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Two children present with diplopia and pain
MRIs and lumbar punctures were performed on both children.
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Patient 1
A 6-year-old girl (patient 1) presented to the emergency room with a 10-day history of headache and blurry vision. She complained of a bitemporal headache that woke her from sleep with associated photosensitivity, nausea and vomiting. Examination revealed no focal abnormalities. The patient was discharged without diagnosis but was scheduled for neurologic examination and an MRI. When the patient returned, she reported new symptoms of stiff neck, neck pain and diplopia.
 Shazia Ahmed |  My Hanh T. Nguyen |
On examination, patient 1’s best corrected visual acuity was 20/60 in the right eye and 20/20 in the left eye. Color vision was full. There was no afferent pupillary defect. Motility examination revealed full extraocular movements in the right eye with a significant abduction deficit in the left eye (60%).
Slit-lamp examination was normal. Dilated fundus examination revealed marked disc swelling bilaterally with dilated, tortuous veins. There were no hemorrhages or exudates (Figures 1a and 1b). Humphrey visual field showed mild enlargement of the blind spot in the right eye with a question of early nasal step in the left eye. An MRI of the head without gadolinium (the family refused gadolinium) was normal. A lumbar puncture revealed an opening pressure of 45 cm water. The cerebrospinal fluid (CSF) contained 200 total cells including 68 white blood cells (77% lymphocytes) with normal protein and normal glucose. CSF culture was negative.
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Images: Smithen LM, Strominger MB |
Patient 2
Three days later, a 9-year-old boy (patient 2) presented with a history that began 1 month before presentation. At that time, the boy had a headache and an earache for 3 or 4 days. These symptoms subsided, but the patient developed severe neck and shoulder pain that persisted until presentation 1 month later. An MRI of patient 2’s neck was negative. The patient noticed no change in vision but reported diplopia on extreme left gaze.
On examination, patient 2’s BCVA was 20/40 in the right eye and 20/30 in the left eye. Color vision was full. There was no afferent pupillary defect. Motility examination revealed full extraocular movements in the right eye; there was a mild abduction deficit in the left eye (90%).
Slit-lamp examination was normal. Dilated fundus exam revealed disc swelling with hemorrhage and cotton-wool spots bilaterally (Figures 2a and 2b). Exudate could be seen in the macula of the left eye. Humphrey visual fields revealed enlarged blind spots in both eyes with an early inferior nasal step in the right eye (Figures 3a and 3b). A lumbar puncture was performed in the emergency room; the opening pressure was not measured. The CSF cell count totaled 100 with 24 white blood cells (84% lymphocytes). The protein and glucose were normal. CSF cultures were negative. An MRI with gadolinium showed disc elevation bilaterally, increased T2 signal intensity around the optic nerves bilaterally and a partially empty sella, all signs of increased intracranial pressure (Figure 4).
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 | MRI with gadolinium (patient 2). This axial T2 image shows convexity at the back of the globe in both eyes (arrow) representing papilledema, increased T2 signal intensity around the right optic nerve (arrow head) and an empty sella (asterisk), all signs of increased intracranial pressure. |
What is your diagnosis?
Children with similar symptoms
The differential diagnosis in children with papilledema, increased intracranial pressure and a sixth nerve palsy includes anything causing a mass effect (tumor, hematoma or abscess), generalized brain swelling, increased venous pressure due to venous sinus thrombosis, decreased CSF absorption (from hydrocephalus, meningitis, subarachnoid hemorrhage or an inflammatory process) and increased CSF production (choroid plexus tumor). Because the MRIs showed no evidence of masses, brain swelling, sinus thrombosis, hydrocephalus or hemorrhage, the differential is limited to meningitis vs. an inflammatory process. The CSF findings led us to a diagnosis of meningitis. The CSF profile seen in our patients (moderately high WBC with lymphocytosis, normal protein, normal glucose, culture negative, total cell count less than 500) is typically reflective of viral meningitis, but some bacteria including Mycoplasma, Listeria, Leptospira, Borrelia burgdorferi, other spirochetes and partially treated bacterial infections can mimic an aseptic/viral meningitis profile. Both of our patients underwent diagnostic testing for Lyme disease. The serum and CSF in both patients were positive for Lyme IgM and IgG antibodies. Western blot analysis confirmed these positive results.
Discussion
Lyme disease is caused by a spirochete, Borrelia burgdorferi, transferred by the bite of Ixodes ticks. In order to pass infection, a tick must be attached for 2 to 3 days. Only 1% of tick bites in endemic regions result in Lyme disease.
