Ayşe Tosun

Subclinical Neurological Abnormalities in Children With Celiac Disease Receiving a Gluten-free Diet

Objectives: Because clinically evident manifestations are frequent in adults with celiac disease (CD), we aimed to investigate whether early neurological abnormalities may be detected in children with CD. Methods: Electroencephalography, electromyography, and somatosensory evoked potentials were performed in children with CD receiving a gluten-free diet. Results: The neurophysiological tests revealed subclinical neurological abnormalities associated with CD in 3 (11%) of 27 children: 2 had peripheral polyneuropathy documented with electromyography, and 1 had prolonged latencies in somatosensory evoked potential. Magnetic resonance imaging showed abnormalities in 2 (7.4%) of children: pontine demyelinization in 1 and cortical atrophy in the other. Conclusions: Because the rate of neurological problems is increased in children with CD, neurological abnormalities should be carefully investigated early after the diagnosis of CD is made.

Neuropsychologic Impairment in Children With Rolandic Epilepsy

Although patients with benign childhood epilepsy with centrotemporal spikes exhibit normal intelligence, they frequently display neuropsychologic abnormalities. Thirty-five patients with rolandic epilepsy were included in this study. They were divided into three subgroups. Group I comprised patients with rolandic focus who were not receiving treatment. Group II comprised patients with rolandic focus who were receiving treatment. Group III comprised patients who demonstrated improved foci and were not receiving treatment. The control group comprised 16 children who were similar to patients in terms of age, sex, and sociocultural level. All children underwent standardized neuropsychologic testing, including the Wechsler Intelligence Scale for Children-Revised subtests, Bender Gestalt Test, Stroop Test, Visual Aural Digit Span, Reading and Writing Performance, and Dichotic Listening Test. Patients exhibited significantly impaired visuomotor and reading ability and attention to verbal stimuli compared with control subjects. Reading disability persisted in patients in remission from seizures and epileptic discharges. Contrary to the presumed benign nature of rolandic epilepsy, this disorder may cause learning disabilities. Therefore, patients must be followed longitudinally to identify any learning problems.

Convulsive status epilepticus in children: Etiology, treatment protocol and outcome

This study aimed to determine the etiology, treatment protocol and outcome of convulsive status epilepticus (SE) in children. An institutional treatment protocol using benzodiazepines (diazepam and midazolam) was assessed in a retrospective case study. The treatment protocol (Ege Pediatric Status Epilepticus Protocol or EPSEP) was developed based on an operational definition of pediatric SE according to the duration of seizure activity. Pediatric SE is divided into three categories: initial SE (20-30 min), established SE (30-60 min) and refractory SE (>60 min). Eight (30%) of the studied episodes were initial SE, 10 (37%) were established SE, and 9 (33%) were refractory SE. With respect to the etiological spectrum of SE, 11 (40%) children had meningitis or encephalitis. Febrile SE was identified in 7 (26%) patients. Only 2 episodes of initial SE (7.5%) were controlled with first step of the protocol (two concomitant-doses of rectal diazepam). Midazolam bolus and infusions (up to 1.2 μg/kg/min) were used to treat 22 episodes of SE (9 refractory SE, 10 established SE and 3 initial SE). Complete arrest of convulsive SE was achieved in 21 of 22 (95%) episodes with midazolam infusion. We concluded that the combined use of benzodiazepines (diazepam+midazolam) was safe and effective in the treatment of convulsive SE in children.

Febrile Seizures: Interleukin 1β and Interleukin-1 Receptor Antagonist Polymorphisms

