P. Toti et al., CELIAC-DISEASE WITH CEREBRAL CALCIUM AND SILICA DEPOSITS - X-RAY SPECTROSCOPIC FINDINGS, AN AUTOPSY STUDY, Neurology, 46(4), 1996, pp. 1088-1092
An increased incidence of seizures and cerebral calcifications, usuall
y bilateral and located in the occipital cortex, has been reported in
celiac patients. The histology of cerebral lesions is not well defined
, and their pathogenesis is only speculative. We report the autopsy re
sults of a patient with celiac disease, seizures, and cerebral calcifi
cations who died following a cerebral hemorrhage caused by Fisher-Evan
s syndrome. Calcifications were restricted to the cortical gray matter
and composed of aggregates of small calcified spicules. Calcium depos
ition was present as psammoma-like bodies, along small vessels, and wi
thin neurons. X-ray spectroscopy of the calcified areas revealed that
calcium (43%) and silica (57%) were present in the lesions. High silic
a content was also found in the cerebral hemorrhagic fluid. Silica tox
icity has to be considered in regard to the pathogenesis of the cerebr
al lesions and of the seizures.