HUNTER SYNDROME COMPLICATED BY HYDROCEPHALUS AND AN ENDOGENOUS ANTICOAGULANT

This case illustrates the features of Hunter syndrome (mucopolysacchar idosis type 2) complicated by hydrocephalus and a prolonged activated partial thromboplastin time (APTT). Ventriculo-peritoneal shunt insert ion was performed but was complicated by an extradural haematoma which nas successfully drained. Other neurological complications of Hunter syndrome include compressive cervical myelopathy, diffuse cerebral whi te matter changes and punctate lesions in the basal ganglia. This case is the first report of a coagulation defect in a patient with Hunter syndrome.