Ruzica Kravljanac

Outcome of status epilepticus in children treated in the intensive care unit: A study of 302 cases

Purpose:  The aim of the study was to evaluate the outcome of status epilepticus (SE) in children and to define predictors for morbidity, mortality, and SE recurrence.

Etiology, clinical features and outcome of epilepsia partialis continua in cohort of 51 children

The objective of the study was to evaluate etiology, clinical characteristics and outcome in children with epilepsia partialis continua (EPC). The investigation included 51 children with EPC aged 0.2-18 years treated in the period 1993-2009. The median period from the onset of underlying disorder to EPC was 6 months (0-72 months). EPC was caused by different pathologies: inflammatory and immune-mediated (52%), metabolic (13.7%), structural brain abnormalities (11.8%), cryptogenic (7.8%), vascular (5.9%), dual (5.9%), postoperative (2%). Median duration of EPC was 15 days (1-200 days). EPC involved more frequently the right side of the body comparing to the left one. The outcome was assessed at the end of the follow up period (mean 6.5 years, ranged 0.2-16 years). Unchanged neurological status was observed in 10 (19.6%) children, neurological consequences in 33 (64.7%) children and lethal outcome in 8 (15.7%) children. The most frequent etiology in our cohort was inflammatory and immune-mediated disease of central nerve system including Rasmussens encephalitis. The duration of EPC was prolonged, most frequently involving the right upper limb. The outcome of EPC in children was unfavorable.

The efficacy of bromides, stiripentol and levetiracetam in two patients with malignant migrating partial seizures in infancy

The syndrome of malignant migrating partial seizures in infancy is a devastating, age-specific, epileptic encephalopathy, which still presents an aetiological, pathophysiological and therapeutic problem. In this study, we present two patients who were diagnosed with the disease, based on electroclinical symptoms. The patients were treated with a combination of sodium bromide, stiripentol and levetiracetam. The first patient unequivocally responded, following a course of ineffective conventional drugs, and the second, who was diagnosed and treated immediately, showed a more significant therapeutic response. Antiepileptic drugs, previously reported to be beneficial in case reports, when given concomitantly, may substantially reduce the number and severity of seizures, without influence on psychomotor development.

Long-term outcome in children with infantile spasms treated with vigabatrin: a cohort of 180 patients.

Evaluation of efficacy of vigabatrin as the first drug in infants with previously untreated infantile spasms (IS) and reporting the long‐term outcome.

Rasmussen syndrome and long-term response to thalidomide

We report a 13-year-old female who experienced symptoms and signs of Rasmussen encephalitis for the first time at the age of 5 years. Various therapeutic procedures, including conventional and new antiepileptic drugs, steroids, immunoglobulin, plasma exchanges, and partial hemispherectomy, were applied, but their results were unsatisfactory. During one of the exacerbations, when the patients life was endangered, thalidomide was administered. Frequency and intensity of epileptic seizures were reduced significantly, and the quality of her life improved. Except for moderate neutropenia, the other adverse effects were not recognized. In our opinion, thalidomide is not a first-choice drug for Rasmussen encephalitis but is a good alternative only for cases refractory to other well-known and accepted therapeutic procedures.

Basal ganglia lesions in the early stage of Menkes disease

An 11-month-old boy presented with progressive truncal hypotonia with poor head control and myoclonic seizures. His hair was sparse and stiff, his skin hypopigmented. Magnetic resonance imaging (MRI) (Fig. 1) demonstrated slight volume loss of the supratentorial white matter, symmetrical T2-weighted (T2W) hyperintense signal of the putamens and isolated round lesions of the left caudate head. The basal ganglia lesions showed restricted diffusion on diffusion-weighted imaging (DWI) (Fig. 2). Light microscopy of the boy’s hair demonstrated pili torti. Low levels of plasma copper and caeruloplasmin confirmed the diagnosis of Menkes disease. Menkes disease is an X-linked recessive disorder of metal metabolism, due to a defect in intracellular– intercompartmental copper transport Bindu et al. (2007), Arita et al. (2009). Typical MRI findings are progressive degeneration of grey and white matter, followed by

Relapsing inappropriate antidiuretic hormone secretion in an anti-aquaporin-4 antibody positive paediatric patient

Paediatric patients with the syndrome of an inappropriate antidiuretic hormone secretion (SIADH), as a manifestation of inflammatory demyelinating disorders of the central nervous system, have been rarely described until now, in only a few cases of neuromyelitis optica spectrum disorders (NMOSDs). We present a case of relapsing SIADH associated with NMOSD, in an anti-aquaporin-4 antibody positive 14-year-old girl, who is, to our best knowledge, the first reported paediatric patient with relapsing SIADH and NMOSD. Additionally, our case further supports the notion that paediatric encephalomyelitis associated with SIADH should suggest the diagnosis of NMOSD.

