Acute psychosis in propionic acidemia: 2 case reports

Journal of Child Neurology
C Dejean de la BâtiePascale de Lonlay

Abstract

Propionic acidemia is an inborn deficiency of propionyl-coenzyme A (CoA) carboxylase activity, which leads to mitochondrial accumulation of propionyl-CoA and its by-products. Neurologic complications are frequent, but only a few cases presenting with psychiatric symptoms have been reported so far. We report 2 cases of children with chronic psychiatric symptoms who presented with an acute psychotic episode as teenagers. Both patients had hallucinations, panic and grossly disorganized behavior, for several weeks to several months. They had signs of moderate metabolic decompensation at the beginning of the episode, although the psychiatric symptoms lasted longer than the metabolic imbalance. We propose that these episodes were at least partially imputable to propionic acidemia. Such episodes require psychiatric examination and antipsychotic treatment, which may have to be adapted in case of cardiomyopathy or long QT syndrome.

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Citations

Sep 11, 2014·Orphanet Journal of Rare Diseases·Matthias R BaumgartnerAnupam Chakrapani
Sep 12, 2015·Transplant International : Official Journal of the European Society for Organ Transplantation·Chiara ArrizzaJean-Marc Nuoffer
Feb 17, 2015·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·F Charbit-HenrionL Dupic
Oct 23, 2016·Journal of Psychiatric Research·Mark A FryeJoanna M Biernacka
Jan 24, 2017·Addiction Biology·Ian R GizerKirk C Wilhelmsen
Sep 1, 2017·Journal of Inherited Metabolic Disease·Caroline Dejean de la BâtieLisa Ouss
May 20, 2018·International Journal of Mental Health Nursing·Sjobha R N van den BergStasja Draisma
May 24, 2019·Journal of Inherited Metabolic Disease·Hanneke A HaijesPeter M van Hasselt
Feb 18, 2021·Journal of Inherited Metabolic Disease·Patrick FornyMatthias R Baumgartner

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