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CASE REPORTS
Year : 2019  |  Volume : 2  |  Issue : 3  |  Page : 134-135

Wishbone pattern of iron accumulation: A pathognomonic sign of type III GM1 gangliosidosis


1 Department of Clinical Neuroscience, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India; Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India
2 Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India
3 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India

Correspondence Address:
Dr. Pramod K Pal
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru 560029, Karnataka.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AOMD.AOMD_17_19

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Type III GM1 gangliosidosis is the adult or chronic variant of a lysosomal storage disorder, which occurs secondary to deficiency of β-galactosidase. The wishbone pattern of iron deposition which involves the medial and latter parts of the globus pallidus is pathognomonic of this disease. The diagnosis of type III GM1 gangliosidosis should be considered in patient with young onset, progressive generalized dystonia with prominent facial dystonia, and a wishbone pattern of iron deposition.


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