Extrapyramidal symptoms and medication use in Mucopolysaccharidosis type III.
BACKGROUND
We report the case of a 16-year-old male with Mucopolysaccharidosis III type A (Sanfilippo syndrome) who was commenced on risperidone for behaviour management. He rapidly developed extrapyramidal symptoms that have not resolved.


METHODS
The medication histories of 20 patients with Mucopolysaccharidosis III seen at a Lysosomal Storage Diseases Clinic were reviewed to determine the incidence of extrapyramidal side effects.


RESULTS
Six patients had been treated with risperidone, olanzapine, or lamotrigine. Five of these patients developed extrapyramidal side effects.


CONCLUSIONS
The incidence of extrapyramidal side effects was considerably higher than expected. We suggest that these medications be used with considerable caution in these patients.
Tchan
Michel C
MC
The Children's Hospital at Westmead, Sydney, Australia. michelt@chw.edu.au
Sillence
David
D
D000293:Adolescent; D000328:Adult; D000927:Anticonvulsants / Q000009:adverse effects* / Q000627:therapeutic use; D014150:Antipsychotic Agents / Q000009:adverse effects* / Q000627:therapeutic use; D001480:Basal Ganglia Diseases / Q000139:chemically induced* / Q000453:epidemiology; D001569:Benzodiazepines / Q000009:adverse effects / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D000077213:Lamotrigine; D008297:Male; D001523:Mental Disorders / Q000188:drug therapy* / Q000209:etiology*; D009084:Mucopolysaccharidosis III / Q000523:psychology*; D000077152:Olanzapine; D018967:Risperidone / Q000009:adverse effects / Q000627:therapeutic use; D014227:Triazines / Q000009:adverse effects / Q000627:therapeutic use; D055815:Young Adult
D000927:Anticonvulsants; D014150:Antipsychotic Agents; D014227:Triazines; D001569:Benzodiazepines; D018967:Risperidone; D000077152:Olanzapine; D000077213:Lamotrigine