Abstract Details

Title Neuromyelitis Optica Spectrum Disorders and Systemic Lupus Erythematosus: Case Series from an University Center

Topic Autoimmune Neurology

Presentation(s) P13 - Poster Session 13 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-006

Objective NA

Background

Neuromyelitis optica spectrum disorders (NMOSD) are immunomediated inflammatory diseases of central nervous system (CNS), which preferentially affect optic nerves and spinal cord (SC). Anti-aquaporin 4 antibody (anti-AQP4) is a specific serological marker. Systemic lupus erythematosus (SLE) is a rheumatologic disease that may affect CNS. There are several reports about the coexistence of NMOSD and autoimmune diseases.

Design/Methods Case 1: 34-year-old woman with SLE presented with dysphagia, nausea, vomiting, hiccups, vertigo, blurred vision, hands paraesthesia, bilateral horizontal nystagmus, left trigeminal neuralgia, right peripheral facial paralysis and gait ataxia. Brain and SC MRI evidenced poorly delimited lesion in area postrema and medulla, suggesting rhomboencephalitis. Cerebrospinal fluid showed protein levels of 44mg/dL, negatives PCRs for Herpes simplex 1 and 2 and Dengue, Zika and Chikungunya virus serologies. Serum anti-AQP4 was positive. She received intravenous methylprednisolone, with good recovery. Case 2: 34-year-old woman with SLE presented with hands paresthesia and amaurosis in left eye. On evaluation, apalesthesia with abatiesthesia in upper limbs, pseudoathetosis in the hands, specially with eyes closed, absent direct left photomotor reflex with Marcus Gunn pupil and left papilledema. Cyclophosphamide pulse was performed initially. Brain and orbits MRI revealed left optic neuritis. Cervical spine MRI demonstrated hyperintensity from C2 to C7, compatible with central extensive myelitis. Serum anti-AQP4 was positive. Finally, plasmapheresis was performed, with partial improvement. Case 3: 44-year-old woman with SLE presented 14 years earlier with acute paraplegia, legs paresthesia and visual blurring, with initial negative serum anti-AQP4. In 2019, she acutelly presented nausea, vomiting, hiccups, worsening of vision and upper limbs weakness. Plasmapheresis was performed, with significant improvement. New dosage of serum anti-AQP4 was positive.

Results NA

Conclusions

In view of a possible pathophysiological association between NMOSD and SLE, clinicians should keep in mind this possible co-occurence, specially when characteristically neurological phenotypes occur.

Authors/Disclosures
Vanessa C. Colares Lessa, MD (Hospital Universitario Antonio Pedro) PRESENTER	Dr. Colares Lessa has nothing to disclose.
Caroline L. Medeiros, Jr., MD (Hospital Universitario Antonio Pedro)	Dr. Medeiros has nothing to disclose.
Luis Felipe Haberfeld Maia, MD (Universidade Federal Fluminense)	No disclosure on file
Arthur Ramalho Monfredinho, MD (University Hospital Antonio Pedro - Federal Fluminense University)	Dr. Ramalho Monfredinho has nothing to disclose.
	No disclosure on file
Maria Luisa R. Prata, MD (Universidade Federal Fluminense)	No disclosure on file
	No disclosure on file
Joao Marcos C. Ferreira, Sr., MD (Joao Marcos Campos Ferreira)	No disclosure on file
	No disclosure on file
Caroline B. Braune, MD (Fluminense Federal University Department of Neurology)	Dr. Braune has nothing to disclose.
Gabriel R. De Freitas, MD, PhD (Universidade Federal Fluminense)	Dr. De Freitas has nothing to disclose.
	No disclosure on file
Osvaldo J. Nascimento, MD, PhD, F�鶹��ýӳ�� (Fluminense Federal University)	Dr. Nascimento has nothing to disclose.

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