Muzaffer Polat

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.

Premedication during micturating cystourethrogram to achieve sedation and anxiolysis

Micturating cystourethrogram (MCUG) is an imaging technique indicated in the diagnosis and follow-up of many diseases. We investigated the reliability and the efficacy of midazolam and chloral hydrate in sedation and anxiolysis during micturating cystourethrogram. Fifty-three children of similar ages (39 girls, 14 boys, mean age of 5.8±3.5xa0years) were randomized to midazolam (n=17), chloral hydrate (n=18) and control groups (n=18). Oral midazolam 0.6xa0mg/kg or chloral hydrate 25xa0mg/kg or saline were administered to the study groups 15–30xa0min prior to the urinary catheterization. Brietkopf and Buttner, Frankl and Houpt scales and Spielberger’s State Anxiety Inventory and parent’s impressions were used to assess the level of sedation and anxiety. The Brietkopf and Buttner classification of emotional status and Houpt behavior rating scale demonstrated a significantly better emotional status and sedation in the midazolam group when compared to controls (P=0.01 and P=0.018, respectively). The catheterization was described as a more unpleasant and distressing event by the parents of the control and the chloral hydrate groups when compared to the parents of the midazolam group (P<0.05). Bladder capacity and frequency of detection of residual urine were not statistically different between the three study groups (P>0.05). Vital signs did not change significantly in any child. Sedation with midazolam does not have adverse effects on the results of micturating cystourethrogram, while it reduces the discomfort in children undergoing this radiological technique.

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.

Assessment of Sleep-Related Breathing Disorders in Patients With Duchenne Muscular Dystrophy

Background Respiratory failure is a significant cause of morbidity and mortality in neuromuscular diseases. Although clinical findings and respiratory function tests aid in diagnosing sleep-related breathing disorders, polysomnography is the gold standard for the diagnosis of these disorders. We aimed to investigate the role of sleep-wake symptoms and clinical findings of patients with Duchenne muscular dystrophy (DMD) in predicting sleep-related breathing disorders through the comparison of polysomnography findings. In addition, we evaluated the sleep architecture of our patients. Methods A total of 35 children (12 patients with DMD and 23 controls) were included in this cross-sectional study. Activity status and clinical severity of the patients were determined by history and clinical findings such as scoliosis, obesity. All subjects were hospitalized for one night in the Sleep Unit and their polysomnography examinations were performed. Sleep, breathing, arousals and limb movements were scored manually according to the American Sleep Disorders Association criteria. Results Nocturnal and daytime symptoms were present in 50% of patients with DMD, 40.8% were wheelchair-bound and 58% had scoliosis. Obstructive sleep apnea was noted in 16.6% of patients with DMD. The apnea-hypopnea index, leg movement index were significantly higher in the DMD group as compared to the control group (P < 0.05). The number of desaturations, total arousal index and the percentage of total superficial sleep were significantly higher in patients with wheelchair, scoliosis, sleep-wake symptoms. Conclusions Being wheelchair-bound or having scoliosis do not predict sleep-related breathing disorders, so patients with DMD should be followed-up via polysomnography. Sleep-wake symptoms should be carefully questioned in these patients and symptomatic patients should be referred to pediatric respiratory units.

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.