A Headache of a Diagnosis

The new england journal of medicine Clinical Problem-Solving Caren G. Solomon, M.D., M.P.H., Editor A Headache of a Diagnosis Robert M. Stern, M.D., Marlise R. Luskin, M.D., M.S.C.E., Roger P. Clark, D.O., Amy L. Miller, M.D., Ph.D., and Joseph Loscalzo, M.D., Ph.D. In this Journal feature, information about a real patient is presented in stages (boldface type) to an expert clinician, who responds to the information by sharing relevant background and reasoning with the reader (regular type). The authors’ commentary follows. A 24-year-old man presented to a university health center in January with a persistent From the Department of Medicine, Brig- headache that began abruptly 6 days earlier. He noted nonpulsating discomfort in the ham and Women’s Hospital, and Har- vard Medical School — both in Boston. right retro-orbital area that was not relieved by ibuprofen (400 mg four times daily). Address reprint requests to Dr. Miller at He reported no prodromal symptoms, sensitivity to light or sound, neurologic symp- Brigham and Women’s Hospital, 75 Fran- toms, nausea, or vomiting and had no history of headaches. A day before presenta- cis St., Boston, MA 02115, or at almiller@ bwh . harvard . edu. tion, he noted subjective fevers. The patient was evaluated, and amoxicillin was pre- scribed for presumed acute rhinosinusitis. N Engl J Med 2018;379:475-9. DOI: 10.1056/NEJMcps1803584 Copyright © 2018 Massachusetts Medical Society. In a young, healthy patient, a viral syndrome would appear to be the most likely explanation for this patient’s clinical presentation. This history is not typical for a patient with a primary headache syndrome. Tension headaches are more com- monly bilateral. His headache is not accompanied by the characteristic features of migraine headaches, including aura, a pulsating character, and associated nausea, vomiting, or hypersensitivity to light or sound. Cluster headache, primary cough headache, and primary stabbing headache all have different presentations. Further- more, the absence of a history of headache and the duration of symptoms argue against a primary headache syndrome. Although many patients receive a diagnosis of and treatment for headaches associated with rhinosinusitis, true sinus headaches are uncommon. Many patients who receive a diagnosis of sinus headache instead have migraine headaches trig- gered by an upper respiratory infection or accompanied by nasal symptoms due to vasomotor instability. Although fever can be attributable to sinusitis, this patient has no other symptoms that suggest sinusitis. Most often, the combination of fever and headache is due to a systemic infectious process, but nonvascular intracranial disorders (e.g., central nervous system [CNS] lymphoma) and cervical or cranial vascular disorders (e.g., subarachnoid hemorrhage) can also cause these symptoms. I would favor further evaluation before initiating empiric antibiotic therapy. The patient’s headache and fever persisted. Two days after presentation, he noted new neck stiffness and myalgias. He presented to the emergency department for further evaluation, reporting malaise, subjective weakness, and decreased appetite. He had no upper respiratory symptoms, rash, arthralgias, or nausea. The development of neck stiffness suggests the possibility of meningitis. Although the classic presentation in patients with meningitis includes fever, neck stiffness, and altered mental status, fewer than two thirds of patients with meningitis present with all three symptoms. Given this patient’s subacute course, it is unlikely n engl j med 379;5 nejm.org August 2, 2018 475 The New England Journal of Medicine Downloaded from nejm.org by LUIS ERNESTO GONZALEZ SANCHEZ on August 8, 2018. For personal use only. No other uses without permission. Copyright © 2018 Massachusetts Medical Society. All rights reserved. The new england journal of medicine that he has acute purulent meningitis (from in- urine) that has contaminated water or soil. This fection with Neisseria meningitidis or Streptococcus condition has two clinical phases: an initial phase pneumoniae), but viral, zoonotic, fungal, or tuber- of fever, rigors, myalgias, and headache and a culous infection is possible. A careful history of severe second phase that can include meningitis, exposures should be obtained. Aseptic meningi- kidney failure, or liver failure. Finally, relapsing tis caused by medications, autoimmune disease, fever is associated with Borrelia turicatae infection, or cancer should also be considered. which is also transmitted by a tick bite and leads to the sudden onset of high fever followed by The patient’s medical history was unremarkable: recurrent febrile episodes with myalgias, chills, he had no allergies and took no medications regu- and abdominal discomfort. Additional infections larly. He was a graduate student. He reported no endemic to Texas or the northeastern United recent sexual activity but indicated that he had States, including the arboviruses, Lyme disease, used condoms with female sexual partners in the anaplasmosis, and ehrlichiosis, are less likely to past. He drank alcohol socially, smoked two ciga- be acquired during the winter months. rettes a month, and smoked marijuana occasion- ally. There was no family history of cancer. He had On physical examination, the temperature was lived in Chicago, Maryland, and Massachusetts. 