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Retrobulbar optic neuritis (RON) is an unusual complication of Lyme disease. The diagnosis of early Lyme disease is difficult, and the relationship between RON and Lyme disease remains controversial. None of the 14 cases of optic neuritis described in the literature in association with Lyme disease fulfilled the Halperin and Sibony criteria for active Lyme disease. We report the first case of acute Lyme disease complicated by RON established using the Halperin and Sibony criteria.
Lyme disease is a multisystem infectious disease caused by tick borne spirochetes of the Borrelia burgdorferi group. Diagnosis of this infection can be difficult and serological testing such as western blot can be useful. Cranial neuropathies are common but RON has been reported in a few isolated cases.1 A causal link between optic neuritis and Lyme disease has not been established and remains controversial. We report a case of active neuro‐Lyme disease complicated by RON.
A 67‐year‐old man who lives in a wooded area of southwest France developed an erythema migrans 3 days after a tick bite on his right arm. He was admitted to hospital 2 weeks later with fatigue, myalgia, painful radiculopathy, facial weakness, ptosis and diplopia. Physical examination showed fever (38°C), cervical radiculoneuropathy with radicular pain and paresis in the right arm, and peripheral right facial palsy with involvement of the IIIth, Vth and VIth right cranial nerves. Two days after hospital admission, he developed retrobulbar pain that increased with eye movements, rapid blurred vision and diminished colour perception in the right eye. Ophthalmological examination showed decreased visual acuity (right eye: 5/10 and left eye: 8/10) with central scotoma in the right eye.
Eye fundus revealed bilateral symmetric intermediate uveitis without retinal vasculitis. Visual evoked potentials (VEP) revealed a delayed P100 latency to 136 ms in the right eye whereas it was normal (99 ms) after left eye stimulation. The amplitude of the right P100 wave was slightly decreased (15 μV) in comparison with the left side (20 μV) and was associated with desynchronisation (P100 duration: 125 ms on the right side vs 38.9 ms on the left side). Brain and optic nerve MRI was normal without contrast enhanced lesions. Syphilis serology was negative. Lyme ELISA IgG antibodies were elevated in serum (99 U/ml; positive serum >24 U/ml). Serum western blot against Borrelia garinii showed three IgG bands (41, 67 and 83 kDa) and one IgM band (41 kDa). Serum Lyme western blot IgG antibody titre was 2 U and Lyme IgM titre was 20 U (positive values 10 U). Ten days later, Lyme IgG titre was 12 U (positive values 10 U or titre increased at least twofold between two successive measurements) and Lyme IgM titre was 18 U. CSF analysis revealed a white cell count of 21/mm3 (normal <5/mm3) with 95% lymphocytes, a protein level of 1.11 g/l (normal <0.40 g/l), normal glucose level of 3.9 mmol/l (blood level was 5.2 mmol/l) and negative culture. CSF Lyme western blot IgG titre was 13 U (positive values 4 U). CSF analysis also demonstrated oligoclonal synthesis of IgG and intrathecal Lyme antibody production (CSF to serum Lyme index IgG of 38.4, positive index >1.2).
The patient was treated with a 2 week course of intravenous antibiotherapy (ceftriaxone) followed by intramuscular injections for 1 week, without corticotherapy. Three months after antibiotherapy initiation, the radiculoneuropathy and multiple cranial involvement had regressed completely. Visual acuity had improved to 10/10 in both eyes and ophthalmological examination was normal. VEP obtained after right eye stimulation had improved, with normalisation of P100 latency (95 ms) and amplitude (20 μV). During the same period, serum Lyme western blot IgG (6 U) and IgM (3 U) antibodies had decreased.
This is the first report of acute Lyme disease complicated by RON and confirmed by VEP. Independent of bilateral intermediate uveitis which modifies the amplitude of response on the right side, a delayed P100 latency was also observed after right eye stimulation. This finding suggests the fortuitous association of bilateral uveitis and unilateral RON. Uveitis alone could not explain why P100 latency was delayed, as previously reported in four cases of RON associated with human T lymphotropic virus type 1 uveitis.2 Our case fulfilled the criteria for acute Lyme disease3 with positive western blot according to European criteria (European Union Concerted Action on Lyme Borreliosis: EUCALB)4 and with strong evidence of a causal link between optic neuritis and Lyme disease, as described by Sibony and colleagues1 and Halperin and colleagues.5 According to Sibony's recommendations,1 strong evidence of optic neuritis associated with active Lyme disease requires the following elements: optic neuritis, endemic exposure, negative VDRL, exclusion of multiple sclerosis and a positive serum titre (ELISA or indirect fluorescent antibody), in association with at least one of the following: (1) encephalitis or meningitis with CSF pleocytosis, intrathecal antibody production or CSF PCR positive for Borrelia burgdorferi DNA and a positive serum or CSF western blot; (2) recent signs of Lyme disease, such as facial nerve palsy, arthritis or radiculoneuritis, with positive serum ELISA confirmed by western blot; and (3) recent physician diagnosed erythema migrans.
Among these cases of Lyme disease with optic neuritis, none fulfilled the Halperin5 or Sibony1 criteria because of negative Lyme ELISA or western blot in both serum and CSF. Two of these patients (patient Nos 3, 4) were diagnosed as having multiple sclerosis 12 years later. In our case, multiple sclerosis cannot be definitely excluded but Lyme disease is a criterion of exclusion for multiple sclerosis. However, CSF showed intrathecal antibody Lyme production with an elevated protein level, brain MRI was normal and the patient was in good health 2 years later. Moreover, we ruled out a diagnosis of vasculitis because there was no papillitis or retina vasculitis in the eye fundus, autoimmune laboratory tests were negative and RON had completely regressed without corticotherapy.
We report the first case of strong evidence of a causal link between RON and acute Lyme disease. Neuro‐ophthalmologic manifestations of Lyme disease remain underdiagnosed. VEP may be helpful in confirming early RON for which a relationship with Lyme disease may be established using the Halperin5 and Sibony1 criteria.
Competing interests: None.