NEUROAXONAL DYSTROPHY IN CHILDHOOD Report of Two Second Cousins with Hallerworden‐Spatz Disease, and a Case of Seitelberger's Disease
- 1 November 1982
- journal article
- case report
- Published by Wiley in Acta Paediatrica
- Vol. 71 (6) , 1045-1049
- https://doi.org/10.1111/j.1651-2227.1982.tb09574.x
Abstract
Three late infantile cases of neuroaxonal dystrophy are presented, two of them second cousins with Hallerworden‐Spatz disease and one sporadic case with Seitelberger's disease. At about 1 1/2 years of age the patients with Hallerworden‐Spatz disease developed clinical signs including progressive extrapyramidal motor disorder and mental retardation. They died at 8 and 11 years. Iron deposits and axonal dystrophy were found in the pallidum. The changes are compared to those in a case of infantile neuroaxonal dystrophy (Seitelberger's disease), the first to be reported in Scandinavia.Keywords
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