Abstract Details

Title First Report of Confirmed CIDP in a Patient with Neuromyelitis Optica Spectrum Disorder (NMOSD)

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-106

Objective We describe a young woman with seronegative NMOSD, who developed CIDP and autoimmune markers.

Background NMOSD is a central nervous system disease without involvement of the peripheral nervous system. There are no reports of patients with both CIDP and NMOSD in the literature.

Design/Methods A 28-year-old woman presented with transient visual obscurations, bilateral papilledema and gait difficulty in October 2017. She reported a history of 6 episodes of nausea, vomiting, diplopia, left hand tremor and facial numbness between 2009-2014. Multiple MRI Brain images from 2009-2014 showed lesions in the medulla. MRI C-Spine in 2011 showed a T2 hyperintense, contrast enhancing lesion from C7-T2 with cord expansion. In 2015, she had an episode of diffuse weakness and gait difficulty. Patient reported remarkable improvement of each attack with steroid treatment.

Results MRI Brain and Orbits showed a non-enhancing lesion in the dorsal medulla, enlarged extraocular muscles and optic nerve edema. MRI C-Spine showed a non-enhancing longitudinal lesion extending from C7-T2. MRI L-Spine showed enlarged, enhancing nerve roots extending to the cauda equina. Aquaporin-4 Ab was 1.7 (negative). Patient had elevated CSF protein > 600, CSF was otherwise normal. EMG/NCS study showed prolonged F-responses, reduced conduction velocities and prolonged distal latencies, suggestive of CIDP. ANA was 1:80, but other autoimmune markers were normal. Gallium scan showed increased uptake in the lacrimal and salivary glands. Lacrimal gland biopsy showed chronic inflammation, but no granulomatous tissue. Repeat autoimmune work up in March 2018 showed positive ANA 1:160, SSA-A-Ab 1.3 and anti-histone-Ab 2.9. She was started on low dose oral steroid therapy, monthly IVIg and Mycophenolate mofetil, with remarkable improvement in her symptoms.

Conclusions

We present the first case of confirmed CIDP in a patient with NMOSD. We suspect that this patient has NMOSD and CIDP due to an underlying autoimmune overlap syndrome.

Authors/Disclosures
Maitreyi Murthy, MD (Peacehealth Southwest Medical Group) PRESENTER	Dr. Murthy has nothing to disclose.
Machteld E. Hillen, MD, FAAN (Rutgers-NJMS)	The institution of Dr. Hillen has received research support from Genentech.

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