Hyperammonaemia as a cause of psychosis in an adolescent.
Diseases that cause hyperammonaemia usually appear during the neonatal period or during the first months of life as severe neurological metabolic distress. In some cases, as the one reported here, the age of onset and initial symptoms are non-specific and the episodes of acute metabolic encephalopathy may be attributed to encephalitis, poisoning or psychiatric problems. Our patient had N-acetyl glutamate synthetase deficiency due to a lack of activation by L-arginine. Treatment with N-carbamylglutamate was successful in maintaining normal ammonia levels.


CONCLUSIONS
We emphasise the importance of measuring ammonia levels in patients with neurological or psychiatric symptoms as part of their diagnostic work-up.
Bélanger-Quintana
Amaya
A
Unidad de Enfermedades Metabólicas, Servicio de Pediatría, Hospital Ramón y Cajal, Ctra de Colmenar Km 9.1, 28034 Madrid, Spain. abelanger@eresmas.com
Martínez-Pardo
Mercedes
M
García
María José
MJ
Wermuth
Bendicht
B
Torres
Julián
J
Pallarés
Esperanza
E
Ugarte
Magdalena
M
D000293:Adolescent; D000641:Ammonia / Q000032:analysis / Q000378:metabolism*; D005500:Follow-Up Studies; D005971:Glutamates / Q000008:administration & dosage*; D006801:Humans; D022124:Hyperammonemia / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D011618:Psychotic Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D005971:Glutamates; C006895:N-carbamylglutamate; D000641:Ammonia