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NEUROAXONAL DYSTROPHY IN CHILDHOOD Report of Two Second Cousins with Hallerworden‐Spatz Disease, and a Case of Seitelberger's Disease
Author(s) -
MALMSTRÖMGROTH A. G.,
KRISTENSSON K.
Publication year - 1982
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1982.tb09574.x
Subject(s) - medicine , disease , dystrophy , pediatrics , degenerative disease , pathology
. 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.