Abstract Details

Title An Atypical NMO Presentation with Locked-In Syndrome

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-012

Objective Neuromyelitis optica is a condition characterized by optic neuritis and transverse myelitis, with associated MRI lesions classically located along the brainstem and spinal cord. We are reporting a case of NMO with rapid-onset locked-in syndrome and atypical MRI findings.

Background The patient is a 56 year old African American female with unconfirmed prior diagnosis of SLE who initially presented with fevers and abdominal pain. She was diagnosed with Legionella pneumonia and started on treatment with Levofloxacin. Over the next three days, the patient developed progressive dysarthria and ataxia, finally culminating into quadriparesis and a tenuous respiratory drive with no cough or gag reflex. The patient was fully conscious and able to communicate by moving her eyes in a specified direction. MRI showed extensive T2 hyperintensities located symmetrically along the white matter of the pons, thalami, midbrain, and cerebellar peduncles. The patient was started empirically on IVIG and steroids, with little improvement in symptoms. Serum studies were remarkable for a positive ANA titer (1:160) and elevated SS-A. LP demonstrated neutrophilic pleocytosis and elevated myelin basic protein, oligoclonal bands, and aquaporin-4 antibody. After the diagnosis was confirmed with the CSF findings, she was initiated on plasmapheresis therapy, with modest improvement in symptoms thus far.

Design/Methods NA

Results NA

Conclusions Unique to this case is the extensive nature of the brainstem lesions, which delayed early diagnosis, as there was consideration of alternate diagnoses including Bickerstaff encephalitis, autoimmune rhomboencephalitis, and ADEM. It can also be argued that the patient was more susceptible to developing NMO given her underlying, undiagnosed autoimmune disease, as multiple cases of NMO as a first presenting symptoms of systemic lupus have been reported. We hope that by presenting this case, NMO remains high on the differential in patients with known rheumatologic disease who present with a rapidly progressive demyelinating condition, despite atypical MRI findings.

Authors/Disclosures
Mahnoor Rehman, MD (Advocate Medical Group) PRESENTER	No disclosure on file
Lara Basovic, MD (Massachusetts General Hospital, Epilepsy Office)	No disclosure on file

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