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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.
Footnotes
Author contributions: Dr. Flanagan was involved in drafting and revising the manuscript for content, including medical writing for content, study concept and design, analysis and interpretation of data, and acquisition of data. Dr. Gavrilova was involved in revising the manuscript for content and analysis and interpretation of data. Dr. Boeve was involved in revising the manuscript for content and analysis and interpretation of data. Dr. Kumar was involved in revising the manuscript for content and analysis and interpretation of data. Dr. Jelsing was involved in revising the manuscript for content and analysis and interpretation of data. Dr. Silber was involved in drafting and revising the manuscript for content, including medical writing for content, study concept and design, analysis and interpretation of data, acquisition of data, and study supervision.
Study funding: No targeted funding reported.
Disclosure: The authors report no disclosures relevant to this manuscript. Go to Neurology.org for full disclosures.
- Received June 29, 2012.
- Accepted August 22, 2012.
- © 2012 American Academy of Neurology
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