An 8-month-old girl of hypomelanosis of Ito associated with hemimegale
ncephaly had frequent seizures beginning 44 h after birth. The seizure
s were secondarily generalized or unilateral initially, followed by in
fantile spasms at about 1.5 months of age. Frequent partial seizures a
ppeared at 4 months of age. [I-123]N-Isopropyl-p-iodoamphetamine (IMP)
single photon emission computed tomography (SPECT) was performed seri
ally during an interictal period at 1, 3 and 7 months of age. At 1 and
3 months, IMP-SPECT showed a marked increase of IMP uptake in the pat
hological left hemisphere and electroencephalography (EEG) revealed le
ft-sided dominant hypsarrhythmia. At 7 months of age, a reversal was s
een, there being decreased uptake on SPECT in the pathological hemisph
ere and abundant high amplitude background activity mingled with epile
ptic discharges on EEG in the non-pathological hemisphere. These seria
l changes of IMP uptake on SPECT seemed to reflect either changes in e
pileptic activity or maturational changes in cerebral perfusion in hem
imegalencephaly.