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NEUROLOGY 1996;46:1088-1092
© 1996 American Academy of Neurology

Celiac disease with cerebral calcium and silica deposits

X-ray spectroscopic findings, an autopsy study

P. Toti, MD, P. Balestri, MD, M. Cano, P. Galluzzi, MD, T. Megha, MD, M. A. Farnetani, MD, M.L.D. Palmeri, MD, M. Vascotto, MD, C. Venturi, MD and A. Fois, MD

From the Departments of Pathology (Drs. Toti, Megha, and Palmeri) and Pediatrics (Drs. Balestri, Farnetani, Vascotto, and Fois), University of Siena, Siena, Italy; the Department of Pathology (M. Cano), University of Nebraska, Omaha, NE; and the Division of Neuroradiology (Drs. Galluzzi and Venturi), Siena, Italy.
Supported by M.U.R.S.T. 60%.
Received April 10, 1995. Accepted in final form July 26, 1995.
Address correspondence and reprint requests to Dr. Paolo Toti, Instituto di Anatomia Patologica, Via delle Scotte, 53100 Siena, Italy.

An increased incidence of seizures and cerebral calcifications, usually 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 results of a patient with celiac disease, seizures, and cerebral calcifications who died following a cerebral hemorrhage caused by Fisher-Evans syndrome. Calcifications were restricted to the cortical gray matter and composed of aggregates of small calcified spicules. Calcium deposition was present as psammoma-like bodies, along small vessels, and within neurons. X-ray spectroscopy of the calcified areas revealed that calcium (43%) and silica (57%) were present in the lesions. High silica content was also found in the cerebral hemorrhagic fluid. Silica toxicity has to be considered in regard to the pathogenesis of the cerebral lesions and of the seizures.

NEUROLOGY 1996;46: 1088-1092




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