Atypical optic neuritis – a case with a new surprise every visit

oc000138 10.3205/oc000138 urn:nbn:de:0183-oc0001382 Case Report Atypical optic neuritis – a case with a new surprise every visit Kaur Kaur Kirandeep K Dr.

Aravind Eye Hospital, Cuddalore Main Road, Thavalakuppam, Pondicherry 605007, IndiaAravind Eye Hospital, Pondicherry, India

beingkirandeep@gmail.com author Gurnani Gurnani Bharat B

Aravind Eye Hospital, Pondicherry, India

author Devy Devy Nirmala N

Aravind Eye Hospital, Chennai, India

author German Medical Science GMS Publishing House

Düsseldorf

610 neuritis demyelination tuberculosis 20200227 engl This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). 2193-1496 10 GMS Ophthalmology Cases GMS Ophthalmol Cases 11 Demyelination is the most common cause of optic neuritis. Typical optic neuritis needs intravenous steroids followed by tapering dose oral steroids. Atypical optic neuritis entails clinical manifestations that deviate from the classic pattern of features. Atypical ON can have devastating visual results if not treated in a timely fashion. Thus, it is critical that cases of atypical optic neuritis are recognized early to initiate proper treatment and preserve vision. Our case presented as an atypical case of optic neuritis. The patient had painless onset and was not responding to steroids, but presentation in the other eye and systemic administration of antivirals helped in management of this case. IntroductionOptic neuritis refers to inflammation of the optic nerve . As per western literature, Optic neuritis occurs in about 50% of patients with multiple sclerosis and it is the presenting feature in 20% of cases . The incidence of acute optic neuritis in general population has been reported as 1/100,000 . A female-to-male ratio of 5:1 is reported . The young age group (20–40 years) is most commonly affected . Demyelination has been reported as the most common cause . There is no need of laboratory investigations, i.e. ESR or ANA. The need for MRI to assess multiple sclerosis risk has been highlighted. Case descriptionHistoryA 56-year-old male patient, driver by occupation, came to our hospital for a medical fitness certificate. He reported left eye redness, which he had suffered from for 6 m</PlainText></TextGroup>onths on and off, with no associated complaints of irritation, pain, or photophobia. Uncorrected visual acuity (UCVA) in the right eye was 6/9 and 6/36 in the left eye, with a best corrected visual acuity (BCVA) of 6/6 in the right eye and 6/9 in the left eye.</Pgraph><SubHeadline>Examination</SubHeadline><Pgraph>Anterior segment examination of the right eye was normal with brisk reacting pupil and sluggish consensual reaction. The examination of the left eye was normal, except for a grade 1 relative afferent pupillary defect (G1 RAPD). Posterior segment examination was normal in the right eye, but examination of the left eye showed blurred disc margins (Figure 1 <ImgLink imgNo="1" imgType="figure"/>) with choroidal folds.</Pgraph><SubHeadline>Ancillary testing</SubHeadline><Pgraph>To identify the causative factor, we advised the patient to undergo color vision, blood investigations, chest X-ray, Mantoux test, and MRI of the brain, all of which turned out to be normal. Disc leakage was seen on fluorescein angiography (FFA) (Figure 2 <ImgLink imgNo="2" imgType="figure"/>), and Humphrey field analyzer (HFA) 30-2 showed superior arcuate scotoma (Figure 3 <ImgLink imgNo="3" imgType="figure"/>). Visual evoked potential (VEP) showed reduced amplitude and prolonged P100 latency.