Biomaterial Database

Extrapyramidal symptoms and medication use in Mucopolysaccharidosis type III. 2009

Michel C Tchan, and David Sillence

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

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009084	Mucopolysaccharidosis III	Mucopolysaccharidosis characterized by heparitin sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.	Polydystrophic Oligophrenia,Sanfilippo's Syndrome,Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase Deficiency,Heparan Sulfate Sulfatase Deficiency,MPS 3 A,MPS 3 B,MPS 3 C,MPS 3 D,MPS III A,MPS III B,MPS III C,MPS III D,MPS IIIA,MPS IIIB,MPS IIIC,MPS IIID,MPS3A,MPS3B,MPS3C,Mucopolysaccharidosis 3,Mucopolysaccharidosis Type 3 A,Mucopolysaccharidosis Type 3 A Sanfilippo Syndrome,Mucopolysaccharidosis Type 3 B,Mucopolysaccharidosis Type 3 C,Mucopolysaccharidosis Type 3 D,Mucopolysaccharidosis Type IIIA,Mucopolysaccharidosis Type IIIB,Mucopolysaccharidosis Type IIIC,Mucopolysaccharidosis Type IIID,N-Acetyl-alpha-D-Glucosaminidase Deficiency,N-Acetylglucosamine-6-Sulfatase Deficiency,N-Acetylglucosamine-6-Sulfate Sulfatase Deficiency,NAGLU Deficiency,San Filippo's Syndrome,Sanfilippo Syndrome,Sanfilippo Syndrome A,Sanfilippo Syndrome B,Sanfilippo Syndrome C,Sanfilippo Syndrome D,Sulfamidase Deficiency,Acetyl CoA:alpha Glucosaminide N Acetyltransferase Deficiency,Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase Deficiencies,Deficiencies, Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase,Deficiencies, N-Acetyl-alpha-D-Glucosaminidase,Deficiencies, N-Acetylglucosamine-6-Sulfatase,Deficiencies, N-Acetylglucosamine-6-Sulfate Sulfatase,Deficiencies, NAGLU,Deficiencies, Sulfamidase,Deficiency, Acetyl-CoA:alpha-Glucosaminide N-Acetyltransferase,Deficiency, N-Acetyl-alpha-D-Glucosaminidase,Deficiency, N-Acetylglucosamine-6-Sulfatase,Deficiency, N-Acetylglucosamine-6-Sulfate Sulfatase,Deficiency, NAGLU,Deficiency, Sulfamidase,MPS IIIDs,Mucopolysaccharidosis IIIs,Mucopolysaccharidosis Type IIIAs,Mucopolysaccharidosis Type IIIBs,Mucopolysaccharidosis Type IIICs,Mucopolysaccharidosis Type IIIDs,N Acetyl alpha D Glucosaminidase Deficiency,N Acetylglucosamine 6 Sulfatase Deficiency,N Acetylglucosamine 6 Sulfate Sulfatase Deficiency,N-Acetyl-alpha-D-Glucosaminidase Deficiencies,N-Acetylglucosamine-6-Sulfatase Deficiencies,N-Acetylglucosamine-6-Sulfate Sulfatase Deficiencies,N-Acetyltransferase Deficiencies, Acetyl-CoA:alpha-Glucosaminide,N-Acetyltransferase Deficiency, Acetyl-CoA:alpha-Glucosaminide,NAGLU Deficiencies,Oligophrenia, Polydystrophic,Oligophrenias, Polydystrophic,Polydystrophic Oligophrenias,San Filippo Syndrome,San Filippos Syndrome,Sanfilippo Syndromes,Sanfilippos Syndrome,Sulfamidase Deficiencies,Sulfatase Deficiencies, N-Acetylglucosamine-6-Sulfate,Sulfatase Deficiency, N-Acetylglucosamine-6-Sulfate,Syndrome, San Filippo's,Syndrome, Sanfilippo,Syndrome, Sanfilippo's,Syndromes, Sanfilippo
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000077152	Olanzapine	A benzodiazepine derivative that binds SEROTONIN RECEPTORS; MUSCARINIC RECEPTORS; HISTAMINE H1 RECEPTORS; ADRENERGIC ALPHA-1 RECEPTORS; and DOPAMINE RECEPTORS. It is an antipsychotic agent used in the treatment of SCHIZOPHRENIA; BIPOLAR DISORDER; and MAJOR DEPRESSIVE DISORDER; it may also reduce nausea and vomiting in patients undergoing chemotherapy.	2-Methyl-4-(4-methyl-1-piperazinyl)-10H-thieno(2,3-b)(1,5)benzodiazepine,LY 170053,LY-170052,Olanzapine Pamoate,Zolafren,Zyprexa,LY 170052,LY170052
D000077213	Lamotrigine	A phenyltriazine compound, sodium and calcium channel blocker that is used for the treatment of SEIZURES and BIPOLAR DISORDER.	3,5-Diamino-6-(2,3-dichlorophenyl)-1,2,4-triazine,3,5-Diamino-6-(2,3-dichlorophenyl)-as-triazine,BW-430C,Crisomet,Labileno,Lamictal,Lamiktal,BW 430C,BW430C
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults