Cant pee, cant climb a tree: Seropositive myelin oligodendrocyte glycoprotein (MOG) antibodies in acute disseminated encephalomyelitis (ADEM)
a b s t r a c t
When weighing the costs and benefits of “choosing wisely,” in a healthcare climate that continues to stress cost- saving practices, it is difficult to argue with approaching low-risk patients with conservative approaches and treatments. In defense of liberal and broad approaches to patient workups, however, one must also weigh the bounce-back emergency department (ED) visit, which may represent either a failure of initial evaluation or a suc- cess of appropriate return precautions. An 18-year-old male presented to the ED with two days of urinary reten- tion, abdominal pain, and subjective fever, was discharged with urology follow-up and doxycycline, and subsequently returned to the ED in <24 h with inability to stand and loss of reflexes in bilateral lower extremities. Magnetic Resonance Imaging (MRI) of the brain and spine demonstrated extensive and multifocal areas of signal abnormalities consistent with active demyelination concerning for Acute disseminated encephalomyelitis . Additional lab workup demonstrated seropositive myelin oligodendrocyte glycoprotein (MOG) anti- bodies, further supporting the diagnosis of ADEM, an immune-mediated disorder which can lead to rapid multi- focal Neurologic dysfunction.
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case presentation“>Acute disseminated encephalomyelitis (ADEM) is a rare immune- mediated multifocal demyelinating disorder affecting the brain and spi- nal cord [1]. Symptoms can be preceded by febrile illness, and include sudden onset multifocal Neurological deficits, such as ataxia, motor and sensory deficits, meningeal signs, and coma within hours to days of initial presentation, with a mortality rate of 10-15% [2,3]. The disor- der is more common in early childhood and is thought to occur in post-infectious or post-immunization periods [1,2]. Myelin oligoden- drocyte glycoprotein (MOG) is a protein found only on the outer surface of oligodendrocytes and myelin sheaths in both serum and cerebrospi- nal fluid (CSF) [4]. The presence of anti-MOG antibodies is highly spe- cific (98.5%) for inflammatory demyelinating disorders, such as ADEM, however the likelihood of capturing this fleeting seropositivity in ADEM with current tests is low (sensitivity 36.4%) [4,5]. The following case describes an afebrile, sexually active 18-year-old male who initially
? This study was reviewed and deemed non-human subjects research by the Prisma Health-Upstate Institutional Review Board.
* Corresponding author at: Prisma Health-Upstate Department of Emergency Medicine, Greenville, SC, United States.
E-mail address: [email protected] (M.K. Wurzelmann).
presented with Urinary retention and re-presented to the ED <24 h later with inability to stand.
- Case presentation
An 18-year-old male with a past medical history of asthma initially presented to the ED with urinary retention and abdominal pain for two days. Associated symptoms included subjective fever, chills, and hot flashes. Patient denied presence of flank pain, dysuria, penile dis- charge, testicular or Scrotal pain and swelling, genital lesions, trauma to genital region, constipation, back pain, saddle anesthesia, Extremity weakness, or numbness. Patient denied history of sexually transmitted infections (STI) but endorsed unprotected intercourse. Patient was doc- umented as alert and oriented x3, with Cranial nerves 2-12 intact, and GCS 15 in nurse practitioner’s (NP) neurologic exam. Vitals were within normal limits: Blood pressure 128/67 mmHg, heart rate 84 beats per minute, temperature 99.2 ?F/37.3 ?C. Catheterization produced
>1000 mL of urine. No leukocytosis noted on complete blood count . Gonorrhea and Chlamydia cultures were collected. Urinalysis was notable for a few white blood cells and presence of nitrites. The pa- tient received one dose of ceftriaxone in the ED and was discharged on doxycycline for possible chlamydia infection with urology follow-up.
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The patient returned to the ED <24 h later with inability to stand and areflexia of lower extremities. On neurologic exam, the physician noted inability to lift bilateral lower extremities and absent bilateral patellar and Achilles reflexes, however patient was able to bend knees and wig- gle toes bilaterally. No other portions of neurologic exam were docu- mented. Vitals documented: BP 105/53 mmHg, HR 88 bpm, Temp
98.9 ?F/37.2 ?C. MRI of the cervical, thoracic, and lumbar spine (Images 2, 3, and 4) were obtained in the ED and demonstrated multifocal cervicothoracic signal abnormalities, consistent with active demyelin- ation. Lumbar puncture results were significant for monocellular pleocytosis and elevated protein. Patient denied family history of auto- immune diseases but reported that his sister had viral meningitis two weeks prior. brain MRI (Image 1) significant for extensive areas of ab- normal signal in supratentorial and infratentorial regions.
