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.


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.


CONCLUSIONS
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.
Department of Pediatrics, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162, Japan.
Akiko
A
Fujii
Hirokazu
H
Oguni
Yoshiko
Y
Hirano
Mutsuki
M
Shioda
Makiko
M
Osawa
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D058256:Brain Waves / Q000502:physiology; D016009:Chi-Square Distribution; D002675:Child, Preschool; D003545:Cysteine / Q000031:analogs & derivatives; D004569:Electroencephalography; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality / Q000502:physiology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D015609:Organotechnetium Compounds; D049268:Positron-Emission Tomography; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology*; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed
D015609:Organotechnetium Compounds; D019788:Fluorodeoxyglucose F18; D000324:Adrenocorticotropic Hormone; C059659:technetium Tc 99m bicisate; D003545:Cysteine