Abstract Details

Title Tumefactive Demyelinating Lesions due to NMO treated with Eculizumab

Topic Autoimmune Neurology

Presentation(s) P14 - Poster Session 14 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-002

Objective

To describe a case of NMO (neuromyelitis optica) presenting with tumefactive demyelinating lesions treated with eculizumab.

Background

A 50-year-old male mechanic with no past medical history presented with one month of confusion, palpitations, headache, right hand weakness and peripheral vision loss. Exam was notable for 0/3 delayed recall, right homonymous hemianopsia, and weak right grip strength. Imaging showed a large infiltrative lesion.

Design/Methods N/A

Results MRI showed an infiltrative lesion that involved the splenium of the corpus callosum and extended into the white matter of the left parietal and occipital lobes. There was incomplete peripheral enhancement and concentric alternating signal intensities. There were also scattered subcortical FLAIR abnormalities. MR spectroscopy demonstrated elevated choline peak, decreased NAA peak, and peak at 2.3ppm (likely glutamate/glutamine).

Brain biopsy of the left parieto-occipital lesion showed “hypervascular brain tissue with marked foamy macrophagic infiltrates.” Additional testing was performed for demyelinating disease. Serum aquaporin-4 antibody IgG resulted positive with titer >80 (normal <3) by ELISA. Serum myelin oligodendrocyte glycoprotein (MOG) antibody and ganglioside GQ1B IgG antibody were negative.

Cerebrospinal fluid (CSF) showed white blood cells 4 cells/uL, red blood cells 0 cells/uL, and protein 32 mg/dL. Myelin basic protein and oligoclonal bands were negative. NMO/AQP4-IgG FACS testing was positive with a 1:64 titer (normal <1:2).

He completed a course of IV steroids with minimal initial improvement. Episodes of confusion were concerning for focal seizures, and he was treated with levetiracetam. He was initiated on eculizumab to decrease risk of recurrence. He has had improvement in symptoms and imaging 5 months later.

Conclusions Tumefactive Demyelinating Lesions (TDL) are often mistaken for neuro-oncologic processes. They are more often seen in multiple sclerosis but have been described in NMO. Patients can be successfully managed with novel agents like eculizumab.

Authors/Disclosures
Kimberly O'Neill, MD PRESENTER	Dr. O'Neill has nothing to disclose.
Lauren Gluck, MD (Montefiore Medical Center)	Dr. Gluck has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeautics. Dr. Gluck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Gluck has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Amgen Rare Disease. Dr. Gluck has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Gluck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bristol Myers Squibb.

��ɫ��

��ɫ��