Evaluation of Sleep Habits and Their Relationship With Quality of Life in Children With Epilepsy.

BACKGROUND: The aim of this study was to investigate sleep habits, quality of life (QoL), and the relationship between them in children with epilepsy. METHODS: In this cross-sectional study, children aged two to 18 years being followed up for epilepsy were assessed using the Children's Sleep Habits Questionnaire (CSHQ) and the Pediatric Quality of Life Inventory (PedsQL). Pearson or Spearman correlation analysis was performed to examine the relationship between normally distributed and non-normally distributed variables, respectively. Linear regression analysis was used to examine independent variables associated with PedsQL total scale score. Level of significance was accepted as P < 0.05. RESULTS: The study included 112 children with a mean age of 10.5 ± 4.4 years (51.8% female). The frequency of poor sleep habits was 96.4%. There was a good level of agreement between children's and parents' PedsQL total, physical health, and psychosocial health scores (P < 0.001). Correlation analysis between QoL and sleep parameters revealed negative correlations between total sleep score and self-assessed PedsQL total scale, physical health, and psychosocial health scores (P < 0.05) and parent-assessed PedsQL total scale and psychosocial health scores (P < 0.05). The results of linear regression analysis indicated that the factors most significantly associated with lower QoL were high CSHQ total sleep score and exclusively daytime seizures (P < 0.001). CONCLUSIONS: It was found that children with epilepsy had poor sleep habits and low QoL and that poor sleep habits have a negative impact on QoL.

Characteristics and clinical value of early electroencephalography (EEG) after a first unprovoked seizure in children.

OBJECTIVE: This study aimed to examine the timing and features of electroencephalography (EEG) as a predictor of seizure recurrence in children with a first unprovoked seizure. METHODS: We retrospectively evaluated the medical records and EEG recordings of pediatric patients who presented within 24 h of a first unprovoked seizure between January 2018-December 2019 and had at least 1 year of pediatric neurology clinical follow-up. RESULTS: The study included 108 patients (53.7% males) with a mean age of 98.75±57.75 months. Sixty-eight patients (63%) had an abnormal initial EEG, of which 55 (80.9%) were focal. The semiology of the first unprovoked seizure was focal in 50% of the patients and correlated with initial EEG findings (p<0.001). Forty-three patients had seizure recurrence during the follow-up period of mean 26.86±7.39 months. Recurrence was observed in the first 6 months in 30 patients and occurred twice in 4 patients. An abnormal EEG after the first unprovoked seizure was found to be an independent risk factor for recurrence, with a 2.42-fold higher recurrence risk in patients with focal EEG abnormalities compared to those with a normal EEG (p = 0.044). Analysis of 7 different timing patterns up to 96 h after the first unprovoked seizure showed that EEG timing was not associated with abnormality detection. DISCUSSION: Our study showed that EEG abnormalities, especially focal abnormalities, after a first unprovoked seizure are a predictor of seizure recurrence. But the rate of detection of EEG abnormalities was not related to the timing of EEG recording, relative to seizure occurrence.

Evaluation of Acute Ataxia in the Pediatric Emergency Department: Etiologies and Red Flags.

BACKGROUND: We aimed to evaluate patients with acute ataxia and to determine the warning clinical factors in the early prediction of neurological emergencies. METHOD: Patients with a history of balance and gait coordination disorder and clinically diagnosed as acute ataxia in pediatric emergency department were included in the study. As a result of final diagnosis, the characteristics of patients with and without clinically urgent neurological pathology (CUNP) were compared. CUNP was defined as any nervous system disorder requiring early diagnosis and prompt medical or surgical treatment and/or intensive care unit admission to prevent disabling or life-threatening evolution. RESULTS: Eighty-eight patients with a median age of 5 years were included in the study (37 [42%] patients with CUNP and 51 [58%] without CUNP). In the CUNP group, the median age of patients and symptom duration were significantly higher (P < 0.001 and P = 0.011, respectively). The most common etiologies were acute post/parainfectious cerebellar ataxias (n = 40 [45.4%]), acute cerebellitis (n = 9 [10.2%]), and Guillain-Barré syndrome (n = 8 [9%]). Hyporeflexia/areflexia and dysmetria were associated with a higher risk of CUNP. Headache, loss of consciousness, and visual dysfunction were the findings appearing exclusively in patients with CUNP. CONCLUSIONS: The most common etiologies in acute ataxia are benign and transient, whereas life-threatening conditions may occur rarely and may require urgent intervention. Older age; prolonged symptom duration; focal neurological deficits such as hemiparesis, hyporeflexia, and visual impairment; and nonspecific findings such as loss of consciousness and headache are the most striking "red flags" of a potential neurological emergency and should alert clinicians to CUNP.

