A long-term, clinical study on symptomatic infantile spasms with focal features

Background: We studied the clinical, neuroradiological and EEG characteristics of patients with infantile spasms (IS) who showed focal features to reveal their long-term prognoses and treatment responses. Subjects and methods: Subjects included 69 patients with IS who consecutively visited our hospital. We tentatively classified the subjects into focal IS (fIS) and diffuse WS (dIS) groups based on the presence and absence of more than two of the following findings, respectively: (1) epileptic spasms (ES) that were asymmetric, (2) a focal epileptic EEG abnormality, (3) a lateralized neurological abnormality, (4) a focal brain MRI and (5) a focal SPECT abnormality. Results: We found 23 cases with fIS and 46 cases with dIS. ES responded more frequently in fIS than dIS group (100% vs. 80%; P = 0.02) to the initial ACTH trial although the subsequent seizure relapse occurred more frequently in fIS than dIS group (74% vs. 38%; P = 0.0006). The second course of ACTH trial brought a short as well as long-term remission in both groups (6/8 cases vs. 5/6 cases). Later in the clinical course, the fIS patients tended to display a focal epileptic EEG abnormality and to develop focal seizures. In our series, approximately one-third of patients with fIS later showed either only a focal epileptic EEG abnormality, a focal epileptic EEG abnormality with focal seizures, or bilateral asymmetric EEG foci with disabling seizures, respectively. Conclusion: It is useful to classify patients with IS into fIS and dIS groups based on various lateralizing signs because the classification provides practical information regarding the long-term outcome and treatment strategy.