</Pgraph><SubHeadline>Treatment</SubHeadline><Pgraph>The final diagnosis of atypical optic neuritis was made, and pulse therapy with intravenous methylprednisolone 1 g/day for 3 days was started, followed by oral steroids and vitamin supplements. On subsequent visits, we stopped steroids as per Optic Neuritis Treatment Trial (ONTT), though the left eye disc margins blurring persisted. Repeat HFA 30-2 was not reliable.</Pgraph><SubHeadline>Review visit</SubHeadline><Pgraph>One month later, the patient presented with chief complaints of painful decreased vision in the right eye associated with photophobia and redness. BCVA in the right eye was noted to be 6/18 and 6/6 in the left eye. Anterior segment examination in the right eye revealed circumcorneal congestion, subepithelial keratitis with 0.5+ cells in the anterior chamber (Figure 4 <ImgLink imgNo="4" imgType="figure"/>). In the left eye, there was persistence of G1 RAPD with blurred disc margins. A diagnosis of right-eye viral keratouveitis and left-eye atypical optic neuritis was made. The patient was started on oral tablets acyclovir 800 mg twice a day for 2 weeks with gancyclovir eye ointment, prednisolone with moxifloxacin eye drops for topical application. Two weeks later, the patient presented with BCVA of 6/6 in both eyes. and surprisingly, fundus evaluation was normal with resolved disc edema. This was a case of atypical optic neuritis with viral aetiology, which responded well to antiviral drug therapy.</Pgraph></TextBlock> <TextBlock linked="yes" name="Discussion"> <MainHeadline>Discussion</MainHeadline><Pgraph>Optic neuritis refers to inflammation of the optic nerve <TextLink reference="1"></TextLink>. Optic neuritis occurs in about 50% of patients with multiple sclerosis, and it is the presenting feature in 20<TextGroup><PlainText>% o</PlainText></TextGroup>f cases <TextLink reference="2"></TextLink>. A female-to-male ratio of 5:1 has been reported in earlier studies <TextLink reference="4"></TextLink>. The young age group (20–40 years) is the most commonly affected <TextLink reference="5"></TextLink>. Demyelination has been reported as the most common cause in western literature <TextLink reference="6"></TextLink>. There is no need of laboratory investigations, i.e. ESR or ANA. MRI has been highlighted to assess multiple sclerosis risk. Classic demyelinating optic neuritis is associated with multiple sclerosis and carries a good prognosis for visual recovery. Atypical optic neuritis has manifestations that deviate from the classic features. Atypical features include lack of pain, simultaneous or near-simultaneous onset, lack of response to or relapse upon tapering from corticosteroids, or optic nerve head or peripapillary hemorrhages <TextLink reference="7"></TextLink>. The “atypical” causes of optic neuritis include neuromyelitis optica (NMO), autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy (CRION), idiopathic recurrent neuroretinitis (NR), and optic neuropathy associated with systemic diseases. Atypical optic neuritis can have devastating visual results if not treated appropriately in a timely fashion. Thus, it is critical that cases of atypical optic neuritis are recognized early to initiate proper treatment and preserve vision as much possible <TextLink reference="8"></TextLink>. Our case presented as an atypical case of optic neuritis. The patient had painless onset and was not responding to steroids, but presentation in the other eye and systemic administration of antivirals helped in management of this case.