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.

Massive gathering of clinical records started quite soon, at the turn of the last century, about 100 years ago. In that period keeping and gathering of medical records gradually evolved from paper medical records to computer-based medical records. Software that we use for this is quite different. We are witnessing tendency that each medical institution is creating software solely for their use, that leads to difference of gathered medical data and their validity for further analysis. In this paper we tested value of gathering and processing medical records through a hospital software, regarding time consumption in everyday work, as well as value of gathered and processed data. Statistically significant difference was found in terms of time needed for complete gathering and processing of medical data of hospitalized patients, as well as most of its segments (administering of admission, forming a medical history, forming a discharge letter). No statistically significant differences were found in terms of value of gathered and processed data in the forms of electronical records, compared to those formed on "traditional" basis. We conclude that the future of gathering, forming and processing of medical documentation of hospitalized patients is solely through interactive computer databases, and that their everyday spreading through all systems and levels of health care is absolutely necessary. Only that way we shall be able to fully use possibilities that such interactive system offers in improving management of patients.

Wolf-Hirschhorn syndrome (WHS) is a rare developmental disorder associated with hemizygous deletion of short arm of chromosome 4. Main phenotype characteristics of WHS are: intrauterine growth retardation, mental retardation, typical facial dysmorphism ("Greek warrior helmet"), microcephaly and midline fusion defects (cleft lip or palate, cardiac septal defects). Other abnormalities, like agenesis of corpus callosum, dysplastic kidneys, coloboma iris, skeletal abnormalities have been described occasionally. We described female newborn baby with 4p deletion, who had most of the phenotype characteristics of Wolf-Hirschhorn syndrome: intrauterine growth retardation, microcephaly, facial dysmorphism, congenital hypotonia and seizures in neonatal period. Other abnormalities included: hypoplastic cervical vertebra C4-C5, renal cyst and partial agenesis of corpus callosum. Patient had not cardiac septal defect. Due to a broad spectrum of possible morphologic abnormalities followed by mental retardation, prenatal diagnosis is very important. Postnatal recognition of the syndrome requires genetic counseling of parents and supportive multidisciplinary treatment.

Carbamazepine is the one of the most prescribed antiepileptic drugs in treatment of partial and generalized tonic-clonic seizures. Its efficacy in treatment of seizures was discovered incidentally during the trials of neuroleptic drugs. Generally it is well tolerated with relatively rare serious side effects. Therapy is introduced gradually, with mean dose between 10-20 mg/kg, and given this way it has the least side effects. Aim of the study was to assess interaction between efficacy, tolerability and overall efficacy of the first prescribed drug, in this case carbamazepine, in the group of patients with partial seizures and generalized tonic-clonic seizures. Patients hospitalized at University Pediatric Hospital in Sarajevo with newly diagnosed epilepsy and started therapy with carbamazepine in period from 07.1999 to 07.2002 were investigated. There were 89 patients that fulfilled the criteria. In 29 patients seizures remitted after the introduction of therapy (32.58%). After the subsequent correction of therapy further 8 patients were seizure free. In total, drug was efficient as a monotherapy in 68 patients (76.40%). Other antiepileptic drugs or polytherapy with carbamazepine were tried in the patients that were not seizure free. Adverse effects were reported in 28 patients (31.46%). The most frequent was benign leucopoenia (16 patients, 17.97%) that did not require changes in therapy. Rash was found in 8 patients (8.98%), out of which in 6 therapy had to be stopped. Compliance with therapeutic regime was slightly better with controlled release formulation (89.98%) then with simplex formulation (77.52%), but generally was good. We think that carbamazepine is still the drug of the first choice in treatment of patients with partial epileptic seizures and generalized tonic-clonic seizures. It showed good overall efficacy with relatively rare serious side effects.

