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Rapoport Ch 31 10/15/08 1:14 PM Page 175

CHAPTER 31 THE WOMAN WITH ACUTE SEVERE

JOHN F. ROTHROCK, MD

Case History • What do the cerebrospinal findings suggest, and what do they rule out? A 25-year-old woman presented with a 3-day history of • How should this patient be managed? uncharacteristic, severe, and unremitting headache. The headache initially was present upon awakening, was moderate in intensity at that time, and subsequently Case Discussion worsened. The was largely nonpulsatile but became This previously healthy and headache-free woman pre- “throbbing” and more severe when she attempted to sented with a severe headache. The most common stimu- walk. The pain was nonlateralized and centered at the lus for this clinical presentation is new onset , vertex. There was no significant positional component. but that diagnosis must be considered suspect for three She described associated and some sono- reasons. First, the International Headache Society (IHS) phobia but no , , or . She described diagnostic criteria for migraine headache stipulate that eye pain with eye movement in all directions of gaze. She an individual must have experienced at least five attacks denied , neck stiffness, recent trauma, or symptoms of characteristic headache in order for that diagnosis to consistent with recent systemic illness. be made with a reasonable degree of certainty. Second, Her past medical history was unremarkable, and she and regardless of whether or not the patient has an estab- specifically denied any prior history of suffi- lished history of migraine, the “worst headache of my ciently severe to inhibit or prohibit daily activity. She life” invariably requires careful diagnostic intervention took no chronically, and she specifically and exclusion of conditions which may mimic migraine. denied recent use of an antibiotic. Lastly, the patient is febrile, and when fever accompanies Her physical examination was normal except for an headache as the primary presenting complaint, infection oral temperature of 37.8˚C. There was no evidence of involving the central must be excluded. neck pain or stiffness with anterior flexion. The most important condition to consider here is sub- She was treated with 6 mg subcutaneous- arachnoid hemorrhage from a ruptured berry aneurysm. ly, and within 30 minutes her headache declined in Clinically devastating aneurysmal rupture often is her- intensity from severe to mild. Lumbar puncture was per- alded by a low volume, “sentinel” leak, and it is impera- formed, and the opening pressure was normal; cere- tive that the correct diagnosis be made at this earlier brospinal fluid (CSF) analysis yielded 257 white blood point. The incidence of mortality or major neurologic cells per cubic mm (98% lymphocytes), no red blood morbidity from high volume aneurysmal hemorrhage is cells, protein concentration 58 mg/dL and glucose 62 distressingly high, and little satisfaction can be taken mg/dL; Gram’s stain was negative for organisms. from establishing the diagnosis after this cataclysmic event has occurred. On the other hand, if the sentinel bleed is diagnosed without delay, and if the aneurysm is Questions about This Case then secured surgically, disaster can be averted. • Given the patient’s history, what diagnoses should have Many other conditions may mimic new onset been considered prior to lumbar puncture? migraine, and a number of these are potentially danger-

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176 / Part II: Secondary and Rare Headache Disorders

