Alien-hand syndrome with mirror movements in hereditary diffuse leukoencephalopathy with spheroids. Issue 4 (24th March 2022)

• Record Type: Journal Article
• Authors: Dongre, Nikhil; Ambwani, Varsha; Mani, Vinita Elizabeth; Paliwal, Vimal Kumar
• Is Part Of: Practical neurology. Volume 22:Issue 4(2022)
• Page Start: 321
• Page End: 323
• Publication Date: 2022-03-24
• Subjects: alien hand -- movement disorders -- neurophysiology -- motor -- dementia; Neurology -- Periodicals; 616.8005
• DOI: 10.1136/practneurol-2021-003237
• Languages: English
• ISSNs: 1474-7758

Abstract

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.