Headaches That Kill
Abstract & Commentary
By Dara Jamieson, MD, Associate Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Jamieson reports she is a retained consultant for Boehringer Ingelheim, Merck, and Ortho-McNeil, and is on the speakers bureau for Boehringer Ingelheim.
Synopsis: Fatal headaches are associated with age over 50, loss of consciousness and collapse, and worst/thunderclap character of the headache.
Source: Lynch KM, Brett F. Headaches that kill: A retrospective study of incidence, etiology, and clinical features in cases of sudden death. Cephalalgia 2012;32:972-978.
This study examined cases of sudden death in which headache was the initial presenting feature. In a retrospective autopsy study, the authors reviewed 55 cases with headache presenting between January 1997 and December 2006 to the Department of Clinical Neurological Sciences, Beaumont Hospital, Dublin. The study population, which was 51% male, ranged in age from 12 to 82 years. The peak incidence of deaths (34.5%) occurred in the 51-70 age group, with 26.7% of that group having multiple medical comorbidities. The number of autopsied deaths per year, with headache as a presenting feature, ranged from 11 (22% of all cases) in 1999, to 1 in 2004, with a mean of 5.5 autopsy cases per year. All 55 patients presented to the emergency room with an associated “red flag” symptom that presumably could indicate potential for an underlying life-threatening etiology for the headache. Red flags that were found in > 20% of patients, ranked from most to least often, were: headache at age > 50 years, seizure/collapse/loss of consciousness, thunderclap headache, worst headache, drowsy/confused/agitated, progressive visual/neurological symptoms, nausea/vomiting, and paralysis/weakness/Babinski sign. The occurrence of a headache in patients > 50 years of age was the most common red-flag feature, presenting in 54.5% (n = 30) of patients. Loss of consciousness or collapse occurred in 52.72% (n = 29); “worst” headache and thunderclap headache in 45.5% (n = 25) and 51% (n = 28), respectively; and nausea and vomiting in 30.9% (n = 17).
There was no correlation between age and the cause of headache-associated death, with the exception of sinus venous thrombosis (SVT) which comprised 8% of the total cause of death associated with headache in the younger age group (median of 40 years). All four cases of SVT occurred in women < 40 years of age with prothrombotic states and with 100% occurrence of two primary red-flag features: a prolonged occipital headache with neck pain and a progressive focal neurological deficit.
The ultimate causes of death found at autopsy in the headache group without evidence of trauma (n = 48) were vascular events in 60.4% (n = 29), primary brain tumors/cysts in 16.7% (n = 8), meningitis in 6.25% (n = 3), and other in 16.7% (n = 8). In cases where aneurysm rupture and subsequent intracerebral hemorrhage were the cause of 20% of headaches (n = 11; male-to-female ratio 1:3), loss of consciousness or collapse, occipital/temporal headache and neck pain, or a focal neurological deficit was the most common red-flag feature in the clinical history.
Headaches are classified as primary (without any underlying “pathological” cause) or secondary (attributable to vascular, neoplastic, or infectious causes, as well as other intracranial pathologies). Primary headache disorders are very common and neuroimaging cannot be obtained on all patients with headaches unless there are specific concerns in the clinical history and examination. The identification of characteristics of a headache that indicate its potential to be a secondary headache, most commonly a vascular etiology, is very useful in directing a more comprehensive evaluation, including neuroimaging. In this study population, the more statistically significant, alarming red-flag features that ultimately heralded a fatal cause of headache were headaches in those > 50 years of age, headaches described as “worst”/thunderclap, a history of seizure/collapse/loss of consciousness, and associated nausea and vomiting. However, migraine headaches, which afflict more than 30 million Americans with disability but without mortality, can affect older individuals, can be very severe and of sudden onset, and are characteristically associated with nausea and vomiting. Use of these criteria will still result in imaging of many non-life threatening headaches to catch the rare secondary headaches.
For the clinician caring for patients with headaches, it is worth remembering that primary headaches are most likely to afflict younger patients and that although the pain of migraines can be very severe, the onset of a very severe headache in an older, non-migraineur warrants further investigation, including neuroimaging. This paper suggests that “CT scan should be the initial test of choice for new-onset headache in adults.” However, MRI with FLAIR and susceptibility weighted imaging can detect even subtle intracranial hemorrhage as well as other potentially fatal pathologies that could be missed on a CT scan of the head. When feasible, MRI is more sensitive in detecting multiple pathological types.
There are limitations to an autopsy study. Although the decrease in the number of headache-associated deaths over the time of the study was attributed to progressively more accurate diagnosis and effective intervention, decreased referral for autopsy is a plausible cause of the decline in autopsied deaths. Would the conclusions of this study be different if all headache-associated deaths, not just those that were autopsied, had been included? The reliance on autopsies to conclude that headache fatalities are decreasing and the recommendation to do CT scanning over MRI scanning may reflect differences in the health care system in Ireland, as compared to the United States.