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Start at low dose (1 - 2 mg/kg/day) increasing gradually to 8 - 10 mg/kg/day in 4 divided doses. The dose is adjusted according to clinical response, monitoring CSF neurotransmitters and prolactin level (8, 87).

25 mg, 50 mg, 100 mg, and 200 mg capsules

PO

Ataxia, headache, insomnia, mania, mental stimulation/agitation, coryza, diaphoresis, tremulousness, hallucinations, dermatomyositis, scleroderma (2 patients), drowsiness, dyspnea, hyperventilation, eosinophilia, eosinophilia-myalgia syndrome, dysarthria, hyperreflexia, increased motor activity, paresthesia, psychosis, sedation, nausea, vomiting, diarrhea, anorexia, stomach pain, palpitations, bradycardia, hypotension, hypertension, transient blurred vision, pupillary dilatation, spontaneous nystagmus.

4

8. Centre for Evidence-Based Medicine. Secondary Centre for Evidence-Based Medicine  2009. http://www.cebm.net/index.aspx?o=1025.

87. Fries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S. Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. J Pediatr 1996;129(3):449-52 doi: S0022347696003678 [pii][published Online First: Epub Date]|.