In order to investigate the association between IL-1beta -511 C-->T and IL-1 receptor antagonist intron 2 variable tandem repeat polymorphisms, and febrile seizures in children, 90 children (mean age, 19.7 +/- 11.2 months) diagnosed with febrile seizure and 106 healthy controls (mean age, 14.2 +/- 3.6 months) with no seizure or neurologic events were included in the study. The polymorphisms were analyzed using restriction fragment length polymorphism and agarose gel electrophoresis methods. In the patient group, the frequencies of IL-1beta genotypes CC, CT, and TT were 24.4%, 52.2%, and 23.3%, respectively, compared with 38.7%, 50.95%, and 10.4%, respectively, in the control group. The TT genotype was significantly more common in the patient group than in the control group (P = 0.044), and the T allele frequency was significantly higher in the patient group (0.50 vs 0.36, P = 0.040). Among the three genotypes (RN1/1, RN1/2, and RN2/2) of the IL receptor antagonist gene variable tandem repeat polymorphisms, the frequency of both the RN2/2 genotype and the RN2 allele were significantly higher in the patient group (P = 0.007). Also RN2 allele frequency was found higher in patient group than controls (0.29 vs 0.15, P = 0.020). IL-1beta -511 and IL-1 receptor antagonist intron 2 variable tandem repeat polymorphisms may be involved in susceptibility to febrile convulsions in children.

Vigabatrin caused rapidly progressive deterioration in two cases with early myoclonic encephalopathy associated with nonketotic hyperglycinemia

Vigabatrin, a structural analogue of γ-aminobutyric acid (GABA), is used for the treatment of generalized and partial seizures in infants. The drug inhibits the GABA transaminase and elevates the GABA concentration in the brain. Here we present the vigabatrin experience in two patients with early myoclonic encephalopathy owing to nonketotic hyperglycinemia (glycine encephalopathy). Both patients had early infantile seizures characterized by fragmentary myoclonic jerks associated with burst-suppression pattern on electroencephalography. Nonketotic hyperglycinemia was diagnosed with elevated cerebrospinal fluid and plasma glycine levels. The seizures were initially thought to be infantile spasms, and vigabatrin (50 mg /kg/day) was started for the treatment of seizures. Rapidly progressive deterioration was noticed after a few days. Acute encephalopathy associated with sleepiness and respiratory failure developed. Vigabatrin produced acute encephalopathy, which regressed in a few days after vigabatrin was stopped in the first patient. However, in the second case, despite the discontinuation of vigabatrin, there was no recovery of general conditions. Our observations in two cases indicate the risk of using vigabatrin in patients with nonketotic hyperglycinemia. The elevated GABA concentration in the brain can enhance the encephalopathy, together with the elevated levels of glycine. (J Child Neurol 2006;21:82—84).

Cerebrospinal fluid interleukin-6 levels in patients with west syndrome

Elevated cytokine response has been reported in patients with epileptic seizures. The objective of this study was to investigate the possible role of interleukin-6 (IL-6) in the pathogenesis of infantile spasms in West syndrome (WS). We measured IL-6 levels in cerebrospinal fluid (CSF) obtained from the newly diagnosed patients with WS. Twelve patients with WS (Group I) were classified as symptomatic WS (Group IA) in eight and as cryptogenic WS (Group IB) in four. The results were compared with control groups including patients with tonic-clonic seizures associated with two different kind of inflammation of central nervous system; Group IIA (infection): bacterial meningitis/encephalitis and Group IIB (trauma): post-traumatic seizures. There was no statistically significant difference between the mean values of CSF IL-6 levels in patients with WS (2.95 +/- 2.31 pg/ml) and those of subgroups of WS (Group IA: 2.26 +/- 2.01 pg/ml and Group IB: 4.33 +/- 2.52 pg/ml). Both control groups had highly increased IL-6 levels in CSF (Group IIA: 193.05 +/- 185.52 pg/ml and Group IIB: 112.74 +/- 167.44 pg/ml) than those of the patients with WS. Elevated IL-6 response in patients with tonic-clonic seizures associated with inflammation of central nervous system might be due to the seizures themselves or related to the underling etiology (infection or trauma). However, no elevated IL-6 response was found in patients with infantile spasms.

The effect of depression on academic achievement in children with epilepsy

In this cross-sectional study our aim was to evaluate the effect of depression on academic achievement in children with epilepsy and low school performance. Fifty-one children with epilepsy and low school performance were evaluated with the Childrens Depression Inventory (CDI) to measure depressive symptoms. School performance was evaluated with Achenbachs Child Behavior Checklist (CBCL) and the Teacher Report Form (TRF). Children diagnosed with depressive spectrum disorders received medical therapy. All tests were administered in the first interview and repeated at the end of 6 months of therapy. Forty-three children completed the study. The patients were evaluated with DSM-IV diagnostic criteria. Accordingly, 9 (20.9%) children had Major Depressive Disorder (MDD) and 4 (9.3%) had Depressive Disorder, Not Otherwise Specified (DD-NOS). All children with MDD and DD-NOS received antidepressant medication, but only seven of them completed treatment. Posttreatment CDI scores were significantly lower, and TRF scores also improved. Pediatric neurologists should be aware of the possibility of depressive disorders in children with epilepsy, especially in those with low school performance.

