Yüksel Yılmaz

Increased QT dispersion in epileptic children

Aim: Epilepsy is a common paroxysmal disorder in childhood. Tachyarrhythmia, bradyarrhythmia, asystole, atrioventricular block, ventricular fibrillation or sudden death may occur during seizures. Mutations of ion‐channel coding genes are found in patients with idiopathic or cryptogenic epilepsy. The ion channels also play a role in arrhythmogenesis. QT dispersion is a non‐invasive method for assessment of regional repolarization differences within the myocardial tissue. This study investigated QT and QTc dispersion (QTcd) and the risk of dysrhythmia in epileptic children. Methods: The first group included 28 patients with newly diagnosed epilepsy and not taking antiepileptic treatment (range 10 mo to 15 y, mean ± SD 6.86 ± 3.92 y), the second group included 34 patients taking antiepileptic treatment (range 1–14 y, mean ± SD 7.51 ± 3.68y) and the control group included 52 healthy children (range 4 mo to 15 y, mean ± SD 6.94 ± 3.92 y). Twelve‐lead ECGs were obtained and heart rate, RR interval, P wave amplitude and duration, PR interval, QRS duration, QRS axis and QT intervals were measured, and QTc, QTd, QTcd were calculated in all subjects. The measurements were repeated in the first group under antiepileptic treatment. Results: While no significant difference in terms of heart rate, RR interval, P wave amplitude and duration, PR interval, QRS duration, QRS axis, QT intervals or QTc intervals was found, QTd and QTcd values were significantly increased in epileptic children compared with the control group. QTd was 58.1 ± 13.4 ms and 35.9 ± 9.3 ms and QTcd was 91.0 ± 22.9 and 68.6 ± 18.0 ms in patients and controls, respectively. Antiepileptic treatment did not affect QT dispersion.

Prognostic Value of Auditory Brainstem Response for Neurologic Outcome in Patients With Neonatal Indirect Hyperbilirubinemia

To investigate the value of the auditory brainstem response as a reliable test for the neurologic prognosis of infants with neonatal indirect hyperbilirubinemia, auditory brainstem response studies were performed in 22 infants. The patients were followed up until 12 months of age. Two patients demonstrated pathologic auditory brainstem response consistent with auditory neuropathy but had no neurologic finding except a lack of speech at 12 months of age. Two other patients had neurologic sequelae, one showing severe dyskinetic cerebral palsy, the other mild hypotonia and motor retardation, but their auditory brainstem response results were normal. These results suggested that auditory brainstem response examination might not provide reliable information for the neurologic prognosis. Neurologic disturbances resulting from bilirubin neurotoxicity can be seen in patients with a normal auditory brainstem response, but patients with an abnormal auditory brainstem response may not have any neurologic dysfunction apart from speech retardation. (J Child NeuroL 2001;16:772-775).

MAGNETIC RESONANCE IMAGING CHARACTERISTICS OF BENIGN MACROCEPHALY IN CHILDREN

Benign macrocephaly of infancy is a common problem in the child neurology practice. The radiologic features of this entity are not well defined. In most of the previous studies, macrocephalic patients were evaluated by computed tomography. To define the radiologic characteristics of this entity, 20 children with macrocephaly with normal neurologic examinations were enrolled in the study. All the patients were evaluated by magnetic resonance imaging studies. Sixty-five percent of patients had enlargement of the subarachnoid space and 35% of patients had megalencephaly. None of the patients had subdural collections. The mean age of patients with enlargement of the subarachnoid space was found to be younger than those with megalencephaly. The cases with parental histories of macrocephaly demonstrated both enlargement of the subarachnoid space and megalencephaly. Our results suggest that the radiologic finding of benign macrocephaly can be both enlargement of the subarachnoid space and megalencephaly. (J Child Neurol 1999;14:678-682).

Thalamic involvement in a patient with kernicterus

Abstract. We report the MR imaging findings of a 16-month-old boy with dyskinetic cerebral palsy resulting from kernicterus. T2-weighted images showed symmetric bilateral hyperintensity in the thalamus in addition to the globus pallidus.

Cerebral vein thrombosis in Behçet’s disease

Behçets disease is a chronic, relapsing multisystem disorder, and nervous system involvement is one of the serious manifestations. Neuro-Behçet is rarely reported in children and may present with a wide variety of symptoms because the entire neuraxis may be affected. A case of cerebral vein thrombosis secondary to Behçets disease is presented. The patient has recovered without any visual loss and had no complaints at 1-year follow-up.

