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OBJECTIVE: Febrile seizures (FS) are the most common neurological emergency in childhood. They are divided into two groups accordingly clinical features, simple febrile seizure and complex febrile seizure. Until now laboratory tests have not been used as a parameter of classification of them. The objective of this study is to estimate the usefulness of the hematogical parameters especially neutrophil-to-lymphocyte ratio (NLR) and red blood cell distribution width (RDW) in the differentiation of febrile seizure types. PATIENTS AND METHODS: A retrospective review was conducted on patients from 6 months to 6 years old presenting with first febrile seizure admitted to a tertiary care hospital. Epidemiological and laboratory variables of the patients were collected. RESULTS: The mean NLR in the simple FS and complex FS groups was 2.18 ± 1.9 and 3.8 ± 4.2 respectively, and the difference was significant (p = 0.024). The mean serum red blood cell distribution width in the simple FS and complex FS groups was 16.1 ± 1.1 and 16.6 ± 0.8 respectively, and the difference was significant (p = 0.019). NLR and RDW values in complex FS patients were statistically higher than simple febrile patients. ROC analysis showed that if the chosen cut-off point for NLR is 1.98 the sensitivity and specificity are 66.7% and 60.3% respectively. These were statistically significant (p = 0.040 AUC 0.623, CI 0.503-0.743). If the chosen cut-off point for RDW is 16.350, the sensitivity and specificity are 59.0% and 58.6%, respectively. These were statistically significant (p = 0.037 AUC 0.626, CI 0.515-0.736) too. CONCLUSIONS: NLR and RDW were simple, effective and practical predictors for differentiation of FS types. They will have potential values in public health practice for management of FS patients. [ABSTRACT FROM AUTHOR]

