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Young woman has persistent headache, disc edema
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A 25-year-old Native American woman presented for an acute care visit reporting a 2-week history of double vision. She also complained of a new onset headache persisting for the last 3 weeks, accompanied by nausea, dizziness, chills, neck pain, tinnitus, and bilateral paresthesia and numbness of her hands and forearms. Her ocular and medical history was otherwise negative.
Images: Runyon RB
She was previously seen by physicians at four other facilities for her persistent headaches. The patient was diagnosed with altitude sickness and was prescribed Zofran (ondansetron, GlaxoSmithKline) and Flexeril (cyclobenzaprine HCl, McNeill). Both medications provided no relief of headache symptoms, and she was subsequently diagnosed with complex migraines and treated unsuccessfully with Topamax (topiramate, Janssen). The patient reported that the recent onset of visual symptoms prompted her to seek additional medical attention.
On examination, the patient appeared uncomfortable with pain and shaking chills despite multiple layers of clothing. Her affect was blunted. However, she was afebrile with a normal pulse and respiratory rate. Her blood pressure was 124/81 mm Hg and her body mass index was 28.8 (overweight).
Ocular examination noted pinhole acuities at 20/20 OU. Pupils were equally round and reactive to light and without a relative afferent pupillary defect in each eye. Confrontation visual fields and extraocular muscles were full in each eye. The patient was grossly orthophoric on cover test at distance and near, but Maddox rod testing revealed a 10 prism diopter uncrossed binocular diplopia suggesting the presence of an eso-deviation with no vertical component. Amsler grid revealed small relative scotomas, more so in the left eye than the right; however, the findings were not consistent with a neurological deficit.
The anterior segment ocular health examination was unremarkable. Posterior segment evaluation revealed severe bilateral disc edema with 360 degrees of blurred disc margins with associated disc hemorrhages and cotton-wool spots. Examination was negative for Paton folds and spontaneous venous pulse. The macula and fovea were intact and without exudates, and no holes, tears or detachments were present in the periphery.
The patient was immediately referred to the emergency department for further evaluation.
What’s your diagnosis?
See answer on the next page.
Papilledema only occurs concurrently with elevated intracranial pressure (ICP). Causes of pseudopapilledema include optic neuritis, congenital disc anomalies and buried drusen, all of which should be considered and ruled out in patients presenting with bilateral disc edema without headaches.
Symptoms of elevated ICP are headaches that often are related to postural changes, dizziness, double vision (secondary to an acquired sixth nerve palsy), visual obscurations and disturbances, tinnitus, neck pain, and distal limb paresthesia and numbness. These symptoms were all part of our patient’s clinical presentation.
Conditions that may cause papilledema and elevated ICP include intracranial tumors and masses (hematomas, infectious abscess, aneurysms, granulomas), congenital conditions (arteriovenous malformations, aqueductal stenosis) and CNS infections (meningitis, encephalitis). Other causes of elevated ICP include malignant hypertension, anemia, obstructive sleep apnea, renal failure, primary CNS lymphoma, endocrine diseases (i.e., Addison’s, Cushing’s, hypothyroidism) and several types of medications (most commonly tetracyclines, nitrofurantoin and excess vitamin A). Finally, in the absence of a detectable etiology, the cause of elevated ICP and resulting papilledema may be idiopathic. According to the Modified Dandy Criteria, idiopathic intracranial hypertension (IIH) is always a diagnosis of exclusion.
After a clinical discovery of papilledema, the patient’s blood pressure and temperature should be measured and recorded. High blood pressure may indicate a hypertensive crisis, whereas a fever may indicate an infectious optic neuritis or meningitis. Blood pressure and temperature readings will help direct the referral and allow you to make better recommendations to the receiving doctor or institution.
A battery of serologic tests and neuroimaging is needed in order to identify and rule out causes of elevated ICP. A CBC with differentials and CMP should be ordered to rule out anemia, renal failure and acute infections. While papilledema is a rare manifestation of bacterial meningitis, it is much more common in viral encephalopathies, thus DNA testing for herpes simplex and herpes zoster should be done in cases of suspected aseptic meningitis. An MRI and MRV or a CT and CT venography should be performed, followed by a lumbar puncture and CSF studies if meningitis is suspected. It is important to understand that neuroimaging must be performed prior to a lumbar puncture in order to rule out intracranial lesions, as performing a lumbar puncture on a patient with an intracranial space-occupying lesion may induce a Chiari malformation. Once mass lesions have been ruled out, a lumbar puncture can safely be performed.
An emergent (same-day) referral is indicated in a symptomatic patient, although in asymptomatic patients, the referral priority may be reduced to urgent.
This patient’s diagnosis
Optic nerve findings, along with persistent headache and associated symptoms, indicated an emergent referral for further systemic and neurologic evaluation. Blood pressure and temperature were both normal. While the patient reported having a MRI ordered by one of the previous providers, her records were not able to be accessed, nor was there an MRI on site at that facility. While MRI is preferred, CT of the head and CT venogram will exclude most mass-effect lesions and cerebral venous sinus thrombosis when MRI is not available. Thus, a CT of the head was ordered without contrast to rule out the presence of space-occupying lesions. The CT was negative for masses, hemorrhages or infarcts, and the ventricles were normal in size and shape. A lumbar puncture was then performed by the ED physician. The lumbar puncture and subsequent CSF analysis yielded the results indicated in the accompanying table.
