P. Pagnotta
Neurology Associates PAAuthor Of 1 Presentation
P0747 - Rapid improvement in neurologic symptoms of neuromyelitis optica spectrum disorder with eculizumab monotherapy: Case report (ID 895)
Abstract
Background
Treatment of neuromyelitis optica spectrum disorder (NMOSD) involves high‑dose corticosteroids for the initial symptoms and maintenance immunosuppressant therapy for relapse prevention. However, 25–60% of patients receiving these medications continue to have recurrent attacks.
Objectives
To describe a case in which a patient with NMOSD was treated with the terminal complement inhibitor eculizumab as monotherapy.
Methods
Case report of a patient with NMOSD, based on neurologic examinations conducted before and after initiation of eculizumab monotherapy.
Results
A 37-year-old African-American woman was hospitalized on January 13, 2020, following a 2-week period of progressive dysesthesia, weakness, ataxia, fatigue, and urinary retention. She was diagnosed with aquaporin-4-positive NMOSD following spinal and brain magnetic resonance imaging. She received treatment with intravenous methylprednisolone for 2 days and plasma exchange therapy for 5 days, and was discharged from hospital on January 22. Although her visual acuity had improved, the patient continued to have significant disability, including ataxic gait requiring use of a cane. She was referred to the authors’ clinic on January 27 for further evaluation. The following results were obtained on neurologic examination: muscle strength – scores of 4 or 5- (full strength = 5) for all extremities; sensory – decreased sensation in right upper and left lower extremities; reflexes – hyper throughout; coordination – bilateral dysmetria; gait – ataxia, unsteadiness, scissoring, and positive Romberg test; vision – normal cranial nerve but patient-reported visual obscurations (visual acuity not assessed). The patient was vaccinated against Neisseria meningitidis on February 8, and initiated eculizumab monotherapy on February 24 at a starting dose of 900 mg once per week for 4 weeks, then 1200 mg every 2 weeks. Reassessment on March 9 showed: muscle strength – score of 5- for right upper extremity, otherwise normal; sensory – normal; reflexes – hyper throughout; coordination – intact; gait – wide-base stable without ataxia; vision – normal cranial nerve, returned to baseline function. Although she had slight difficulties walking and intermittent slightly blurred vision, the patient was subsequently able to return to work. The patient had no adverse events associated with eculizumab and continues treatment.
Conclusions
Eculizumab monotherapy was associated with rapid resolution of neurologic symptoms with no adverse events in a patient with first-episode NMOSD, allowing the patient to return to work.
Presenter Of 1 Presentation
P0747 - Rapid improvement in neurologic symptoms of neuromyelitis optica spectrum disorder with eculizumab monotherapy: Case report (ID 895)
Abstract
Background
Treatment of neuromyelitis optica spectrum disorder (NMOSD) involves high‑dose corticosteroids for the initial symptoms and maintenance immunosuppressant therapy for relapse prevention. However, 25–60% of patients receiving these medications continue to have recurrent attacks.
Objectives
To describe a case in which a patient with NMOSD was treated with the terminal complement inhibitor eculizumab as monotherapy.
Methods
Case report of a patient with NMOSD, based on neurologic examinations conducted before and after initiation of eculizumab monotherapy.
Results
A 37-year-old African-American woman was hospitalized on January 13, 2020, following a 2-week period of progressive dysesthesia, weakness, ataxia, fatigue, and urinary retention. She was diagnosed with aquaporin-4-positive NMOSD following spinal and brain magnetic resonance imaging. She received treatment with intravenous methylprednisolone for 2 days and plasma exchange therapy for 5 days, and was discharged from hospital on January 22. Although her visual acuity had improved, the patient continued to have significant disability, including ataxic gait requiring use of a cane. She was referred to the authors’ clinic on January 27 for further evaluation. The following results were obtained on neurologic examination: muscle strength – scores of 4 or 5- (full strength = 5) for all extremities; sensory – decreased sensation in right upper and left lower extremities; reflexes – hyper throughout; coordination – bilateral dysmetria; gait – ataxia, unsteadiness, scissoring, and positive Romberg test; vision – normal cranial nerve but patient-reported visual obscurations (visual acuity not assessed). The patient was vaccinated against Neisseria meningitidis on February 8, and initiated eculizumab monotherapy on February 24 at a starting dose of 900 mg once per week for 4 weeks, then 1200 mg every 2 weeks. Reassessment on March 9 showed: muscle strength – score of 5- for right upper extremity, otherwise normal; sensory – normal; reflexes – hyper throughout; coordination – intact; gait – wide-base stable without ataxia; vision – normal cranial nerve, returned to baseline function. Although she had slight difficulties walking and intermittent slightly blurred vision, the patient was subsequently able to return to work. The patient had no adverse events associated with eculizumab and continues treatment.
Conclusions
Eculizumab monotherapy was associated with rapid resolution of neurologic symptoms with no adverse events in a patient with first-episode NMOSD, allowing the patient to return to work.