SMA Patient Experienced Aseptic Meningitis With Severe Hypoglycorrhachia After Nusinersen Infusion (2528)

2020 
Objective: To discuss the finding of aseptic meningitis with severe hypoglycorrhachia in an adult patient receiving Nusinersen. Background: Nusinersen is the first FDA-approved drug for spinal muscular atrophy (SMA), an autosomal recessive motor neuron disorder causing severe progressive motor weakness in children and adults. It is an intrathecally-administered antisense oligonucleotide that promotes expression of full-length survival motor neuron (SMN) protein, which is deficient in SMA. Design/Methods: We describe the case of a 39-year-old man with SMA2 who developed aseptic meningitis after his first dose of Nusinersen. Results: Our patient was administered Nusinersen via fluoroscopic-guided lumbar puncture. The week after his first dose, he reported mild body aches and temperatures of 99–101 degrees Fahrenheit which were relieved by Tylenol. His CSF at the time of his first infusion was normal. The day of the second dose of Nusinersen, CSF was abnormal: glucose was markedly reduced to 30mg/dL with protein of 701mg/dL. The patient remained asymptomatic. At the third dose, the patient’s CSF was markedly abnormal: protein 299mg/dL, glucose Conclusions: Adults receiving Nusinersen may experience a self-limited aseptic meningitis with flu-like symptoms which has not previously been reported. Disclosure: Dr. Janarious has nothing to disclose. Dr. Habib has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion and Argenx. Dr. Habib has received research support from Ra, Alexion, Acceleron, Catalyst, Immunovant, and UCB. Dr. Mozaffar has nothing to disclose. Dr. Kak Korb has nothing to disclose.
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