Encéphalopathie hyperammoniémique au valproate de sodium

Granel, B.; Gavaret, Martine; Baut, X. Le; Sautereau, N.; Rodriguez, D.; Rossi, P.; Bagnères, D.; Demoux, A.-L.; Francès, Y.

doi:10.1016/j.revmed.2010.06.010

Cited by 8 publications

(2 citation statements)

References 18 publications

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“…In fact, in most published cases, serum VPA levels are usually within the normal therapeutic range (50-100 mg/L). 6,8,[20][21][22][23] Similarly, we observed that most of our patients (Table 1), had VPA levels in the therapeutic range and this was not related with the decrease in ammonia levels.…”

Section: Discussionsupporting

confidence: 68%

“…16 Hyperammonemic encephalopathy presents clinically as impaired consciousness (seizure, somnolence, lethargy, confusion, and even stupor and coma). 21,22 Other symptoms include vomiting and nausea, low-grade fever, ataxic gait, blurred vision, and focal neurological deficit. These symptoms may be difficult to differentiate from the underlying disease itself and they can be misdiagnosed as therapeutic failure instead of an adverse event related to the use of VPA.…”

Section: Discussionmentioning

confidence: 99%

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Use of carglumic acid in valproate‐induced hyperammonemia: 25 pediatric cases

et al. 2020

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Hyperammonemic encephalopathy is a rare but potentially dangerous complication of the antiepileptic drug (AED) sodium valproate (VPA). We report a retrospective study of 25 pediatric patients, (15 females [60%]; age: 7.6 ± 4.9 years), with different underlying disorders, who suffered from hyperammonemia due to VPA and who were treated with carglumic acid (CA). The duration of treatment with VPA was 15 ± 1 month, with a dose of 40 ± 16.6 mg/kg/d. VPA blood levels were 75.5 ± 60 mg/L with seven patients being overdosed (>100 mg/L). Twenty‐three patients received concomitant treatment with other AEDs. The initial dose of CA was 100 mg/kg. Subsequently, CA doses of 25 mg/kg were given to 22 patients every 6 hours (average treatment length 2.17 ± 1.1 days) until ammonemia was normalized. In nine patients, CA was used in combination with other drugs to treat hyperammonemia. In all cases, blood ammonia levels were brought under control and symptoms of hyperammonemia resolved. Two hours after CA administration, the average reduction in ammonium levels was 53 ± 29 and 88.6 ± 47.5 μmol/L at 24 hours, resulting in a statistically significant decrease when compared to pretreatment levels. There were no statistically significant differences between sexes, in the presence or not of cognitive impairment or previous carnitine treatment. There were no statistically significant differences when comparing treatment with CA plus ammonia scavengers vs CA alone. In 17 patients (68%) VPA was discontinued and 62% of the patients who maintained treatment had recurrent episodes of hyperammonemia.

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