It has been recognized that duplication of LMNB1 gene or mutation in its promoter leading to increased expression of the encoded protein is a genetic cause of adult-onset autosomal dominant leukodystrophy (ADLD), which is a fatal progressive neurological disease characterized by widespread central nervous system demyelination [2, 44, 45]. Here, LMNB1 is linked to adult-onset autosomal dominant demyelinating leukodystrophy.