(Kiosk 3) Late Onset of Neuromyelitis Optica in an Elderly Female Without Optic Neuritis: A Case Report

Case Diagnosis: Neuromyelitis Optica (NMO)

Case Description or Program Description: 88-year-old female presented to the hospital after falling due to sudden leg weakness. Examination showed leg weakness, left worse than right, decreased light touch and temperature sensation, and mixed upper/lower motor neuron signs. MRI showed a longitudinally extensive spinal cord lesion from C4 to the conus medullaris. Serum studies returned positive for ​​Aquaporin-4 (AQP4) Antibodies, confirming the diagnosis of NMO. Despite receiving intravenous steroids, plasmapheresis, and Inebilizumab her symptoms progressed. She was subsequently transferred for acute inpatient rehabilitation.

Setting: Acute Inpatient Rehabilitation Hospital

Assessment/Results: Despite receiving treatment and rehabilitation, minimal functional progress was seen. The decision was made to transfer the patient to a skilled nursing facility. Unfortunately, transfer was delayed due to multiple medical complications, including urinary tract and Clostridium difficile infections, multiple episodes of delirium and lastly development of sepsis from pneumonia. The patient was transferred to an acute-care hospital where she expired.

Discussion (relevance): NMO is an autoimmune inflammatory syndrome involving antibodies targeting AQP4 channels on glial cells, causing demyelination of neurons in the Central Nervous System (CNS). NMO attacks are induced by infection or pre-existing autoimmune disease. Typical age of onset is 30-40 years of age. Presentation includes optic neuritis and/or transverse myelitis. With this patient’s presentation at later age and without one the hallmark symptom of Optic Neuritis, diagnosis and treatment was delayed, possibly contributing to her lack of improvement and poor rehabilitation course.

Conclusions: Initial NMO symptoms m may include sudden weakness; optic neuritis may not be involved. Immediate recognition of transverse myelitis on MRI and positive serum AQP4 antibodies is vital in making an early diagnosis so appropriate treatment may begin as soon as possible. NMO may present outside age 30-40. Older age of onset may confer poorer prognosis and need be considered if limited medical and/or functional progress is made despite intensive rehabilitation.