A case of early-onset benign occipital seizure susceptibility syndrome: decreased cerebral blood flow in the occipital region detected by interictal single photon emission computed tomography, corresponding to the epileptogenic focus.
Early-onset benign childhood occipital seizure susceptibility syndrome (EBOSS) recently described by Panayiotopoulos, is an early-onset variant of benign childhood epilepsy with occipital paroxysms. EBOSS is characterized by partial seizures that are predominantly manifested at night and associated with deviation of the eyes, vomiting and impairment of consciousness, but without ictal visual symptoms or postictal headache. The clinical features of our case were consistent with those of EBOSS, and we therefore diagnosed the patient as having a typical form of EBOSS. Neuroimaging by CT, MRI and MR angiography did not reveal a focal lesion. Interictal single photon emission computed tomography (SPECT) revealed decreased cerebral blood flow in the right occipital region corresponding to the epileptogenic focus shown on EEG. It remains unclear whether our finding on SPECT reflects secondary hypoperfusion due to minor morphological abnormality or immediate functional hypoperfusion. No reference to SPECT in a case of EBOSS has appeared in the literature to date. This report provides a better understanding of benign childhood epileptic syndromes with occipital spikes.
Sakagami
M
M
Department of Pediatrics, Tenri Municipal Hospital, Tenri, Japan.
Takahashi
Y
Y
Matsuoka
H
H
Hoshida
T
T
Izaki
K
K
Nouka
S
S
Yoshioka
A
A
D002560:Cerebrovascular Circulation / Q000502:physiology*; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D009778:Occipital Lobe / Q000098:blood supply* / Q000000981:diagnostic imaging; D012640:Seizures / Q000000981:diagnostic imaging / Q000503:physiopathology*; D015899:Tomography, Emission-Computed, Single-Photon