Cerebrospinal fluid shunts in the management of behavioural problems in Sanfilippo syndrome (MPS III)

Abstract

Our experience suggests cerebrospinal fluid shunting should be formally evaluated as an adjunct to conventional forms of treatment of extreme behavioural disturbance in MPS III.

Correlation of MR Imaging and MR Spectroscopy Findings with Cognitive Impairment in Mucopolysaccharidosis II

“…II, 17,18 because chronic intraventricular high pressure can lead to behavioral disturbances, optic nerve lesions, and brain stem damage. Because of probable outcomes, different authors recommend neurosurgical intervention for patients with MPS, mainly types I, III, and VI.…”

Neurosurgical Interventions in Children with Maroteaux-Lamy Syndrome

“…There have been several reports of hydrocephalus communicans in children with Hurler’s syndrome (MPS1), Sanfilippo’s syndrome (MPS3) and Maroteaux-Lamy syndrome (MPS6) [1, 10, 11]. Patients may present with signs of raised intracranial pressure, macrocephaly, behavioral disturbance or visual impairment due to optic atrophy.…”

Extrapyramidal symptoms and medication use in Mucopolysaccharidosis type III

“…Cleary and Wraith reported the use of both thioridazine and haloperidol as being moderately successful agents, with the precaution that haloperidol may induce extrapyramidal sideeffects (EPS) (Cleary & Wraith, 1993). Other experimental approaches to behaviour management have included CSF shunting (Robertson, Klug, & Rogers, 1998) and hormone treatment (Kushner & Guze, 2005).…”