Retrobulbar optic neuritis in neuroborreliosis

Medical topics with questions, information and discussion related to Lyme disease and other tick-borne diseases.
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Retrobulbar optic neuritis in neuroborreliosis

Post by RitaA » Thu 28 May 2015 20:34

Note: EUNOS = European Neuro-ophthalmology Society ... 510_MH.pdf
12th EUNOS Congress


53rd ISCEV Symposium and 12th EUNOS Congress joint day

Wednesday, 24 June 2015

(The very last entry is):

PE122 Retrobulbar optic neuritis in neuroborreliosis: a case report
Monika Sarnat-Kucharczyk, Dorota Pojda-Wilczek, Ewa Mrukwa-Kominek (POLAND) ... stello.pdf
Title: Inflammatory Optic Neuropathies: Distinguishing Optic Neuritis from Potential Mimics

Author: Fiona Costello, MD, FRCP, Associate Professor, Departments of Clinical Neurosciences and Surgery, University of Calgary


1. To review optic neuritis as a clinical entity versus the spectrum of diagnostic considerations
2. To discuss potential “red flags” which herald an optic neuritis mimic, and review ways to avoid clinical pitfalls in misdiagnosis
3. To outline and approach to the investigation and management of different inflammatory optic neuropathies


Many potential causes of optic nerve inflammation exist. Idiopathic optic neuritis (ON) is the most common inflammatory optic neuropathy. This entity is heralded by pain, and often has a self-limited course. While ON may be a sporadic event for some patients, one in every 5 individuals affected by multiple sclerosis (MS) will present with ON as the first manifestation of their disease. Therefore, recognizing the cardinal clinical features of ON is important, because the diagnosis may have potential long-term implications. By extension, it is also crucial that clinicians be aware of the red flags that may indicate the presence of an alternative diagnosis, including age of onset greater than 45 years, absence of pain, atypical systemic symptoms and signs, and poor recovery. Alternatively, conditions mimicking ON may require a different course of management. Therefore, an in-depth understanding of inflammatory optic neuropathies is paramount, because it can help reduce the morbidity associated with these conditions.


Infectious Optic Neuropathies

Optic neuritis is rarely infectious in nature. More commonly, optic nerve involvement can occur in the clinical setting of neuroretinitis, which is characterized by vision loss, optic disc swelling, and exudative maculopathy, commonly referred to as a ‘‘macular star’’.

Despite the ever growing list of infectious, neoplastic, and inflammatory conditions linked with neuroretinitis, approximately 50% of cases are idiopathic. A complete workup including cranial imaging, lumbar puncture, and serologic evaluation in patients presenting with acute neuroretinitis is often necessary because treatments are directed toward the underlying pathogen. The investigations involve testing for potential sources of infection, including syphilis, Lyme disease, histoplasmosis, brucellosis, chlamydia, HIV, West Nile virus, toxoplasmosis, Epstein-Barr virus, viral hepatitis B and C, and tuberculosis.


Lyme Optic Neuropathy

Lyme disease is caused by the spirochete Borrelia burgdorferi, which is a tick-borne pathogen. This condition is often characterized by a classic rash called erythema chronicum migrans. CNS involvement may occur days to weeks after the initial infection. Patients with Lyme disease may manifest inflammation of the ocular anterior segment, exudative retinal detachment, papilledema, and cranial neuropathies. Papilledema caused by raised intracranial pressure in Lyme meningitis occurs more frequently in children, although some adult cases have been reported. Occasionally, cases of retrobulbar optic neuritis, papillitis, neuroretinitis, and ischemic optic neuropathy have been associated with Lyme neuroborreliosis. In cases of Lyme associated optic neuritis, vision loss is typically acute, and pain may occur at initial presentation. Visual acuity deficits range from mild to severe in cases of optic neuritis, whereas, for patients with perineuritis, visual acuity is generally well preserved. Optic disc edema can be observed in in cases of papillitis and neuroretinitis. The MRI findings in Lyme disease are often not specific and may include meningeal involvement and cranial nerve enhancement. The CSF may show a lymphocytic pleocytosis with or without protein elevation. The standard screening test is an ELISA, whereas the Western blot assay is used to distinguish false-positive ELISA results from true infection. In cases of optic neuritis associated with CSF lymphocytic pleocytosis and cranial neuropathies, neuroborreliosis should be strongly considered, particularly in Lyme-endemic areas. Detection of intrathecal antibody against Lyme is diagnostic. Treatment for neuroborreliosis typically involves 2 g/d of IV ceftriaxone for 1 month.

Posts: 2768
Joined: Thu 1 Jul 2010 8:33

Re: Retrobulbar optic neuritis in neuroborreliosis

Post by RitaA » Thu 28 May 2015 20:39

Just to put this in context, retrobulbar optic neuritis was (and probably still is) considered a rare finding in neuroborreliosis:
Curr Opin Ophthalmol. 2012 Nov;23(6):485-490.

Optic nerve involvement in Lyme disease.

Träisk F, Lindquist L.


a Department of Clinical Neuroscience and Clinic of Neuro-Ophthalmology, Karolinska Institute, St Erik Eye Hospital, Stockholm b Department of Medicine and Clinic for Infectious Diseases, Karolinska Institute, Karolinska University Hospital, Huddinge, Sweden.



The tick-borne spirochete Borrelia burgdorferi sensu lato can cause several neural manifestations from the peripheral and central neural system. There are several case reports in the literature of optic nerve involvement in association with Lyme neuroborreliosis, but clinical guidelines as to when Lyme disease should be considered in optic neuropathy is lacking.


Papilledema caused by raised intracranial pressure in Lyme meningitis seems mainly to affect children, although some adult cases have been reported. Very few cases of retrobulbar optic neuritis, papillitis, neuroretinitis and ischemic optic neuropathy have shown evidence of a strong association with Lyme neuroborreliosis.


Optic neuropathy in Lyme neuroborreliosis is rare. The cases reported in the literature are not sufficient for making a list of clinical 'red flags'. However, in adult cases, special attention seems reasonable in patients with painless visual loss, bilateral optic nerve head swelling with or without an elevated cerebrospinal fluid opening pressure. In endemic areas, any optic neuropathy may still be considered for a Lyme neuroborreliosis work-up. The use of accepted criteria for establishing the diagnosis of Lyme neuroborreliosis is emphasised.

PMID: 23047166 [PubMed - as supplied by publisher]

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