</Pgraph></TextBlock> <TextBlock linked="yes" name="Conclusions"> <MainHeadline>Conclusions</MainHeadline><Pgraph><OrderedList><ListItem level="1" levelPosition="1" numString="1.">Optic neuritis can have varied presentation.</ListItem><ListItem level="1" levelPosition="2" numString="2.">The clinical presentation and response to treatment outweighed the investigations in this patient.</ListItem></OrderedList></Pgraph></TextBlock> <TextBlock linked="yes" name="Notes"> <MainHeadline>Notes</MainHeadline><SubHeadline>Competing interests</SubHeadline><Pgraph>The authors declare that they have no competing interests.</Pgraph><SubHeadline>Declaration of patient consent</SubHeadline><Pgraph>The authors certify that they have obtained appropriate consent from the patient. In the consent form, the patient has given his/her consent for his/her images and other clinical information to be published in the journal. </Pgraph><SubHeadline>Study location</SubHeadline><Pgraph>The work was carried out at Aravind Eye Hospital and Post-Graduate Institute of Ophthalmology, Thavalakuppam, Cuddalore Main Road, Pondicherry 605007, India.</Pgraph></TextBlock> <References linked="yes"> <Reference refNo="1"> <RefAuthor>Hoorbakht H</RefAuthor> <RefAuthor>Bagherkashi F</RefAuthor> <RefTitle>Optic neuritis, its differential diagnosis and management</RefTitle> <RefYear>2012</RefYear> <RefJournal>Open Ophthalmol J</RefJournal> <RefPage>65-72</RefPage> <RefTotal>Hoorbakht H, Bagherkashi F. Optic neuritis, its differential diagnosis and management. Open Ophthalmol J. 2012;6:65-72. DOI: 10.2174/1874364101206010065</RefTotal> <RefLink>https://doi.org/10.2174/1874364101206010065</RefLink> </Reference> <Reference refNo="2"> <RefAuthor>Kale N</RefAuthor> <RefTitle>Optic neuritis as an early sign of multiple sclerosis</RefTitle> <RefYear>2016</RefYear> <RefJournal>Eye Brain</RefJournal> <RefPage>195-202</RefPage> <RefTotal>Kale N. Optic neuritis as an early sign of multiple sclerosis. Eye Brain. 2016;8:195-202. DOI: 10.2147/EB.S54131</RefTotal> <RefLink>https://doi.org/10.2147/EB.S54131</RefLink> </Reference> <Reference refNo="4"> <RefAuthor>Sahraian MA</RefAuthor> <RefAuthor>Moinfar Z</RefAuthor> <RefAuthor>Khorramnia S</RefAuthor> <RefAuthor>Ebrahim MM</RefAuthor> <RefTitle>Relapsing neuromyelitis optica: demographic and clinical features in Iranian patients</RefTitle> <RefYear>2010</RefYear> <RefJournal>Eur J Neurol</RefJournal> <RefPage>794-9</RefPage> <RefTotal>Sahraian MA, Moinfar Z, Khorramnia S, Ebrahim MM. Relapsing neuromyelitis optica: demographic and clinical features in Iranian patients. Eur J Neurol. 2010 Jun 1;17(6):794-9. DOI: 10.1111/j.1468-1331.2009.02928.x</RefTotal> <RefLink>https://doi.org/10.1111/j.1468-1331.2009.02928.x</RefLink> </Reference> <Reference refNo="5"> <RefAuthor>Galetović D</RefAuthor> <RefAuthor>Bojić L</RefAuthor> <RefAuthor>Karlica D</RefAuthor> <RefAuthor>Bućan K</RefAuthor> <RefAuthor>Rogošić V</RefAuthor> <RefAuthor>Znaor L</RefAuthor> <RefTitle>Optic Neuritis and Multiple Sclerosis: Our Experience</RefTitle> <RefYear>2007</RefYear> <RefJournal>Acta Clin Croat</RefJournal> <RefPage>75-8</RefPage> <RefTotal>Galetović D, Bojić L, Karlica D, Bućan K, Rogošić V, Znaor L. Optic Neuritis and Multiple Sclerosis: Our Experience. Acta Clin Croat. 2007;46(Suppl 1):75-8.