Autonomic dysreflexia associated with intramedullary astrocytoma of the spinal cord

Abstract

Autonomic dysreflexia is a life-threatening syndrome that may lead to seizures, myocardial infarction, stroke, or even death. It is characterised by severe responses to visceral or cutaneous stimuli that include acute hypertensive episodes, sweating, and headaches. Although occurrences of autonomic dysreflexia are well documented in patients with spinalcord injury, we were able to find only one report of autonomic dysreflexia associated with an acute intramedullary haemorrhage from a spinal haemangioma at the T4–5 level. In March 1995, a 61-year-old man was admitted to the Toronto Western Hospital with incomplete paraplegia due to an extensive intramedullary low grade (pilocytic) astrocytoma extending from C4 to T2, accompanied by syrinx cavities, rostral and caudal to the tumour (figure 1). He had a 5-year history of progressive myelopathy and initially had reduced sensation in his right arm, which progressed to weakness of arms and legs associated with further sensory loss and urinary incontinence. A C7–T3 laminectomy and subtotal microsurgical resection of the tumour was done. The tumour was invasive, despite a low-grade pathology, so the patient was treated with postoperative fractionated radiotherapy (50 Gy). After treatment, the patient made a partial neurological recovery and was able to leave hospital. By 1998, the patient experienced worsening paraparesis associated with syringomyelia, rostral and caudal to the tumour. He reported episodes of profuse sweating, often with severe constipation. A T3–4 laminectomy with insertion of a syringopleural shunt was done in September 1998. The patient was able to walk within a few days of surgery and was discharged from hospital. In October 2001, he again developed progressive leg weakness and underwent laminectomy from C6–T6 for repeat subtotal resection of the intramedullary astrocytoma and re-insertion of the syringopleural shunt. While in hospital, the patient developed a typical episode of autonomic dysreflexia characterised by hypertensive crises (arterial blood pressure suddenly reached 204/109 mmHg after previous blood pressure measure of 160/93 mmHg) accompanied by sweating, pounding headache, and anxiety. His arterial blood pressures decreased to 165/95 mmHg when he sat bolt upright and received a potent analgesic. Furthermore, the patient was constipated over 4 days and rectal examination confirmed fecal impaction. After rectal evacuation, blood pressure stabilised and the patient was discharged home. During the follow-up period the patient admitted to frequent episodes of headache and sweating in association with bladder distention or constipation. These symptoms promptly resolved after emptying his bladder or evacuating his bowel. There are two major characteristics in this case that should be emphasised. First, the type of tumour is unusual. Astrocytomas are the most common intramedullary spinal-cord tumours in children (59%), and are second only to Autonomic dysreflexia associated with intramedullary astrocytoma of the spinal cord