PURPOSE To assess outcome of children diagnosed with infantile spasms (IS) during the six-year-period (2002-20006), at the Pediatric Clinic of Clinical Center of University of Sarajevo, as well as to present other important clinical characteristics in this group of patients. METHODS All patients had medical histories with detailed description orvideo recordings of their seizures, as well as profound neurological exam, series of video-EEG registrations, neuroimaging studies and laboratory studies that were possible to perform. RESULTS Total of 19 patients with IS were treated (14 male, 5 female). Etiologically symptomatic IS were present in 78.9% of cases, cryptogenic in 21.1%. Flexor and mixed spasms were the most common (47.4% and 31.6% respectively). Therapeutic response was satisfactory: 42.1% of patients were seizure-free, 47.4% had partial response with more than 50% decrease of seizures, 10.5% had poor therapeutic response. Most of the patients were treated with polytherapy. The follow-up period was 15-70 months (mean 42.5 months). At last check-up four patients had normal development and were without seizures, two were lost to follow-up, two patients have died (21.4%, 10.5% and 10.5% respectively). Out of remaining patients seven (36.8% of total) had a severe psychomotor retardation with spastic tetraparesis, while the rest had hemiparesis and developmental difficulties. DISCUSSION Treatment of infantile spasms presents a great challenge, especially in the developing countries like Bosnia and Herzegovina in which the treatment modalities are limited. Our results indicate that despite the lack of the proper treatment options, outcome of the patients regarding control of seizures and latter psychomotor development did not differ significantly from the reports from the other countries. CONCLUSION Although prognosis for most patients with infantile spasms remains poor, further studies identifying predictors of favorable prognosis and recent advances in understanding the pathophysiology of infantile spasms offer hope of safer and more-effective therapies that improve long-term outcome.

Discontinuation of therapy is always challenging after successful control of epilepsies. Numerous parameters have to be taken in account, and relationship between benefits and any possible damages has to be assessed. The final decision should be made with the parents and the patient. We assessed the role of various factors on the possibility of relapse of epileptic seizures after discontinuation of antiepileptic therapy in patients with complex partial epilepsies. We studied a cohort of children in whom discontinuation of therapy was performed from 20012007. Study included 59 children divided into groups according to age of diagnosis, introduction of antiepileptic therapy, aetiology, neurological and psychological status, results neuroimaging studies, family history, history of febrile seizures, antiepileptic therapy, EEG registration before discontinuation of the treatment, speed of tapering therapy. Multivariable analysis was made, binary logistic regression. Factors that showed statistical significance were: age at the diagnosis, duration of active epilepsy, neurological and psychological status, duration of seizure free period, aetiology, MRI scan and length of tapering the therapy. We conclude that identified factors should be discussed with parents and patients, and to consensus has to be reached.

Central nervous system (CNS) malformations represent important factor of morbidity and mortality in children. The aim of the study was to determine the incidence, type and clinical features of CNS malformations in children who were admitted at the Neonatal and Child Neurology Department, Neonatal Intensive Care Unit and Paediatric Intensive Care Unit of Paediatric Clinic, University of Sarajevo Clinics Centre, from January 1st, 2002 to December 31st, 2006. There were total of 16520 admissions at the Paediatric Clinic over the studied period. CNS malformations, solitary or multiple, have been diagnosed in 100 patients (0,61%). The total number of various CNS malformations was 127. Lethal outcome was established in 9/100 cases (9%). The most frequent CNS malformations were neural tube defects 49/127 (38,6%). Hydrocephalus was seen in 34/127 (26,8%), microcephaly in 24/127 (18,9%), agenesis of corpus callosum in 10/127 (7,9%), Dandy Walker malformation in 6/127 (4,7%) and other CNS malformations in 4/127 (3,1%). In 20/100 of patients neural tube defect was associated with hydrocephalus (20%). CNS malformations were prenatally diagnosed in 13/100 of patients (13%). Primary prevention of CNS malformations can be improved in our country by better implementation of preconceptional folic acid therapy for all women of childbearing age. Secondary prevention by prenatal diagnosis requires advanced technical equipment and adequate education of physicians in the field of foetal ultrasonography. In our circumstances, prenatal diagnostics of CNS malformations is still not developed enough.

