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.
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.
Crohn's disease (CD) is a chronic inflammatory disease characterized by unpredictable and severe course. Most clinicians use simple laboratory parameters of inflammatory activity such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), the number of leukocytes (Le) and platelet count to assess disease activity. The aim of this paper was to determine the value of various laboratory parameters in the assessment of Crohn's disease activity. The study included 36 patients, 15 men and 21 women, treated for Crohn's disease at the Gastroenterohepatology Clinic, Clinical Centre, University of Sarajevo, in period 2006 and 2007. Assessed patients were divided into three groups, according to the degree of disease activity, which was determined using the Crohn's Disease Activity Index (CDAI). Mild disease (MD) was present in 15, moderately severe disease (MSD) in 14, and severe disease (SD) in 7 patients. Statistical significance of association between values of laboratory parameters with the degree of severity of illness was investigated using the 95% Confidence Interval test. CRP showed a statistical significance for the relationship between MD-MSD (95% CI 6,645-74,333) and the relationship between MD-SD (95% CI 4,114-135,278), while the relationship between MSD-SD was not statistically significant. MPV showed statistical significance only for the relationship between MD-MSD (95% CI 0,060-2,909). Sedimentation rate and the number of red blood cells showed statistical significance for the relationship MD-SD (95% CI 10,638-62,943 and 0,077-1,080). Haemoglobin, hematocrit and platelet count showed statistical significance for the relationship between MD-MSD and MD-SD. Number of leukocytes did not show any statistically significant relationship with clinical activity of disease. The most reliable indicators of activity of Crohn's disease in this study were CRP, MPV, haemoglobin, hematocrit and platelet count, while leukocyte number did not correlate with disease activity. Neither parameter was sensitive enough to distinguish the relationship between moderately severe disease and severe disease. Until finding a "gold standard" for assessment of clinical disease activity, various laboratory and other parameters must be combined and compared with other indicators, such as endoscopic findings and radiological results.
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.
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