CEREBROSPINAL-FLUID SHUNTS IN THE MANAGEMENT OF BEHAVIORAL-PROBLEMS IN SANFILIPPO-SYNDROME (MPS-III)

Severe behavioural disturbance is a very common feature of Sanfilippo syndrome (mucopolysaccharidosis III, MPSIII), and one of the more diff icult aspects of the disease to treat. We describe a series of six pat ients with MPS III who had cerebrospinal shunts inserted in an attempt to ameliorate behaviour that had proved refractory to conventional tr eatment. Symptoms improved significantly in all six but removal of the shunt was necessitated in one patient due to shunt blockage and infec tion. Conclusion Our experience suggests cerebrospinal fluid shunting should be formally evaluated as an adjunct to conventional forms of tr eatment of extreme behavioural disturbance in MPS III.