</RefTotal> </Reference> <Reference refNo="6"> <RefAuthor>Voss E</RefAuthor> <RefAuthor>Raab P</RefAuthor> <RefAuthor>Trebst C</RefAuthor> <RefAuthor>Stangel M</RefAuthor> <RefTitle>Clinical approach to optic neuritis: pitfalls, red flags and differential diagnosis</RefTitle> <RefYear>2011</RefYear> <RefJournal>Ther Adv Neurol Disord</RefJournal> <RefPage>123-34</RefPage> <RefTotal>Voss E, Raab P, Trebst C, Stangel M. Clinical approach to optic neuritis: pitfalls, red flags and differential diagnosis. Ther Adv Neurol Disord. 2011 Mar;4(2):123-34. DOI: 10.1177/1756285611398702</RefTotal> <RefLink>https://doi.org/10.1177/1756285611398702</RefLink> </Reference> <Reference refNo="7"> <RefAuthor>Gaier ED</RefAuthor> <RefAuthor>Boudreault K</RefAuthor> <RefAuthor>Rizzo JF 3rd</RefAuthor> <RefAuthor>Falardeau J</RefAuthor> <RefAuthor>Cestari DM</RefAuthor> <RefTitle>Atypical Optic Neuritis</RefTitle> <RefYear>2015</RefYear> <RefJournal>Curr Neurol Neurosci Rep</RefJournal> <RefPage>76</RefPage> <RefTotal>Gaier ED, Boudreault K, Rizzo JF 3rd, Falardeau J, Cestari DM. Atypical Optic Neuritis. Curr Neurol Neurosci Rep. 2015 Dec;15(12):76. DOI: 10.1007/s11910-015-0598-1</RefTotal> <RefLink>https://doi.org/10.1007/s11910-015-0598-1</RefLink> </Reference> <Reference refNo="8"> <RefAuthor>Malik A</RefAuthor> <RefAuthor>Ahmed M</RefAuthor> <RefAuthor>Golnik K</RefAuthor> <RefTitle>Treatment options for atypical optic neuritis</RefTitle> <RefYear>2014</RefYear> <RefJournal>Indian J Ophthalmol</RefJournal> <RefPage>982–984</RefPage> <RefTotal>Malik A, Ahmed M, Golnik K. Treatment options for atypical optic neuritis. Indian J Ophthalmol. 2014 Oct; 62(10): 982–984. DOI: 10.4103/0301-4738.145986</RefTotal> <RefLink>https://doi.org/10.4103/0301-4738.145986</RefLink> </Reference> <Reference refNo="3"> <RefAuthor>Zavoreo I</RefAuthor> <RefAuthor>Jurasic MJ</RefAuthor> <RefAuthor>Lisak M</RefAuthor> <RefAuthor>Jadrijevic Tomas A</RefAuthor> <RefAuthor>Zrinscak O</RefAuthor> <RefAuthor>Basic Kes V</RefAuthor> <RefTitle>Recurrent Atypical Optic Neuritis As The Leading Sign of Fabry Disease</RefTitle> <RefYear>2019</RefYear> <RefJournal>Acta Clin Croat</RefJournal> <RefPage>550-5</RefPage> <RefTotal>Zavoreo I, Jurasic MJ, Lisak M, Jadrijevic Tomas A, Zrinscak O, Basic Kes V. Recurrent Atypical Optic Neuritis As The Leading Sign of Fabry Disease. Acta Clin Croat. 2019 Sep;58(3):550-5. DOI: 10.20471/acc.2019.58.03.22</RefTotal> <RefLink>https://doi.org/10.20471/acc.2019.58.03.22</RefLink> </Reference> </References> <Media> <Tables> <NoOfTables>0</NoOfTables> </Tables> <Figures> <Figure format="png" height="370" width="482"> <MediaNo>1</MediaNo> <MediaID>1</MediaID> <Caption><Pgraph><Mark1>Figure 1: Fundus image of left eye showing blurred disc margins with choroidal folds</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="488" width="474"> <MediaNo>2</MediaNo> <MediaID>2</MediaID> <Caption><Pgraph><Mark1>Figure 2: Fundus flourescein angiography of left eye depicting disc leakage</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="714" width="689"> <MediaNo>3</MediaNo> <MediaID>3</MediaID> <Caption><Pgraph><Mark1>Figure 3: Humphrey field analysis 30-2 showing left superior arcuate scotoma</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="595" width="808"> <MediaNo>4</MediaNo> <MediaID>4</MediaID> <Caption><Pgraph><Mark1>Figure 4: Slit-lamp photo showing circular subepithelial infiltrates in the right eye</Mark1></Pgraph></Caption> </Figure> <NoOfPictures>4</NoOfPictures> </Figures> <InlineFigures> <NoOfPictures>0</NoOfPictures> </InlineFigures> <Attachments> <NoOfAttachments>0</NoOfAttachments> </Attachments> </Media> </OrigData> </GmsArticle>