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A Rare, Novel Case of Autoimmune Mediated Meningoencephalomyelitis

Sindhoori R. Nalla, DO, MHSA (McGaw Medical Center of Northwestern University (SRAL) PM&R Program, Chicago, Illinois); Steven Nussbaum, MD

Meeting: AAPM&R Annual Assembly 2020

Categories: Neurological Rehabilitation (2020)

Session Information

Session Title: Virtual Poster Hall

Session Time: None. Available on demand.

Disclosures: Sindhoori R. Nalla, DO, MHSA: No financial relationships or conflicts of interest

Case Description: A 25 year old female presented with several days of headaches, neck pain, and dysphagia. She was initially diagnosed with a peritonsillar abscess, but developed progressive quadriplegia, occulomotor abnormalities, and respiratory distress requiring intubation. Imaging showed pons, medulla, and cervical spine hyperintensities. Autoimmune encephalitis panel was positive for glial fibrillary acidic protein antibody (GFAP-Ab). Other than an indeterminate ovarian mass, a definitive source was not found. She was treated for meningoencephalomyelitis of unclear origin with intravenous steroids, plasma exchange therapy, mycophenolate, and steroid taper. After ventilator weaning, she admitted to AIR requiring maximum assistance for activities of daily living (ADLs) and total assistance for mobility. Rehabilitation course was interrupted for planned laparoscopic salpingo-oophorectomy, ultimately confirming an ovarian dermoid cyst. Repeat neuroimaging revealed improvement in prior enhancement. She returned to AIR and discharged home with modified independence for transfers/ADLs and supervision for ambulation.

Setting: Acute Inpatient Rehabilitation (AIR)

Patient: 25 year old female with progressive quadriplegia and encephalopathy Assessment/

Results: Patient’s functional status improved with immunosuppressive agents, tumor removal, and intensive rehabilitation.

Discussion: GFAP-Ab mediated meningoencephalomyelitis is a rare autoimmune process first defined in 2016. In cases published, it is associated with fevers, headaches, vision changes, encephalopathy, myelitis, and typically affects adults over forty years old. There is a lack of uniform diagnostic criteria, and concurrent neural antibodies potentially obscure findings. In one study, 34% of patients with GFAP-Ab mediated disease also had a neoplasm. Although ovarian tumors have been documented, the type of neoplasm varies widely. While standard treatment regimen is not established, tumor removal and immunosuppression are recommended for patients with associated neoplasms. This atypical case highlights a young, healthy female with a subacute neurological presentation who improved after immunosuppression, surgery, and rehabilitation.

Conclusion: GFAP-Ab mediated meningoencephalomyelitis is a rare, newly discovered process that can be seen in our neurorehabilitation population.

Level of Evidence: Level V

To cite this abstract in AMA style:

Nalla SR, Nussbaum S. A Rare, Novel Case of Autoimmune Mediated Meningoencephalomyelitis [abstract]. PM R. 2020; 12(S1)(suppl 1). https://pmrjabstracts.org/abstract/a-rare-novel-case-of-autoimmune-mediated-meningoencephalomyelitis/. Accessed May 9, 2025.
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