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Show Journal of Nemv- Ophthalmologij 14( 1): 2- 5, 1994. © 1994 Raven Press, Ltd., New York Isolated Fascicular Abducens Nerve Palsy and Lyme Disease J. A. Mastrianni, M. D., Ph. D., S. L. Galetta, M. D., E. C. Raps, M. D., G. T. Liu, M. D., and N. J. Volpe, M. D. A patient with Lyme disease developed an isolated sixth nerve palsy at the end of a 1 month course of oral antibiotics. Magnetic resonance imaging disclosed high-signal abnormality at the left pontomedullary junction, implicating involvement of the distal sixth nerve fascicle. Although facial numbness ensued during a subsequent course of intravenous antibiotics, corticosteroid therapy was associated with prompt improvement of neurologic signs, suggesting an immunologic mechanism for the central nervous system dysfunction. Key Words: Lyme disease- Abducens nerve palsy- Magnetic resonance imaging. From the Departments of Neurology ( J. A. M., S. L. G., E. C. R., G. T. L., N. J. V.) and Ophthalmology ( S. L. G., G. T. L., N. J. V.) and the Neuro- Ophthalmology Unit ( S. L. G., G. T. L., N. J. V.), Hospital of the University of Pennsylvania, Scheie Eye Institute, and Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, U. S. A. Address correspondence and reprint requests to Dr. Steven L. Galetta, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, U. S. A. The cranial neuropathies associated with Lyme neuroborreliosis typically result from an infectious process involving the meninges ( 1- 3). Here we report a patient with Lyme disease who developed an isolated sixth nerve palsy from a lesion of the ventral pons as disclosed by multiplanar magnetic resonance imaging ( MRI). CASE REPORT In June 1992, a 40- year- old man from northeast Philadelphia removed a small tick from his right forearm. One day later, a small area of erythema developed at the site of the bite, which progressed over several days to form a " bull's- eye" rash. He was evaluated at a local emergency ward where the diagnosis of Lyme disease was made and a 10- day course of tetracycline was prescribed. The patient did well until 3 months later when he again pulled a tick from his arm. An erythematous patch at the site appeared, and within a few days he developed a flulike syndrome with severe frontal headaches, fever, stiff neck, and arthralgias that lasted 10 days. Because of medical insurance problems, he was seen at a local clinic 2 months later, at which time his serum Lyme titer was found to be highly positive. He was treated with oral Doxycy-cline 100 mg twice a day for 1 month. Near the end of this treatment, the patient developed mild neck stiffness and headache. He also noted diplopia in left gaze and at distance. The patient was seen in the Department of Neurology at the Hospital of the University of Pennsylvania in March 1993, where he was noted to have a left sixth nerve palsy with an otherwise normal neurologic examination ( Fig. 1). Cerebrospinal fluid ( CSF) analysis disclosed an opening pressure of 210 mmHzO; glucose, 56 mg/ dl; protein, 27 mg/ dl; negative oligo-clonal bands; negative VDRL; negative microbio- 2 NERVE PALSY AND LYME DISEASE 3 FIG. 1. External photograph demonstrating left abduction deficit that followed course of Doxycycline. logic cultures; and negative cytology. The white blood cell count was 4/ mm3 ( 99% lymphocytes). Erythrocyte sedimentation rate was 4 mm/ h; anti-nuclear antibody, 1: 80; rheumatoid factor, 1: 160, with a negative MHA- TP ( microhemagglutination test for T. pallidum). Serum and CSF Lyme titer analyses were performed by Imugen Laboratories ( Norwood, MA). Lyme values for antibody capture enzyme immunoassay were abnormal as follows: serum IgM, > 32.4; CSF IgM, 8.4; serum IgG, 1.9; CSF IgG, 21.5; CSF IgA, > 33.7 ( normal range for CSF and serum: < 1). CSF/ serum ratios were