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Abstract Details

Neuroimaging Correlates of Longitudinally Extensive Transverse Myelitis in AQP4-IgG Positive and AQP4-IgG Negative Patients
MS and Related Diseases
P02 - (-)
154
BACKGROUND: LETM is a characteristic feature of neuromyelitis optica (NMO), but it can also occur in several other inflammatory diseases of the Central Nervous System. Early recognition of NMO spectrum disorders is of critical importance because appropriate therapy can prevent disease relapses and the associated severe disability.
DESIGN/METHODS: in this retrospective study with longitudinal follow-up we performed a chart and neuroimaging review of patients with a first episode of LETM admitted at the Multiple Sclerosis Center of our institution between 2006 and 2011. Sera obtained from all patients were tested for AQP4-IgG by a cell-based assay.
RESULTS: Clinical record review identified 31 patients (21 females, 10 males) with a first-ever episode of LETM. The follow-up mean was 27 months (range 18-51). Fourteen patients were diagnosed as having NMO, fulfilling the Wingerchuck et al. 2006 criteria, and 3 patients had a diagnosis of NMO Spectrum Disorder (NMOSD) (relapsing LETM and AQP4-IgG seropositivity) at the last follow-up. Eleven NMO patients resulted AQP-4 IgG positive while 3 were negative. Among AQP4-IgG negative patients without NMO (14) the diagnoses at the last follow-up were: monophasic LETM, in 7 patients, relapsing LETM in 5, paraneoplastic myelopathy in one and sarcoidosis in one. We did not find any difference in the frequency of cervical or thoracic cord involvement between AQP4-IgG positive and AQP4-IgG negative patients. MRI of the spinal cord and the brainstem revealed a contiguous inflammatory lesion extending into the medulla and involving the area postrema in 12/14 (85%) of the AQP4-IgG positive patients. Involvement of the area postrema was not observed in any of the AQP4-IgG negative LETM patients (p=<0.0001).
CONCLUSIONS: involvement of the area postrema in the setting of LETM is highly specific for AQP4-IgG positive NMO.
Authors/Disclosures

PRESENTER
No disclosure on file
Domenico Plantone No disclosure on file
Valentina Damato (University of Florence) Valentina Damato has nothing to disclose.
Jeffrey J. Raizer, MD No disclosure on file
No disclosure on file
Giovanni Frisullo, PhD (Univ Catt S Cuore Polic Gemelli) Dr. Frisullo has nothing to disclose.
Anna P. Batocchi No disclosure on file