RT Journal Article
SR Electronic
T1 Alien-hand syndrome with mirror movements in hereditary diffuse leukoencephalopathy with spheroids
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 321
OP 323
DO 10.1136/practneurol-2021-003237
VO 22
IS 4
A1 Nikhil Dongre
A1 Varsha Ambwani
A1 Vinita Elizabeth Mani
A1 Vimal Kumar Paliwal
YR 2022
UL http://pn.bmj.com/content/22/4/321.abstract
AB 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.