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Subdural hematomas are most common in infants and elderly adults and result from bridging veins’ tears. Subdural haemorrhage has been described in several neurometabolic diseases with cerebral atrophy, including MPS II and IIIB. Few reports describe impaired hemostasis in MPS I and II presumably due to anticoagulant activities of glycosaminoglycans.Routine brain MRI of 8-year-old boy with MPS IIIB and developmental regression revealed chronic subdural hematoma of right hemisphere and cerebral atrophy. There was no indication on preceding trauma. Three weeks later the boy presented with seizure episode, whereupon he started to have hemiparetic gait, got drowsy. Brain KT displayed acute haemorrhage in the region of the chronic subdural hematoma. Laboratory studies demonstrated fluctuating thrombocytopenia, impaired platelet aggregation to ADP and arachidonic acid, decreased level of plasma fibrinogen. The patient was treated with platelet concentrate transfusion, etamsylate, folic acid and closed external drainage of subdural hematoma was performed. Three months after subdural hematoma surgery the patient’s status remained stable.According to presented data we conclude that regular neurovisualization and coagulation tests should be included in the standard workup for patients with neuronopathic mucopolysaccharidoses, as constellation of abnormal hemostasis and progressive cerebral atrophy may present a risk of intracranial haemorrhage.