neurological dysfunction, including cerebellar signs and subnormal intelligence; cataracts; premature atherosclerosis; and pulmonary and adrenal insufficiency. In cerebrotendinous xanthomatosis, cholestanol levels increase in both plasma and xanthomas [7). In contrast, spinal cord signs were the only neurological abnormality in our patient. Furthermore, plant sterols, sitosterol as well as cholestanol, were found in high concentrations in both plasma and xanthoma of our patient; this finding was diagnostic of sitosterolemic xanthomatosis 19, 101.
The mechanism of formation of tendon and tuberous xanthomas is poorly understood in sitosterolemia. Possible explanations include increased intestinal absorption coupled with decreased removal of sterols [ 1, 3,7, 111, the abnormal cellular uptake andor degeneration of low-density lipoprotein [lZ], and reduced bile acid synthesis related to inhibition of cholesterol 7 cihydroxylase [ 13). Accumulation of sitosterol in tendon, skin, and epidural spaces may have initiated further cholesterol deposition in these tissues, either by attracting cholesterol from plasma by way of net transfer or by stimulating biosynthesis of cholesterol locally. In our patient, intradural-extramedullary xanthomatosis certainly was the basis for the neurological manifestations, and sitosterolemia should be considered as a cause of spinal cord complication with paraplegia. We are deeply indebted to Drs Y . Seyama and T. Kasama for highpressure liquid chromatography analysis of sitosterol. Lahat E, Katz Y, Bistritzer T, Eshel G, Aladjem M. Recurrent seizures in children with Shigellu-associated convulsions. Ann Neurol 1990;28:393-395 Convulsions are among the most frequent extraintestinal manifestations of shigellosis in children. It has been previously suggested that shigellosis-related seizures may represent simple febrile convulsions 11-31.