Hypomelanosis of Ito presenting with adult-onset dementia and marked enlarged Virchow-Robin spaces.

A 52-year-old woman presented with a 10-year-history of memory loss and executive dysfunction. Examination disclosed hypopigmented whorls and linear streaks following the lines of Blaschko in different body segments (Figure 1), typical of Hypomelanosis of Ito (HI). Brain MRI showed marked enlarged Virchow-Robin spaces (Figure 2). HI or incontinentia pigmenti achromians (MIM#300337) represents a heterogeneous group of early-onset sporadic neurocutaneous disorders, resulting from chromosomal mosaicism1. Neurological manifestation may include: cognitive impairment, epilepsy, spasticity, cerebellar ataxia and microcephaly2. Adult-onset neurological symptoms may occur1. MRI abnormalities include: white-matter lesions, multiple cerebral infarcts, hypoplasia of the corpus callosum, and enlarged Virchow-Robin spaces2.