Gesundheit!!!
Chief Complaint:
Headache
HPI:
30-year-old female, G1 P0 5 weeks estimated by LMP, states at approximately 08:15 this morning was getting ready to go to work when she sneezed and immediately developed a severe 8 out of 10 headache. Shortly after, she vomited and had diplopia that lasted for 1 to 2 minutes. Diplopia has resolved but nausea and headache have persisted. The headache is a diffuse throbbing pain that has now moved to the back of her neck. She typically will get a headache approximately once a month that is less intense and responds to Tylenol. Today she has not taken anything for her headache because of its severity she came to the emergency department. She has not had neurologic symptoms or vomiting with headaches in the past. Nothing she does makes it worse. No changes with light or sound. She recalls a worse headache about one month ago while driving to a hockey game.
No family history of aneurysms or polycystic kidney disease
Pertinent Exam Findings:
Neurological: Awake, alert, oriented. Motor grossly intact with 5 out of 5 strength upper and lower extremity. Speech is normal and patient is able to ambulate without ataxia. cranial nerves II through XII intact, finger-to-nose intact.
ED Course:
Discussed with patient given the report of sudden onset, severe, atypical, associated neurologic symptoms and vomiting need to rule out subarachnoid. Discussed risk benefits of CTA head and neck in the setting of being pregnant, patient okay with proceeding.
IVF, Reglan and diphenhydramine for sx tx with minimal relief
Additional 10mg Reglan given, no relief
Dilaudid given after imaging read
Neuro IR and NSGY consulted and planned for cerebral angiogram and MRI, control BP <160
Diagnostic Studies:
CT HEAD
1. Subarachnoid hemorrhage is noted within the sellar and suprasellar cisterns, prepontine and interpeduncular cisterns, perimesencephalic and left sylvian cisterns. Hyperdensity appears within the region of the pituitary stalk and raises concern for pituitary apoplexy or lymphocytic hypophysitis in this pregnant patient though would be unusual at this early gestation. Recommend MRI brain and pituitary without and with contrast to better evaluate.
CTA HEAD
1. No aneurysm identified. No large vessel occlusion or hemodynamically significant stenosis.
CTA NECK
1. No hemodynamically significant stenosis or evidence of cervical dissection.
DISCUSSION:
Background:
While nine out of every 100,000 people will experience a subarachnoid hemorrhage (SAH), not even one in 100,000 will experience perimesencephalic nonaneurysmal hemorrhage (PMH). Aneurysm rupture is responsible for about 80% of non-traumatic SAH with location and size of aneurysm being risk factors for rupture. Approximately 10% of non-traumatic SAH cases are attributed to PMH. Timely diagnosis is extremely important considering a 25% mortality rate within 2 hours of initial bleeding. This percentage rises to 40% mortality after one week.
Diagnosis:
Patients with PMH have a similar presentation to those with SAH. Most PMH patients will present with sudden onset of pain with nausea and vomiting and rarely transient focal symptoms may be present. Classic red flags include rapid onset, worst headache of life, exertional headache, syncope with headache, neck stiffness, and vomiting. CTA is typically ordered when these patients present, CTA is without aneurysmal bleed, and the CT head portion is revealing of the PMH diagnosis. Radiographic criteria for diagnosis includes bleeding patterns directly involving the midbrain or pons, any or all perimesencephalic cisterns, proximal anterior interhemispheric fissure, and basal region of Sylvian fissure. If obvious intraventricular hemorrhage is absent but some combination of previously listed patterns is present, then the physician can be confident in the diagnosis. The usefulness of lumbar puncture (LP) following a negative CT is debated within clinical literature. LP may be useful in detecting xanthochromia and may detect blood missed by CT. The American College of Emergency Physicians has a clinical policy with a Level B Recommendation that LP is the criterion standard for diagnosing SAH. However, the literature tends to support foregoing an LP in a patient presenting with a sudden-onset headache, negative neurologic exam, negative non-contrast head CT in the setting of symptomatic onset within 6 hours prior.
Management in ED:
Initial management should consist of symptomatic treatment. There are several “headache cocktails” that are used by emergency physicians which consists of different medications and routes. The perfect medication should provide fast sustained release with little to no side effects. Unfortunately, the perfect medication does not exist. Whether you choose antidopaminergics, triptans, steroids, acetaminophen, antihistamines, valproic acid, ketamine or others is not important. However, initial avoidance of NSAIDs makes pathophysiological sense in order to avoid platelet inhibition in a possible intracranial hemorrhage. Hematoma growth within the first 24 hours is an important mortality predictor, with spontaneous ICH posing a high expansion risk. In such patients blood pressure control is very important and a goal systolic blood pressure between 140-180 mm Hg can be achieved with Nicardipine. While waiting on the infusion a bolus of IV Labetalol may be used. In the case of atraumatic head-bleeds the use of seizure medications such as Phenytoin is not indicated and may even negatively impact cognitive recovery due to their vasospastic and pyrexic effects.
Prognosis:
PMH carries a much better prognosis than SAH and life expectancy is not altered. While there are reports of re-bleeding, recurrent severe headache, episodic amnesia, delayed cerebral ischemia (DCI), cranial nerve palsies, or delayed hemorrhagic or ischemic events, none of these have proven consistent. In one study patients two years post PMH had no difference in quality of life compared to controls.
Differential of “Thunderclap headache”:
While we often hear “thunderclap headache” dramatized on Scrubs, Grey’s Anatomy, its use in the ED triggers a serious response as the differential includes severe and acute possibilities. The most likely diagnoses include but are not limited to SAH, cervical artery dissection, cerebral venous thrombosis (CVT), hypertensive crisis, ischemic stroke, and pituitary apoplexy. Additional consideration should be given to less likely diagnoses such as spontaneous intracranial hypotension, reversible cerebral vasoconstriction syndrome (RCVS), and hypertensive encephalopathy. Pregnancy raises concern for thrombotic events due to a hypercoagulable state including pituitary apoplexy.
It was initially thought that on CT our patient had pituitary apoplexy. Pituitary apoplexy occurs with hemorrhage or infarction of the pituitary gland. The most common causes are pituitary adenoma, pregnancy (usually 3rd trimester or peri-postpartum), radiation or bromocriptine therapy. In addition to headache, pituitary apoplexy usually results in decreased visual acuity, ophthalmoplegia, visual field disturbances, and vomiting. Due to disruption of the HPA axis, abnormal lab findings typically include hypoglycemia, hyperkalemia, and hyponatremia. This adrenal crisis patient is also frequently associated with hypotension refractory to fluids and pressors. Stress dose steroids are life-saving in these patients.
Main Points:
In evaluating a patient presenting with a thunderclap headache the use of imaging studies in combination with a thorough history are essential as the neurological exam may be negative in cases of SAH
Key red flags in a patient’s history include rapid onset headache, worst headache of life, exertional headache, syncope with headache, neck stiffness, and vomiting
Non-contrast CT Head along with CTA head and neck are the diagnostic modalities in the ED for SAH, however cerebral angiograms are the gold standard
While PMH is rare, it carries a much better prognosis than SAH and typically does not require invasive treatment measures
Antiepileptic medications should not be used in atraumatic bleeds
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Authors
Aaron Wolfe, DO, FACEP
Joshua Bridge, MSII
Sarah Rokhlin, MSI