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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 0028-3886
Vol. 53, No. 3, 2005, pp. 333-334
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Bioline Code: ni05116
Full paper language: English
Document type: Research Article
Document available free of charge
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Neurology India, Vol. 53, No. 3, 2005, pp. 333-334
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Recurrent stupor due to lysinuric protein intolerance
Ahsan Moosa NV, Minal D, Rao AnanthN, Kumar Anand
Abstract
Recurrent stupor in children is an uncommon clinical problem with a wide differential diagnosis; inherited metabolic disorders account for a vast majority. We report a 9-year-old girl with recurrent episodes of stupor. Initial episode was treated as viral encephalitis and the second episode was managed as non-convulsive status epilepticus. Hyperammonemia was detected in the last episode. Metabolic work-up after dietary protein challenge revealed classical biochemical features of lysinuric protein intolerance. She was managed with protein-restricted diet, which resulted in marked neurological improvement. LPI is a rare inherited metabolic disorder due to membrane transport defect of cationic amino acids.
Keywords
Lysinuric protein intolerance, hyperammonemia, recurrent stupor.
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