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Cerebrospinal fluid shunts in the management of behavioural problems in Sanfilippo syndrome (MPS III) (1998)

Robertson, S. P. ; Klug, G. L. ; Rogers, J. G.

Springer

European journal of pediatrics 157 (1998), S. 653-655

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Details

ISSN: 1432-1076

Schlagwort(e): Key words Sanfilippo disease ; Mucopolysaccharidosis ; Cerebrospinal fluid shunt ; Hyperactivity

Quelle: Springer Online Journal Archives 1860-2000

Thema: Medizin

Notizen: Abstract Severe behavioural disturbance is a very common feature of Sanfilippo syndrome (mucopolysaccharidosis III, MPSIII), and one of the more difficult aspects of the disease to treat. We describe a series of six patients with MPS III who had cerebrospinal shunts inserted in an attempt to ameliorate behaviour that had proved refractory to conventional treatment. Symptoms improved significantly in all six but removal of the shunt was necessitated in one patient due to shunt blockage and infection. Conclusion 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.

Materialart: Digitale Medien

URL: http://dx.doi.org/10.1007/s004310050904

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