Nesrin Şenbil

Nasal Midazolam Effects on Childhood Acute Seizures

Sixteen children, aged from 2 months to 14 years, with a diagnosis of acute seizures and seen at Dr. Sami Ulus Child Health and Disease Center, were included in this study. Midazolam (5 mg/mL) 0.2 mg/kg was administered intranasally in 30 seconds by an injector. The heart rate, respiratory rate, blood pressure, and oxygen saturation were recorded at 0, 5, and 10 minutes after administration. The seizures of three (18.7%) patients terminated within 1 minute, of seven (43.7%) patients in 1 to 2 minutes, and of three (18.7%) patients in 2 to 5 minutes. However, three (18.7%) patients did not respond to treatment. As a result, it was concluded that intranasal midazolam administration is easy and effective. The half-life of midazolam is shorter than diazepam, and midazolam has fewer complications when compared with diazepam. It is easier to use in nasal drop and spray forms. (J Child Neurol 2000;15:833-835).

Epileptic and non-epileptic cerebral palsy: EEG and cranial imaging findings

The aims of the study were to compare the clinical types, electroencephalogram (EEG) and cranial magnetic resonance imaging/computed tomography findings of epileptic and non-epileptic cerebral palsy (CP) patients. Seventy-four patients with CP were evaluated in 2 years. Tetraplegic CP had a higher incidence of epilepsy (60.5%). EEG was confirmed abnormal in epileptic CP as 90.3%, and in non-epileptic CP as 39.5%. Focal epileptiform activity, generalized slowing, and multifocal epileptiform activity were significantly frequent in epileptic CP. There were cranial imaging abnormalities of 74.2% in epileptic and 48.8% in non-epileptic CP. Although there was not any statistically significant difference between the two groups, epileptic group revealed more structural abnormalities. Further studies concerning a possible risk of epilepsy development and its relations with the EEG and cranial imaging findings are needed in presenting the other risk factors involved and the factors affecting the CP prognosis.

Prevalence of some risk factors in children with epilepsy compared to their controls

AIM The goal of this case-control study was to identify the significance of certain risk factors for epilepsy in Turkey. METHOD A total of 805 cases, aged 1-16 years, followed-up for epilepsy at the Pediatric Neurology Department and a control group consisting of 846 age-matched cases without epilepsy were included in the study. The risk factors examined were gender, neurological impairment, febrile convulsion, head trauma, central nervous system infections, parental consanguinity, family history of epilepsy, prenatal and natal risk and newborn jaundice. Data regarding the investigated epilepsy risk factors were obtained through a questionnaire via personal interviews and the medical records and were assessed using univariate and multivariate analysis. RESULT Univariate analysis showed an increased risk for epilepsy with a history of atypical febrile seizure (21.97-fold), severe and moderate head injury (27.76- and 7.09-fold respectively), CNS infection (4.76-fold), history of epilepsy in first-, second- or third-degree relatives (6.42-, 3.09- and 2.66-fold, respectively), presence of maternal hypertension (4.31-fold), an apgar score < or =6 at any time (7.78-fold) and neonatal jaundice (3.12-fold). Abnormal neurological signs increased the epilepsy risk 5.92 times in univariate analysis and 30.26 times in multivariate analysis. CONCLUSION The most important risk factors for epilepsy in this study were neurological impairment, history of atypical febrile seizures, severe head injury and a low apgar score. Other important risk factors were moderate head trauma and a history of epilepsy in the family.

Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm.

A 13‐year‐old girl was referred for assessment of severe gastrointestinal tract bleeding. Her liver function tests were normal, and she had no evidence of chronic liver disease or history of significant trauma. Clinical and sonographic findings suggested the presence of a portal vein aneurysm associated with a hepatoportal arteriovenous fistula. Abdominal angiography confirmed the diagnosis. The arteriovenous fistula was congenital, and the associated portal vein aneurysm was either congenital or secondary to hemodynamic changes in the portal venous system.

