• Tumefactive demyelinating lesions (TDL) is an acute, inflammatory presentation of large acute demyelinating lesions (>2 cm) that can be associated with mass effect17.

• TDLs are rare but can mimic brain neoplasms or abscess because of their associated edema within the cerebral hemisphere18.

• TDLs are also known as tumefactive multiple sclerosis lesions, tumor-like demyelinating lesions, demyelinating pseudotumor18.

• They primarily affect females, ages 2 through 70 with the average age being 37.

• Tumefactive demyelination is a rare neurological disorder that can present with multiple symptoms: cognitive, sensory, motor, visual and cerebellar.

• Patients with visual symptoms may initially present with sub- acute or acute vision loss but may not have any associated neurological symptoms at the time.

• This case will discuss the clinical quandaries of suspecting demyelinating changes.

BackgroundVision loss, visual field loss, decreased color vision

and pallor of optic nerves warrants an urgent

work-up, both ophthalmic as well as systemic.

• DDX: optic neuritis/ optic neuropathy

• ischemic, inflammatory, infectious, compressive,

neoplastic or hereditary etiology.

• Inflammatory demyelinating disease spectrum

• Multiple sclerosis (MS), neuromyelitis optica

spectrum disorder (NMOSD), acute

disseminated encephalomyelitis (ADEM)

• Within the MS category - tumefactive

demyelinating lesions

• Tumefactive lesions may be the initial

presentation of relapsing MS seen in about 50-

70%7.

• TDLs occupy more space and can cause seizures,

hemiparesis, neglect or encephalopathy7.

In this case of tumefactive demyelination, time may be a factor in getting the best visual outcome. MS can appear with ocular related symptoms early on. • Clinical presentations can vary from mild

symptoms to severe.• Presentation can vary from typical MS cases.• In some cases, asymptomatic patients remained

untreated and were shown to improve on their own.

• Our patient reported her symptoms worsening over 2–3-month period, justifying treatment.

Treatment: IV Solumedrol + Plasma Exchange (PLEX)• It is imperative to have a clear history of

patient’s presenting symptoms and associated review of systems.

Follow-Up x 2 weeks

VA20/70+20/70-

23-year-old white female was referred to the Neuro-ophthalmology clinic at Bascom Palmer Eye Institute.Chief complaint:• Mild superior field vision loss OS• Severe vision loss OD

ROS:• Neurological: Numbness of left side of face,

index finger, thumb, left leg• Constitutional: ImbalanceMedical history: NoneMedications: NoneFamily history: • Father: DM, Heart disease.• Mother: Lung problemsExamination:

• Ishihara Color Plates: 1/11 OD, 1/11 OS• Anterior segment unremarkable

Imaging:

1.Adamek, Dariusz, Radwanska, Edyta, Rog, Teresa, Grzywna, Ewelina, & Herman-Sucharska, Izabela. (2013). Tumefactive demyelinating lesion. Trying to find unity in diversity. Comparison of two cases. Clinical Neurology and

Neurosurgery, 116(C), 90-92.

2.Al-Louzi, Omar, & Saidha, Shiv. (2016). Chapter 12 - Pathophysiology of Optic Neuritis. In Multiple Sclerosis (pp. 281-309). Elsevier.

3.Altintas, A, Petek, B, Isik, N, Terzi, M, Bolukbasi, F, Tavsanli, M, . . . Siva, A. (2012). Clinical and radiological characteristics of tumefactive demyelinating lesions: Follow-up study. Multiple Sclerosis, 18(10), 1448-1453.

4.Association, N. G. O. N. B. O. C. M., Immunology, N. C. O. C. S. F., & Association, I. S. O. C. S. (2017). Chinese Guidelines for the Diagnosis and Management of Tumefactive Demyelinating Lesions of Central Nervous System. Chinese

Medical Journal, 130(15), 1838. https://link.gale.com/apps/doc/A499766736/AONE?u=miami_richter&sid=AONE&xid=3fab8ed5

5.Brod, Staley A, Lindsey, J William, & Nelson, Flavia. (2019). Tumefactive demyelination: Clinical outcomes, lesion evolution and treatments. Multiple Sclerosis Journal - Experimental, Translational and Clinical, 5(2),

2055217319855755.

6.Dwivedi, R A, Dwivedi, R E, Durnian, J M, & Young, C A. (n.d.). Tumefactive demyelination: An unusual cause of a spontaneously resolving homonymous hemianopia. BMJ Case Reports, 2013(Jun21 1), Bcr2013009363.

7.Fabian, Michelle T, Krieger, Stephen C, & Lublin, Fred D. (2016). Multiple Sclerosis and Other Inflammatory Demyelinating Diseases of the Central Nervous System. In Bradley's Neurology in Clinical Practice (Seventh ed., pp. 1159-

1186.e4).

8.Harmel, Jens, Ringelstein, Marius, Ingwersen, Jens, Mathys, Christian, Goebels, Norbert, Hartung, Hans-Peter, . . . Aktas, Orhan. (2014). Interferon-beta-related tumefactive brain lesion in a Caucasian patient with neuromyelitis

optica and clinical stabilization with tocilizumab. BMC Neurology, 14(1), 247.

