title: Propionic acidemia associated with visual hallucinations.
abstract: Propionic acidemia, an autosomal recessive disorder, is a common form of organic aciduria resulting from the deficiency of propionyl-CoA carboxylase. It is characterized by frequent and potentially lethal episodes of metabolic acidosis often accompanied by hyperammonemia. A wide range of brain abnormalities have been reported in propionic acidemia. We report recurrent visual hallucinations in 2 children with propionic acidemia. Four visual hallucination events were observed in the 2 patients. Three episodes were preceded by an intercurrent illness, and 2 were associated with mild metabolic decompensation. The 2 events in one patient were associated with a seizure disorder with abnormal electroencephalogram. Brain magnetic resonance imaging showed abnormal basal ganglia and faint temporo-occipital swelling bilaterally. This is probably the first report of visual hallucinations in propionic acidemia and should alert the treating clinicians to look for visual hallucinations in patients with organic acidurias, especially in an unusually anxious child.
authors.affiliation: Department of Pediatrics, King Abdulaziz University, Jeddah, Saudi Arabia.
authors.forename: Taghreed
authors.initials: T
authors.lastname: Shuaib
authors.forename: Nadia
authors.initials: N
authors.lastname: Al-Hashmi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Ghaziuddin
authors.forename: Eman
authors.initials: E
authors.lastname: Megdad
authors.forename: Dejene
authors.initials: D
authors.lastname: Abebe
authors.forename: Amr
authors.initials: A
authors.lastname: Al-Saif
authors.forename: Alaa
authors.initials: A
authors.lastname: Doubi
authors.forename: Hesham
authors.initials: H
authors.lastname: Aldhalaan
authors.forename: Mohei Eldin
authors.initials: ME
authors.lastname: Abouzied
authors.forename: Mohammed
authors.initials: M
authors.lastname: Al-Owain
mesh_terms: D000293:Adolescent; D002648:Child; D004252:DNA Mutational Analysis; D038524:Diffusion Magnetic Resonance Imaging; D004569:Electroencephalography; D019788:Fluorodeoxyglucose F18; D006212:Hallucinations / Q000150:complications* / Q000000981:diagnostic imaging / Q000235:genetics; D006801:Humans; D008297:Male; D043586:Methylmalonyl-CoA Decarboxylase / Q000235:genetics; D009154:Mutation / Q000235:genetics; D049268:Positron-Emission Tomography; D056693:Propionic Acidemia / Q000150:complications* / Q000000981:diagnostic imaging / Q000235:genetics
chemical_list: D019788:Fluorodeoxyglucose F18; D043586:Methylmalonyl-CoA Decarboxylase