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.
"Regarding patients with a first event involving an isolated spinal cord lesion, it is well recognized that partial myelitis is highly predictable of progression to MS
[15,25]. Previous studies in IATM have reported a conversion rate of 0%
[14,18] to 11.4%
, with a mean follow-up ranging from 2 to 4.8 years. "
[Show abstract][Hide abstract] ABSTRACT: In 2002, the Transverse Myelitis Consortium Working Group (TMCWG) proposed the diagnostic criteria for idiopathic acute transverse myelitis (IATM) to delimit and unify this group of patients. This study aimed to describe the conversion rate to multiple sclerosis (MS) and variables associated with conversion, and to analyze functional outcome and prognostic factors associated with functional recovery in patients who fulfilled the current TMCWG criteria for definite and possible IATM.
Eighty-seven patients diagnosed with IATM between 1989 and 2011 were retrospectively reviewed. Two patients with positive neuromyelitis optica IgG serum antibodies were excluded. Epidemiological, clinical, laboratory, magnetic resonance imaging (MRI) data and outcome of 85 patients were analyzed.
Eleven (13%) patients converted to MS after a median follow-up of 2.9 years (interquartile range 1.0-4.8). Early-age onset of symptoms was related to conversion to MS. Only 9.4% of patients with IATM were unable to walk unassisted at the end of follow-up. Urinary sphincter dysfunction (odds ratio [OR] 3.37, 95% confidence interval [CI] 1.04-10.92) and longitudinally extensive transverse myelitis (LETM) on MRI (OR 12.34, 95% CI 3.38-45.00) were associated with a poorer outcome (Rankin >= 2).
At least 13% of patients who fulfill the TMCWG criteria for definite and possible IATM will convert to MS. Functional recovery in IATM is poorer in patients with urinary sphincter dysfunction at admission or LETM on MRI.
"This is likely due to the small number of overall patients in each group. In patients with spinal cord CIS, conversion to MS has been reported to vary between 41% and 61% [33, 34]. The proportion of patients with brainstem syndromes who develop MS varies between 53% and 60% . "
[Show abstract][Hide abstract] ABSTRACT: Background. Clinically isolated syndrome (CIS) is the first neurologic episode of multiple sclerosis (MS). Magnetic resonance imaging (MRI) and clinical features are used to predict risk of conversion to MS.
Objectives. The aim of this prospective study is to evaluate predictors of conversion of CIS to McDonald MS.
Method. 97 patients with CIS have been followed for 2 years. Age of onset, gender, initial clinical presentation, and MRI brain and spine were assessed. The 2010 revised McDonald criteria were applied.
Results. Fifty-nine patients (60.8%) with CIS converted to McDonald MS after 10.1 + 4.2 months. Thirty-seven (38.1%) of the convertors satisfied the diagnostic criteria based on the radiological parameters, while 21.7% sustained their second clinical events. A multivariate regression analysis revealed that high number of lesions in MRI (P = 0.001) and earlier age of onset (P = 0.043) predicted the conversion of CIS to McDonald MS. Gender (P = 0.5) and initial clinical presentation (optic pathway (P = 0.4), supratentorial (P = 0.91), brain stem/ cerebellum (P = 0.97), and spinal (P = 0.76)) were not statistically significant.
Conclusion. Age of onset and MRI parameters can be used as predictors of CIS conversion to McDonald MS. Application of the 2010 revised McDonald criteria allows an earlier MS diagnosis.
[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.
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