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Background: Sandifer Syndrome is an uncommon clinical entity characterized by gastroesophageal reflux, irritability and abnormal movements of the body and contortions of the neck. The majority of paroxysmal cases, in particular, tend to show an association with epilepsy. Methods: The clinical, laboratory and 6-month observation results of the four patients (two boys, two girls) have been presented. Results: The 6-month prospective observation/treatment of four patients aged between 2 and 14 months (mean age, 6.5 +/-2 months) with a diagnosis of Sandifer Syndrome has been investigated. Due to paroxysmal extensor jerks, two of the patients were misdiagnosed with infantile spasm and they were treated accordingly. In the clinical observations of the patients, abnormal neurobehavioral attacks (irritability, crying, head/eye version, torticollis, extensor spasm and dystonic posture) 5-10 times daily were observed. In two of the patients, motor growth retardation was observed, in one patient, bronchospasm attacks were observed, and in all the patients iron deficiency anemia was observed. The electroencephalograms of the patients which were taken during the routine, sleepless and paroxysmal behaviors were normal; the gastroesophageal scintigraphies were positive in the manner of reflux. Management of the infant with gastroesophageal reflux disease, in addition to nonpharmacological interventions pharmacologic therapy, including metoclopramide HCl and Fe (6 mg/kg per day, oral) was useful for the patients, and their paroxysmal attacks decreased dramatically (0-2 attacks/day). Conclusion: These findings suggest that infants or children with these atypical movements should be evaluated for Sandifer Syndrome. Expensive and comprehensive neurologic examination may be unnecessary. Early diagnosis permits prompt treatment and relief of the problem. Medical management is usually successful. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study is to examine the factors affecting seizure recurrence in pediatric patients diagnosed with epilepsy., Methods: Three hundred patients presenting to the pediatric neurology clinic between 2015 and 2018 and diagnosed with epilepsy and treated with single antiseizure drug were included in the study. Medical histories and clinical and laboratory findings were retrieved retrospectively from the hospital data system. The combined and adjusted effects of risk factors on seizure recurrence were evaluated using multivariate binary logistic regression analysis., Results: Boys had a higher rate of seizure recurrence than girls. Seizure recurrence was also higher in patients with abnormal neurological examinations at the time of diagnosis compared to those with normal neurological examinations. Seizure recurrence was significantly higher in patients with global growth retardation. Epilepsy patients with abnormal MRI findings also had a higher rate of seizure recurrence than patients with normal neuroimaging findings. In addition, seizure recurrence was significantly higher in epilepsy patients with comorbidities such as cerebral palsy and autism spectrum disorders compared to patients without comorbidities. No significant association was observed between seizure recurrence and the first drug, perinatal asphyxia history, localization of epileptiform discharges on EEG, family history of epilepsy, family history of febrile seizures, history of stay in the neonatal intensive care unit during the perinatal period, or preterm delivery., Conclusion: Abnormal neurological examination, abnormal neuroimaging and accompanying comorbidities, and global growth retardation at the time of diagnosis are important factors affecting seizure recurrence in pediatric patients with epilepsy., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Increasing evidence suggests that vasoactive neuropeptides such as pituitary adenylate cyclase-activating polypeptide-38 (PACAP-38), substance P, calcitonin gene-related peptide, and vasoactive intestinal peptide are involved in the pathophysiology of migraine in adults, but their role in pediatric migraineurs remains unclear. We prospectively investigated plasma levels of these vasoactive neuropeptides in pediatric migraine patients without aura and compared the results with those of age-matched healthy controls., Methods: Thirty-eight children aged 6-18 years with migraine without aura and 20 age-matched control subjects were included in the study. Neuropeptides in plasma samples from the controls, and in either the ictal or interictal periods in pediatric migraine without aura, were measured using ELISA., Results: PACAP-38 and vasoactive intestinal peptide levels in both ictal and interictal plasma were higher in the patients with pediatric migraine without aura than in the controls ( p  < 0.001), although calcitonin gene-related peptide and substance P levels remained unchanged. Otherwise, no significant difference was determined between ictal and interictal periods in terms of all neuropeptide levels., Conclusions: This study demonstrates increased plasma PACAP-38 and vasoactive intestinal peptide levels, but not calcitonin gene-related peptide and substance P levels, in pediatric patients with migraine during both attack and attack-free periods. The study findings suggest that PACAP-38 and vasoactive intestinal peptide may be implicated in the pathophysiology of migraine, particularly in pediatric migraineurs.

Background: In the present study, the effects of fever and hyperthermia, and different anti hyperthermia treatment modalities on the brain by was investigated by using experimental animal model, Materials and Methods: Endogenous hyperthermia (41 degrees C) was induced by lipopolysaccharide (LPS) injection, and the signs of probable neuronal damage were evaluated by healthy, necrotic and apoptotic cells, and heat-shock proteins (HSP 27 and HSP 70) in cerebral cortex, cerebellum and hypothalamus. The animals were treated with widely used treatment modalities for high fever in pediatric practice, namely hypothermia, dexamethasone, paracetamol and diclofenac, and their effect on the hyperthermia-induced brain changes were evaluated., Results: Generalized seizure was observed in fifteen rats of which rectal temperature achieved 41 degrees C (15/36, 41%); five of them died on second day (5/15, 33%). LPS-induced endogenous hyperthermia; (i) caused significant increase of necrotic cells in cerebral cortex and cerebellum and apoptotic cells in all three regions (p < 0.05), (ii) caused significant decrease of healthy cells in cerebral cortex (p < 0.05), and (iii) no significant change of HSP 27 and 70 in all three neuronal locations (p > 0.05). For the treatment modalities applied; (i) paracetamol had an effect of increasing the healthy cell count in cerebral cortex and hypothalamus and decreasing the necrotic cell count in cerebellum and hypothalamus (p < 0.05)., Conclusion: The neuronal tissue in different regions of brain can show various degrees of damage in response to endogenous hyperthermia and the applied medications have varying degree of protection (Tab. 3, Fig. 6, Ref. 44).

