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Teenager presents with new onset diplopia, ptosis
The patient reported mild headache, upper respiratory infection symptoms and mild fatigue.
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A 17-year-old white boy with myopia presented urgently to the pediatric ophthalmology department for new onset diplopia and ptosis of the left upper lid. He had developed binocular diplopia for 4 days before presentation, as well as left upper lid ptosis, which worsened in the last 2 days. He denied any other changes in vision but did complain of pain in the right eye with eye movement.
Review of systems was positive for a mild headache, a 1-month history of upper respiratory infection symptoms and mild fatigue. He had mild paresthesias on the right side of his face. He also reported multiple erythematous ring-like lesions on his body, treated as tinea corporis with a topical antifungal. Medical history was significant for attention deficit disorder treated with Concerta (methylphenidate, Janssen Pharmaceuticals). Family history was unremarkable. He denied tobacco or alcohol use and had no known drug allergies. He lives in a heavily wooded area and has pulled ticks off his skin in the past.
Examination
Best corrected visual acuity was 20/20 in both eyes. Pupils were round and equally reactive to light with no afferent pupillary defect. IOP was normal in both eyes. Confrontational visual fields were full. There was a –2 abduction deficit in the right eye. Motility was full in the left eye. He had an esotropia in primary gaze that worsened in right gaze. Stereopsis was decreased at 400 seconds of arc. Anterior segment exam showed trace to 1+ conjunctival injection and a quiet anterior chamber. Posterior segment exam revealed trace blurred disc margins in both eyes and a flat 1-mm choroidal nevus in the mid-periphery of the left eye.
Images: Liu E, Yoo S
What is your diagnosis?
Multiple cranial nerve palsies
The differential diagnosis for a patient with multiple cranial nerve palsies includes myasthenia gravis, thyroid ophthalmopathy, multiple sclerosis, mass or infiltrate, viral meningitis and Guillain-Barré syndrome. His history of rash, headache, fatigue and upper respiratory infection symptoms, followed by multiple cranial nerve palsies, is consistent with Lyme disease, particularly given his frequent exposure to ticks. His symptoms did not fluctuate or worsen throughout the day, as would be expected in myasthenia gravis. It would be unusual for a mass or infiltrate to solely affect innervation to the levator without other third nerve involvement or causing bilateral cranial nerve palsies. In Guillain-Barré syndrome, ataxia and areflexia would likely be present, in addition to ophthalmoplegia.
Diagnosis and management
MRI of the brain and orbits with contrast showed scattered areas of white matter signal abnormality and enhancement of cranial nerves III, V and VI. A complete blood count, Lyme titers, acetylcholine receptor antibodies, thyroid panel and complete metabolic panel were ordered. All returned within normal limits except for the Lyme titers, which were positive for IgG and IgM. The patient was admitted to pediatrics and started on IV ceftriaxone. After 2 days, he had mild improvement in his ocular symptoms, his headache had resolved, and he was discharged with a PICC line to continue IV ceftriaxone. At 5 weeks, the left lid levator function and diplopia continued to improve, and the pain with eye movement resolved. At 2 months, he had minimal left upper lid ptosis and regained fusion in primary gaze. Diplopia was present only in right gaze with a small esotropia. At 6 months, his symptoms had resolved with full motility, no diplopia and no ptosis. He received a total of 5 weeks of IV ceftriaxone, extended from the 21-day course initially prescribed.
Discussion
Lyme disease is the most common vector-borne disease in the United States. Approximately 30,000 cases are reported yearly to the CDC, but the incidence continues to rise and may be underreported by as much as tenfold. Lyme disease is caused by the spirochete Borrelia burgdorferi, which is transmitted to humans by ticks of the genus Ixodes. The distribution of ticks is highly geographical and should be taken into consideration when ordering and interpreting Lyme testing. Transmission from an infected tick typically takes more than 24 hours of attachment and can be prevented by prompt detection and removal of any ticks.
Stage 1 of the disease, 1 day to 30 days after infection, is localized and manifests with a characteristic bull’s eye rash, flu-like symptoms and headache. Conjunctivitis is common. Stage 2 is early disseminated Lyme, takes place 3 weeks to 6 weeks after infection and can present with multiple erythema migrans lesions, migratory polyarthritis, and cardiac and neurologic involvement. Ophthalmoplegia, as was seen in our case, is uncommon.
The most commonly involved cranial nerve is cranial nerve VII and can be bilateral. Ocular inflammation is rare but has a highly variable presentation, including reports of keratitis, iritis, panuveitis, vitritis, retinal vasculitis, optic neuropathy and orbital myositis. Stage 3 disease commonly manifests as chronic arthritis and can have neuropsychiatric sequelae and peripheral neuropathies.
The rate of under- and overdiagnosis of Lyme disease is high, making pre-test probability an important consideration when testing for Lyme disease. Risk factors to consider include geographic location (highest in the Northeast, Atlantic Midwest and Upper Midwest) and time spent outdoors in areas of possible tick exposure. The current gold standard test is “whole cell” EIA or ELISA as the initial screen, followed by Western blot if the screening is positive or equivocal. A minimum set of bands must be reactive to be considered positive. False negatives can arise in patients who have not seroconverted, which can take 6 weeks to 8 weeks. False positives can occur in the setting of other diseases, including lupus, reactive arthritis, syphilis and a variety of viral infections. Neuroborreliosis is recommended to be confirmed with CSF studies.
MRI findings, as was seen in our case, may be nonspecific or may show enhancement that is not consistent with presenting symptoms. Serologic testing after treatment is not indicated due to persistent presence of antibodies. Newer testing with C6 ELISA may offer greater specificity, obviating the need for a two-step test; however, additional research is needed to confirm this as a reliable test.
Recurrence after treatment is unlikely although reinfection is possible and not uncommon. Antibodies do not offer future protection, and people tend to continue activities that put them at risk. Early treatment of Lyme disease offers a good prognosis for recovery. Treatment is typically with oral or intravenous antibiotics for 21 days, although symptoms may persist despite adequate treatment. Current medical literature does not support extended courses of antibiotics, although there is controversy in the general community.
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- For more information:
- Erica Liu, MD, and Sylvia Yoo, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Kristen E. Dunbar, MD, and Kendra Klein, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston MA 02111; website: www.neec.com.