In this review, we address adult onset autosomal dominant leukodystrophy (ADLD), recently shown to be caused by increased expression of lamin B1 resulting from duplication of the gene encoding lamin B1, LMNB1[18-24], or possibly its dysregulation [25]. The gene discussed is LMNB1; the disease is adult-onset autosomal dominant demyelinating leukodystrophy.