Cerebral complications in Schimke immuno-osseous dysplasia.

Abstract:

UNLABELLED:Schimke immuno-osseous dysplasia is a multisystem disorder consisting of spondylo-epiphysial dysplasia, progressive renal insufficiency due to focal segmental glomerulosclerosis, and immunodeficiency. Cerebrovascular complications have only been described in five patients. Here we report a patient with prominent neurological symptoms most likely caused by transient ischaemic attacks. CONCLUSION:Neurological symptoms consisted of repeated brief spells of hemiparaesthesia, motoric aphasia and diplopia. MRI studies of the CNS revealed progressive white matter lesions. Morphological changes as well as neurological deficits are compatible with cerebral ischaemia.

journal_name

Eur J Pediatr

authors

Schmidt B,Christen HJ,Herkenrath P,Benz-Bohm G,Müller-Berghaus J,Querfeld U

doi

10.1007/s004310050714

subject

Has Abstract

pub_date

1997-10-01 00:00:00

pages

789-91

issue

10

eissn

0340-6199

issn

1432-1076

journal_volume

156

pub_type

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