No abstract

Cardiac abnormalities have potential long-term hemodynamic consequences that justify an early diagnosis. Thus, for any patient with NF1, a cardiologic assessment is mandatory at the time of diagnosis and with regular follow-up intervals.

Objectives:The numerous antiepileptic drug (AED) withdrawal studies published in the last 40 years have relied mainly on heterogeneous study groups. There is still no general agreement on the criteria to predict safe discontinuation. The goal of this study was to assess the outcome of AED withdrawal in epileptic children.Materials and Methods:Three hundred and eight children with epilepsy were enrolled, and these patients followed at least 1 year after drug withdrawal. Time to seizure relapse and predictive factors were analyzed by survival methods.Results:Among the 308 patients, 179 (58.1%) were boys and 129 (41.9%) were girls and the mean age at the seizure onset was 60.41 ± 36.54 months (2-144 months). The recurrence occurred in 73 (23.7%) patients. Mental retardation, history of febrile seizure, etiological of epilepsy, abnormal first electroencephalogram (EEG), abnormal neuroimaging findings, and total number of AED before remission were significantly associated with relapse risk according to univariate analysis. In the multivariate analysis, abnormal first EEG and number of AED before remission (polytherapy) were the risk factors influencing seizure recurrence.Conclusions:In our study, recurrence rate was 23.7% in children and most occurred during the 1st year. The potential risk factors of recurrence are history of febrile seizure, mental retardation, etiological of epilepsy, abnormal first EEG, abnormal neuroimaging findings, and total number of AED before remission. However, we found abnormal first EEG and polytherapy as risk factors of recurrence in multivariate analysis.

The aim of this retrospective study was to determine the risk factors associated with intractability to therapy in childhood epilepsy. Fifty children with intractable epilepsy as evidenced by at least 1 epileptic fit per month were included in the study group, whereas the control group consisted of children who did not experience any recurrent seizure for at least 1 year at the time of the study. A chi( 2) test was used to evaluate the relationship between the test variables for the 2 groups, and the estimated relative risk (odds ratio) for each variable was calculated. The risk factors were subsequently determined by logistic multiple regression analysis. Univariate analysis showed that mental retardation, neurological abnormality, neuroradiological abnormality, perinatal anoxia, neonatal convulsion, presence of status epilepticus, and symptomatic etiology were significant risk factors for the development of refractory epilepsy (P < .05). For multivariate logistic regression analysis, age at seizure onset, status epilepticus, mixed type of seizures, and history of frequent seizures (more than once a month) were all found to be significant and independent risk factors for refractory epilepsy, and the number of drugs used in the study group was significantly higher than that in the control group (P < .05). In line with these findings, it was concluded that children who present with epilepsy and have these risk factors should be referred to a center where epileptic surgery is carried out without delay.

Levetiracetam (LEV) is a new antiepileptic drug (AED)that is effective in adults and children with partial-onset seizures or idiopathic or symptomatic generalized seizures. LEV does not bind to plasma proteins, and is eliminated by the kidneys. It has been argued that LEV can act on the N-type Ca 2? channel and can reverse the gammaaminobutyric acid (GABA) and glycine-gated currents [1]. Side effects include fatigue, somnolence, infection, headache, behavioral changes and skin rashes [2]. Herein, we present the first case of rhabdomyolysis in a child treated with LEV.A 13-year-old girl presented with a history of myalgia (mainly in her lower extremities) since last 2-3 days to our hospital. She was born of an uneventful full-term pregnancy. Developmental milestones were normal. On medical history, she had partial onset of secondarily generalized seizures during sleep since 2 months. The seizure activity consisted of twitching of the right face, tonic deviation of the mouth involving the lips, tongue, and pharyngeal and laryngeal muscles, resulting in speech arrest and drooling and progressing into bilateral tonic stiffening of the arms and legs. The EEG revealed left centrotemporal spikes and was subsequently diagnosed with benign epilepsy with centrotemporal spikes. The patient was administered LEV monotherapy. LEV had started 10 mg/kg/day, but her brief partial seizures continued. The dose was increased to 20 mg/kg/day (500 mg/day), and her seizures were ceased. One week after starting LEV therapy, she experienced mild myalgia. On her examination, the vital signs, including the blood pressure were normal. Her height and weight were in normal limit for her age. The physical and neurological examinations were normal. There was no evidence of trauma, exercise or infection.On laboratory examination, blood urea was 29 mg/dL (normal range 17-43), creatinine 1.0 mg/dL (normal range 0.6-1.2), myoglobin 78 ng/mL (normal range 14.3-65.8), and creatinine phospho kinase (CPK) was 986 U/L (normal range 27-168). Plasma carnitine (42 lmol/L; reference range 19-59 lmol/L) and free carnitine levels (35 lmol/L; reference range 12-46 lmol/L) were normal as acylcarnitine profile. Brain MRI showed no abnormal findings.Rhabdomyolysis was diagnosed with clinical and biochemical findings. We thought LEV may be the cause of her complaints and ceased LEV therapy. Hydration therapy were started to avoid potential complications. After stopping LEV, serum CPK and myoglobin levels gradually decreased and returned into normal values. Muscle biopsy was not performed.