IgG, 11.3 and IgA, > 67.4 ( normal: < 1). A serum Lyme IgG immunoblot was positive, showing six abnormal bands. The DNA from Borrelia burgdorferi was not detected in the spinal fluid by polymerase chain reaction ( performed by Dr. D. Persing, Rochester, MN). Brain MRI demonstrated an isolated area of hyperintensity on long TR images at the ventral aspect of the left pontomedullary junction. The lesion did not enhance with gadolinium ( Fig. 2). The patient was treated with intravenous ceftriaxone 2 g daily for 1 month. Three weeks into this course the patient developed a fever to 102° F without meningismus or other neurologic signs. A complete blood count and blood cultures were negative. The fever resolved in 2 days. Five days later, the patient developed mild facial swelling and numbness of the right side of his face in a V2 dermatomal distribution. Repeat CSF analysis showed an opening pressure of 160 mmH20; white blood cells, 6/ mm3; glucose, 55 mg/ dl; and protein 38 mg/ dl. All serum and CSF Lyme titers remained remarkably elevated except the serum IgG level, which was negative. A repeat MRI did not demonstrate trigeminal nerve enhancement, nor were there any changes in the brain or brainstem from the previous scan. Since the patient continued to developed symptoms despite adequate treatment with antibiotics he was begun on prednisone 60 mg daily. The facial swelling and numbness improved within days and the sixth nerve function showed marked improvement at the end of the prednisone taper 4 weeks later ( Fig. 3). After corticosteroid therapy, the patient had no further Lyme- related symptoms. On follow- up 4 months later, he was asymptomatic with a 6- diopter esophoria in the primary position. A repeat brain MRI showed no change in the ventral pontine lesion. DISCUSSION Our patient developed an isolated sixth nerve palsy from a ventral pontine lesion in the setting of clinical and serologic evidence of Lyme disease, an association previously unrecognized. Several authors have described isolated cranial nerve palsies resulting from vasculopathic brainstem lesions as detected by neuroimaging ( 4- 7). Isolated fascicular sixth nerve palsies associated with infarctions of the pontine tegmentum have been documented in diabetic ( 5) and hypertensive patients ( 7). Slavin and colleagues ( 8) have recently reported a patient with luetic meningoencephalitis who had an isolated abducens palsy from an MRI- documented pontocerebellar mass ( 8). Our patient's lesion at the ventral pontomedullary junction probably interrupted the sixth nerve fascicle near its exit from the brainstem ( 9). In a recent review of the neuro- ophthalmologic complications of Lyme disease, three of six patients had sixth nerve palsies ( 10). One had a unilateral deficit associated with increased intracranial pressure and the other two had bilateral palsies without mention of their CSF opening pressure. Two patients had an unremarkable MRI and each improved with intravenous antibiotics. A third patient with MRI- documented periventricular high-signal abnormality failed antibiotic therapy, which suggested an immunologic or vasculitic mecha- I Ncuw- Ophthalmol, Vol. 14, No. 1, 1994 4 /. A. MASTRIANNI ET AL. FIG. 2. ( A): Sagittal T2- weighted ( TE = 90, TR = 2,700) image demonstrates high- signal abnormality at pontomedullary junction ( arrow). ( B): Axial section ( TE = 90, TR = 2,700) confirms high- signal abnormality at ventral aspect of pontomedullary junction ( arrow). ( C): Coronal section ( TE = 18, TR = 2,700) shows left pontomedullary high- signal abnormality ( arrow). nism of injury rather than a direct infection. The role of humoral and cellular autoimmunity in central nervous system ( CNS) Lyme borreliosis has been emphasized previously ( 11- 13). Aberer and coworkers ( 14) have demonstrated that Borrelia burgdorferi flagella