Rapidly Evolving Acute Brainstem Syndrome With Respiratory Insufficiency due to Intracranial Dural Arteriovenous...

A 52-year-old female presented with one week of progressive quadriparesis followed by the development of diplopia, dysphonia and dysphagia over 48 h. Initial impression was of an inflammatory myelitis given mild cerebrospinal fluid pleocytosis and diffuse cervical cord swelling extending to the lower brainstem on MRI. Treatment with intravenous methylprednisolone was commenced. Her condition continued to progress with a brief cardiac arrest secondary to diaphragmatic insufficiency due to brainstem involvement. She survived the cardiac arrest after a period of cardiopulmonary resuscitation and was admitted to the intensive care unit. Subsequent MRI suggested a chronic right sigmoid venous sinus thrombosis with venous congestion in the brainstem and upper cervical cord. Digital subtraction angiogram revealed a right dural arteriovenous fistula (DAVF) between the right transverse venous sinus and perimesecephalic veins. The dural arteriovenous fistula was embolised with subsequent rapid improvement in bulbar function. Limb weakness also improved, and she was transferred to a rehabilitation unit for physiotherapy. Our case highlights the importance of rapid recognition of an intracranial DAVF mimicking an inflammatory myelitis in the setting of an acute brainstem syndrome. Symptoms can progress quickly if untreated with the risk of progression following the administration of corticosteroids.