Author: Rachel Wightman, MD (Senior EM Resident, NYU) // Editors: Adaira Landry, MD, & Justin Bright, MD
HPI: 29 year old female with a prior history of headaches, presented with two days of gradual onset, atraumatic, right sided headache that is throbbing in nature. The patient reported heaviness about the eye but no visual changes or disturbances. No neck pain, fevers, chills. She described feeling slightly light-headed but no balance loss. She had a mechanical trip and fall yesterday without head trauma, and her headache had been present for a day prior to the fall.
ROS: otherwise normal.
PMH/PSH: headaches, depression, anxiety, asthma
SH: no smoking, no etoh, no drugs
Allergies: Penicillin (rash)
Vitals: 98.6F, BP: 156/85 P: 101, RR: 16, O2: 98%RA
Gen: A&Ox3, well-developed, well-nourished
HEENT: normocephalic, atraumatic, conjunctiva wnl, EOM wnl, PERRL, normal fundoscopic exam, crisp optic discs, normal ROM neck/supple
Neuro: CN2-12 intact, normal reflexes, normal muscle tone, normal coordination
Labs: Serum HCG negative
Imaging: None ordered
ED Course: The patient was believed to be experiencing a migraine headache. She had no evidence of head trauma, no signs of infectious etiology, and had no clinical findings or hx for SAH. She was administered Toradol, IVF and Reglan, and discharged with instructions to follow up with neurology and possibly have an outpatient MRI.
Discharge Dx: Headache
Bounce Back Visit
The patient re-presented to the Emergency Department 14 hours later with worsening headache and nausea. Minimal additional history was obtained. The physical exam was documented as unchanged from the prior exam, and the patient was again diagnosed with a likely migraine. Her pain was uncontrolled with Toradol, Reglan, and IVF. The patient subsequently received dexamethasone and magnesium with improvement in pain. The new discharge plan was a steroid taper and outpatient neurology follow-up. At time of discharge patient reported worsening pain and requested a neurology consult. A head CT was also ordered at that time.
Neurology Consult Note:
29 year old obese female on OCPs with R frontal headache radiating to left, stabbing, unremitting. Headache associated with worsening on valsalva maneuvers, dizziness, pain on eye movement, but no vomiting. History tension-type headache in the past, but this headache is different, failing ibuprofen 400mg therapy which usually relieves her headache. FH: Maternal Grandmother with venous clots resulting in stroke, mother with PFO and stroke at age 40 unknown if hypercoagulability workup performed.
In addition to the medication given above, a head CT was performed. CT images demonstrated a right transverse and straight sinus thrombosis.
DIAGNOSIS: Cerebral Venous Sinus Thrombosis
Cerebral venous sinus thrombosis (CVST) is a rare but serious cause of neurologic symptoms. CVST results from venous outflow obstruction causing increased intracranial pressure, increased retrograde venous pressure, a decrease in cerebral blood flow, decreased cerebral perfusion pressure and eventually venous infarction. CVST is estimated to account for fewer than 1-2% of all strokes. It affects mostly younger adults and children with the mean age of presentation at 39 years. Women are affected more often than men with a 3:1 female predominance. This gender imbalance is thought to be due to the increased risk for CVST associated with pregnancy, puerperium, and with use of oral contraceptives. More than 85% of pts with CVST have at least one identifiable risk factor and multiple risk factors may be found in about half of patients with CVST. The most frequently cited risk factors include pro-thrombotic conditions including genetic and acquired conditions. Other less common risk factors include CNS/sinus infection, head injury, inflammatory bowel disease and cirrhosis.
The clinical presentation for CVST is highly variable and non-specific. Signs and symptoms are divided into three categories: 1) isolated intracranial hypertension 2) a focal syndrome including focal deficits, seizures or both 3) encephalopathy causing multifocal signs, mental status changes, stupor, or coma. Patients can present acutely, sub-acutely, or with chronic symptoms. Other variables affecting presentation include site and number of occluded vessels, presence of parenchymal brain lesions, patient age and gender. CVST cannot be diagnosed on clinical grounds alone and neuroimaging is required for diagnosis.
Non-contrast head CT is typically the first study performed in patients with suspected CVST in the ED because it is readily available and the most useful test to evaluate for conditions that can mimic CVST clinically. Common findings of CVST on head CT are generalized swelling or localized areas of hyper-density indicating cerebral infarction (seen in up to 40% cases). Direct signs of CVST can sometimes be seen on CT scan and include the dense triangle sign, as seen on CT scan in our patient, which indicates fresh thrombus in the posterior aspect of the superior sagittal sinus. Non-contrast head CT can be normal in 25-30% of patients with CVST. In hospitals without MRI capability, CT venogram is the confirmatory test for CVST. However, MRI/MRV is the most sensitive and specific imagining modality for CVST and can best determine the location and age of a thrombus.
Anticoagulation with heparin is the standard therapy for CVST. At this time no clinical guideline exists to recommend un-fractionated heparin (UFH) versus enoxaparin (LMWH) for therapy and there are no prospective, randomized trials of systemic anticoagulation use in treatment CVST. Hemorrhagic infarction is associated with 40% of CVST, however, most experts recommend use of anticoagulation in patients with CVST even in the setting of known hemorrhagic infarction on imaging. Systemic TPA and direct endovascular thrombolysis are considered experimental therapy at this time and cannot be routinely recommended. Patients with CVST will require anticoagulation beyond hospital discharge. The duration of anticoagulation is dependent on the etiology of thrombosis.
The patient was admitted to the stroke unit for anticoagulation of right transverse and straight venous sinus thrombosis. She was started on a heparin drip, transitioned to enoxaparin, and then bridged to warfarin. Thrombophilia studies demonstrated a low anti-thrombin level and an elevated anti-2 beta glycoprotein antibody, suggesting a hypercoagguable genetic disorder. The patient was discharged home on hospital day seven with no residual deficits on warfarin with plans to repeat coagulation studies in six weeks.
Errors in Evaluation
Lack of full history: Patient risk factors for CVST were not elicited on first visit. Additionally, changes in headache symptoms from previous headaches were not adequately explored. Any alteration in patient headache symptoms should trigger further evaluation and consideration of possible secondary cause of headache.
Anchoring bias: Anchoring to the diagnosis of possible migraine headache from the initial visit likely led to delayed diagnosis and near-miss on second visit to the ED. It is important to ask all patients with reported history of migraines how the diagnosis was made because many patients with headache sometimes attribute any and all headaches to “migraine.” Making a new diagnosis of migraine headache in the Emergency Department should cause providers to pause and consider other possible etiologies of headache.
Failure to broaden differential: CVST is rare diagnosis with variable presentation and can often be missed on initial presentation. Physicians need to expand their differential and think about CVST in order to make the diagnosis. Not all headaches are migraines so we should be sure to document our thought process well when seeing these patients.
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Singh A, Soares WE. Management Strategies for Acute Headache in the Emergency Department. Emergency Medicine Practice. June 2012. 14(6):1-42. EB Medicine.net.
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