9.Jeong, In Hye, Kim, Su-Hyun, Hyun, Jae-Won, Joung, AeRan, Cho, Hyo-Jin, & Kim, Ho Jin. (2015). Tumefactive demyelinating lesions as a first clinical event: Clinical, imaging, and follow-up observations. Journal of the Neurological

Sciences, 358(1), 118-124.

10.McGuire, Jennifer L, Fridman, Vera, Wüthrich, Christian, Koralnik, Igor J, & Jacobs, Dina. (2011). Progressive multifocal leukoencephalopathy associated with isolated CD8 T-lymphocyte deficiency mimicking tumefactive MS. Journal

of Neurovirology, 17(5), 500-503.

11.Moreira Ferreira, Vanessa F, Meredith, David, & Stankiewicz, James M. (2019). Tumefactive demyelination in a patient with relapsing-remitting MS on ocrelizumab. Neurology : Neuroimmunology & Neuroinflammation, 6(5), E589.

12.Nagappa, M, Taly, A. B, Sinha, S, Bharath, R. D, Mahadevan, A, Bindu, P. S, . . . Shankar, S. K. (2013). Tumefactive demyelination: Clinical, imaging and follow‐up observations in thirty‐nine patients. Acta Neurologica Scandinavica,

128(1), 39-47.

13.Pessini, Lucas M, Kremer, Stéphane, Auger, Cristina, Castilló, Joaquín, Pottecher, Julien, De Sèze, Jérome, . . . Rovira, Alex. (2020). Tumefactive inflammatory leukoencephalopathy in cocaine users: Report of three cases. Multiple

Sclerosis and Related Disorders, 38, 101496.

14.Roy, Ujjawal, Saini, Dinesh Satyanarayan, Pan, Koushik, Pandit, Alak, Ganguly, Goutam, & Panwar, Ajay. (2016). Neuromyelitis Optica Spectrum Disorder with Tumefactive Demyelination mimicking Multiple Sclerosis: A Rare Case.

Frontiers in Neurology, 7, 73.

15.Ueno, Tatsuya, Kinoshita, Iku, Arai, Akira, Suzuki, Chieko, & Tomiyama, Masahiko. (2020). Combined central and peripheral demyelination with tumefactive demyelinating lesions in the brainstem and longitudinally extensive optic

neuritis: A case report. Neurological Sciences, 41(6), 1601-1603.

16.Verma, R., & Kumar, C. (2019). Tumefactive Demyelination Associated with Bilateral Optic Neuritis in Neuromyelitis Optica Spectrum Disorders. Journal of Neurosciences in Rural Practice, 10(4), 693–696. https://doi.org/10.1055/s-

0039-3399614

17.Xia, Lei, Lin, Song, Wang, Zhong-cheng, Li, Shao-wu, Xu, Li, Wu, Jing, . . . Gao, Chuan-chuan. (2009). Tumefactive demyelinating lesions: Nine cases and a review of the literature. Neurosurgical Review, 32(2), 171-179.

18.Yachnis, Anthony T., MD, & Rivera-Zengotita, Marie L., MD. (2014). Tumefactive Demyelinating Lesions (TDL). In Neuropathology (pp. 289-290).

Acknowledgements: Dr. Shanaz Miri Dr. Byron Lam

MRI orbits and brain with and without contrast: • Numerous FLAIR hyperintense lesions with

incomplete peripheral rim of enhancement and confluent FLAIR signal.

• Asymmetric increased T2 signal and enhancement of the left optic nerve.

MRI cervical spine with and without contrast: • Multiple cervical cord lesions

Labs of interest:ANA: positiveMyelin Basic Protein 140: highCSF: protein 71 H, Glc 61, WBC 0, RBC 0Oligoclonal bands: Three (3) oligoclonal bands were observed in the CSF, which were not detected in the serum sample.*Note: not all labs are included

Case Report

Tumefactive Demyelination

Purpose

Discussion Conclusion

Sydney Madrigal, OD, MPHBascom Palmer Eye Institute | Miami, Florida

References

8/2020: Mild Superior vision

loss OS

9/2020: Dramatic vision

loss OD

10/2020: Numbness first

noticed

10/2020: Seen in Neuro-Ophthalmology

clinic

VARICELLA ZOSTER VIRUS ASSOCIATED WITH PRESEPTAL CELLULITISSamantha Wang O.D. and Zanna Kruoch O.D., F.A.A.O.

Malcom Randall VA Medical Center, Gainesville, FL

ABSTRACTThis is a case report of a patient with varicella-zoster infection with a

secondary bilateral preseptal cellulitis. This report highlights secondary

bacterial infections as one of the possible complications of a varicella

infection.

CASE HISTORYDemographics: 67-year-old Asian male

Chief Complaint

Periorbital pain and eyelid swelling OU that began 5 days prior.

