Adult-onset autosomal dominant leukodystrophy presenting with REM sleep behavior disorder

Abstract

Adult-onset autosomal dominant leukodystrophy (ADLD) is a slowly progressive hereditary disease of the white matter caused by duplication of the nuclear lamina protein lamin B1 on chromosome 5q23.2.1 Patients usually present in the 4th–5th decade with autonomic symptoms followed by pyramidal and cerebellar dysfunction.2 In ADLD, MRI head reveals symmetric T2-signal hyperintensities in the subcortical white matter, brainstem, and middle cerebellar peduncles.2 REM sleep behavior disorder (RBD) is a parasomnia characterized by dream enactment behavior and REM sleep without atonia.3 It has been reported most commonly with synucleinopathies such as Parkinson disease and may precede the diagnosis by decades.3 To our knowledge, RBD has not been reported with leukodystrophies. Herein we report a case of ADLD presenting with RBD as the initial symptom.