Clinical Characteristics and Visual Outcomes of Pediatric Optic Neuritis: A Single Center Experience

INTRODUCTION: The aim of this study was to describe the clinical characteristics, visual outcomes of pediatric patients presenting with first-episode of optic neuritis. METHODS: We reviewed medical records of the patients newly diagnosed with optic neuritis younger than 18 years between January 2014 and December 2018 retrospectively. RESULTS: Twenty-eight patients were included to this study. The mean age at first onset of optic neuritis was 13.2+-3.1 years (range 6.2-17.3 years). The mean follow-up period was 4.2+-3.2 (range 0.6-13.08) years. 7 of 28 (25%) patients had recurrent optic neuritis. Optic neuritis involvement was unilateral in 17 of 28 (60%) patients. Forty percent of the patients had idiopathic optic neuritis. Of the six patients with demyelinating lesions in cranial magnetic resonance imaging (MRI) at the first admission, three were diagnosed with multiple sclerosis (MS) at the time of first optic neuritis attack, and three were diagnosed within 13.4+-4.8 months after the first episode. Eight of 21 optic neuritis patients (38%) had oligoclonal band positivity and the incidence of MS was significantly higher in these patients (p=0.014). The mean visual acuity at nadir was 0.48+-0.27 at admission. Whereas it was 0.74+-0.31 and 0.76+-0.33 at 1 and 6 months respectively. There was a strong correlation between first and sixth-month visual acuity (r=0.98, p=0.00). DISCUSSION AND CONCLUSION: Our study demonstrated that poor visual acuity (worse than 0.5) at 1 month can predict poor vision at 6 months. The patients with demyelinating lesions in cranial MRI at their first optic neuritis episode, are more likely to develop MS during the follow-up

Seizures in a Pediatric Intensive Care Unit: A Prospective Study

Background: The aim of the research is to determine the etiology and clinical features of seizures in critically ill children admitted to a pediatric intensive care unit (PICU). Methods: A total of 203 children were admitted from June 2013 to November 2013; 45 patients were eligible. Age ranged from 2 months to 19 years. Seizures were organized as epileptic or acute symptomatic. Pediatric risk of mortality score III, Glasgow coma scale, risk factors, coexistent diagnosis, medications administered before admission, type and duration of seizures, drugs used, requirement and duration of mechanical ventilation, length of stay and neuroimaging findings were collected as demographic data prospectively. Results: The male-female ratio was 0.8. Mean age was 5.4. The most common causes of seizures were acute symptomatic. Most frequent coexistent diagnosis was infectious diseases, and 53.3% had recurrent seizures. Medications were administered to 51.1% of the patients before admission. Seizures were focal in 21 (46.7%), generalized in 11 (24.4%) and 13 (28.9%) had status epilepticus. Intravenous midazolam was first-line therapy in 48.9%. Acute symptomatic seizures were usually new-onset, and duration was shorter. Epileptic seizures tended to be recurrent and were likely to progress to status epilepticus. However, type of seizures did not change severity of the disease. Also, laboratory test results, medications administered before admission, requirement and duration of ventilation, mortality and length of stay were not significant between epileptic/acute symptomatic patients. Conclusion: Seizures in critically ill children, which may evolve into status epilepticus, is an important condition that requires attention regardless of cause. Intensified educational programs for PICU physicians and international guidelines are necessary for a more efficient approach to children with seizures.WoSScopu

Early-Onset Mild Type Leukoencephalopathy Caused by a Homozygous EARS2 Mutation.

Childhood leukoencephalopathies are a broad class of diseases, which are extremely rare. The treatment and classification of these disorders are both challenging. Nearly half of children presenting with a leukoencephalopathy remain without a specific diagnosis. Leukoencephalopathy with thalamus and brain stem involvement and high lactate (LTBL) is a newly described childhood leukoencephalopathy caused by mutations in the gene encoding a mitochondrial aminoacyl-tRNA synthetase specific for glutamate, EARS2 Magnetic resonance images show a characteristic leukoencephalopathy with thalamic and brain stem involvement. Here, we report a different clinical course of LTBL supported by typical MRI features in a Turkish patient who presented with a history of failure to walk. The EARS2 gene mutation analysis identified a c.322C>T transition, predicting a p.R108W change. This is the first reported early-onset mild type LTBL caused by a homozygous EARS2 mutation case in the literature

Normal neonatal electroencephalography and maturation of electroencephalography during neonatal period

Despite evolving technologies, electroencephalography (EEG) remains a powerful tool for neurological diagnosis and prognosis in both preterm and term neonates. Neonatal EEG is different from children and adult's EEG technically and also because it changes week by week as a result of brain maturation and growth. An EEG finding normal in a developmental stage may be abnormal in a different developmental stage owing to these rapid changes of brain growth. So it is important to know normal patterns of neonatal EEG in different conceptional weeks and behavioral states (sleep, awakeness). In this section, technical and qualitative features of normal neonatal EEG will be mentioned, normal graphoelements and maturational changes on the EEG background of neonates going through preterm to term age will be described