Objective: To assess the clinical characteristics of patients with migraine., Methods: The medical records of 76 patients diagnosed with migraine were reviewed using the ICHD-II 2004 diagnosis criteria. The patients were classified into three age groups: 3-6 yr olds (group I), 7-12 yr olds (group II), and 13-17 yr olds (group III)., Results: Migraine was the most common cause of headache in the patients of present pediatric neurology outpatient clinic (57.1%, 76/133). The mean age of patients was 11.08 ± 3.27 (3.25-17) yrs. The number of girls as the age increased (groups II and III). The mean headache attacks rate was 2.5 ± 1.5 per wk, which resulted in worsening of school performance (n = 26, 34.2%). In the majority of patients (n = 54, 71.1%), there was a family history of migraine or headache in the close relatives. Prophylaxis was found effective for all given medications (flunarizine: 46/54, propranolol: 19/21, topiramate: 10/10, sodium valproate: 1/1)., Conclusions: These findings indicate that: (a) migraine is the most frequent cause of headache in pediatric patients, (b) it has negative effects on school performance and daily activities, (c) the family history is important for making the diagnosis and (d) prophylaxis is significantly effective.

Henoch-Schönlein purpura is characterized by nonthrombocytopenic purpura with multisystem involvement. Nervous system involvement was reported, characterized by headaches, mental-status changes, seizures, paresis, coma, or encephalopathy. Peripheral neuropathy is rarely reported. We describe a 12-year-old boy with Henoch-Schönlein purpura who presented with abdominal pain and underwent a laparatomy before the onset of palpable purpuric rash. Neuropathic findings in the left lower brachial plexus developed while he was receiving steroid treatment for gastrointestinal involvement. He responded well to intravenous pulse steroid therapy. Both sensory and motor dysfunction returned to normal after 3 months of treatment. His steroid dose was gradually withdrawn and stopped. He was symptom-free at month 12 of follow-up., (Copyright 2010 Elsevier Inc. All rights reserved.)

An 8.5-year-old girl evaluated for central cyanosis, hypoxia and normocarbia was found to have aorticopulmonary window and pulmonary hypertension. The diagnosis of Eisenmenger syndrome (ES) was made and treatment with bosentan was started. Four months later she was diagnosed to have juvenile rheumatoid arthritis and naproxen treatment was started. The case was remarkable in that she showed clinical improvement with new generation treatment of ES although pulmonary arterial pressure did not decrease significantly and the diagnosis of juvenile rheumatoid arthritis was made during follow-up.

The aim of this study was to investigate possible correlations of the cognitive impairment with abnormalities of regional cerebral blood flow and electroencephalogram in children with (Down's Syndrome) DS. Nine patients with DS were evaluated by single photon emission computed tomography (SPECT) in combination with clinical findings, electroencephalography (EEG), and magnetic resonance imaging (MRI). In cases with IQs below 40, there were one or more findings of abnormal EEG/MRI and brain perfusion SPECT. In 6 cases (66.7%) EEG findings were normal, but 3 (33.3%) had abnormal EEG findings. Perfusion abnormalities were most pronounced in the fronto-parieto-temporal region in the form of hypoperfusion (n = 5) and in the right hemisphere (n = 5) than the left hemisphere (n = 1). These findings suggest that the children with DS had varying levels of structural, perfusion, and electrophysiological abnormalities in the brain and these abnormalities were reflected by measurable alterations of the cognitive functions.

