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Extrapyramidal symptoms and medication use in Mucopolysaccharidosis type III

Michel C Tchan1, David Sillence

  • 1The Children's Hospital at Westmead, Sydney, Australia. michelt@chw.edu.au

Journal of Intellectual & Developmental Disability
|August 15, 2009
PubMed
Abstract

Insights

Antipsychotics like risperidone can cause severe extrapyramidal symptoms in patients with Sanfilippo syndrome (Mucopolysaccharidosis III). This study found a high incidence of these side effects, suggesting cautious use of these medications.

Area of Science:

  • Neurology
  • Genetics
  • Pharmacology

Background:

  • Mucopolysaccharidosis III (Sanfilippo syndrome) is a rare genetic disorder affecting the central nervous system.
  • Behavioral issues are common in Sanfilippo syndrome, often necessitating pharmacological intervention.
  • Risperidone is a commonly used antipsychotic for managing behavioral symptoms.

Observation:

  • A 16-year-old male with Mucopolysaccharidosis III type A developed severe extrapyramidal symptoms after starting risperidone.
  • Review of 20 patients with Mucopolysaccharidosis III revealed that 5 out of 6 treated with risperidone, olanzapine, or lamotrigine experienced extrapyramidal side effects.

Findings:

  • The incidence of extrapyramidal side effects from risperidone, olanzapine, and lamotrigine in Mucopolysaccharidosis III patients is significantly higher than anticipated.
  • Extrapyramidal symptoms, including movement disorders, were observed in 83% of patients treated with these psychotropic medications.

Implications:

  • These findings highlight a heightened susceptibility to extrapyramidal side effects in individuals with Sanfilippo syndrome when treated with certain antipsychotics and mood stabilizers.
  • Clinicians should exercise extreme caution when prescribing risperidone, olanzapine, or lamotrigine for behavioral management in patients with Mucopolysaccharidosis III.
  • Further research is warranted to understand the underlying mechanisms and explore safer therapeutic alternatives for this population.

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