Benign epilepsy with centro-temporal spikes (BECTS): relationship between unilateral or bilateral localization of interictal stereotyped focal spikes on EEG and the effectiveness of anti-epileptic medication.

BACKGROUND AND AIM: Benign epilepsy with centro-temporal spikes (BECTS) is one of the most frequent epileptic syndromes in children. It is placed among the idiopathic localization-related epilepsies. However, the relationship between unilateral or bilateral localization of interictal stereotyped focal spikes on electroencephalogram (EEG) and the effectiveness of anti-epileptic drugs has not been studied yet. PATIENTS AND METHODS: We studied 55 neurodevelopmentally normal children who had been diagnosed with BECTS. Children were subdivided into two groups, based on EEG findings: Group A comprised 30 children with unilateral findings on EEG and Group B 25 children with bilateral findings on EEG. All patients in the present study were started on an anti-epileptic medication after the third seizure (Sodium Valproate, Carbamazepine, Oxcarbazepine) and we studied the response to medications. RESULTS: Children with bilateral findings on EEG had the same response to treatment with either Sodium Valproate or Carbamazepine or Oxcarbazepine. Other side, children with unilateral findings on EEG corresponded best to Carbamazepine or Oxcarbazepine. CONCLUSIONS: Children diagnosed with BECTS and bilateral discharges on EEG have good response to treatment with either Sodium Valproate or Carbamazepine or Oxcarbazepine.

Idiopathic acute transverse myelitis: Complete recovery after intravenous immunoglobulin.

Idiopathic acute transverse myelitis is a focal inflammatory disorder of the spinal cord of unknown etiology diagnosed according to established criteria. As it occurs rarely in children herein we report a case of a 4 year old boy who developed clinical and radiological manifestations of myelitis, 10 days after a recent respiratory tract infection. Diagnostic workup failed to reveal a causative factor. After the administration of corticosteroids a clinical deterioration was observed and intravenous immunoglobulin was administered. Symptoms resolved within a 48-hour period, suggesting an immune-mediated pathogenetic mechanism.

Evaluation of thyroid gland function in children with obstructive apnea hypopnea syndrome.

Patients with obstructive sleep apnea hypopnea syndrome (OSAHS) and thyroid function abnormalities, such as hypothyroidism and Hashimoto's thyroiditis, usually have closely resembling clinical features. Differentiation between these disorders is made more difficult because hypothyroid patients are also at risk for secondary sleep-disordered breathing. The aim of our study is to evaluate the prevalence of thyroid function abnormalities in children with OSAHS. Forty-four children (15 females: 29 males), 2.5-14.5 (7.43±2.98) years old were studied with overnight polysomnography. Biochemical screening of thyroid gland function was also carried out. Patients were judged to have OSAHS based mainly on the evaluation of Apnea Hypopnea index per hour of sleep (AHI). 15/44 (34.1%) children had mild OSAHS, 17/44 (38.6%) moderate and 12/44 (27.3%) severe OSAHS. Hypothyroidism was recorded only in 5/44 (11.4%) and Hashimoto's thyroiditis in 3/44 (6.8%) of OSAHS patients. Two patients with hypothyroidism showed mild and three severe OSAHS, while from the 3 children with Hashimoto's thyroiditis one presented mild, one moderate and one severe degree of OSAHS. Although the majority of studies in bibliography worldwide do not consider necessary the systemic evaluation of thyroid gland function in patients with breathing disorders during sleep, it seems that in children this type of screening is required for the differential diagnosis between primary sleep apnea and hypothyroid sleep-disordered breathing in order to differentiate these two conditions. Therefore, the laboratory investigation of thyroid gland function could be considered necessary.

Maternal smoking during pregnancy and childhood lymphoma: a meta-analysis.

