title: Acute psychosis in propionic acidemia: 2 case reports.
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.
authors: 1Service de Pédopsychiatrie, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France.
authors: C
authors: C
authors: Dejean de la Bâtie
authors: V
authors: V
authors: Barbier
authors: V
authors: V
authors: Valayannopoulos
authors: G
authors: G
authors: Touati
authors: A
authors: A
authors: Maltret
authors: A
authors: A
authors: Brassier
authors: J B
authors: JB
authors: Arnoux
authors: D
authors: D
authors: Grévent
authors: B
authors: B
authors: Chadefaux
authors: C
authors: C
authors: Ottolenghi
authors: P
authors: P
authors: Canouï
authors: P
authors: P
authors: de Lonlay
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D014150:Antipsychotic Agents / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D002648:Child; D002908:Chronic Disease; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D056693:Propionic Acidemia / Q000523:psychology*; D011618:Psychotic Disorders / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism; D013997:Time Factors
chemical_list: D014150:Antipsychotic Agents
keywords: antipsychotics; hallucinations; long-QT syndrome; organic acidemia; organic psychotic episode