Incontinentia Pigmenti is a rare X-linked multisystem disorder with well 
described and pathognomonic skin manifestations. Neurological manifestations are 
found in 30% of IP patients, forming one of the major causes of morbidity and 
mortality of the condition. In this review, clinical and brain imaging data of 
45 IP patients with a neurological phenotype are reviewed. Several clinical 
presentations could be identified, comprising seizures, infantile 
encephalopathy, acute disseminated encephalomyelitis and ischemic stroke. Most 
neurological features presented during the neonatal period. No patients 
presented during adolescence or at adult age. Seizures of different type are 
reported in about 20% of the patients at young age and seem to correlate with 
the degree of cerebrovascular damage. Brain MRI findings include periventricular 
and subcortical white matter disease, haemorrhagic changes, corpus callosum 
hypoplasia, cerebral atrophy and cerebellar hypoplasia. Ocular findings comprise 
a range of retinal vascular changes and optic atrophy, but also developmental 
defects like microphthalmia and cataract. Most findings may reflect changes 
following brain injury. Both (ischemic) vascular and inflammatory components may 
play a role in the cerebral and ocular phenotype. However, a role of disturbed 
apoptosis during development may also be a contributing factor.