Clinical Reasoning
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RESIDENT & FELLOW SECTION Clinical Reasoning: Section Editor A 20-year-old man with headache and John J. Millichap, MD double vision Khaled Moussawi, MD, SECTION 1 resolved with closing one eye. He denied neck stiff- PhD A 20-year-old man presented to the emergency ness, focal weakness, numbness, or other neurologic Anoopum Gupta, MD, department with 1 week of headaches and double symptoms. He denied recent rashes, infections, or 8 PhD vision following 2 days of fever (102 F), nausea, tick bites. He lives on a farm in central Brazil. He Haatem Reda, MD and vomiting. His headache was progressively wors- arrived in Massachusetts 2 months before his presen- ening, throbbing behind his right eye, nonpositional, tation to visit family members. He had no significant and associated with photophobia, blurry vision, and medical history and took no medications. He did not Correspondence to pain with eye movement. Occasionally, it was severe smoke or use drugs. Dr. Moussawi: enough to wake him up from sleep. Horizontal [email protected] Question for consideration: double vision ensued soon after the headache. His diplopia was worse looking at a distance, improved 1. What is the localization of his presenting on leftward gaze, worsened on rightward gaze, and symptom? GO TO SECTION 2 Supplemental data at Neurology.org From Harvard Medical School (K.M., A.G., H.R.), Boston; Department of Neurology (K.M., A.G., H.R.), Massachusetts General Hospital, Boston; and Department of Neurology (K.M., A.G.), Brigham and Women’s Hospital, Boston, MA. Dr. Moussawi is currently with the National Institute on Drug Abuse; and the Department of Psychiatry, Johns Hopkins Medicine, Baltimore, MD. Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. e162 © 2016 American Academy of Neurology ª 2016 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 2 sensitivity for meningitis), nausea, and photopho- Binocular horizontal diplopia that is worse with dis- bia, one must consider and rule out meningitis tance and on rightward gaze is most suggestive of right even in the absence of neck stiffness (28% sensitiv- abducens nerve (cranial nerve [CN] VI) palsy. How- ity for meningitis) or altered mental status (67% ever, without further information, the differential diag- sensitivity).e1 nosis remains broad and encompasses pathologies On examination, his vital signs and mental status anywhere between the eye itself and the brainstem. were normal. His neck was supple. No skin rashes These include but are not limited to eye muscle disease were seen. His pupils were normal and visual acuity (myositis), orbital lesions (tumors, infections), thyroid was 20/20 in each eye. External examination of the disease, neuromuscular junction disorders (myasthenia eyes and orbits was normal. The examination of the gravis, botulism), orbital apex infiltrating pathology extraocular movements showed a right eye abduction (tumors, inflammation), cavernous sinus disease (infec- deficit without nystagmus. His visual fields were full. tion, inflammation, thrombosis), clival tumor (chordo- Fundoscopic examination revealed bilateral optic ma), cerebellopontine angle tumor, ischemic nerve nerve head swelling. Otherwise, his neurologic exam- injury, brainstem disease (glioma, demyelination, ination was unremarkable. ischemia), leptomeningeal disease, infectious cranial Question for consideration: neuropathy (Lyme, tuberculosis, viral), and increased intracranial pressure (ICP). 1. How would the examination narrow your locali- Headaches have poor localizing value in general. zation? What would you do next to evaluate the However, when associated with fever (85% patient? GO TO SECTION 3 Neurology 87 October 11, 2016 e163 ª 2016 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 3 immunoglobulin G (IgG) index (0.54), and no xantho- His overall clinical presentation of progressive head- chromia. The headache improved immediately after the aches, diplopia, bilateral papilledema, and right CN LP. Complete blood count, comprehensive metabolic VI palsy is most consistent with elevated ICP. With panel, erythrocyte sedimentation rate, and C-reactive a prodrome of fever, nausea, and vomiting, CNS protein were normal. MRI of the brain with contrast infection needs to be ruled out urgently. demonstrated optic nerve sheath expansion and flatten- Noncontrast head CT scan was normal. This was ing of the posterior aspects of the globes. No abnormal followed promptly by a lumbar puncture (LP). The meningeal or parenchymal enhancement was appreci- opening pressure was 40 cm H2O. CSF analysis re- ated (figure e-1 at