Combined Treatment With Subcutaneous Interferon-α, Oral Isoprinosine, and Lamivudine for Subacute Sclerosing Panencephalitis

We compared patients with subacute sclerosing panencephalitis who received treatment according to our protocol for at least 6 months (19 patients) with the patients who could not receive any treatment (13 patients). The treatment protocol consisted of oral isoprinosine (100 mg/kg/day), subcutaneous interferon alpha-2a (10 mU/m2/three times a week), and oral lamivudine (10 mg/kg/day). There were no statistical differences between the two groups according to Neurological Deficit Index, clinical stage, and average age on admission and also on the final evaluation after treatment. The mortality rates of both groups were similar: 3 (15.7%) for the treatment group and 6 (46%) for controls. The remission rates for the treatment and control groups were 7 of 19 (36.8%) and 0 of 13 (0%), respectively, and the difference was statistically significant (P = .036). The mean survival period of the treatment group was significantly longer than that of the control group (P = .01). In conclusion, this combination treatment protocol resulted in higher remission rates and longer survival periods when compared with controls, as well as a remission rate that was better than the spontaneous remission rate of 5%. For this reason, and as well as because interferon-α therapy has an easier route of application and a higher family compliance, we have considered this an alternative protocol for patients with subacute sclerosing panencephalitis. (J Child Neurol 2003; 18:104—108).

Central Pontine and Extrapontine Myelinolysis Owing to Disequilibrium Syndrome

Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients.Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor, and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement. After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients. (J Child Neurol 2003;18:292—296).

Prothrombotic risk factors in children with hemiplegic cerebral palsy

Background: The purpose of the present paper was to investigate the prevalence of prothrombotic risk factors associated with hemiplegic cerebral palsy (CP).

Nonconvulsive Status Epilepticus on Electroencephalography in a Case With Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5Z\x-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury. The magnetic resonance image of the brain and computed tomographic scan of the head were normal. His electroencephalogram (EEG) showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. After treatment with phenytoin for 2 days, an EEG showed periodic high-amplitude sharp-and-slow-wave complexes, which were also not suppressed with intravenous diazepam. Since the patient had measles at 5 months of age, subacute sclerosing panencephalitis was considered, and the diagnosis was confirmed by the presence of measles antibodies in cerebrospinal fluid. (J Child Neurol 2006;21:256—260; DOI 10.2310/7010.2006.00056).

Paroxysmal non-kinesigenic and hypnogenic dyskinesia associated with Streptococcal infection

© 2008 Japan Pediatric Society Central nervous system diseases associated with group A beta hemolytic Streptococcus (GABHS) infections were recently called post-streptococcal autoimmune disorders of the central nervous system. After the description of Sydenham’s chorea as a classic model for this entity, pediatric autoimmune neuropsy chiatric disorders (PANDAS) associated with streptococcal infection were described. 1 More recently, myoclonus, 2 acute disseminated encephalomyelitis, 3 dystonia, 4 paroxysmal dystonic choreathetosis, 5 chorea encephalopathy, 6 and parkinsonism 7 were reported to be associated with GABHS in the literature. The paroxysmal dyskinesias are a group of intermittent movement disorders such as dystonia, chorea, athetosis, ballismus, or any combination of these hyperkinetic disorders. They are divided into four main categories: kinesigenic, non-kinesigenic, hypno genic, and exercise-induced. Additionally, they can be classifi ed etiologically as primary or secondary in relation to other underlying neurological diseases. 8

Cerebrospinal Fluid Nitric Oxide Levels in Subacute Sclerosing Panencephalitis

Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in subacute sclerosing panencephalitis (n = 47) and age-matched control (n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 micromol/L) and nitrate (median, 6.14 micromol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of subacute sclerosing panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 micromol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in subacute sclerosing panencephalitis patients with myoclonus (median, 9.26 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.