• Users Online: 548
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 4  |  Page : 396-399

Bilateral demyelinating optic neuropathy with retinal pigment epithelial mottling and concurrent central serous chorioretinopathy due to the domino effect of severe Vitamin B12 deficiency


1 Medical Officer, Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India
2 Medical Officer, Department of Glaucoma and Research, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India
3 Chief Medical Officer, Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India
4 Head of the Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India
5 Medical Officer, Department of Vitreo-Retinal Services, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India
6 Optometrist, Mahathma Eye Hospital Private Limited, Tiruchirappalli, Tamil Nadu, India

Date of Submission01-Apr-2021
Date of Acceptance17-Sep-2021
Date of Web Publication21-Dec-2021

Correspondence Address:
Dr. Prasanna Venkatesh Ramesh
Department of Glaucoma and Research, Mahathma Eye Hospital Private Limited, No 6, Tennur, Seshapuram, Tiruchirappalli - 620 017, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_33_21

Rights and Permissions
  Abstract 


A 35-year-old female presented with severe headache and left-sided hemifacial spasm to the ophthalmic outpatient department. Ocular examination revealed normal visual acuity and color vision, with a presumably normal posterior pole in OU, except for parafoveal central serous chorioretinopathy (CSCR) in OD. Infrared imaging of both fundi revealed generalized retinal pigment epithelial (RPE) mottling, despite the color fundus appearing apparently normal in OU. Complete history taking, systemic workup, and hematological workup was performed and was normal, except for severe Vitamin B12 deficiency. Visual evoked potential was performed and showed delayed latency in OU, diagnosing subclinical demyelinating optic neuropathy. The patient was then started on methylcobalamin 1500 mcg and was reviewed. Signs resolved completely at 1 month follow-up. RPE disturbance (due to severe Vitamin B12 deficiency) may be a possible indirect contributor for CSCR. Hence, Vitamin B12 deficiency manifesting as bilateral optic neuropathy with extensive RPE mottling should be closely monitored for the evolution of CSCR.

Keywords: CSCR, Optic Neuropathy, RPE Mottling, Vitamin B12 Deficiency


How to cite this article:
Ramesh SV, Ramesh PV, Rajasekaran R, Ramesh MK, Balamurugan A, Ray P, Aji K. Bilateral demyelinating optic neuropathy with retinal pigment epithelial mottling and concurrent central serous chorioretinopathy due to the domino effect of severe Vitamin B12 deficiency. TNOA J Ophthalmic Sci Res 2021;59:396-9

How to cite this URL:
Ramesh SV, Ramesh PV, Rajasekaran R, Ramesh MK, Balamurugan A, Ray P, Aji K. Bilateral demyelinating optic neuropathy with retinal pigment epithelial mottling and concurrent central serous chorioretinopathy due to the domino effect of severe Vitamin B12 deficiency. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2022 Sep 29];59:396-9. Available from: https://www.tnoajosr.com/text.asp?2021/59/4/396/333168



A 35-year-old vegetarian female presented with severe headache and left-sided hemifacial spasm to the ophthalmic outpatient department. Her best-corrected visual acuity was 20/20 in both eyes (OU). Fundus evaluation with TrueColor Confocal scanner (Eidon, iCare, Finland) revealed central serous chorioretinopathy (CSCR) in OD not involving the fovea [Figure 1]a, in an otherwise apparently normal posterior pole in OU [Figure 1]a and [Figure 1]b. But on infrared (IR) imaging with swept-source optical coherence tomography (SS-OCT) with enhanced depth imaging (EDI) (Spectralis, Heidelberg Engineering, Germany), generalized pigment mottling of the posterior pole was revealed in OU [Figure 1]c and [Figure 1]d, despite the color fundus appearing apparently normal in OU. Color vision testing using Ishihara plates appeared normal. Visual fields showed areas of generalized depression [Figure 2]. Complete history taking, systemic, and hematological evaluation were normal, except for Vitamin B12 deficiency (serum level <50 ng/L). History of mental stress, usage of steroids, and insomnia was ruled out after physician and psychiatric evaluation. The patient has a balanced vegetarian diet intake, with no obvious signs of loss of appetite, malnourishment, and gastrointestinal abnormalities, being ruled out after nutritionist and gastroenterologist consultation. Hypercortisolism, Type A personality, abdominal pain from Helicobacter pylori, pregnancy, 3,4Methylenedioxymethamphetamine usage, and overnight polysomnography to rule out obstructive sleep apnea, were the systemic evaluations performed.[1] Hematological evaluations included a complete blood count and a serum Vitamin B12 level. Since elevated levels of serum homocysteine and methylmalonic acid are highly sensitive markers for Vitamin B12 deficiency, both the parameters were also tested and confirmed the severe Vitamin B12 deficiency.[2]
Figure 1: (a) Central serous chorioretinopathy of OD sparing the fovea (red arrows). (b) Normal fundus photograph of OS. (c and d) Infrared reflectance image showing hyper reflective retinal pigment epithelium mottling in OD and OS respectively. (e and f) Autofluorescence image of OD and OS respectively

Click here to view
Figure 2: (a and b) Visual fields of OD and OS showing nonspecific diffuse depression

