and distances to certain anatomical landmarks seemed to reach adult size in different developmental periods. In this context, the findings of this study may seem to emphasize the importance of preoperative radiological evaluation on skull base, related to EACC, for multidisciplinary surgeon teams during childhood surgeries in terms of patients' positioning, and the selection of appropriate surgical approach.

Identification of a Novel Mutation in GRIN2A Gene with Global Developmental Delay and Refractory Epilepsy.

We report a 2.5-year-old Turkish boy who first presented with nystagmus, lack of eye contact, and hypotonia at 2 months of age and developed refractory seizures when 6 months old. Extensive metabolic tests and imaging being noncontributory, whole-exome sequencing was carried out which revealed a heterozygote NM_001134407.2:C.3299A>G (p.Glu1100Gly) novel mutation in GRIN2A gene. Topiramate was started and seizures were rapidly brought under control. GRIN2A mutations may result in altered GluN2A membrane trafficking and response to glutamate. This report illustrates the clinical variability of GRIN2A mutations according to the age of onset of symptoms and suggests considering mutations in this gene in cases of global developmental delay, refractory epilepsy, and nystagmus.

Levetiracetam-Related Mania-Like Symptoms: An Adolescent Case.

Levetiracetam is an antiepileptic agent that is used for partial and generalized epilepsy. Although it is well tolerated in most cases, behavioral and nonbehavioral adverse effects may be observed. Among behavioral symptoms, depression, hostility, and agitation have been frequently reported. However, mania or mania-like symptoms are relatively rare, especially in children and adolescents. Hereby, we report mania-like symptoms with levetiracetam use in a 15-year-old boy. Mania-like symptoms emerged 3 weeks after starting levetiracetam and disappeared after adding risperidone to ongoing levetiracetam treatment.

A Rare Cause of Refractory Epilepsy: Posterior Periventricular Nodular Heterotopia.

A 15-year-old male patient presented to our outpatient clinic with drug-resistant seizures. Magnetic resonance imaging of the brain showed bilateral posterior nodular heterotopia and left cerebellar dysgenesis. The patient was diagnosed with cortical developmental malformation and medically refractory epilepsy. The filamin A gene mutation was negative. Posterior periventricular nodular heterotopia is a rarer and a more different entity from classical periventricular nodular heterotopia with no gender difference and negative filamin A gene mutation. There is a limited number of case studies on posterior periventricular heterotopia. Therefore, this patient was presented to emphasize that epilepsy may be more refractory to treatment, and central congenital abnormalities including posterior fossa abnormalities are more frequent in patients with posterior periventricular nodular heterotopia.

Attention-Deficit Hyperactivity Disorder (ADHD) in Epilepsy and Primary ADHD: Differences in Symptom Dimensions and Quality of Life.

PURPOSE: We aimed to (1) compare quality of life (QOL) among children with epilepsy, epilepsy and attention-deficit hyperactivity disorder (ADHD), and primary ADHD and (2) compare ADHD symptom dimensions and subtypes between children with epilepsy-ADHD and primary ADHD. METHODS: A total of 140 children; 53 with epilepsy, 35 with epilepsy-ADHD, and 52 with primary ADHD were included. KINDL-R (quality of life measure), Turgay DSM-IV Disruptive Behavior Disorders Rating Scale (T-DSM-IV-S), and Conners' Parent Rating Scale (CPRS) were completed. Neurology clinic charts were reviewed for epilepsy-related variables. RESULTS: Children with epilepsy-ADHD had the lowest (poorest) KINDL-R total scores. Epilepsy-ADHD group had more inattentiveness symptoms, whereas primary ADHD group had more hyperactivity/impulsivity symptoms. The frequencies of ADHD combined and inattentiveness subtypes were 60% and 40% in children with epilepsy-ADHD and 80.7% and 19.3% in children with primary ADHD, respectively ( P = .034). CONCLUSION: ADHD in epilepsy is associated with a significantly poor quality of life and predominantly inattentiveness symptoms.