ous. Patients with infectious may present with ry of recent antibiotic use exclude untreated or partially acute, severe headache, and fever and neck stiffness are treated bacterial meningitis. The CSF profile is consistent not always present. Although tuberculous meningitis typ- with viral , but the patient did not express ically is more subacute in onset, patients with bacterial or symptoms or exhibit signs referable to the itself. viral meningitis become ill quickly, and it is virtually impossible to distinguish between the two without CSF analysis. Patients with often complain Management Strategies of headache; if there is alteration of consciousness, focal Management of this patient should hinge primarily on neurologic deficit, activity, or some combination the question: is hospitalization for general support thereof, this diagnosis should be considered. [Editors’ required? If the patient is able to maintain oral hydra- note: from Borrelia burgdorferi should be tion, her head pain can be managed with oral or subcuta- considered in some parts of the world. A history of tick bite, neous medication, and there can be someone at home bull’s-eye rash, or residence in an area with a large deer who will observe her and confirm that her course is con- population is usually present.] sistent with resolving viral meningitis, then hospitaliza- There are noninfectious conditions which may mimic tion makes little sense. On the other hand, dehydration new onset migraine. Patients with primary intracerebral will aggravate and prolong the symptoms of meningitis, hemorrhage from hypertensive arteriopathy, recreational and intravenous fluids may be required to avoid this. drug abuse, or other causes frequently complain of To the author’s embarrassment, this patient was admit- headache which may possess migrainous features, but ted by his department’s service despite near total resolu- parenchymal hemorrhage is with few exceptions accom- tion of her headache following administration of suma- panied by focal neurologic signs. Patients with extra-axial , her ability to maintain oral hydration, and the (subdural or epidural) hematoma from recent head availability of concerned and informed family members at injury may present with headache as the chief complaint, home. She was placed in an isolation room, and antibac- and focal neurologic signs may be subtle or absent. terial and antiviral were administered intra- Patients with low from a dural tear venously. A brain imaging study was performed and was and associated CSF leak may present with acute normal. An electroencephalogram was requested. headache, but during the first few days to weeks that None of this makes very good sense. Medical resources headache typically has a strong positional component needlessly were utilized, and worse, the patient suffered (much worse while the individual is upright and relieved unnecessary discomfort and some risk exposure. Such by lying flat); a history of an inciting traumatic event is aggressive diagnostic and therapeutic intervention should not always elicited. be reserved for patients with presumed viral meningitis This patient’s positive response to treatment with whose presentations are more atypical (examples include sumatriptan did not assist in establishing a diagnosis. antibiotic treatment prior to lumbar puncture, atypical Sumatriptan is nonspecific and has been report- CSF profile, or of consciousness). Straight- ed to be effective in patients with postictal headache or forward viral meningitis should be managed in a straight- headaches from aneurysmal . forward fashion. The same can be said for any medication used to treat acute migraine headache. A variety of stimuli will acti- vate the peripheral trigeminovascular system and central Case Summary pathways which generate and modulate head pain, and • Patients presenting with uncharacteristically severe acute any agent which exerts its pharmacologic effect within head pain require meticulous diagnostic evaluation. those areas may work to oppose pain, regardless of the • In such circumstances, aneurysmal subarachnoid hem- stimulus involved. orrhage must be excluded. Taken along with her clinical presentation, the results • Viral meningitis is a common, temporarily disabling of this patient’s CSF analysis indicate that the correct but typically benign condition which most often can diagnosis is viral meningitis. The normal glucose level, be managed with simple supportive therapy only. lymphocytic pleocytosis, and elevated protein concentra- tion are characteristic of aseptic meningitis of viral ori- gin. There was no evidence of recent subarachnoid Overview of Aseptic Meningitis hemorrhage; no fresh red cells were present, and the pro- “Aseptic meningitis” implies of the tein concentration was not elevated to the level one meninges in the absence of bacterial or fungal infection would expect from recent crenation of a high volume of and without symptoms or signs referable to the brain, red cells. Results of the Gram’s stain and the lack of histo- brain stem, or . Afflicted patients present with Rapoport Ch 31 10/15/08 1:14 PM Page 177

