Hereditary diffuse leukoencephalopathy with spheroids is a rare genetic disorder caused by mutations of the colony-stimulating factor 1 receptor gene. It is an adult-onset leukodystrophy, with a wide spectrum of neurological and psychiatric manifestations that includes Parkinsonism, dementia, seizures, limb weakness, spasticity and abnormal motor behaviour. Alien-hand syndrome and mirror movements are rare manifestations of this and other neurodegenerative disorders. We describe a woman with progressive limb and trunk rigidity, Parkinsonism and dementia, who also had involuntary left arm levitation (part of the posterior variant of alien-hand syndrome) and left-hand mirror movements. We discuss the different types of alien-hand syndrome, and the likely mechanisms of arm levitation and mirror movements.
- alien hand
- movement disorders
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Contributors ND collected data and wrote the initial draft. VA collected data and prepared video and images and wrote legends. VEM revised the manuscript. VKP conceived, wrote and revised the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally reviewed by David Lynch, London, UK and Huw Morris, London, UK.
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