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Neurological rarities
Alien-hand syndrome with mirror movements in hereditary diffuse leukoencephalopathy with spheroids
  1. Nikhil Dongre,
  2. Varsha Ambwani,
  3. Vinita Elizabeth Mani,
  4. Vimal Kumar Paliwal
  1. Department of Neurology, SGPGIMS, Lucknow, Uttar Pradesh, India
  1. Correspondence to Dr Vimal Kumar Paliwal, Department of Neurology, SGPGIMS, Lucknow, Uttar Pradesh, India; dr_vimalkpaliwal{at}rediffmail.com

Abstract

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
  • neurophysiology
  • motor
  • dementia

https://doi.org/10.1136/practneurol-2021-003237

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    Footnotes

    • 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.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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