The abnormal presence of white blood cells in the CSF, along with leukocytosis (elevated white blood cell count) in the blood indicated a potential underlying infection. Ultimately this patient was admitted to the Adult Care Unit with suspected meningitis, and treatment was initiated with IV ceftriaxone, IV acyclovir and acetazolamide 500 mg twice daily. After no improvement of symptoms in 72 hours, she was transferred to a university medical center and placed under the care of neurology and infectious disease. After 9 days of continued acetazolamide treatment, her symptoms began to improve. After consulting neurology, infectious disease and oncology specialists, infectious causes were ruled out, and the cause of elevated ICP was determined to be idiopathic. The patient was discharged with acetazolamide 500 mg twice daily and tramadol every 4 to 6 hours as needed.
Case discussion
Although the patient represented the classic demographic of patients with IIH (a young, overweight female with no known systemic conditions or environmental risk factors), a complete neurologic and systemic work-up was required to rule out other potential causes of elevated ICP.
Despite evaluation by several primary care and emergency department physicians, the cause of her headaches remained elusive until she was evaluated by an eye care provider. Papilledema was the key clinical finding, which ultimately redirected the systemic work-up and management to the appropriate course.
General signs of serious infections are fever, elevated white blood cell count and change in affect. While the patient was afebrile, her affect was blunted. She was generally responsive but clearly in a level of discomfort and pain that consumed her. She was visibly shaking from the chills despite wearing layers and being wrapped in a hospital blanket. The severity and ongoing nature of these symptoms along with the presence of white blood cells in the CSF heightened the level of concern for meningitis, which resulted in overnight hospital admission and initiation of IV antibiotics and IV antivirals.
Given the severity and length of the patient’s signs and symptoms, immediate treatment with acetazolamide was indicated until further laboratory and serologic testing was completed. Acetazolamide is the mainstay treatment for IIH and, in this case, it was used before ruling out infectious causes, but only after a lumbar puncture had been performed. Acetazolamide inhibits carbonic anhydrase, an enzyme required for CSF production, thereby lowering the pressure in the head and spinal column. Typically, it is dosed 500 mg twice daily, but the dosing can reach a ceiling of 4 G/day, depending on the clinical response and the patient’s tolerance of side effects. The dose should be titrated slowly to avoid such side effects.
Source: Runyon RB
The medication has well-known side effects of metallic taste and tingling sensations in the extremities, but more serious side effects including electrolyte imbalance and metabolic acidosis may occur with chronic dosing. Therefore, patients on chronic acetazolamide should have routine blood work and be closely followed by their primary care provider. Potassium supplements should be concurrently prescribed in patients on chronic acetazolamide treatment. In recalcitrant cases, serial lumbar punctures or shunts may be considered.
Topiramate is a weak carbonic anhydrase inhibitor and can be considered particularly when managing the headache component of IIH. Generally, headaches improve after the first month of treatment, but in many patients, headaches persist even after the resolution of elevated ICP.
Long-term weight loss has been shown to reduce the incidence of headaches. A 5% to 10% reduction in body weight along with acetazolamide has been shown to be most effective in reducing symptoms of IIH and has also been shown to reduce ICP more than acetazolamide alone.
Vision loss is the most common morbidity of IIH, with 10% of cases resulting in bilateral blindness. However, enlarged blind spots and nasal steps, as was noted in this patient, were found to be the most common visual field defects, according to the Idiopathic Intracranial Hypertension Treatment Trial. Visual field defects may be permanent, and their severity may worsen or improve as the condition stabilizes. In most cases, the macula is spared.
The duration of IIH is difficult to predict, but it is likely that the patient will remain on chronic acetazolamide treatment even after the papilledema and elevated ICP has resolved. The medication may then be slowly tapered with close monitoring for the return of signs and symptoms. In most cases, IIH is a chronic disease that fluctuates with weight changes. Routine examinations are indicated in these patients, and OCT and HVF are helpful ancillary tools for monitoring the ocular sequelae. IIH treatment should be comanaged with a primary care physician and a neurologist.
The cause of IIH is still unclear. Current theories include anatomical abnormalities, stenosis of the transverse sinuses or abnormal vitamin A metabolism, but research is still ongoing. The Modified Dandy Criteria is the current clinical guideline for evaluating patients with papilledema. In 2013, revised criteria were proposed by Friedman and colleagues, but Modified Dandy Criteria remains the standard clinical guideline.
- References:
- Friedman, DI, et al. Neurology. 2013;doi:10.1212/WNL.0b013e3182a55f17
- Friedman, DI. Papilledema. In: Miller NR, Walsh FB, Hoyt WF, eds. Walsh and Hoyt’s Clinical Neuro-ophthalmology. Philadelphia: Lippincott Williams & Wilkins; 2005:237-291.
- Mollan, SP, et al. J Neuro Neurosurg Psychiatry. 2016;doi:10.1136/jnnp-2015-311302.
- Mollan, SP, et al. Pract Neurol. 2014;doi:10.1136/practneurol-2014-000821.
- Smith, JL. J Clin Neuroophthalmol. 1985;5:55–56.
- Wall, M, et al. Invest Ophthalmol Vis Sci. 2016;doi:10.1167/iovs.15-18626.
- For more information:
- Stephanie Loftus, OD, is completing her residency in primary care and ocular disease within Indian Health Service. She can be reached at Stephanie.Loftus@ihs.gov.
- R. Brandon Runyon, OD, FAAO, is residency coordinator and staff optometrist within Indian Health Service. He can be reached at Richard.Runyon@ihs.gov.
- Edited by Leo P. Semes, OD, FAAO, a former professor of optometry, University of Alabama at Birmingham, and a member of the Primary Care Optometry News Editorial Board. He can be reached at leopsemes@gmail.com.
Disclosure: Loftus and Runyon have no relevant financial disclosures. Semes is an advisor or on the speakers bureau for Alcon, Allergan, Bausch + Lomb, Genentech, Maculogix, OptoVue, Shire and ZeaVision. He is a stockholder with HPO.