We present a sibship with a rare syndrome characterized by mental reta
rdation, dense calcification of the lateral ventricular choroid plexus
, and increased CSF protein. Neurophysiologic studies yielded nonspeci
fic results, and endocrine studies, including parathormone levels, wer
e normal. Simultaneous measurements of CSF and serum calcium, magnesiu
m, and other electrolytes were normal, but the CSF/serum ratio of phos
phate was low, suggesting a possible role in the pathogenesis of this
syndrome.