Devic's disease with serological confirmation [Doença de Devic com Confirmação Serológica]

• Type: Article in International Scientific Journal
• Date: 2006

Authors

Joana Guimaraes (Author) FMUP; Wingerchuk, D (Author) Other; Sa, MJ (Author) Other

Journal

Title: Sinapse

Vol. 6

Pages: 69-74

ISSN: 1645-281X

Publisher: Sociedade Portuguesa de Neurologia

Indexing

Scopus - 4 Citations

Other information

Authenticus ID: P-007-FR4

Abstract (EN): Introduction: In Devic's disease (Neuromyelitis Optica, NMO), a rare demyelinating disease of CNS, there have been several proposed specific and precise diagnostic criteria; however, the clinical spectrum of presentation and the variability of the clinical course, make, sometimes, this diagnosis difficult. The detection of NMO-IgG, a specific marker autoantibody of NMO, is a recently described serological study that allows a precise diagnosis and makes NMO distinguishable from other neuroimmunological disorders. Case report: A 48 year-old woman wa s admitted at our Department in 2003, because of sudden right crural paresis; neurological examination showed a degree 2 monoparesis, bilateral deep and superficial hypoesthesia, with a level by T7. She had a past history of left optic neuritis 10 years ago. Cervical spinal MRI revealed cord widening, with central hypointensity on T1 and hyperintensity on T2, from C4 to L1. Cerebral MRI disclosed unspecific T2 hyperintense lesions in the white matter. The cerebrospinal fluid (CSF) study showed increased protein and positive IgG oligoclonal bands. She did a pulse of i.v. MP with partial recovery and then a pulse of i.v. immunoglobulins (IVIG) with progressive motor improvement. Spinal MRI, one moth later, showed reduction of the central hyperintensity signal. The patient was discharged to the MS Outpatient Clinic with the diagnosis of multiple sclerosis (MS). Five months later she had sudden clinical worsening, with paraparesis and urinary retention, and the imaging results were similar. She started glatiramer acetate and IVIG, with progressive clinical recovery. In 2004, she developed a right optic neuritis. Since 2005 she got progressively worse, with spastic paraparesis, and stays wheel-chair bounded. Oral azathioprine was added and we decided to make the determination of serum NMO-IgG. This antibody was positive and allowed NMO diagnosis. Conclusion: In this patient, the initial clinica l picture, the CSF changes and the cerebral MRI findings favoured the diagnosis of MS. However, the clinical evolution and the imaging aspects of the spinal cord raised diagnostic doubts. We would like to reinforce that the serological NMO-IgG specific detection was crucial to settle the definitive diagnosis, allowing the reformulation of the treatment and prognosis.

Language: Portuguese

Type (Professor's evaluation): Scientific