Indian Journal of Dermatology
CASE REPORT
Year
: 2015  |  Volume : 60  |  Issue : 3  |  Page : 290--292

Self-mutilation in neurodegeneration with brain iron accumulation


Sadanandavalli Retnaswami Chandra1, Pawan Raj1, Thomas Gregor Issac2 
1 Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
2 Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India

Correspondence Address:
Sadanandavalli Retnaswami Chandra
Faculty Block, Neurocentre, NIMHANS Bangalore 560 029, Karnataka
India

Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.


How to cite this article:
Chandra SR, Raj P, Issac TG. Self-mutilation in neurodegeneration with brain iron accumulation.Indian J Dermatol 2015;60:290-292


How to cite this URL:
Chandra SR, Raj P, Issac TG. Self-mutilation in neurodegeneration with brain iron accumulation. Indian J Dermatol [serial online] 2015 [cited 2021 Sep 18 ];60:290-292
Available from: https://www.e-ijd.org/article.asp?issn=0019-5154;year=2015;volume=60;issue=3;spage=290;epage=292;aulast=Chandra;type=0