Longitudinally extensive transverse myelitis (LETM) is a condition shown to confer high risk of conversion into neuromyelitis optica (NMO). Increasing evidence from immunological and histopathological studies suggests that LETM is an autoimmune disorder caused by pathogenic antibodies to aquaporin-4 (AQP4-Ab), the most abundant water channel in the CNS, at least in a subset of patients. However, cases of infectious or parainfectious NMO/LETM (mostly associated with herpes zoster) have been repeatedly reported in the previous literature, raising the question of aetiological diversity in NMO/LETM. Here we present a case of acute LETM in a 63-year-old patient occurring two weeks after reactivation of varicella zoster virus (VZV). Serological testing revealed antibodies to AQP4. Plasma exchange was paralleled by disappearance of AQP4-Ab and sustained clinical improvement. Our observations provide further evidence for a pathogenic role of AQP4-Ab in LETM and suggest that AQP4-Ab associated auto-immunity should be considered also in apparently infectious/parainfectious settings.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Journal of the Neurological Sciences
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- The spectrum of neuromyelitis optica.Lancet Neurol. 2007; 6: 805-815
- Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis.Ann Neurol. 2006; 59: 566-569
- Revised diagnostic criteria for neuromyelitis optica.Neurology. 2006; 66: 1485-1489
- Clinical viral infections and multiple sclerosis.Lancet. 1985; 1: 1313-1315
- Viral infections trigger multiple sclerosis relapses: a prospective seroepidemiological study.J Neurol. 1993; 240: 417-422
- Neuromyelitis optica and longitudinally extensive transverse myelitis following thymectomy for myasthenia gravis.Mult Scler. 2007; 13: P534
- Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis.Arch Neurol. 2008; 65: 913-919
- Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica.J Neurol. 2004; 251: 47-52
- The clinical course of neuromyelitis optica (Devic's syndrome).Neurology. 1999; 53: 1107-1114
- Neuromyelitis optica (Devic's disease) following chicken pox.Trop Doct. 1994; 24: 75-76
- Devic's neuromyelitis optica and varicella.J Neurol. 1993; 240: 450-451
- Neuromyelitis optica following CMV primo-infection.J Intern Med. 2007; 261: 500-503
- Post-dengue neuromyelitis optica: case report of a Japanese-descendent Brazilian child.J Infect Chemother. 2006; 12: 396-398
- Neuromyelitis optica (Devic disease) following varicella infection.J Pediatr. 1979; 95: 737-738
- NMO-IgG/Aqp4-Ab belongs to the IgG1 subclass and activates complement in vitro.Mult Scler. 2007; 13: P533
- Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica.Neurology. 2007; 69: 2221-2231
- Mechanisms of Disease: aquaporin-4 antibodies in neuromyelitis optica.Nat Clin Pract Neurol. 2008; 4: 202-214
Accepted: August 15, 2008
Received in revised form: August 13, 2008
Received: May 26, 2008
© 2008 Elsevier B.V. Published by Elsevier Inc. All rights reserved.