Neuromyelitis Optica and Anti-MOG Disease Poster Presentation

P0688 - Area postrema syndrome and longitudinally extensive transverse myelitis in a patient with prostatic adenocarcinoma and Aquaporin-4 antibodies. (ID 1872)

Speakers
  • A. Dinoto
Authors
  • A. Dinoto
  • A. Bosco
  • A. Sartori
  • M. Cheli
  • G. Bellavita
  • F. Pasquin
  • A. Bratina
  • P. Manganotti
Presentation Number
P0688
Presentation Topic
Neuromyelitis Optica and Anti-MOG Disease

Abstract

Background

Neuromyelitis optica spectrum disorder (NMOSD) is not defined as a “classical” paraneoplastic neurological syndrome (PNS), however there are growing evidences that NMOSD may be associated with cancer.

Objectives

The aim of the present report is to describe the clinical presentation of a patient with NMOSD of probable paraneoplastic origin.

Methods

A 79-years old man presented with acute onset of mild dysphagia associated with intractable hiccups and vomiting one month after radiotherapy for prostatic adenocarcinoma (cT2N0M). Two weeks later, he developed subacute lower-limb weakness and hypoesthesia that rapidly spread involving the trunk and upper limbs. MRI showed an extensive T2 hyperintense, tumefactive spinal cord lesion, extending from C2 to the conus and similar lesion in the area postrema. Cerebrospinal fluid analysis showed increased cell count (mononuclear cells) and protein concentration. He resulted positive for Aquaporin-4-IgG (AQP4) antibodies on serum. He was treated with intravenous steroids with mild improvement.

Results

Our patient fulfills the criteria for a “probable PNS”, according to PNS diagnostic criteria, since NMOSD is a “non-classical” PNS and cancer occurred within two years from the diagnosis. It has been recently highlighted that older male patients (>45 years) presenting with longitudinally extensive transverse myelitis or patient with an “area postrema” syndrome at onset have higher risk for neoplasm associated NMOSD. Appropriate tumor screening should be always performed in patients with the aforementioned clinical features.
Since prostatic cells express AQP4, we hypothesize that AQP4-IgG could be part of the immune response against cancer cells or alternatively that radiotherapy could have led to a break of tolerance against AQP4, given the close temporal relationship with disease onset.

Conclusions

Paraneoplastic NMOSD is a rare disease that is becoming more frequently recognized. Further studies should elucidate the immunological relationship between cancer and AQP4-IgG.

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