L. Marti-bonmati et al., Tuberous sclerosis: Differences between cerebral and cerebellar cortical tubers in a pediatric population, AM J NEUROR, 21(3), 2000, pp. 557-560
Citations number
15
Categorie Soggetti
Radiology ,Nuclear Medicine & Imaging","Neurosciences & Behavoir
BACKGROUND AND PURPOSE: Histologic evidence of cortical tubers is pathognom
onic of tuberous sclerosis (TS) disease, Cerebellar tubers, however, are un
commonly found, Our objective was to evaluate the prevalence and characteri
stics of cerebellar tubers in a large series of pediatric patients with TS
studied with MR imaging, In particular, their relationship with volume loss
and age will be analyzed,
METHODS: MR images of 34 children with TS and cortical tubers were reviewed
. There were 17 female and 17 male patients. The mean age was 8.9 +/- 4.5 y
ears (mean +/- SD), with a range from 2 to 14 years, The number and locatio
n (supratentorial, infratentorial) of tubers and volume loss of the underly
ing parenchyma were recorded, The relationship between tuber location, pati
ent age, and number of tubers was studied using the Student's t test for in
dependent samples. The relationship between tuber location and presence of
volume loss was established using the Pearson chi(2) test.
RESULTS: The mean number of cortical tubers was 14.3 +/- 8.5. Fifteen (44.1
%) patients had cerebellar tubers associated with cerebral lesions. Patient
s with cerebellar and cerebral tubers had significantly more global cortica
l lesions than did patients with isolated cerebral tubers (17.9 +/- 8.9 ver
sus 11.4 +/- 7.2 tubers, P = .026), Patients with cerebellar tubers were si
gnificantly older than those with isolated supratentorial tubers (11.3 +/-
3.4 versus 7.1 +/- 4.4 years, P = .005), In only four (11.8%) patients were
tubers associated with focal parenchymal volume loss. The tubers in all of
these cases were located in the cerebellum-indicating a significant relati
onship with volume loss (Pearson chi(2) test, P = .017),
CONCLUSION: In this series, cerebellar tubers were frequent and were always
present in association with cerebral cortical tubers. They were seen in ol
der children with a larger total number of tubers. Cerebellar tubers may be
associated with focal volume loss.