Evaluation of clinical and electroencephalographic findings in patients with early childhood epilepsy and inborn errors of metabolism.

INTRODUCTION: Epilepsy is one of the leading chronic diseases of childhood, and an underlying IEM is an etiology that can easily be overlooked. The aim of this study was to determine the frequency of metabolic disease in patients diagnosed with epilepsy in the first two years of life, as well as to determine the clinical, radiological, and electroencephalographic (EEG) characteristics of the metabolic disease subtypes associated with epilepsy and evaluate treatment response in our study. MATERIALS AND METHODS: The records of patients diagnosed with epilepsy before the age of 2 years in our pediatric neurology clinic between 2014 and 2021 were reviewed retrospectively. Those diagnosed with an IEM and followed up in the pediatric neurology and pediatric metabolism departments of our hospital were included in the study. RESULTS: A total of 990 patients under the age of 2 years were diagnosed with epilepsy in the pediatric neurology clinic of our hospital and 74 (7.5%) of them had IEM. Thirty-nine (52.7%) of the 74 patients were female. The median age at admission was 144 days (min-max: 0-284). Of the 74 patients diagnosed with metabolic epilepsy, 38 patients were diagnosed with amino acid metabolism disorder, 17 with lysosomal storage disease, 9 with energy metabolism disorder, 5 with vitamin/cofactor/trace element metabolism disorders, 2 with fatty acid metabolism disorder, 2 with peroxisomal disease, and 1 with carbohydrate metabolism disorder. Epilepsy was refractory despite appropriate treatment in 39 patients (52.7%). CONCLUSION: Inborn errors of metabolism are a rare cause of epilepsy, in regions like our country with high rates of consanguineous marriage, IEM should be considered in patients presenting with seizures that do not respond to conventional antiepileptic treatments.

Evaluation of the Anxiety Level of Mothers of Children with Epilepsy during the COVID-19 Pandemic Period.

BACKGROUND AND AIM: Although anyone can be affected by the COVID-19 pandemic, it may cause additional concern for people with chronic conditions. Epilepsy is the most common neurological disease in childhood and adolescence. The aim of this study was to determine anxiety levels among the mothers of children under follow-up for epilepsy in our clinic during the COVID-19 pandemic. METHODS: The study group consisted of the mothers of epilepsy patients who were under follow-up in the pediatric neurology outpatient clinic of the tertiary care center and were scheduled for a routine examination during the COVID-19 pandemic. The mothers' anxiety levels according to the Beck Anxiety Inventory and their opinions about COVID-19 in relation to their child were assessed and compared based on whether the mother/patient attended their appointments in person and whether the child had frequent or infrequent seizures. RESULTS: There was no statistically significant difference in anxiety level between the mothers of 64 children with epilepsy who attended their appointment during the pandemic and those of the mothers of 52 who did not attend their appointment. However, the mothers of children with frequent seizures had significantly higher anxiety levels. CONCLUSION: Anxiety level of mothers whose children have frequent seizures was significantly higher compared to mothers whose children have infrequent seizures. It is important to be aware about this point and using telemedicine approach in suitable population and postpone routine outpatient follow-up appointments as much as possible.

Acute necrotizing encephalopathy of childhood: a single-center experience

Background/aim: Acute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it. Materials and Methods: Patients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study. Results: Nine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1­6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1­4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died. Conclusion: Our study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.

Evaluation of the international severity score for FMF (ISSF) scores in Turkish children diagnosed with FMF: a single-center experience.

AIMS: The aim of this study is to evaluate our patients with the newly developed international severity score for FMF (ISSF) and make comparisons with the literature. METHODS: This cross-sectional study included patients with FMF, were between 6 months and 18 years old, and were using colchicine/colchicine+IL-1 inhibitor for at least 6 months. The patients were classified as mild, intermediate, and severe based on their scores between 1 and 10. In addition to scoring, those who have additional pathological or silent mutation were compared based on these classifications. RESULTS: Our patients consist of 88 women 72 men total 160. The mean age, age of onset, and age of diagnosis were 12 ± 4.3, 4.9 ± 3.7, and 7.5 ± 4 years, respectively, and the mean diagnostic delay was 2.6 ± 3years. When our large patient population is evaluated with ISSF, the disease severity is mostly intermediate. According to ISSF, mild, intermediate, and severe diseases were 21.3%, 70.5%, and 8.1%, respectively. The duration of attacks shorten with increasing age (p > 0.05), and there is an increase in the involvement of other organs during the attacks among patients older than 7 years. CONCLUSIONS: ISSF appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. Key Points • Our article is the first study to evaluate ISSF performance in the pediatric population. • International severity score for FMF (ISSF) appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. • We think that the addition of pathogenic mutations and inheritance model to the ISSF scoring system as important determinants of disease severity, especially when comparing different patients, can be evaluated in future studies.