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Serial IMP-SPECT and EEG studies in an infant with hemimegalencephaly
Insights
This study tracked seizures in an infant with hypomelanosis of Ito and hemimegalencephaly. Serial SPECT scans revealed changing brain perfusion patterns correlating with seizure activity and development.
Area of Science:
- Pediatric Neurology
- Neuroimaging
- Epileptology
Background:
- Hypomelanosis of Ito is a rare genetic disorder associated with neurological abnormalities.
- Hemimegalencephaly, characterized by an enlarged cerebral hemisphere, often presents with intractable epilepsy in infants.
- Infantile spasms and partial seizures are common seizure types in affected children.
Abstract:
An 8-month-old girl of hypomelanosis of Ito associated with hemimegalencephaly had frequent seizures beginning 44 h after birth. The seizures were secondarily generalized or unilateral initially, followed by infantile spasms at about 1.5 months of age. Frequent partial seizures appeared at 4 months of age. [123I]N-Isopropyl-p-iodoamphetamine (IMP) single photon emission computed tomography (SPECT) was performed serially 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 pathological left hemisphere and electroencephalography (EEG) revealed left-sided dominant hypsarrhythmia. At 7 months of age, a reversal was seen, there being decreased uptake on SPECT in the pathological hemisphere and abundant high amplitude background activity mingled with epileptic discharges on EEG in the non-pathological hemisphere. These serial changes of IMP uptake on SPECT seemed to reflect either changes in epileptic activity or maturational changes in cerebral perfusion in hemimegalencephaly.