CEREBRAL COMPLICATIONS IN SCHIMKE IMMUNO-OSSEOUS DYSPLASIA

Schimke immune-osseous dysplasia is a mul tisystem disorder consisting of spondylo-epiphysial dysplasia, progressive renal insufficiency due to focal segmental glomerulosclerosis, and immunodeficiency. Cerebrov ascular complications have only been described in five patients. Here we report a patient with prominent neurological symptoms most likely c aused by transient ischaemic attacks. Conclusion Neurological symptoms consisted of repeated brief spells of hemiparaesthesia, motoric aphas ia and diplopia. MRI studies of the CNS revealed progressive white mat ter lesions. Morphological changes as well as neurological deficits ar e compatible with cerebral ischaemia.