protein shares antigenic properties with human tissue, including nerve cells and axons of the central nervous system. Cells secret-m \ FIG. 3. External photograph showing resolution of the abduction deficit after course of corticosteroids. / Ncuro- Ophtlialnwl, Vol. 14, No. 1, 1994 NERVE PALSY AND LYME DISEASE 5 ing autoantibodies to myelin basic protein also have been detected in the cerebrospinal fluid of patients with neuroborreliosis ( 15). However, the precise role of these autoimmune factors in the development of CNS Lyme borreliosis remains uncertain ( 15). Our patient appears to have had an immunologic process for multiple reasons: 1. His sixth nerve palsy occurred near the completion of oral Doxycycline therapy. 2. V2 involvement was noted during 1 month of intravenous ceftriaxone therapy. 3. His CSF Lyme PCR was negative. 4. He exhibited dramatic improvement of his ab-ducens palsy after corticosteroid administration. Although a delayed response to antibiotic therapy might be an alternative explanation, the continued evolution of symptoms after several weeks of both oral and intravenous antibiotics argues against a direct infectious process as a sole mechanism of injury. Our patient provides supportive evidence that brainstem lesions may result in isolated cranial neuropathies and that Lyme disease may present in unusual ways, earning its title as the next " great imitator" after syphilis. Further, an immunologic response to Borrelia infection should be considered in patients who clinically progress despite intensive antibiotic treatment. REFERENCES 1. Steere AC, Bartenhagen NH, Craft JE, et al. Clinical manifestations of Lyme disease. Zentralbl Bakteriol Mikrobiol 1986; 263: 201- 5. 2. Finkel MF. Lyme disease and its neurologic complications. Arch Neurol 1988; 5: 99- 103. 3. Winterkorn JMS. Review in medicine: Lyme disease: neurologic and ophthalmic manifestations. Surv Ophthalmol 1990; 35: 191- 204. 4. Hopf HC, Gutmann L. Diabetic 3rd nerve palsy: evidence for a mesencephalic lesion. Neurology 1990; 40: 1041- 5. 5. Fukutake T, Hirayama K. Isolated abducens nerve palsy from pontine infarction in a diabetic patient. Neurology 1992; 42: 2226. 6. Kamei T, Uchiyama F, Hukuyama J. Primary tectal mesencephalic hemorrhage with isolated trochlear nerve palsy: a case report. Clin Neurol 1987; 27: 1167- 9. 7. Kim JS, Kang JK, Lee SA, Lee MC. Isolated or predominant ocular motor nerve palsy as a manifestation of brain stem stroke. Stroke 1993; 24: 581- 5. 8. Slavin ML, Haimovic I, Patel M. Sixth nerve palsy and pontocerebellar mass due to luetic meningoencephalitis. Arch Ophthalmol 1992; 110: 322. 9. Glaser JS, Bachynski B. Intranuclear disorders of eye movement. In: Glaser JS, ed. Neuro- ophthalmology. Philadelphia: JB Lippincott; 1990: 361- 418. 10. Lesser RL, Kornmehl EW, Pachner AR, et al. Neuro-ophthalmologic manifestations of Lyme disease. Ophthalmology 1990; 97: 699- 706. 11. Pachner AR, Steere AC, Sigal LH, Johnson CJ. Antigen specific proliferation of CSF lymphocytes in Lyme disease. Neurology 1985; 35: 1642- 4. 12. Sigal LH, Tatum AH. IgM in the sera of patients with Lyme neurologic disease bind to cross- reacting neuronal and Borrelia burgdorferi antigens. Ann NY Acad Sci 1988; 539: 422- 4. 13. Martin R, Ortlauf J, Sticht- Groh V, Mertens HG. Borrelia burgdorferi as a trigger for autoimmune T- cell reactions within the central nervous system. Ann NY Acad Sci 1988; 539: 400- 1. 14. Aberer E, Brunner C, Suchanek G, et al. Molecular mimicry and Lyme borreliosis: a shared antigenic determinant between Borrelia burgdorferi and human tissue. Ann Neurol 1989; 26: 732- 7. 15. Shahid B, Olsson T, Hojeberg B, Link H. Cells secreting antibodies to myelin basic protein in cerebrospinal fluid of patients with Lyme neuroborreliosis. Neurology 1991; 41: 581- 6. / Neuw- Ophthalmol, Vol. 14, No. 1, 1994 |