ABSTRACT
Purpose To explore brain MRI features in newborn infants with incontinentia pigmenti,and to enhance the understanding and diagnosis level of this disease.Materials and Methods A retrospective analysis on MRI data of 13 neonates with incontinentia pigmenti admitted to Hunan Children's Hospital from January 2009 to December 2016 and further confirmed by clinical,pathology and gene diagnosis were conducted.Such conventional MRI sequences as T1WI and T2WI as well as DWI and SWI sequences wereincorporated.Results Five out of 13 newborn infants with incontinentia pigmenti were abnormal in the MRI features.In the conventional sequence,slightly short T1 signals in bilateral frontal lobe and right parietal occipital cortex were identified in 3 cases,right basal ganglia hemorrhage in 1 case.Slightly short TI and long T2 signals in bilateral basal ganglia were observed in 1 case;1 case of subarachnoid hemorrhage and 1 ease of bilateral lateral ventriculomegaly were noticed as well.The lesion sites of 4 cases were observed high density signal in DWI findings,three of which were widely distributed in abnormal signals.Lobes deep in bilateral cerebral hemispheres and subcortical white matter,bilateral basal ganglia,thalamus,internal capsule,corpus callosum,brain stem and cerebellum were the most affected areas.DWI could detect lesion area earlier and more accurate thanconventional sequences.Conclusion Incontinentia pigmenti among newborn infants demonstrates a high incidence.MRI examination should be adopted the moment neurological symptoms occur.Incontinentia pigmenti is relatively distinct in DWI findings,and hence is helpful for early detection of lesions and evaluation of clinical prognosis.