By: Breanne Jaqua, DO, MPH, PGY-3
As part of the PGY-3 rotation that includes dedicated pediatric shifts in the ED and medical student sim lab teaching, residents now engage in a Quality Improvement (QI) project. I did my QI project on the differential diagnosis of headache, and presented my findings during Grand Rounds (#EMConf) on 8/28/2019. This article runs through a quick differential of headache, followed by my QI project findings, and final thoughts from our grand rounds discussion.
Part 1: Differential Diagnosis
First, here is a large list of diagnoses (please note that this list is not all inclusive):
Subarachnoid hemorrhage
Epidural hematoma (EDH)/Subdural hematoma (SDH)/Intraparenchymal Hemorrhage (IPH)
Meningitis
Temporal arteritis
Pre-eclampsia
Central venous thrombosis
Cryptococcal meningoencephalitis
CNS Histoplasmosis
Toxoplasmosis
Rocky Mountain Spotted Fever
Increased ICP (VP shunt malfunction or pseudotumor cerebri)
Mass/cancer
Carbon monoxide
Migraine headache
Tension headache
Cluster headache
SAH
The headache from subarachnoid hemorrhage (SAH) is often described as “thunderclap”, where the pain reaches maximal intensity shortly after onset (usually around 5 minutes). Patients may characterize their pain as the worst headache of their life. For patients with frequent headaches, they may describe this as feeling different from their typical headaches. Some patients may have associated syncope, and the headache may wake people from sleep. Risk factors include hypertension, cigarette smoking, and family history of SAH. Personal or family history of polycystic kidney disease, connective tissue disorders, recent cocaine or methamphetamine use, and prior brain aneurysms can also increase the risk of SAH.
EDH/SDH/IPH
For patients with recent head trauma and history of coagulopathy or anticoagulant use, intracranial hemorrhage should be high on your differential. This can also occur in patients with severe uncontrolled high blood pressure who develop a sudden and severe headache.
Meningitis
Infection of the meninges classically manifests with symptoms such as fever, headache, altered mental status, photophobia, and meningismus. Consider bacterial, viral, and fungal causes of meningitis in your history-taking to help guide your treatment.
Giant cell/temporal arteritis
This pathology usually presents in patients older than 50 years old. Symptoms include new onset temporal headache and jaw claudication. The patient may also have proximal myalgias. Visual changes are a late finding. The number one test in the ED for this potential etiology is ESR, with diagnosis later confirmed by temporal artery biopsy.
Cryptococcus/Histoplasmosis/Toxoplasmosis
Key things to ask in the history might include HIV status, whether or not the patient is on chemotherapy, has a prior organ transplant, and/or has a history of IV drug use (basically think immunocompromised). It is also important to consider any recent travel to areas where these pathogens are common.
Increased ICP
Patients with a CSF shunt may have headaches if there is a shunt malfunction or CSF infection. Also, Idiopathic Intracranial Hypertension (AKA pseudotumor cerebri) primarily affects overweight women of childbearing age. It commonly results in visual disturbances, cranial nerve deficits, tinnitus, and other neurologic symptoms. Use your ultrasound skills to check optic nerve sheath diameters for an easy estimate of ICP.
Preeclampsia/Eclampsia
The exact mechanism of how preeclampsia and eclampsia cause headache is
unknown. However, it is suspected that generalized endothelial cell dysfunction leads to vasospasm of the cerebral vasculature in response to severe hypertension. Poor cerebrovascular autoregulation can also lead to vasospasm, sometimes resulting in cerebral vasospasm and ischemic/hemorrhagic changes in the posterior hemispheres. Look out for other severe symptoms such as visual changes and seizures.
Mass/Lesion
Key elements of the history that may point toward this differential include known malignancy, progressive headaches, headaches that worsen with lying down, headaches that are worse first thing in the morning, or new onset headaches in patients over 50 years old.
Cerebral Venous Thrombosis
Symptoms might include cranial nerve palsies or deficits, proptosis, fevers, chemosis, and periorbital edema. A high clinical suspicion is required to make this diagnosis, as an MR venogram is the best test to assess for it. Consider it in patients with coagulopathies predisposing patients to clotting more quickly (pregnancy, cancer, obesity, collagen vascular diseases), inflammatory bowel disease, intracranial hypotension, sickle cell anemia, iron deficiency, and dehydration.
Carbon Monoxide Poisoning
Especially during winter, it is important to ask whether the patient and/or their companions are using grills or space heaters indoors. Often, other family members (and even pets) will have similar symptoms as the patient.
Rocky Mountain Spotted Fever
This is particularly pertinent for patients who spend a large amount of time outdoors and live in endemic regions. Look for the classic centripetal rash involving the hands and feet before the trunk.