Medically intractable epilepsies are defined as seizures that are not controlled after an adequate trial with 2 first-line antiepileptic drugs (AED). Evidence in the literature show that these patients have many dysfunctions in their lifes. Lamotrigine is part of group of "newer antiepileptic drugs". Goal of this paper was to show what benefit is expected with introduction of add-on therapy with lamotrigine in patients with medically intractable epilepsies. Study was done in period 2002-2007 at Paediatric Hospital in Sarajevo. Inclusion criteria were: established diagnose of epilepsy, medical intractability defined as seizures not controlled after an adequate trial with 2 first-line antiepileptic drugs, age of more than 2 years and less than 18 years. Total of 61 children were assessed, 35 male and 26 female. Average age was 61.3 months at diagnosis of epilepsy. Add-on therapy with lamotrigine started in average about sixteen months after the diagnosis, with average age at starting the therapy of 77.4 months. Predominant type of seizures were partial seizures (with or without secondary generalization) in 67.20% cases, primarily generalized tonic clonic seizures in 13.11% cases, typical and atypical absences in 11.48% cases and myoclonic seizures in 8.20% cases. Reduction in seizure frequency was very good (76-100% reduction) in 37.70% of patients, good (51-75%) in 21.31%, fair (26-50%) in 9.84 and poor (less than 25%) in 31.14%. Chances of poor outcome were greater in patients with partial seizures. Side effects were noticed in 8.2% patients (6,56% with skin rash). Lamotrigine has showed good efficacy and safety profile. It is providing new efficient and well tolerated options for treatment for medically intractable epilepsies.

In this case report, the boy with familiar spastic paraplegia, the relatively rare genetic disorder and Klinefelter syndrome that was found during investigation, has been presented. The diagnosis of the disease has been established by anamnesis, clinical features and relevant diagnostic procedures, so the criteria for autosomal dominant type of the familiar spastic paraplegia have been fulfilled. The therapeutic possibilities are limited to the physical therapy and orthopedic treatment of feet deformities.

Early posttraumatic epilepsies (EPTE) are epileptic attacks that appear in first seven days after brain injury, with incidence of 3-5%. Predictors for development of EPTE are: impressive skull fracture with rupture of dura, intracranial haemorrhage, neurogical deficit (brain contusion), and posttraumatic amnesia longer than 24 hours. It is more common in children than in adolescents and adults. It carries four times increased risk for development of late posttraumatic epilepsy. Aspects of pharmacological prophylaxis was often considered, but scientifically neglected, without clear standings regarding controversial data in literature. Patients with severe head injury, hospitalised at Neurosurgical Hospital and Pediatric Hospital, Clinical Centre University of Sarajevo, in period from 6th of April 1992 till 1st of July 1994, were included in study. Prophylaxis of EPTE was carried out with phenobarbital (2-3 mg/kg) or phenytoin (3 mg/kg) parenterally. Decision was made upon clinical findings. CT scan was done in 13.5% patients, and in 31.9% patients serum concentrations of antiepileptic drugs were monitored. 310 patients aged 0-18 years (105 patients 0-10 years, and 205 patients 11-18 years) were investigated. Predictors of EPTE presented were posttraumatic amnesia longer than 24 hours in 90.6%, neurogical deficit in 86.45%, impressive skull fracture with rupture of dura in 81.3% and intracranial haemorrhage in 40.6%. Only two boys developed EPTE in first 24 hours after injury. This study has showed that use of antiepileptic drugs can decrease incidence of EPTE. However, problem remains, management of injured patients is still highly individualised, based on different experiences of doctors that treat patient, and without clear guidelines.

Known as D trisomy, Patau syndrome is the third chromosomopathy according to frequency. One of the 5000 newborn carries the trisomy 13. In over 80% cases there is fresh mutation with non separation in myeosis of older mother. The mosaic or translocation forms are not rare. The mail newborn with Patau syndrome is shown in this article. We notice: microcephalia, dolihocephalia, microphthalmia, cheilognatopalatoshisis, polydactilia, and found ultrasound changes at the brain, hearth and genitourinary system. Cytogenetic finding show: mail cariotype with aberrations 47, XY + 13, Sy Patau.

Intracranial haemorrhage (ICH) is the common name for periventricular and intraventricular haemorrhage. We analyzed patients diagnosed as ICH in period January 2001 till May 2002. In 29/323 (8.9%) pts was verified ICH, 16/29 (55.1%) were male sex. Birth weight under 1000 grams had 6/29 (20.6%), birth weight 1000-1499 grams was 10/29 (34.4%), than 1500 to 2499 grams 8/29 (27.5%) and over 2500 grams 5/29 (17.2%). APGAR score were under 7 in 20/29 (68.9%), and four of tham 4/20 (20%) had severe and 16/20 (80%) pts had modest and mild forms of perinatal asphyxia. According to Papile classification of ICH, we found: I degree ICH had 12.29 (41.3%) pts, II degree 8/29 (27.5%) pts, while severe forms III and IV degree of of ICH had 9/29 (31.2%) pts. In 3/29 (10.4%) pts, posthemoragic hydrocephalus were registrated. Risk-factors for development of ICH were low birth weight, small gestational age and perinatal asphyxia.