The Woman with Acute Severe Headache / 177

fever, headache, signs of meningeal irritation, and char- demand that encephalitis or other conditions which acteristic CSF findings. Many have come to regard aseptic involve the brain itself be considered. meningitis and viral meningitis as being synonymous, Viral meningitis is evanescent; the patient rapidly and although it is true that viral infection is the leading becomes sick and miserable and almost as rapidly cause of this syndrome, the CSF profile associated with improves to his or her normal state of health. Long term aseptic meningitis may be produced by conditions as sequelae are very rare. The viral agents incriminated most widely varied as treponemal infection, Behçet’s disease, commonly in cases of aseptic meningitis are enteroviruses and exposure to certain medications or contrast agents (echovirus and coxsackievirus), but the mumps virus, (e.g., nonsteroidal anti-inflammatory drugs, gamma herpesviruses (notably, type 2), lymphocytic chori- globulin, iohexol). Aside from viruses, infections which omeningitis virus, and Epstein-Barr virus may be culpable may produce aseptic meningitis include leptospirosis, as well. Benign recurrent aseptic meningitis (Mollaret’s Lyme disease, syphilis, mycoplasma, and chlamydia. meningitis) may result from reactivation of a herpesvirus. Parainfectious causes include partially treated bacterial Human immunodeficiency virus may cause aseptic meningitis, parameningeal infection, and endocarditis. meningitis early in its course, and the meningitis may Among the noninfectious causes not already mentioned coincide with or predate seroconversion. are sarcoidosis, collagen vascular diseases, and migraine. The peculiar intersection of migraine and aseptic If the term is employed in its broadest sense, encompass- meningitis deserves a brief mention. Both are common ing both acute and more chronic processes, the list of clinical conditions, and it is inevitable that a certain per- causative conditions expands significantly, and there can centage of individuals with established migraine will be said to be no single characteristic clinical presentation. contract viral meningitis. Beyond the coincidental rela- For example, a patient with acute aseptic meningitis of tionship, however, there is some evidence to suggest that viral origin or from exposure to contrast material used in migraine itself may induce a meningeal response similar myelography may present rapidly and with prominent to that observed with aseptic meningitis and that in this symptoms, in contrast to a patient with human immuno- situation efforts to establish an infectious or other non- deficiency virus (HIV)-related syphilitic meningitis who infectious source for the meningitis will be fruitless. may express no symptoms whatsoever. Compounding the potential for diagnostic confusion, The CSF profile in aseptic meningitis generally demon- patients with migraine-associated aseptic meningitis strates a white cell pleocytosis which numbers in the tens may exhibit progressive obtundation or express sensori- to hundreds, and the cells are chiefly lymphocytes. The motor symptoms. Suffice it to say that the incidence and glucose concentration is normal, and the protein concen- biogenesis of “migraine meningitis” remain obscure and tration is moderately elevated. The degree of abnormality that the diagnosis of this condition should be considered present in the CSF tends to parallel the severity of the tenuous at best. patient’s symptoms. In a minority of cases, and especially when the meningeal inflammatory response is intense and lumbar puncture is performed early in the course, the Selected Readings white cell count may be higher, a greater proportion of Beghi E, Nicolosi A, Kurland LT, et al. Encephalitis and aseptic neutrophils may be present, and the glucose concentration meningitis, Olmstead County, Minnesota, 1950–1981: epi- may be decreased. In that event, bacterial or tuberculous demiology. Ann Neurol 1984;16:283–94. meningitis becomes of greater concern, and it may be safer Ratzan KR. Viral meningitis. Med Clin North Am 1985;69: to initiate appropriate antibiotic therapy and to continue 399–413. such treatment until cultures initially obtained return as negative and repeat lumbar puncture demonstrates find- ings consistent with evolving aseptic meningitis. Editorial Comments As indicated previously, most acute aseptic meningitis is Aseptic meningitis is usually a self-limiting disorder with- viral in origin, and patients presenting with viral meningi- out sequelae. However, the diagnosis is not always easy or tis may appear quite ill, exhibiting fever, headache, lethar- straightforward, and the differential diagnoses include gy, irritability, and varying degrees of neck stiffness; the several serious and life threatening disorders. Dr. Rothrock last is variable and may be absent. Photophobia may be leads us through an erudite overview and discussion of prominent, and patients often complain of pain with eye this entity. He points out the pitfalls in diagnosis and pro- movement. On rare occasions the examiner will find evi- vides sensible management strategies. He even mentions dence of early , but alteration of conscious- the entity of “migraine meningitis,” an obscure disorder, ness, focal neurologic signs, or seizure activity are typically somewhat familiar to neurologists and physicians dealing inconsistent with the diagnosis of aseptic meningitis and with acute headache.