Fragile X syndrome is an inherited disorder caused by a defective gene on the X chromosome. It is associated with developmental or behavioral symptoms and various degrees of mental retardation. Morphologic abnormalities and altered perfusion of various brain areas can underlie these functional disturbances. The aim of this study was to investigate the cerebral perfusion state in patients with fragile X syndrome using single-photon emission computed tomography (SPECT). Structural and functional assessment was also performed by magnetic resonance imaging (MRI) and electroencephalography (EEG). Eight boys with cytogenetically confirmed fragile X syndrome (mean age 8.8 +/- 4.4 years, range 5-18 years), were included. All patients had mental retardation, with a mean IQ of 58.9 +/- 8.8 (range 40-68), and additional neurobehavioral symptoms. SPECT revealed cerebral perfusion abnormalities in six patients (75%), most commonly in the frontoparietotemporal area and prominent in the right hemisphere. The SPECT and EEG findings were concordant: hypoperfused areas in SPECT corresponded to regions of persistent slow-wave paroxysms on EEG. On the other hand, cranial MRI was abnormal qualitatively only in two patients (25%) showing cerebellar and vermal hypoplasia and cerebral hemispheric asymmetry. Our results indicate that cerebral perfusion abnormalities, which are correlated with electrophysiologic findings but not necessarily with anatomic abnormalities, can underlie the pathogenesis of the clinical findings observed in fragile X syndrome.

Anthrax is primarily a disease of herbivores, but it also causes cutaneous, respiratory and gastrointestinal infections in humans. Bacillus anthracis is an uncommon cause of meningitis and generally produces a haemorrhagic meningoencephalitis. We present the CT and MR findings of anthrax meningoencephalitis due to the cutaneous form of anthrax in a 12-year-old boy. They showed focal intracerebral haemorrhage with leptomeningeal enhancement.

Fragile X syndrome (FXS) is known as the most common form of inherited mental retardation. In our study, brain perfusion single photon emission computed tomography (SPECT) was performed in a 6 year-old boy diagnosed with FXS. Diffuse bilateral uptake of Technetium-99m hexamethyl propylene amine oxime (99mTc-HMPAO) was noted in his orbits, as well as cortical perfusion defects (hypoperfusion in the right parietal and the left temporal lobe). Ophthalmologic examination showed no pathological findings. Magnetic resonance imaging (MRI) revealed no abnormality in the orbital structures, although hypoplasia of cerebellum and vermis was visualized. Since the patient was crying during the injection, the increased blood flow or the increased metabolism of the eyes and/or ocular muscles may be responsible for this orbital finding. Alternatively, the enhanced uptake of HMPAO in the orbits may reflect the pathology associated with FXS, because patients with FXS might have visual-motor abnormalities. To the best of our knowledge, there has been no report documenting such an orbital uptake of HMPAO. Moreover, the visualization of decreased cerebral perfusion, with the normal findings of MRI, indicates that brain SPECT imaging with HMPAO is helpful for detecting brain abnormalities in children with FXS.

Mucopolysaccharidoses are characterized by involvement of multiple organs, including blood vessels. We present a case of mucopolysaccharidosis IIIB admitted with recurrent subdural hematoma; further evaluation and dural biopsy revealed an association with cerebral vasculopathy.

Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and cranial Doppler studies. Brain MRI and Doppler studies of the all cases were normal. In the brain SPECT study, six patients were determined to have cerebral perfusion abnormalities. Although six patients responded well to the therapy, two girls who had hypoperfusion in the right frontal region as well as hypo- / hyperperfusion in the basal ganglia did not respond to therapy. While the chorea form of the patients who have cerebral perfusional abnormality was generalized, the clinical picture of the patients with normal cerebral perfusion was in the form of hemichorea.

Segmental myoclonus is described as the involuntary contractions of contiguous muscles innerved by the brain stem or by spinal cord. The underlying causes of segmental myoclonus in children are demyelinating diseases and intrinsic tumors. Here, we report a case who was presented with segmental myoclonus on his left arm and later diagnosed as atypical monosymptomatic presentation of acute disseminated encephalomyelitis (ADEM). The case represents the first in the literature in which ADEM is considered as the possible cause of segmental myoclonus. Our findings demonstrate that: (i) in focal movement disorders such as segmental myoclonus, a careful neuroradiological examination of the neuroanatomical region for the possible presence of organic lesions might be rewarding, (ii) ADEM might be one of the potentially reversible causes of myoclonus.