Results from epidemiological studies exploring the association between childhood lymphoma and maternal smoking during pregnancy have been contradictory. This meta-analysis included all published cohort (n = 2) and case-control (n = 10) articles; among the latter, the data of the Greek Nationwide Registry for Childhood Hematological Malignancies study were updated to include all recently available cases (-2008). Odds ratios (ORs), relative risks and hazard ratios were appropriately pooled in three separate analyses concerning non-Hodgkin lymphoma (NHL, n = 1,072 cases), Hodgkin lymphoma (HL, n = 538 cases) and any lymphoma (n = 1,591 cases), according to data availability in the included studies. An additional metaregression analysis was conducted to explore dose-response relationships. A statistically significant association between maternal smoking (any vs. no) during pregnancy and risk for childhood NHL was observed (OR = 1.22, 95% confidence interval, CI: 1.03-1.45, fixed effects model), whereas the risk for childhood HL was not statistically significant (OR = 0.90, 95% CI: 0.66-1.21, fixed effects model). The analysis on any lymphoma did not reach statistical significance (OR = 1.10, 95% CI = 0.96-1.27, fixed effects model), possibly because of the case-mix of NHL to HL. No dose-response association was revealed in the metaregression analysis. In conclusion, this meta-analysis points to a modest increase in the risk for childhood NHL, but not HL, among children born by mothers smoking during pregnancy. Further investigation of dose-response phenomena in the NHL association, however, warrants accumulation of additional data.

Delayed exposure to infections and childhood lymphomas: a case-control study.

OBJECTIVES: Delayed exposure to common infections during childhood, have been implied to cause strong immunological response to a single infectious agent that eventually triggers leukemogenesis. The aim of the present study was to investigate whether decreased exposure to infections, as reflected in a more seronegative spectrum to several common infectious agents, is associated with increased risk for the development of childhood lymphomas. METHODS: All 125 children (up to 14 years old), with Hodgkin (HL, n = 52) and non-Hodgkin lymphomas (NHL, n = 73) diagnosed through the national network of childhood Hematology-Oncology units during an 8-year period were enrolled in the study along with 125 age- and gender-matched controls. Past exposure to nine common infections [respiratory syncytial virus (RSV), influenza A and B, parainfluenza type 1, adenovirus, Epstein-Barr virus (EBV), cytomegalovirus (CMV), human herpes virus 6 (HHV6), Bartonella henselae] was assessed using serological markers. RESULTS: After controlling for possible confounding factors, the overall seronegativity status upon diagnosis was statistically significantly associated with NHL [odds ratio; 95% CI: 1.45 (1.10-1.93), p = 0.01] and less so with HL risk [odds ratio; 95% CI: 1.30 (0.83-2.05), p = 0.25]. A statistically significant association of seronegativity with the development of NHL was evident for RSV [odds ratio; 95% CI: 7.27 (1.59-33.28), p = 0.01], EBV [odds ratio; 95% CI: 6.73 (1.45-31.20), p = 0.01] and suggestive association for influenza B [odds ratio; 95% CI: 2.60 (0.90-7.55), p = 0.08] and influenza A [odds ratio; 95% CI: 2.35 (0.81-6.80), p = 0.11]. In contrast, there was no evidence for association of HL with negative serology for any of the infectious agents tested. CONCLUSIONS: The risk of lymphomas, especially NHL, might be higher when, due to lower exposure to several infectious agents, the relatively unmodulated immune system of a child is challenged by environmental stimuli that can trigger development of lymphomas. The results, however, need further confirmation, through more pertinent methodological designs.

MR spectroscopy and serial magnetic resonance imaging in a patient with mitochondrial cystic leukoencephalopathy due to complex I deficiency and NDUFV1 mutations and mild clinical course.