Increased QT dispersion in breath-holding spells

Aim: Breath‐holding spells are common in infancy and early childhood, and patients are frequently referred to paediatric cardiology clinics for exclusion of heart disease. Recent data reveal subsequent development of epilepsy and neurocardiogenic syncope. Autonomic dysregulation and increased vagal stimulation leading to cardiac arrest and cerebral ischaemia is considered as the cause. Iron deficiency anaemia may be associated with these spells. We studied QT dispersion for the assessment of ventricular repolarization in these patients. Methods: The study group consisted of 19 girls and 24 boys between 3 and 108 mo of age (mean ± SD = 22.7 ± 17.7 mo); and the control group consisted of 13 girls and 12 boys between 3 and 57 mo of age (mean ± SD = 22.9 ± 15.1 mo). QT interval was measured; corrected QT interval (QTc), QT dispersion (QTd) and QTc dispersion (QTcd) were calculated from 12‐lead surface electrocardiograms of the patients and the control group. Results: There was no statistically significant difference in terms of QT and QTc intervals between patient and control groups, while QTd and QTcd values were significantly increased in patients with breath‐holding spells compared to the healthy children. QT dispersion was 59.5 ± 35.9 ms and 44.8 ± 11.9 ms, respectively, in patients and controls (p < 0.05). QTc dispersion was 102.1 ± 41.9ms and 79.6 ± 24.6ms, respectively (p < 0.01). The presence of iron deficiency did not effect the QT and QTc dispersion.

Pseudotumour cerebri in children: Etiological, clinical features and treatment modalities

To investigate the etiological and clinical features of pseudotumour cerebri (PTC) in children, features of 12 children with PTC were documented. The etiology could be clarified in 6 patients. The other 6 patients were accepted as idiopathic intracranial hypertension (primary PTC). Acetazolamide was used as the first drug and was effective in only 4 patients who had no underlying cause. Repeated lumbar punctures (LP) were performed in 6 patients and 5 of them were unresponsive. Prednisone was used in 3 patients and was effective in only one patient. The CSF pressure continued to be high in five patients and could be normalized only by withdrawing of the precipitating drug in three patients and by ventricular-peritoneal shunting in two patients with cerebral venous thrombosis. Although, medical treatment and repeated LP are usually effective in children with PTC, clarifying and solving the underlying cause can be crucial.

Etiological analysis of presumed perinatal stroke

This study aimed to investigate the maternal, pre- and perinatal, and prothrombotic factors with congenital hemiparesis due to presumed perinatal stroke (PPS). Prothrombotic risk factors including protein C and S, antithrombin III, lipoprotein (a), homocystein, factor VIII levels; anticardiolipin antibodies and lupus anticoagulant; methylenetetrahydrofolate reductase mutations, factor V Leiden, prothrombin G20210A mutations were investigated. Arterial ischemic stroke was detected in 60% and periventricular venous infarction in 40%. At least one prothrombotic risk factor was present in 69%, two in 17%, and three or more in 8.5% of cases. The most common combination was methylenetetrahydrofolate reductase C677T and factor V Leiden heterozygosity. The etiology and pathogenesis of PPS is still unclear. According to this study, most of the patients with PPS might have one or more prothrombotic risk factors and certain prenatal risk factors including intrauterine growth retardation, twin gestation and preeclampsia might be related to PPS.

Clinical Characteristics and Prognostic Factors in Childhood Bacterial Meningitis: A Multicenter Study

OBJECTIVE To evaluate clinical features and sequela in children with acute bacterial meningitis (ABM). STUDY DESIGN Multicenter retrospective study. MATERIAL AND METHODS Study includes retrospective chart review of children hospitalised with ABM at 11 hospitals in İstanbul during 2005. Follow up visits were conducted for neurologic examination, hearing evaluation and neurodevelopmental tests. RESULTS Two hundred and eighty three children were included in the study. Median age was 12 months and 68.6% of patients were male. Almost all patients had fever at presentation (97%). Patients younger than 6 months tended to present with feeding difficulties (84%), while patients older than 24 months were more likely to present with vomitting (93%) and meningeal signs (84%). Seizures were present in 65 (23%) patients. 26% of patients were determined to have at least one major sequela. The most common sequelae were speech or language problems (14.5%). 6 patients were severely disabled because of meningitis. Presence of focal neurologic signs at presentation and turbid cerebrospinal fluid appearance increased sequelae significantly. Childen under 24 months of age developed neurologic sequelae more commonly than older children. CONCLUSION Symptoms and signs were largely depending on the age of the patient. Speech or language problems were the most common sequelae following meningitis.

Is the brain biopsy obligatory or not for the diagnosis of Schilder’s disease? Review of the literature

BackgroundSchilder’s myelinoclastic diffuse sclerosis (Schilder’s disease) is a rare demyelinating disorder. Clinical features and neuroimaging findings of this disease might mimic an intra-cranial mass lesion including an abscess or a tumour.Case reportClinical and radiological findings of two children with the diagnosis of Shilder’s disease are reported, and the role of brain biopsy as a diagnostic tool is discussed.