Abstract Details

Title SMA Patient Experienced Aseptic Meningitis With Severe Hypoglycorrhachia After Nusinersen Infusion

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P8 - Poster Session 8 (8:00 AM-9:00 AM)

Poster/Presentation
Number 1-011

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 <10mg/dL, 51/mm³WBCs (with neutrophil predominance.) The patient was directly admitted to our hospital and treated empirically with antibiotics and antifungals. Examination showed no fever, nuchal rigidity, or encephalopathy. His CBC, BMP, and blood and fungal cultures returned normal. Antibiotics were discontinued after two days and the patient was discharged home. He continued to deny symptoms. At his fourth dose of Nusinersen, CSF was normal. Aseptic meningitis with severe hypoglycorrhachia has not previously been reported in patients receiving Nusinersen. Phase 1 and 2 studies of Nusinersen reported the most common adverse effect as post-lumbar puncture headache but did not report CSF abnormalities. Another study of 19 adult patients receiving Nusinersen found infrequent pleocytosis (up to 21 WBC/mL) without hypoglycorrhachia.

Conclusions

Adults receiving Nusinersen may experience a self-limited aseptic meningitis with flu-like symptoms which has not previously been reported.

Authors/Disclosures
Afra Janarious, MD (Netherfield Place) PRESENTER	Dr. Janarious has nothing to disclose.
Ali A. Habib, MD (University of California, Irvine)	Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for argenx. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovant. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech/Roche. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for NMD Pharma. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon/ Amgen. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Jansen/Johnson & Johnson. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMDSerono/Merck. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH/NINDS. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunis Biomedical. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman-La Roche. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Habib has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for argenx. Dr. Habib has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. Dr. Habib has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Jansen/Joohnson & Johnson. The institution of Dr. Habib has received research support from Alexion. The institution of Dr. Habib has received research support from Horizon/Amgen. The institution of Dr. Habib has received research support from Immunovant. The institution of Dr. Habib has received research support from UCB. The institution of Dr. Habib has received research support from argenx. The institution of Dr. Habib has received research support from CabalettaBio. The institution of Dr. Habib has received research support from Regeneron. The institution of Dr. Habib has received research support from Arcellx. The institution of Dr. Habib has received research support from Novartis. The institution of Dr. Habib has received research support from EMDSerono/Merck. The institution of Dr. Habib has received research support from Cour Pharmaceuticals.
Tahseen Mozaffar, MD, F�鶹��ýӳ�� (University of California Irvine)	Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion Pharmaceuticals. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi-Genzyme. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Amicus. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Grifols. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Astellas Gene Therapy. Dr. Mozaffar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bayer. Dr. Mozaffar has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Mozaffar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Applied Therapeutics. The institution of Dr. Mozaffar has received research support from NIH. The institution of Dr. Mozaffar has received research support from Muscular Dystrophy Association. The institution of Dr. Mozaffar has received research support from Sanofi. The institution of Dr. Mozaffar has received research support from Argenx. The institution of Dr. Mozaffar has received research support from Amicus Therapeutics. The institution of Dr. Mozaffar has received research support from Astellas Gene Therpay. The institution of Dr. Mozaffar has received research support from Cartesian. The institution of Dr. Mozaffar has received research support from Cabaletta. Dr. Mozaffar has received personal compensation in the range of $500-$4,999 for serving as a Study Section Member with NIH.
Manisha Kak Korb, MD	Dr. Kak Korb has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Kak Korb has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CSL Behring. Dr. Kak Korb has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Kak Korb has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen.

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