INCONTINENTIA PIGMENTI - MR DEMONSTRATION OF BRAIN CHANGES

PURPOSE: To describe the MR findings in eight girls and women with inc ontinentia pigmenti, from two families. Four had skin lesions and neur ologic disease, and four had only skin lesions. METHODS: Eight patient s had physical examination, family history, electroencephalogram and M R examination of the brain. MR was repeated in the two cases with more severe changes several years after the first study. RESULTS: MR revea led brain changes only in the four patients who had neurologic disease associated with the cutaneous lesions of incontinentia pigmenti. Abno rmalities were located in the cerebral hemisphere contralateral to the most affected side of the body. In two cases, the MR changes were sub jacent to the scalp areas where the most severe cutaneous lesions were located in the neonatal period. Hypoplasia of the corpus callosum, pr obably secondary to atrophy of one or both cerebral hemispheres, and a bnormal signal and atrophy of the lateral regions of one of the cerebe llar hemispheres also were found in all four cases. Although the chang es were seen in both the T1- and T2-weighted images, they were most ev ident in the latter. The four patients in the fourth stage who had onl y cutaneous lesions without neurologic problems did not reveal any MR abnormalities. CONCLUSIONS: This study demonstrates MR signal changes and focal atrophy of the cerebrum, cerebellum, and corpus callosum in patients with incontinentia pigmenti and neurologic disorders. The MR images appear normal in patients with incontinentia pigmenti who have no neurologic abnormalities.