Although birth-related brachial plexus injury (BPI) was first described more than two centuries ago, it still represents therapeutic dillemma. Incidence is 0.37-2.0 per 1000 live births. The most frequent etiologic cause is extreme lateral traction and excessive fundal pressure in a case of shoulder dystocia. However, in last decade there are reports that cite of BPIs occurring prior to delivery in up to 46% cases--intrauterine maladaptation. In 1998. and 1999. at the Rehabilitation Department of Pediatric Hospital, Clinical University Center in Sarajevo 32 children with BPI were treated. The incidence is 2.67 per 1000 live births. There were 21 cases of Erb's palsy (65.63%), 2 cases of Klumpke's palsy (6.25%). Total plexus palsy was present in 9 children (28.63%), and Horner's Syndrome in 1 case. In 25.77% of cases (8 children) there was-no evidence of shoulder dystocia at delivery. Treatment of the brachial plexus injuries in newborn is still controversial. Proper immobilization in first 7-15 days is of great importance. In this study 25 children (78.13%), in average age of 4.6 days, were brought for examination with improper immobilization. The role of widely applied electrotherapy is controversial. Complete recovery is expected in about one half, and in this study it was achieved in 75% of cases (24 children). Consensus about timing od surgical approach does not exist still. However, there is strong agreement that decision about eventual surgical treatment should be based on clinical motor testing and that MRI should precede the surgery. Although the electrodiagnostic studies have proven to be of limited prognostic value in the evaluation of children with acute obstetrical brachial plexus injuries, electromyography should be performed prior the surgery in the aim of later comparison of the results. According to our data, at this particular moment in BiH surgical treatment in this age is not available, as well as use of MRI in diagnostics of BPIs.

The clinical algorithm is a text format that is specially suited for presenting a sequence of clinical decisions, for teaching clinical decision making, and for guiding patient care. Clinical algorithms are compared as to their clinical usefulness with decision analysis. We have tried to make clinical algorithm for managing status epilepticus in children that can be applicable to our conditions. Most of the algorithms that are made on this subject include drugs and procedures that are not available at our hospital. We identified performance requirement, defined the set of problems to be solved as well as who would solve them, developed drafts in several versions and put them in the discussion with experts in this field. Algorithm was tested and revised and graphical acceptability was achieved. In the algorithm we tried to clearly define how the clinician should make the decision and to be provided with appropriate feedback. In one year period of experience in working we found this algorithm very useful in managing status epilepticus in children, as well as in teaching young doctors the specifities of algorithms and this specific issue. Their feedback is that they find that it provides the framework for facilitating thinking about clinical problems. Sometimes we hear objection that algorithms may not apply to a specific patient. This objection is based on misunderstanding how algorithms are used and should be corrected by a proper explanation of their use. We conclude that methods should be sought for writing clinical algorithms that represent expert consensus. A clinical algorithm can then be written for many areas of medical decision making that can be standardized. Medical practice would then be presented to students more effectively, accurately and understood better.

Vigabatrin has been in clinical use for 18 years. It has not been used widely in our country because of unavailability and costs of therapy. After the end of war in Bosnia and Herzegovina we started to use it in treatment of childhood epilepsies. We studied 19 patients that received vigabatrin as add on therapy and monotherapy. Follow up period was 6 months to 2 years. Nine of those patients were diagnosed as West syndrome, three had tuberous sclerosis, seven had intractable partial epilepsies. In the group with West syndrome 7 patients (5 as add on therapy and 2 as monotherapy) responded with complete control of seizures and disappearing of hypsarrhythmia in electroencephalographic recordings. One patient responded with reduction of seizures for 50%, one did not respond. In the group with tuberous sclerosis 1 was completely seizure free, one had reduction of seizures for 75%, one did not respond. Out of 7 patients with intractable partial epilepsies 3 responded with reduction of seizures between 75%-100%, 1 with reduction of seizures of 25%-50%, and 3 did not respond. Vigabatrin was well tolerated, we did not experience any serous adverse reactions. This antiepileptic drug can be a major improvement in treatment of some of the epileptic syndromes and needs further investigation.