Ratios of Nine Risk Factors in Children With Recurrent Febrile Seizures

Febrile seizures are the most common convulsive disorder of childhood, with a recurrence probability of 33%. The aim of the study was to determine the risk factors for recurrence of febrile seizures in children. In this descriptive, cross-sectional study, nine risk factors of recurrence of febrile seizures were investigated in 259 children with febrile seizures: (1) sex; (2) domicile; (3) income level; (4) family history of febrile seizures; (5) family history of epilepsy; (6) level of fever; (7) duration of fever; (8) type of seizure, simple vs complex; and (9) age at seizure onset. The risk factors were compared for 119 children with isolated febrile seizures (45.9% of the total) and 140 children with two or more febrile seizure recurrences (54.1%). Among the patients with and without recurrent febrile seizures, 32% and 18% were domiciled in nonurban areas, respectively (P = 0.012). There was a family history of febrile seizures in 57% and 44% of cases with and without recurrent febrile seizures, respectively (P = 0.031). According to the logistic regression analysis, a family history of febrile seizures was a risk factor that affected recurrence (P = 0.018; odds ratio OR = 1.933; 95% confidence interval CI = 1.121-3.333). We also found that domicile (P = 0.001) and income (P = 0.013) were risk factors for recurrence. A family history of epilepsy was not a significant risk factor (P = 0.129; OR = 2.110; 95% CI = 0.804-5.539).

Evaluation of the Cases with Acute Disseminated Encephalomyelitis

ObjectiveWe aimed to describe the epidemiologic, clinical, laboratory features, neuroimaging, treatment, and outcome of children with acute disseminated encephalomyelitis in a cohort study.MethodsIn this study, twelve children who were diagnosed as acute disseminated encephalomyelitis were reviewed retrospectively. All of the cases were reevaluated with systemic and neurological examinations, serologic tests, cerebrospinal fluid investigations, magnetic resonance imaging.ResultTheir age ranged between 2.5 and 16 years. Five of the cases had initial infections. Patients presented most often with motor deficits (75%), secondly with loss of conscious (33%), and seizures (33%). Spinal fluid abnormalities occurred in 41.6%. Cranial, and spinal magnetic resonance imaging (MRI) revealed hyperintense signal changes mainly in basal ganglia and thalamus (58%), cortical and subcortical areas (33) in T2 weighted images. Myelitis was determined in two cases. Six patients were treated with steroid, and 3 were treated with intravenous immunoglobulin. Ten patients recovered completely. We observed relapse in one case and recurrence in two cases. These cases responded well to high dose intravenous prednisolone followed by oral prednisolone for 6 months.ConclusionOutlook recovery is generally good in acute disseminated encephalomyelitis. Recurrence and neurological deficits are rarely seen. Early treatment of prednisolone is one of the most important factors to determine the prognosis in this disease.

Arterial ischemic stroke in childhood: risk factors and outcome in old versus new era.

Risk factors of children with arterial ischemic stroke were retrospectively evaluated. The children were grouped according to values on developing diagnostic tools: 13 in the old era (1987-1994) and 18 in the new era (1995-2004). The old era battery included 5 tests: protein C, protein S, antithrombin, lupus anticoagulants, and anticardiolipin antibodies. The new era battery added 5 more tests: homocystine level, factor VIII level, mutations for factor V Leiden and prothrombin G20210A, and lipoprotein (a) level. At least 1 risk factor was found in 5 of 13 children (38.5%) in the old era and in 8 of 18 (44.4%) in the new era. The extended battery for prothrombotic disorders revealed 7 risk factors in 4 children (22.2%) in the new era, whereas the limited battery identified a single risk factor in 1 child (7.7%) in the old era. For the correct etiologic identification, prothrombotic risk factors should be extensively evaluated in patients with arterial ischemic stroke.