We present clinical, magnetic resonance imaging and MR spectroscopic findings of a female patient, first admitted at the age of 9 months for regression of motor milestones and signs of mild spastic diplegia. Magnetic resonance imaging (MRI) demonstrated periventricular white matter abnormalities with sparing of the subcortical white matter. Subsequent MRIs, performed at the ages of 13 and 16 months, demonstrated progression of the white matter changes, progressive white matter rarefaction and cystic degeneration, and additional involvement of the corpus callosum; only the subcortical white matter remained spared. Proton MR spectroscopy revealed lactate elevation in the white matter. Blood lactate and lactate/pyruvate ratio were mildly elevated. Subsequent analysis of mitochondrial function in muscle tissue showed decreases in substrate oxidation and in ATP and CrP production rates. Complex I activity was seriously decreased, whereas mild decreases of complex II and IV activities were also noted. Analysis of the NDUFV1 gene revealed compound heterozygosity for two point mutations, each of them carried by one parent. The further clinical course of the patient was uphill; she slowly regained all previously lost motor milestones. In conclusion, diffuse white matter changes on MRI are compatible with mitochondrial encephalopathy and not necessarily associated with a severe clinical course.

Childhood leukaemias and lymphomas in Greece (1996-2006): a nationwide registration study.

BACKGROUND: Monitoring time trends in the incidence of childhood leukaemias and lymphomas requires efficient and continuous data collecting systems. In countries without official cancer registries, such as Greece, ad hoc nationwide registration of incident childhood leukaemias and lymphomas could help elucidate the underlying aetiology and monitor socioeconomic differentials in health care delivery. METHODS: We registered all cases and produced age, gender, type and immunophenotype specific figures and overall crude and age adjusted annual incidence rates and secular trends for 863 leukaemia and 311 lymphoma incident cases diagnosed in children <15 years of age across Greece during 1996-2006, namely the first 11 years of the Nationwide Registry for Childhood Hematological Malignancies. RESULTS: The epidemiological profiles of leukaemias/lymphomas in Greece are similar to those in industrialised countries. No secular trends are observed for either malignancy during the studied period. However, the calculated incidence for leukaemia (46.60 cases per 1 million children annually) is among the highest in the EU-27 (19% higher than average; p<0.001), whereas that for lymphoma (16.8 cases per 1 million children annually) is around the EU-27 average. CONCLUSIONS: Minimal secular changes in childhood leukaemias/lymphomas have been noted recently in the EU-27, which cannot be easily explained in countries with small populations. Therefore, centralised EU databases such as the Automated Childhood Cancer Information System (ACCIS) should be enlarged to generate sufficient statistical power for monitoring time trends. It would be interesting to explore whether different lifestyle patterns across the EU might be responsible for the observed excess leukaemia incidence in countries such as Greece.

An integrated evaluation of socioeconomic and clinical factors in the survival from childhood acute lymphoblastic leukaemia: a study in Greece.

An evaluation of the role of socioeconomic factors in the survival of children with leukaemia, controlling for major clinical prognostic indicators, has been attempted in very few studies and the role of these factors may be different in various cultural settings. Our investigation aims to study the independent role of socioeconomic factors on the prognosis of childhood acute lymphoblastic leukaemia (ALL) in Greece. During a 7-year period (1996-2002) 293 cases of incident ALL were diagnosed and followed up in four Childhood Haematology-Oncology Units, which covered over half of all childhood ALL cases nationwide. At the time of diagnosis, information concerning age, gender, maternal schooling, maternal marital status, sibship size, distance of residence from the treating centre, attendance of day care centre and clinical information was recorded. The influence of these factors on survival was studied by modelling the data through Cox's proportional-hazards regression. After adjustment for clinical prognostic factors, children of mothers who were not currently married, were of low educational level or were living far from the treating centre tended to have lower survival (P-values 0.02, 0.14 and 0.08, respectively). There was also evidence that two factors that are predictive of disease occurrence, that is sibship size and attendance of day care centre, may also predict survival (P-values 0.04 and 0.26, respectively). In conclusion, socioeconomic factors are likely to influence survival from ALL at least in some sociocultural contexts. Moreover, there is evidence that factors that could affect incidence of ALL through modulation of herd immunity may also have prognostic implications for this disease.