Symptoms constant and severe

Ocular History: Unremarkable

Medical History: Unremarkable

Medications: Oral valacyclovir 1000 mg BID prescribed by primary

care provider 3 days prior

EXAM FINDINGSGross Examination: Crusted necrotic vesicular scab-like lesions

localized to the right forehead and right upper eyelid.

Adnexa: Bilateral periorbital edema and erythema OD>OS (Figure 1)

Figure 1

EOMs, CVFs, Pupils: Could not be assessed due to the severe eyelid

swelling and pain

BCVAs: Unable to be recorded OD, 20/50 OS

No ocular involvement OU to the extent seen

DISCUSSIONVaricella-Zoster Virus (VZV)

About

• A human herpes virus that causes varicella (chickenpox) as a

primary infection followed by latency in peripheral ganglion.1

• The latent VZV may reactive to cause herpes zoster (shingles).1,2

Classic Sign

• Right or left-sided vesicular rash that follows a characteristic

dermatomal distribution.

Ocular Complications

• Postherpetic neuralgia, stromal keratitis, giant cell arteritis,

vasculopathy, and secondary bacteria infections especially in

immunocompromised individuals.1,2,3

Treatment and Prevention

• Oral antivirals and vaccinations

Preseptal CellulitisAbout

• Infection located anterior to the orbital septum involving

periorbital region.4

• Most commonly caused by trauma, insect bites or spread of a

systemic infection.5

• Most common organisms isolated are the Staphylococcus and

Streptococcus species. Very rarely, associated with VZV

(secondary bacterial infection). 4,5,6

Demographics and Prognosis

• More common in childhood and with good prognosis. However,

if left untreated can cause severe complications including

orbital cellulitis, orbital abscess, and cavernous sinus

thrombosis.7

Signs

• Periorbital erythema, edema, and eyelid swelling. Vision and

pupils are usually normal. Typically, no proptosis or

ophthalmoplegia that is seen in orbital cellulitis.7,8

Treatment

• Oral antibiotics in mild infections, hospitalization and

intravenous antibiotics in severe cases.

CONCLUSION AND CLINICAL PEARLS

1. A VZV infection can lead to many complications and secondary

infections.

2. Prompt recognition and initiation of treatment can prevent the

spread of infection and potentially be life-saving.

DIFFERENTIAL DIAGNOSESVZV associated with orbital cellulitis, VZV associated with

preseptal cellulitis, Severe post-herpetic neuralgia from

VZV

INITIAL ASSESSMENTVZV associated with Bilateral Orbital Cellulitis.

TreatmentImmediate hospitalization referral for IV antibiotics. Return for follow-

up two weeks later.

Hospital Notes“A CT scan of his face had shown moderate soft tissue edema

overlying the right lateral orbital region without any focal fluid

collection. There was no sign of fluid crossing the sinuses or orbital

septum”

Patient was diagnosed with preseptal cellulitis, received IV

vancomycin and cefepime for 3 days, and then discharged with oral

clindamycin to take for 7 days.

Two-Week Follow-UpSignificant improvement in both signs and symptoms. Patient still had

vesicular scab-like lesions localized to the right forehead and right

upper eyelid; however, the bilateral eyelid swelling completely

resolved (Figure 2). Rest of the exam was unremarkable.

Figure 2

REFERENCES1. Kennedy P. & Gershon AA. (2018). Clinical Features of Varicella-Zoster Virus Infection. Viruses, 10(11), 609.

https://doi.org/10.3390/v10110609

2. Gershon AA, Breuer J, Cohen JI, et al. (2015). Varicella zoster virus infection. Nature reviews. Disease primers, 1, 15016.

https://doi.org/10.1038/nrdp.2015.16

3. Lee KG & Cheng MC (2012). Varicella-Zoster Infection with Secondary Bacteremia and Extensive Facial Abscesses.

Medical Journal of Malaysia. 2012 Oct;67(5):529. http://www.e-mjm.org/2012/v67n5/varicella-zoster-infection.pdf

4. Cürebal B, Şahin A, & Dalgıç N. (2019). Preseptal Cellulitis in Children: A Single-Center Experience. Sisli Etfal Hastanesi tip

bulteni, 53(4), 409–412. https://doi.org/10.14744/SEMB.2018.75010

5. Qualickuz Z, Zahedi FD, & Husain S. (2017). Varicella zoster causing preseptal cellulitis - uncommon but

possible. Malaysian family physician: the official journal of the Academy of Family Physicians of Malaysia, 12(3), 37–39.

6. Bae C, Bourget D. Periorbital Cellulitis. (2020). In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan.

https://www.ncbi.nlm.nih.gov/books/NBK470408/

7. Cürebal, B, Şahin, A, & Dalgıç, N. (2019). Preseptal Cellulitis in Children: A Single-Center Experience. Sisli Etfal Hastanesi

tip bulteni, 53(4), 409–412. https://doi.org/10.14744/SEMB.2018.75010

8. Danishyar A & Sergent SR. Orbital Cellulitis. (2021). In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021

Jan. https://www.ncbi.nlm.nih.gov/books/NBK507901/