Diagnostic pitfall: nontraumatic spinal epidural hematoma mimicking a brainstem stroke

Abstract

To the Editor: Acute nontraumatic (spontaneous) spinal epidural hematoma is a well-recognized, although uncommon, clinical entity. Reported here is a case of high cervical spinal epidural hematoma presented with compression symptoms of the medulla oblongata that has not been described previously in the literature. An 83-year-old woman presented to the emergency department (ED) complaining of a severe posterior neck pain and right hemiparesis while she was getting up from her bed in the early morning. On arrival at the ED, she was alert and her initial vital signs were as follows: blood pressure 156/68 mm Hg, pulse rate 65 beats/min, respiratory rate 20 breaths/min, and temperature 36.2°C (97.2°F). She had a medical history of hypertension and left internal carotid artery stenosis under long-term aspirin therapy. Neurologic examinations revealed grade 3/5 strength of the right upper and lower limbs and an equivocal facial palsy. Blood coagulation tests were within normal limits. A computed tomographic (CT) scan of her brain was normal. Fifty minutes after arrival, she rapidly deteriorated into coma (Glasgow Coma Scale score of E1M1V1), complete quadriplegia, apnea, bradycardia (pulse rate 42 beats/min), and shock (blood pressure 70/42 mm Hg). She was immediately intubated and resuscitated according to the advanced cardiac life support guidelines. A repeated nonenhanced CT scan focusing on a brainstem or high cervical cord lesion demonstrated a large hematoma extending from the level of C1 to C7 vertebrae (Figure). The patient remained quadriplegic and ventilator-dependent despite decompressive surgery (performed 36 hours after onset). She died of respiratory sepsis 80 days later. Nontraumatic spinal epidural hematoma often associates with anticoagulant or antiplatelet therapy, blood dyscrasias, arteriovenous malformation, spinal manipulations, arterial hypertension, atherosclerosis, and minor vertebral trauma from physical activities of daily living.1.Groen R.J. Ponssen H. The spontaneous spinal epidural hematoma. A study of the etiology.J Neurol Sci. 1990; 98: 121-138Abstract Full Text PDF PubMed Scopus (288) Google Scholar The usual clinical presentation is that of sudden stabbing neck or back pain that progresses toward paraparesis or quadriparesis, depending on the level of the lesion.2.Boukobza M. Guichard J.P. Boissonet M. et al.Spinal epidural haematoma: report of 11 cases and review of the literature.Neuroradiology. 1994; 36: 456-459Crossref PubMed Scopus (126) Google Scholar However, an epidural hematoma at the craniocervical junction, as illustrated in this case, would cause medulla compression with sudden respiratory center dysfunction and cardiovascular collapse. The key to early diagnosis of acute nontraumatic spinal epidural hematoma is a high index of suspicion in addition to an emergency CT scan or magnetic resonance imaging scan. Early recognition and emergency evacuation remain the mainstay management for spinal epidural hematoma. The outcome is essentially determined by the timing of decompressive surgery, the location of the hematoma, and the preoperative neurologic condition of the patient.3.Lawton M.T. Porter R.W. Heiserman J.E. et al.Surgical management of spinal epidural hematoma: relationship between surgical timing and neurological outcome.J Neurosurg. 1995; 83: 1-7Crossref PubMed Scopus (459) Google Scholar In conclusion, spinal epidural hematoma must be considered in patients with coagulopathy who have spontaneous signs of acute cord compression. Epidural hematoma at the craniocervical junction that compresses the brainstem can be rapidly fatal.