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 improve
Valproate-Induced Chorea and Encephalopathy in Atypical Nonketotic Hyperglycinemia
β Scribed by Peter F. Morrison; Raman Sankar; W. Donald Shields
- Book ID
- 116825014
- Publisher
- Elsevier Science
- Year
- 2006
- Tongue
- English
- Weight
- 57 KB
- Volume
- 35
- Category
- Article
- ISSN
- 0887-8994
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We have investigated the molecular lesions of T-protein deficiency causing typical or atypical nonketotic hyperglycinemia (NKH) in two unrelated pedigrees. A patient with typical NKH was identified as being homozygous for a missense mutation in the T-protein gene, a Gto-A transition leading to a Gly
## Abstract Six severe epileptic patients developed stuporous encephalopathy with marked cognitive impairment when topiramate (TPM) and sodium valproate (VPA) were coprescribed for five patients, and when monotherapy with TPM was introduced for one patient. In four patients, ammonaemia increased an