Acute Angle Closure Glaucoma
This can present with a constellation of symptoms, including headache, abdominal pain, nausea, and vomiting. Given the symptoms it can be easy to miss this differential. Key clues are increased intra-ocular pressures and a painful red eye. Act quickly to prevent permanent visual deficits.
Part 2: Chart Review
Throughout this QI project, I found significant variation in documentation from ED providers. Some providers had incomplete neurologic exams in their notes, others lacked critical components of the history, and others either did not know about or did not comment on the patient’s prior visits for the same complaint. I was surprised by this disparity and set out to scrutinize my own neuro exam documentation and help improve that of my colleagues as well.
First, I read through the UpToDate article on neuro exams (Gelb, 2012), and I found that I have been under-documenting key portions of the exam, or not performing critical elements at all (when was the last time I assessed a patient’s gait in the ED?). While going through the article, I started drafting a new neuro template that I now use on shift. This is my new neuro exam (keep in mind there are other elements of the neuro exam that will need to be added depending on patient and complaint such as fundoscopic exam or HINTS exam, among others).
Mental Status: alert & oriented X4, attentive, follows simple and complex commands, no aphasia, no dysarthria
Cranial nerves: CNII-XII grossly intact
Motor: strength testing 5/5 in b/l biceps, triceps, hand interossei, iliopsoas, hamstrings, ankle plantar and dorsiflexors. No pronator drift in b/l upper extremities.
Coordination: smooth and normal finger to nose testing bilaterally without tremor. Heel-knee-shin testing smooth and symmetric b/l without tremor. No involuntary movements noted.
Gait: steady, smooth gait
Sensation: light touch intact to all four distal limbs
The cranial nerve exam can be further broken down to include:
· no gross visual field deficit bilaterally
· visual acuity grossly intact bilaterally
· PERRL, no afferent pupillary defect
· no ptosis, no nystagmus, EOMI
· facial sensation grossly intact and symmetric bilaterally
· symmetric facial expressions b/l, no facial droop
· hearing grossly intact b/l
· symmetric movement of the palate b/l
· sternocleidomastoid and trapezius muscle strength symmetric and strong b/l
· symmetric tongue movement b/l
Part 3:
During my QI presentation (which I designed to be an interactive discussion), one of our attendings raised an important question: what is a good differential diagnosis for a patient with headache AND visual changes? Our on-the-fly differential included:
Temporal arteritis
Idiopathic intracranial hypertension
Preeclampsia
Migraine
Iritis
Acute angle closure glaucoma
Stroke
Reversible cerebral vasoconstriction syndrome
Posterior reversible encephalopathy syndrome
Optic neuritis (multiple sclerosis)
Of these, I wanted to highlight two less-common differentials not discussed above:
PRES Syndrome (Posterior reversible encephalopathy syndrome) – summarized from UpToDate: (Neill, 2018)
This syndrome usually involves the subcortical white matter in the posterior cerebral hemispheres. The patient develops vasogenic edema in the brain interstitium, and if severe can show up on radiographs (can show up on CT, but MRI is best). This disease is classically associated with high blood pressure, but sharp and sudden changes in blood pressure may also play a role. Have a high clinical suspicion in patients who are on immunosuppressive therapies, have renal disease such as lupus nephritis and glomerulonephritis, or autoimmune disorders. Presentation often includes headache, altered mental status, visual disturbances, and seizures.
Multiple Sclerosis (Optic Neuritis) – summarized from UpToDate: (Osborne & Balcer, 2018)
Optic neuritis is strongly correlated with MS. This is an inflammatory and demyelinating process that affects the optic nerve and leads to monocular vision loss most commonly. There is always an afferent pupillary defect present if the other eye is otherwise normal; test for this condition using a swinging flashlight test. Central scotoma is the most common visual field defect. Loss of color more than loss of visual acuity is specific to this condition. Fundoscopic exam and ophthalmologic exam is crucial. Workup includes MRI of the brain and orbits with contrast.
Conclusion
Headache is a common complaint with numerous etiologies. Patients require in-depth histories and physical exams that are accurately documented. The differential diagnoses should reflect the patient in front of you.
References:
Gelb, D. (2012, Sept 7). The detailed neurologic examination in adults. Retrieved Sept 9, 2019 from uptodate.com
Neil, T.A. (2018, Nov 19). Reversible posterior leukoencephalopathy syndrome. Retrieved Sept 9, 2019 from uptodate.com
Osborne, B., & Balcer, L.J. (2018, Jul 17). Optic neuritis: Pathophysiology, clinical features, and diagnosis. Retrieved Sept 9, 2019 from uptodate.com
Edited by: Dr. Sara Graber and Dr. Shyam Murali
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