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Devic neuromyelitis optica (DNMO) is a severe demyelinating disease of the central nervous system (CNS). In 2004, Lennon et al described a new antibody, NMO–immunoglobulin G (IgG), which is highly specific (>90%) and moderately sensitive (approximately 50%–80%) for patients with DNMO. NMO-IgG reacts with the water channel protein aquaporin 4 (AQP4), which is expressed in astrocytic foot processes. The precise mechanism by which NMO-IgG acts is not known, but complement activation leading to astrocytic necrosis has been shown. In this context, the description of several magnitudes of increased concentration of cerebrospinal fluid (CSF) glial fibrillar acidic protein (GFAP) in DNMO is intriguing.1 GFAP is a protein biomarker specific for astrocytosis and gliosis.2 In multiple sclerosis (MS), CSF GFAP levels were only moderately elevated (see references in Petzold et al2) compared with the findings in DNMO.1 This observation raises the question whether CSF GFAP levels have the potential to become an additional, sensitive diagnostic tool for diagnosing DNMO.
Patients and methods
This retrospective study included 188 patients from three centres (Nijmegen, London and Lyon). The study was approved by the local institutional review board, written informed consent was obtained and samples were exchanged using material transfer agreements.
The inclusion criteria for DNMO and high-risk syndromes for DNMO (n=7, Lyon) were as published by Wingerchuk et al in 2007. The inclusion criteria for MS were the 2001 McDonald criteria (n=14, Nijmegen). All the other patients (London) were classified clinically into acute optic neuritis (n=37), myelitis (n=6), benign intracranial hypertension (n=3), unspecified memory problems (n=7), epilepsy (n=6), patients requiring extraventricular drainage for treatment of hydrocephalus (n=6), stroke (n=6), tension-type headache (n=11), encephalitis (n=30), autoimmune-mediated CNS disease (vasculitis with neurological complications, sarcoidosis, stiff-man syndrome and paraneoplastic disorders, n=32), parkinsonian syndromes (n=8), polyneuropathy in …