39.4°C, the heart rate 74 beats per minute, the His only recent travel had been to Texas, where he blood pressure 118/65 mm Hg, the respiratory rate had spent 4 days vacationing on a cattle ranch; his 16 breaths per minute, and the oxygen saturation headache had begun on the last day he spent at the 98% while the patient was breathing ambient air. ranch. He had no recent sick contacts, including He appeared comfortable, with anicteric sclerae, his fellow travelers. moist mucous membranes, and a clear orophar- ynx. The neck was supple, with a full range of The patient’s travel history raises the possibility motion and no lymphadenopathy. Both lungs were of an exposure to a source endemic to Texas or clear on auscultation. Cardiovascular examination associated with cattle, although processes unre- revealed a regular rate and rhythm with normal lated to his recent trip remain possible. Rocky heart sounds. The abdomen was soft, without hep- Mountain spotted fever, caused by infection with atomegaly or splenomegaly. The arms and legs Rickettsia rickettsii, which is transmitted by a tick were warm and well perfused, with no edema. bite, occurs throughout the United States and in There were no rashes or skin lesions. The cranial warm states can be transmitted during the winter nerves, motor strength, sensation, and gait were months. Symptoms are generally nonspecific and normal. Neither Kernig’s sign (the inability to include fever, headache, malaise, myalgias, and extend the knee with the hip at 90-degree flexion) rash. Additional sources of rickettsial infection nor Brudzinski’s sign (spontaneous flexion of the found in Texas, including R. typhi (the cause of hip with passive flexion of the neck) was present. murine typhus), R. prowazekii, and R. parkeri, can result in symptoms similar to those of Rocky The patient has a temperature–pulse dissociation. Mountain spotted fever (although patients with This condition was first described in patients R. parkeri infection often present with an eschar). with yellow fever and is often associated with Q fever (caused by Coxiella burnetii) is spread conditions caused by infection with obligate in- through contact with animal excretions and re- tracellular organisms, including Rocky Mountain sults in a flulike illness that includes high fevers, spotted fever, murine typhus, Q fever, leptospi- fatigue, and headache, but the incubation period rosis, and Salmonella enterica serotype Typhi. Tem- (approximately 3 weeks) is not consistent with perature–pulse dissociation may also be associ- this patient’s history. Brucellosis, transmitted by ated with CNS lesions, lymphoma, fevers related fluids from livestock, including cattle, can lead to medications, and beta-blockade. The absence to a systemic infection that is associated with of a rash does not rule out a rickettsial infection, fever, night sweats, and headache or to a local- since rash is frequently absent on initial presen- ized infection focused in nearly any organ system. tation. The absence of lymphadenopathy, hepato- The disease has an incubation period of 1 to megaly, or splenomegaly argues against lym- 4 weeks, a timeline that is also inconsistent with phoma or Epstein–Barr virus (EBV) infection. this patient’s history. Leptospirosis is spread Both Kernig’s sign and Brudzinski’s sign lack the through exposure to animal urine (including cow sensitivity for the diagnosis of meningitis. 476 n engl j med 379;5 nejm.org August 2, 2018 The New England Journal of Medicine Downloaded from nejm.org by LUIS ERNESTO GONZALEZ SANCHEZ on August 8, 2018. For personal use only. No other uses without permission. Copyright © 2018 Massachusetts Medical Society. All rights reserved. Clinical Problem-Solving The white-cell count was 5100 per cubic milli- Fever, headache, and rash constitute the classic meter (67% neutrophils, 17% lymphocytes, 7% triad found in both Rocky Mountain spotted monocytes, and 7% bands), the hemoglobin level fever and murine typhus. In the medical litera- 12.9 g per deciliter, and the platelet count 132,000 ture, the rash in murine typhus is described as per cubic millimeter. The mean corpuscular vol- beginning on

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