Lyme disease occurs in three stages: early localized, early disseminated and late disseminated. Early localized disease is characterized by erythema migrans. Early disseminated disease consists of intermittent arthritis, cranial nerve palsies and radicular pain (neuritis). The final stage, late disseminated disease, involves prolonged arthritis, chronic encephalitis, myelitis and fibromyalgia-type symptoms.
The diagnosis of Lyme disease is made in two steps. First, an antibody titer is run (either total Lyme or Lyme IgM and IgG). Since there is a high false positive rate with Lyme antibody titer, all positive results are confirmed by Western blot.
The ocular manifestations of Lyme disease are numerous and vary with the stage of systemic disease. In early localized disease, conjunctivitis and photophobia can be seen. In early systemic disease, Lyme disease can cause cranial nerve palsies (most commonly the facial nerve) and blurry vision secondary to papilledema, optic atrophy, optic neuritis or pseudotumor. In the late stages of Lyme disease, the most common ocular manifestations are pars planitis, vitritis and a bilateral, patchy, nummular, stromal keratitis. Other late complications include episcleritis, symblepharon, iritis, intermediate uveitis, posterior uveitis, chorioretinitis, exudative retinal detachment, retinal pigment epithelial detachment, cystoid macular edema, branch artery occlusion, retinal vasculitis and cranial nerve palsies.
The treatment of Lyme disease depends on the extent of disease. After tick exposure, oral doxycycline (200 mg once) administered within 72 hours of exposure will prevent infection. With only arthritic symptoms, the treatment of choice is oral doxycycline (200 mg/day) for 30 days. If neuroborreliosis is present, treatment with IV ceftriaxone for 30 days is indicated. Our patients were treated with IV ceftriaxone with almost immediate resolution of their sixth nerve palsies and slow resolution of their papilledema.
There have been only 25 reported cases of increased intracranial pressure in children with Lyme disease. The majority of cases presented with systemic symptoms. There are only six reported cases of Lyme disease with only neurologic manifestations. Moses and colleagues reported two patients with Lyme disease presenting with headaches. As with patient 1, these patients had an elevated opening pressure on lumbar puncture and exhibited a mild lymphocytic pleocytosis on examination of the CSF. Rothermel and colleagues reported four children with only neurologic manifestations of Lyme disease — two children with optic neuritis that responded well to IV ceftriaxone and IV steroids, one patient with increased intracranial pressure (ICP) that responded well to IV ceftriaxone and one patient with increased ICP, bilateral sixth and seventh nerve palsies, and a question of optic neuritis. This last patient was left with permanent bilateral blindness after treatment with IV ceftriaxone and IV steroids. This is the only reported case of blindness resulting from Lyme disease.
The pathophysiology of ICP in Lyme disease is unclear, but two theories exist. The first states that increased ICP is the result of an immune-mediated process similar to the increased ICP seem in systemic lupus erythematosus. The alternate theory is that increased ICP in Lyme is due to direct infection by the spirochete and low-grade inflammation causing disequilibrium in the production and absorption of CSF.
Our patients represent two cases of Lyme disease with only neurologic manifestations. In both cases, diagnosis was delayed due to the absence of systemic symptoms such as rash or arthritis. It is important to remember that Lyme disease can present variably, including ocular involvement.
For more information:
- Lindsay M. Smithen, MD, and Mitchell B. Strominger, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Shazia Ahmed, MD, and My Hanh T. Nguyen, MD. Drs. Ahmed and Nguyen can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Ahmed and Nguyen have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Eppes SC, Nelson DK, Lewis LL, Klein JD. Characterization of Lyme meningitis and comparison with viral meningitis in children. Pediatrics. 1999;103(5 Pt 1):957-960.
- Kan L, Sood SK, Maytal J. Pseudotumor Cerebri in Lyme disease: a report and literature review. Pediatr Neurol. 1998;18(5):439-441.
- Meyerhoff J. Lyme Disease. Emedicine Web site. Available at: www.emedicine.com/med/topic1346.htm.
- Moses JM, Riesberg RS, Mansbach JM. Lyme disease presenting with persistent headache. Pediatrics. 2003;112(6 Pt 1):477-479.
- Pietrucha DM. Pediatric neurologic Lyme disease. Presented at: 14th International Scientific Conference on Lyme Disease and Other Tick-Borne Disorders; April 21-23, 2001; Hartford, Conn.
- Rothermel H, Hedges TR 3rd, Steere AC. Optic neuropathy in children with Lyme disease. Pediatrics. 2001;108(2):477-481.