[Topography and number of cortical tubers in tuberous sclerosis: comparison between patients with and without West syndrome].
To reveal the role of cortical tubers in the pathophysiology of West syndrome associated with tuberous sclerosis, we studied their numbers and topography, focusing on the differences between patients with infantile spasms and without infantile spasms. We reviewed the clinical date on the seizure types, seizure evolution and developmental status of 13 patients: 8 patients with infantile spasms (the West syndrome group) and 5 patients without infantile spasms (the non-West syndrome group). The number, size and location of the cortical tubers were evaluated on 5 mm-thick T2-weighted MR images. The average number of cortical tubers of the West syndrome group was 11.4 per patient, being larger than that of the non-West syndrome group (7.8). The West syndrome group included two patients with only one tuber. The average number of the cortical tubers in the occipital lobes of the West syndrome group was 2.4, which was larger than that of the non-West syndrome group (0.8). From these results, a large number of cortical tubers, which suggest involvement of larger cerebral cortical regions, increase the possibility of the West syndrome in patients with tuberous sclerosis. On the other hand, some patients with only one or few cortical tubers developed West syndrome, which may suggest the presence of a region critical on the development of West syndrome. Our results also suggest that lesions in the occipital lobes might be more significant than those in other lobes. However, the results of this study are diverse, to some extent, which may have resulted from variable epileptogenicity of the cortical tubers. It is necessary to conduct further study, including a greater number of patients, to reveal topographic pathophysiology in West syndrome.
Hamano
S
S
Division of Neurology, Saitama Children's Medical Center, Iwatsuki.
Tanaka
M
M
Imai
M
M
Nara
T
T
Maekawa
K
K
D002540:Cerebral Cortex / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013036:Spasms, Infantile / Q000209:etiology* / Q000473:pathology; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology