Self-Mutilation in Neurodegeneration with Brain Iron Accumulation |
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Authors: | Sadanandavalli Retnaswami Chandra Pawan Raj Thomas Gregor Issac |
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Affiliation: | From the Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India;1.Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India |
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Abstract: | 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. |
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Keywords: | Childhood Movement Disorders neuro degeneration with Brain Iron Accumulation self-Mutilation |
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