Acute hemorrhagic leukoencephalitis (AHL) is a rare and usually fatal disorder characterized by an acute onset of neurological abnormalities. It may occur in association with a viral illness or vaccination. Radiology and brain biopsy are essential for the diagnosis. We present a case of AHL mimicking or manifesting as intracerebral hemorrhage associated with herpes simplex virus. A 3-year-old boy was admitted to our hospital with cerebral hemorrhage. The findings of cerebrospinal fluid, MRI and electroencephalogram indicated acute hemorrhagic leukoencephalitis associated with herpes simplex virus involving the left parietal cortex (hemorrhage) and the right temporal lobe (encephalitis). The patient received intravenous dexamethasone (1.5 mg/kg/24 h divided q6 (six times a day) for 5 days, tapered slowly to discontinuation over the next 5 days) and aciclovir (15 mg/kg/every 6 h for 14 days). His clinical condition markedly improved, and after 3 weeks he was discharged from the hospital with moderate right hemiparesis. Mild hemiparesis with normal psychometric testing was determined when he was checked 6 months later. AHL remains misdiagnosed as viral encephalitis because it produces different protean clinical forms. Pediatric patients admitted with cerebral hemorrhage and infarct should be evaluated for presence of hemorrhagic encephalitis-causing agents, especially for herpes simplex. Since, as in our case, treatment with corticosteroid and acyclovir markedly improves conditions of herpes simplex-caused AHL, we suggest that early diagnosis and treatment is of importance for the prognosis.

Although controversial, protective and therapeutic effects of valproic acid in various types of cellular injury suggest a potential role for this agent in hypoxic-ischemic brain injury. We therefore investigated the effects of valproic acid in an experimental model of neonatal hypoxic-ischemic brain injury. To examine the effect of valproic acid in this condition, hypoxic-ischemic brain injury was induced in 7-day-old rat pups by ligation of the right common carotid and then the pups were exposed to 1 hour of hypoxia in 8% oxygen. Low (200 mg/kg/day) and high (400 mg/kg/day) doses of valproic acid were administered in a 5-day regimen. Neuropathologic evaluation was performed using the hippocampus, cerebral cortex, and basal ganglia in the coronal plane. The 5-day regimen of valproic acid administration resulted in some protective and therapeutic effects on the brain damage and neuronal apoptosis in both hemispheres in a dose-dependent manner. Administration of valproic acid also decreased the percentage of apoptotic neurons in the contralateral hemisphere (P < .05). These results suggest that valproic acid can have therapeutic and protective effects in hypoxic-ischemic brain injury.

Immunologic studies in relation to clinical status might help to understand the pathogenesis of subacute sclerosing panencephalitis (SSPE) and the effect of treatment. We measured lymphocyte subsets and intracellular TNFalpha and interleukin-4 levels in peripheral blood in SSPE patients. Patients had elevated percentages of CD8+ cells compared to age-matched control children. Rapidly progressive course was associated with increased CD4+ cells. Treatment with interferons and inosiplex altered the percentage of CD3+, CD4+ and CD19+ cells. TNFalpha and interleukin-4 levels had no correlation with course or treatment. The proportions of lymphocyte subsets appear to have a role in the evolution or manifestations of SSPE, if not in the pathogenesis.

Neurofibromas occurring in the breast are very rare. A 10-year-old boy with neurofibromatosis type 1 (NF1) presented with a palpable mass on his left breast. US showed a well-defined and hypoechoic solid mass within the subcutaneous fat tissue in the areolar area. Mass was isointense compared to muscle and hypointense compared to fat tissue on TIW and T2W MR images. Excisional biopsy was applied to the mass. The diagnosis of gynecomastia and neurofibroma was made on the basis of histopathological and immunohistochemical findings.