- Resolution
The patient was started on high dose steroids and admitted to the hospital. Labs were significant for positive MOG antibodies, negative CSF culture, elevated protein and WBC in CSF, and multiple negative au- toimmune and infectious labs. Patient received five days of 1 g Solumedrol IV daily and lower extremity weakness, areflexia, and uri- nary retention resolved. He was discharged with a 12-day Steroid taper and diagnosis of ADEM, with a plan to repeat MRI in 6 weeks. In follow-up neurology appointment, the patient was started on Mycophenalate 500 mg daily, and increased to 1000 mg twice per day over the course of 4 weeks to reduce the risk of recurrence. Repeat MRI demonstrated nearly complete resolution of signal abnormalities throughout parenchyma and spinal cord.
Images: Image 1. Brain MRI demonstrating multiple areas of abnormal signal in supratentorial and infratentorial regions. Image 2, 3, & 4. Multifocal cervical, thoracic, and lumbar spinal cord signal abnormalities, concerning for active demyelination.
This case highlights the Rapid progression of neurologic symptoms in ADEM in young healthy patients. While presentations can vary due to the multifocal nature of demyelination in this disorder, ~25% of patients present with urinary dysfunction, as well as para- or quadriparesis [6].
The differential for urinary retention in young males is broad, encompassing obstructive, infectious, inflammatory, neurologic, mus- culoskeletal, and pharmacologic etiologies. Acute prostatitis, often caused by E. coli, is the most common infectious cause of urinary reten- tion, however urethritis from STIs can cause this [7]. While initially there was concern for STI complications given his history of unprotected intercourse, additional neurological function questions were also appro- priately asked. This report points to the importance of also documenting a history of IV drug use, recent weight loss or respiratory infection, and concern for Epidural abscess. In addition, workup should include a detailed physical exam, including neurologic testing such as reflexes, along with Laboratory workup targeting infectious causes. Imaging to assess signs of obstruction may have also been valuable in the initial workup. Although neurologic causes are less likely in non-traumatic and healthy patients, care should be taken to exclude these etiologies, particularly in young patients with urinary retention >1 L and no other urinary complaints.
Funding
No funding from public, private, or commercial sectors to report.
CRediT authorship contribution statement
Mary K. Wurzelmann: Writing - review & editing, Writing -
original draft. Raymond Chahoud: Writing - review & editing.
Declaration of Competing Interest
I have no conflicts of interest to report.
References
- Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S, et al. Acute disseminated encephalomyelitis. Neurology. 2016;87(9_Supplement_2):S38-45. https://doi.org/10.1212/WNL.0000000000002825.
- Hartung H. ADEM Neurol. 2001;56(10):1257-60. https://doi.org/10.1212/WNL.56.10.
1257.
- Wender M. Acute disseminated encephalomyelitis (ADEM). J Neuroimmunol. 2011 February;231(1-2):92-9. https://doi.org/10.1016/j.jneuroim.2010.09.019.
- Peschl P, Bradl M, Hoftberger R, Berger T, Reindl M. Myelin oligodendrocyte glycopro- tein: deciphering a target in inflammatory Demyelinating diseases. Front Immunol. 2017;8:529. https://doi.org/10.3389/fimmu.2017.00529.
- Narayan R, Simpson A, Fritsche K, Salama S, Pardo S, Mealy M, et al. MOG antibody disease: a review of MOG antibody seropositive neuromyelitis optica spectrum disor- der. Mult Scler Relat Disord. 2018;25:66-72. https://doi.org/10.1016/j.msard.2018.07. 025.
- Huynh W, Cordato DJ, Kehdi E, Masters LT, Dedousis C. Post-vaccination encephalo- myelitis: literature review and illustrative case. J Clin Neurosci: Off J Neurosurg Soc Austral. 2008, December;15(12):1315-22. https://doi.org/10.1016/j.jocn.2008.05. 002.
- Davis NG, Silberman M. Bacterial Acute Prostatitis. [Updated 2022 Jul 17]. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan Available from: https://www.ncbi.nlm.nih.gov/books/NBK459257/.