THE RELATIONSHIP OF ARYLSULFATASE A DEFICIENCY TO PSYCHIATRIC FINDINGS

Objective:

Arylsulfatase A deficiency is a metabolic disorder capable of producing a wealth of psychiatric and neurologic diseases in children and adolescents. The aim of this paper is to review the psychiatric findings seen in this deficiency and its relationship to psychiatric disorders.

Method:

Selected papers and books regarding to diseases caused by arylsulfatase A deficiency, psychiatric findings and neuropsychological tests are reviewed and the consequences are summarised.

Results:

Deficiency of this enzyme may lead to metachromatic leukodystrophy, the principal dysmyelinating disease of childhood as well as psychosis, dementia and learning disabilities in variable frequency. Due to this heterogenity molecular, biochemical, genetic, neurological, radiological and psychiatric investigations are made. Conflicting findings are reported in adults. This deficiency should be borne in mind in the laboratory investigations of cases of childhood dementia and psychosis like presentations in adolescents and young adults. It is possibly related with pervasive developmental disorders and learning disabilities.

Conclusion:

Investigations up to date are handicapped by lack of large screening procedures, highlighting neurological findings and varied diagnostic criteria. Future research in groups of pervasive developmental disorders, Asperger syndrome and mental retardation in children and adolescents using various investigational tools in a collaborative manner will help us to understand this relationship better.