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Clinical/Scientific Note
Adult nonketotic hyperglycinemia (NKH) crisis presenting as severe chorea and encephalopathy
Article first published online: 20 NOV 2003
DOI: 10.1002/mds.10681
Copyright © 2003 Movement Disorder Society
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Additional Information
How to Cite
Hall, D. A. and Ringel, S. P. (2004), Adult nonketotic hyperglycinemia (NKH) crisis presenting as severe chorea and encephalopathy. Mov. Disord., 19: 485–486. doi: 10.1002/mds.10681
Publication History
- Issue published online: 2 APR 2004
- Article first published online: 20 NOV 2003
- Manuscript Accepted: 19 SEP 2003
- Manuscript Revised: 3 SEP 2003
- Manuscript Received: 11 MAR 2003
- Abstract
- Article
- References
- Cited By
Keywords:
- nonketotic hyperglycinemia;
- encephalopathy;
- chorea
Abstract
We report on a patient with mild episodic nonketotic hyperglycinemia (NKH) on valproate who developed episodes of encephalopathy and chorea provoked by a fever and protein loading. After treatment with sodium benzoate and dextromethorphan, the patient's encephalopathy resolved and her chorea improved. The importance of this case is in recognizing this rare syndrome, which may present in the adult population. © 2003 Movement Disorder Society