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An understanding and awareness of secondary syndromes in metabolic disorders is of great importance as it can lead to the early diagnosis of such disorders. Many of these metabolic disorders are progressive and may have illness\u2010modifying treatments available. Earlier diagnosis may prevent or delay damage to the central nervous system and allow for the institution of appropriate treatment and family and genetic counselling. Metabolic disorders appear to result in neuropsychiatric illness either through disruption of late neurodevelopmental processes (metachromatic leukodystrophy, adrenoleukodystrophy, GM2 gangliosidosis, Niemann\u2010Pick type C, cerebrotendinous xanthomatosis, neuronal ceroid lipofuscinosis, and alpha mannosidosis) or via chronic or acute disruption of excitatory\/inhibitory or monoaminergic neurotransmitter systems (acute intermittent porphyria, maple syrup urine disease, urea cycle disorders, phenylketonuria and disorders of homocysteine metabolism). In this manuscript we review the evidence for neuropsychiatric illness in major metabolic disorders and discuss the possible models for how these disorders result in psychiatric symptoms. Treatment considerations are discussed, including treatment resistance, the increased propensity for side\u2010effects and the possibility of some treatments worsening the underlying disorder.<\/jats:p>","DOI":"10.1007\/s10545-013-9618-y","type":"journal-article","created":{"date-parts":[[2013,5,22]],"date-time":"2013-05-22T09:22:43Z","timestamp":1369214563000},"page":"687-702","source":"Crossref","is-referenced-by-count":94,"title":["The neuropsychiatry of inborn errors of metabolism"],"prefix":"10.1002","volume":"36","author":[{"given":"Mark","family":"Walterfang","sequence":"first","affiliation":[{"name":"Melbourne Neuropsychiatry Centre University of Melbourne and Melbourne Health Parkville Australia"},{"name":"Neuropsychiatry Unit Royal Melbourne Hospital Parkville Australia"},{"name":"Royal Melbourne Hospital Level 2, John Cade Building Parkville 3050 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