Click here to view


Neurological evaluation revealed normal magnetic resonance imaging (MRI) with the absence of plaques, ruling out multiple sclerosis [Figure 3]c and [Figure 3]d. An MRI is helpful, but not mandatory to make the clinical diagnosis of demyelinating optic neuropathy. The main indication of MRI is derived from its prognostic value for the development of multiple sclerosis. However, in clinical practice, visual evoked potential (VEP) testing is typically very useful for confirming the diagnosis of very mild demyelinating optic neuritis, which may not be picked up occasionally on MRI. When mild optic neuritis or subclinical optic nerve damage is suspected, VEP testing can be useful in capturing the effects of a prior demyelinating injury.[3] VEP showed prolonged latency with a P100 wave of 110 ms in OD and 103.8 ms in OS diagnosing subclinical demyelinating optic neuropathy in OU (OD greater than OS) [Figure 3]a and [Figure 3]b.
Figure 3: (a and b) Visual evoked potential response showing prolonged latency of P100 wave in OD and OS respectively (red circles). (c and d) Axial segments of MRI brain revealing normal features and absence of multiple sclerosis plaques

Click here to view


Methylcobalamin 1500 mcg strips were started thrice weekly for 3 months along with folic acid 1 mg. After 2 weeks, the signs of CSCR started resolving with complete resolution at 1 month follow-up. The CSCR resolved [Figure 4] and [Figure 5] with systemic resolution of the headache and hemifacial spasm. The possibility of severe Vitamin B12 deficiency especially in vegans and vegetarians should be considered.[4],[5] Although no obvious pigmentary changes due to Vitamin B12 deficiency were elicited on color fundus photograph, IR imaging of OU [Figure 1]c and [Figure 4]d and autofluorescence imaging of OU [Figure 1]e and [Figure 1]f revealed hyperreflective mottling of the posterior pole suggestive of RPE disruption, which jeopardized its barrier function, which in turn caused the CSCR.[6],[7]
Figure 4: (a) Central serous chorioretinopathy of OD sparing the fovea (red arrows) before treatment. (b) Fundus photograph of the same in zoomed view. (c) Resolving central serous chorioretinopathy of OD after treatment (2 week follow-up). (d) Fundus photograph of the same in zoomed view

Click here to view
Figure 5: (a) Swept-source optical coherence tomography (SS-OCT) with enhanced depth imaging (EDI) (Spectralis, Heidelberg Engineering, Germany) of OD showing pigmentary epithelial detachment with central serous chorioretinopathy before treatment. (b) Resolving central serous chorioretinopathy with pigmentary epithelial detachment (2 week follow-up). (c) Resolved central serous chorioretinopathy with minimal reduction in the size of pigmentary epithelial detachment (1 month follow-up)

Click here to view


Although one can argue that the CSCR was an incidental finding which could have resolved naturally; only way to test that hypothesis would be, to not treat the patient with Vitamin B12 therapy, and see if it was spontaneously resolving. But that would be ethically wrong. Moreover, points against idiopathic acute CSCR in this patient were that; there were no likely trigger factors, which were ruled out in the first place with thorough history taking and systemic evaluation. Furthermore, pachychoroid was the first differential diagnosis that was ruled out of the equation after performing Spectralis SS-OCT with EDI, when the patient presented with this clinical picture.


  Conclusion Top


To our knowledge, there are no reported data on severe Vitamin B12 deficiency manifesting as CSCR. RPE disturbance (due to severe Vitamin B12 deficiency) may be a possible indirect contributor for CSCR. The association in this scenario was more a domino effect rather than a direct causal relationship. Hence, Vitamin B12 deficiency manifesting as extensive generalized RPE mottling should be closely monitored with IR imaging for evolution of CSCR.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yavaş GF, Küsbeci T, Kaşikci M, Günay E, Doğan M, Unlü M, et al. Obstructive sleep apnea in patients with central serous chorioretinopathy. Curr Eye Res 2014;39:88-92.  Back to cited text no. 1
    
2.
Carmel R, Green R, Rosenblatt DS, Watkins D. Update on cobalamin, folate, and homocysteine. Hematology Am Soc Hematol Educ Program 2003;2003:62-81.  Back to cited text no. 2
    
3.
Balcer LJ. Clinical practice. Optic neuritis. N Engl J Med 2006;354:1273-80.  Back to cited text no. 3
    
4.
B12 Deficiency Causes | Dr. Schweikart. Available from: https://www.b12-vitamin.com/deficiency-causes. [Last accessed on 2020 Aug 16].  Back to cited text no. 4
    
5.
Dastur DK, Santhadevi N, Quadros EV, Gagrat BM, Wadia NH, Desai MM, et al. Interrelationships between the B-vitamins in B12-deficiency neuromyelopathy. A possible malabsorption-malnutrition syndrome. Am J Clin Nutr 1975;28:1255-70.  Back to cited text no. 5
    
6.
Nicholson B, Noble J, Forooghian F, Meyerle C. Central serous chorioretinopathy: Update on pathophysiology and treatment. Surv Ophthalmol 2013;58:103-26.  Back to cited text no. 6
    
7.
Prünte C, Flammer J. Choroidal capillary and venous congestion in central serous chorioretinopathy. Am J Ophthalmol 1996;121:26-34.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed748    
    Printed24    
    Emailed0    
    PDF Downloaded41    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]