Neurology.org). vealed normal protein (37 mg/dL), normal glucose Question for consideration: (50 mg/dL), elevated nucleated cells (14/mm3, 83% lymphocytes), few red blood cells (127/mm3), normal 1. How would you manage the patient next? GO TO SECTION 4 e164 Neurology 87 October 11, 2016 ª 2016 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 4 in assessing damage to the optic nerves from elevated He was started empirically on IV acyclovir until viral ICP,1 which helps guide further clinical management. meningitis could be ruled out. For his symptomatic acute For example, if the blind spot were to enlarge and the intracranial hypertension, he was started on acetazol- visual fields become restricted, one ought to consider amidee2 and serial large-volume LPs were performed. A more invasive interventions such as optic nerve sheath second LP on day 2 was done after recurrence of his fenestration and/or CSF shunting to avoid permanent 2 headache. Opening pressure was again 40 cm H2O, pro- optic nerve damage and vision loss. tein and glucose remained normal, while the nucleated However, the most important intervention in sim- cell count increased to 26/mm3 (94% lymphocytes). ilar cases remains to identify and treat the primary eti- Both his headache and right eye abduction improved ology of acute intracranial hypertension. after the second LP. Similar improvement was noted Question for consideration: after a third LP on day 4 (opening pressure 26 cm H2O). A neuro-ophthalmologic evaluation showed nor- 1. What is the differential diagnosis for his clinical mal visual fields. Visual field testing is very sensitive presentation? GO TO SECTION 5 Neurology 87 October 11, 2016 e165 ª 2016 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 5 signs such as neck stiffness and altered mental status. This patient had a constellation of transient fever, The CN VI palsy was most likely a result of elevated nausea, headache, intracranial hypertension, and ICP rather than infectious mononeuropathy since it CSF lymphocytic pleocytosis, in the absence of improved with serial LPs. clear focal findings on brain MRI. The differential Neurologic manifestations of acute Lyme disease diagnosis includes infectious meningitis, venous sinus (neuroborreliosis) occur in 12% of patients with thrombosis, carcinomatous meningitis, inflammatory Lyme disease.3 These occur 2 to 18 weeks after infec- granulomatous meningitis, low-grade glioma, lym- tion and can be the only manifestation of the disease. phoma, and idiopathic intracranial hypertension. The most common presentation of neuroborreliosis Our suspicion was highest for infectious meningi- in the United States is facial palsy (9% of patients), tis. The infectious differential was especially broad followed by radiculoneuropathy (4%) and meningitis given his history of exposure on a farm in Brazil, (1%).e5 but we were most concerned about certain bacterial Diagnosis of neuroborreliosis requires a 2-tier infections (syphilis, tuberculosis, brucellosis, myco- serologic test and a CSF antibody index to demon- plasma, Lyme, leptospirosis), fungi (cryptococcosis, strate intrathecal production of Lyme antibody. The coccidiomycosis), parasites (cysticercosis, schistoso- CDC recommends a screening test (usually ELISA). miasis), and viruses (herpes simplex virus, HIV, When positive, it is followed by an immunoblot for enterovirus, Eastern equine encephalitis, West Nile confirmation ($5 bands for IgG and $2 bands for virus). IgM).e6 While venous sinus thrombosis can be associated Our patient’s blood serology results meet the with lymphocytic pleocytosis,e3 fever is not usually CDC criteria for acute Lyme infection. Seroconver- a presenting symptom. Magnetic resonance venogra- sion from IgM to IgG can take up to 2 months,4 phy was performed and was negative. The lack of which could explain the positive IgM and negative contrast enhancement on MRI is atypical for neuro- IgG on repeat testing 1 month after initial presenta- sarcoidosis or carcinomatous meningitis. Serum and tion. While the definitive diagnosis of Lyme menin- CSF angiotensin-converting enzyme levels were nor- gitis requires demonstration of intrathecal production mal and there were no signs of pulmonary sarcoid on of Lyme antibodies, in early neuroborreliosis, the chest x-ray. CSF flow cytometry and cytology were CSF antibody index can be negative in 20% to also negative. Idiopathic intracranial hypertension is 30% of patients in the first 6 weeks after symptom not expected to present with fever or lymphocytic onset.5,6 pleocytosis. This is the third reported case of acute intracranial There were no HIV or treponemal antibodies hypertension from