| Popis: |
West syndrome hasn't been thoroughly investigated in Georgia. The purposes of our study were a) to assess the clinical and etiological peculiarities of West syndrome, based on MRI data and its relation to the long-term outcome; b) to assess the evolution of West syndrome and its relation to patient characteristics; c) to compare the efficacies of treatments with ACTH and antiepileptic drugs (AEDs); d) to evaluate the neuropsychological outcome after 12 and 24 months and their early predictors. We evaluated 31 patients (17 male, 14 female) with infantile spasms. Mean age of seizure onset was 6.3 months. Inclusion criteria were newly diagnosed patients with infantile spasms from 2 to 18 months, abnormal EEG and written informed consent of parents/ caregivers. We collected birth, family and seizure detailed history. All patients were examined neurologically, investigated with prolonged sleep and awake video - EEG, brain MRI, developmental screening tests (AgesStages Questionnaires®, Third Edition, ASQ-3™) at the time of admission. Spasm diary was given and filled by every parent/caregiver. The video-recording of seizures to study the detailed phenomenology of event was done in all cases as well. In 94% of patients (n=29) spasm were observed in clusters. EEG investigation revealed hypsarrhythmia in majority of cases (n=20; 65%). 19% (n=6) and 16% (n=5) patients had modified hypsarrhythmia and other types of EEG changes respectively. In 19 (61%) cases neurological examination was normal. 7 patients (22.6%) showed normal neuropsychological development. In remaining 12 (38.7%) and 12 (38.7%) cases moderate and severe delay of development was revealed accordingly. MRI investigation revealed no abnormality in 16% (n=5). 16 (52%) individuals were treated with ACTH only. In 12 (39%) cases ACTH and AED were used simultaneously and 2 (6.5%) cases were treated with AED only. One year follow-up assessments were provided in 22 (74%) cases. One patient died during the study. Neuropsychological development was with no changes in 12 (55%) individuals. In 3 (9.7%) cases some improvement was detected and the deterioration of development was identified in 6 (19%) individuals. In all cases with developmental deterioration, seizures were started before seven month of age, this association was statistically significant (Pearson Chi-Square 6.3; df 1; p=0.019). There was no relationship between developmental changes and sex, birth from full term pregnancy, infantile spasm type, treatment, or EEG pattern, however. Despite the fact, that the number of included patients was small, preliminary findings are in accordance to the other published data. The current study is still in process. 12-24 months follow-up with clinical, EEG and neuropsychological monitoring is planned for all cases. |
