Clinically isolated acute transverse myelitis: prognostic features and incidence
ABSTRACT Demyelinating acute transverse myelitis may be the first presentation of multiple sclerosis or remain a clinically isolated syndrome. North Canterbury, New Zealand provides a well circumscribed population to study acute transverse myelitis. Objective: to identify prognostic features, clinical outcomes and incidence of ATM in North Canterbury, New Zealand. All patients with acute transverse myelitis as a first neurological presentation diagnosed from January 2001 to December 2005 at a single institution providing all neurological care for North Canterbury were assessed for clinical data, MRI findings, cerebrospinal fluid results and clinical outcomes. CHAMPS, Barkhof/Tintore and Swanton criteria were applied to brain MRI. Sixty-one patients were identified with a mean duration of follow-up of 30 +/- 17 months. Fifty percent of patients with ATM with brain lesions by CHAMPS criteria converted to clinically definite multiple sclerosis. No patients with idiopathic acute transverse myelitis converted to clinically definite multiple sclerosis. There was a strong association with conversion to clinically definite multiple sclerosis and abnormal brain MRI by CHAMPS criteria (hazard ratio, 5.63; 1.83-17.3), Barkhof/Tintore criteria (hazard ratio, 6.43; 2.31-17.9) and Swanton criteria (hazard ratio, 4.53; 1.67-12.3). The age standardized annual incidence of acute transverse myelitis was 24.6 (18.2-31.1) per million, of definite and possible idiopathic acute transverse myelitis was 6.2 (2.9-9.6) per million, and of acute transverse myelitis with brain lesions was 4.7 (1.9-7.6) per million. Patients with idiopathic acute transverse myelitis are at low risk for conversion to clinically definite multiple sclerosis. Abnormal brain MRI by CHAMPS criteria is a sensitive predictor of conversion to clinically definite multiple sclerosis. The annual incidence of acute transverse multiple sclerosis in North Canterbury, New Zealand is significantly higher than previously reported.
Article: Transverse myelitis.[Show abstract] [Hide abstract]
ABSTRACT: Acute transverse myelitis (ATM) is an etiologically heterogeneous syndrome with acute or subacute onset, in which inflammation of the spinal cord results in neurologic deficits, manifesting as weakness, sensory loss and autonomic dysfunction. It is frequently associated with infectious or systemic autoimmune diseases, but its etiology remains unknown in a substantial portion of cases, which are classified as idiopathic. Unifying diagnostic criteria for idiopathic and disease-associated ATM were proposed in 2002. Although they have been applied to a few cohorts of patients, the limited information provided in the relevant publications has not yet yielded many new insights on the clinical characteristics, disease course, and outcome of adult patients with idiopathic ATM compared to older studies that did not always distinguish between the various etiologies of ATM. There is, however, some new epidemiological data indicating that the incidence of idiopathic ATM is considerably higher, and the female preponderance greater, than previously recognized. In addition, new data on children with ATM show that the prognosis in pediatric patients is not always as benign as previous studies had indicated. The combination of ATM and optic neuritis characterize Devic's syndrome or neuromyelitis optica (NMO). A seminal discovery was the identification of an antibody that is a specific marker not only for NMO, but also of some of its characteristic manifestations in isolation, including longitudinally extensive TM. This has resulted in the proposal that all of the disorders that are associated with NMO-IgG positivity constitute part of an NMO spectrum of disorders. This antibody recognizes aquaporin-4, which represents the most abundant water channel of the central nervous system. There is growing evidence that the antibodies targeting this channel protein have pathogenic potential, thereby providing insights into the possible pathogenetic mechanisms of at least one type of ATM.Autoimmunity reviews 05/2011; 11(3):231-48. DOI:10.1016/j.autrev.2011.05.018 · 7.10 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins.DNA research: an international journal for rapid publication of reports on genes and genomes 09/2011; 9(3):417-28. DOI:10.2174/157015911796557948 · 2.35 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Although multiple sclerosis (MS) is the most common demyelinating disorder of the central nervous system (CNS), it lacks any definitive diagnostic test. Instead, diagnosis of MS primarily depends upon clinical criteria, supported by abnormalities characteristic of MS on para-clinical investigations including magnetic resonance imaging of the brain and spine, in the absence of an alternative explanation for underlying neurologic symptoms. While many of the potential disorders that may mimic MS in routine clinical practice are either extremely rare, or associated with specific and characteristic distinguishing diagnostic features, some inflammatory demyelinating disorders of the CNS may be particularly challenging to distinguish from MS, especially during initial presentation. In particular, acute disseminated encephalomyelitis, neuromyelitis optica, and idiopathic transverse myelitis may closely resemble MS, impeding prompt and accurate diagnosis. In this review, we describe the clinical features, diagnosis, pathology, and treatment of these other CNS demyelinating disorders. In addition, we review relevant features of other CNS inflammatory disorders that may mimic MS, including Sjögren's syndrome, systemic lupus erythematosus, Behçet's disease, and primary CNS vasculitis.Journal of Neurology 09/2011; 259(5):801-16. DOI:10.1007/s00415-011-6240-5 · 3.84 Impact Factor