[Neuroimaging findings of hemiconvulsions, hemiplegia, epilepsy (HHE) syndrome].
A 32-year-old man with hemiconvulsions, hemiplegia, epilepsy (HHE) syndrome is described. He was well developed with a normal pregnancy and delivery, but at age 10 months, he had status epilepticus during a febrile illness. Thereafter, he was noted to have left hemiparesis and mental retardation with recurrent hemiconvulsions. Magnetic resonance (MR) images showed atrophy and degeneration of the right cerebral cortex and white matter, homolateral thalamus, caudate nucleus, and hippocampus, with hyperintensities in both T2-weighted (TR/2200, TE/90) and proton (TR/2200, TE/30) images. There were also slight bilateral cerebellar atrophies. Quantitative single photon emission computed tomographic (SPECT) images using technetium-99m-ethyl cysteinate dimer (99mTc-ECD) revealed markedly reduced cerebral blood flow (CBF) in the right cerebral hemisphere, homolateral thalamus, caudate nucleus and bilateral cerebellum. Bilateral putamen and the medial occipital lobe showed normal findings on MR images and normal regional CBF in SPECT images. We suppose these selective neuronal injures in this case of HHE syndrome will be mainly due to histotoxic factors in epileptic brain damage.
Department of Neurology, Sakai Municipal Hospital.
H
H
Oe
R
R
Yuasa
M
M
Tsuchiyama
K
K
Kitamura
D000328:Adult; D001284:Atrophy; D001921:Brain / Q000473:pathology; D002560:Cerebrovascular Circulation; D004827:Epilepsy / Q000175:diagnosis*; D006429:Hemiplegia / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009410:Nerve Degeneration; D012640:Seizures / Q000175:diagnosis*; D013577:Syndrome; D015899:Tomography, Emission-Computed, Single-Photon