Epileptic seizures provoked by bathing with water at room temperature

We report two Caucasian boys with seizures induced by bathing in lukewarm water. Different mechanisms of provocation were observed; in one boy a complex partial seizure was provoked by pouring water over the body, while in the other boy, a complex partial seizure with secondary generalisation was provoked by immersion. Since thewater was not hot in either of the cases, the pathophysiological mechanism was not clear and the seizures could not be explained as hyperthermic-related events. We suggest that in the ILAE classification of epilepsies and epileptic seizures, bathing epilepsy should be added as a separate category, distinct from “hot-water epilepsy”.

New-onset seizure presenting as status epilepticus: etiology and clinical characteristics in a cohort of 236 children

PURPOSE Evaluation of the etiology, clinical characteristics and outcome of the first status epilepticus (fSE) event in children. METHOD The patients with fSE hospitalized in our Institute from 1995 to 2011 were included. The etiology was characterized as either known (symptomatic) or unknown (cryptogenic). Outcome was assessed at the end of hospitalization. Logistic regression analyses were used to assess predictors of the outcome, with odds ratio adjusted by age as a measure effect. RESULTS The study included 236 patients with a median age of 2.0 years (IQR 4.0). Etiology was identified as secondary to: defined electroclinical syndromes 108 (45.8), acute symptomatic conditions 63 (26.7%), unknown 24 (10.1%), progressive encephalopathy 23 (9.7%), or remote symptomatic 18 (7.6%). Recurrence rate was 16.9%, neurological consequences were in 24.6% and case-fatality ratio was 4.7%. The main predictors were for: a) death - progressive encephalopathy (OR = 14.68, 95% CI 4.06-23.11. p = 0.001); b) neurological sequelae - acute symtomatic (OR 3.44, 95% CI 4.82-6.47) p = 0.001, remote symptomatic (OR = 13.84, 95% CI 4.34-44.12. p = 0.001), progressive encephalopathy (OR = 3.94, 95% CI 1.64-9.56. p = 0.002), seizure duration >60 min (OR = 0.44, 95% CI 0.24-0.81. p = 0.001); c) seziure recurrence - acute symptomatic etiology (OR = 3.59, 95% CI 41.76-7.21. p = 0.001), seizure duration >60 min (OR = 0.30, 95% CI 0.15-0.61. p = 0.001). CONCLUSIONS In children with fSE, exploring acute disorders and immediate etiological treatment is essential. The outcome of fSE is favorable concerning the recurrence rate, while neurological sequelae are seen in one quarter of the patients. The etiology and fSE duration are the main determinants of outcome.

Long-term outcome in children with neonatal seizures: A tertiary center experience in cohort of 168 patients

PURPOSE The purpose of this study was to evaluate long-term outcome and assess predictors of prognosis in children with neonatal seizures (NS). METHOD This retrospective study includes children with NS treated at our Institute from January the 1st 2005 until December the 31st 2015. The data were collected from medical charts and the electroencephalogram (EEG) database at the Institute. The predictive value was evaluated for following parameters: (1) characteristics of the patients, such as gender, gestational age, birth body weight, Apgar score, artificial ventilation; (2) etiology; (3) characteristics of seizures such as type, time of onset, resistance to treatment; and (4) EEG background activity and paroxysmal discharges. The outcome of NS was assessed at the end of the follow-up period and was categorized as one of the following: (1) lethal outcome, (2) neurological abnormalities, (3) intellectual disability, and (4) epilepsy. Univariate and multivariate logistic regression analyses were used to assess predictors of NS outcome. RESULTS The study included 168 children with NS (of which 109 are males, and 59 are females), mean aged 5.6 (SD 3.5) years at the end of the follow-up (with a range of 1 to 12 years). There was normal neurological development without epilepsy in 131 patients (78%), neurological abnormality in 31 (19.0%), intellectual disability in 28 (17.2%), epilepsy in 12 (7.4%), and lethal outcome in 7 patients (4.17%). CONCLUSIONS Long-term outcome in children with NS could be favorable in most patients, and it appears to be related to specific early clinical and paraclinical variables. Newborns with an abnormal background EEG activity, with seizures resistant to antiepileptic drugs and/or low Apgar score are at a higher risk of a poor outcome. Females are at a much higher risk of lethal outcome than males.