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.
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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.ISRN neurology. 12/2012; 2012:792192.
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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.BMC Neurology 10/2013; 13(1):135. · 2.56 Impact Factor
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ABSTRACT: The term "acute transverse myelitis (ATM)" comprises various non-traumatic disorders that eventually can be associated with a focal myelopathy. Patients characteristically present with an acutely occurring paraparesis/plegia and require a comprehensive and timely diagnostic work up for the initiation of an appropriate treatment. We present a case of a 36-year-old female patient with a rare genetic disorder (ANE1: Acute Necrotizing Encephalopathy due to a RANBP2 mutation) who presented with an acute quadriplegia. Following an acute pulmonal infection, she rapidly (< 24 h) developed a severe quadriplegia (total motor score 38) with some facial sensory symptoms (perioral hypoesthesia). Magnetic resonance imaging (MRI) revealed a combination of longitudinal extensive transverse myelitis and symmetrical thalamic lesions. A work-up for infectious and systemic diseases was negative; specifically, no findings related to multiple sclerosis, neuromyelitis optica or vascular disorders. After empirical high dose steroid treatment and rehabilitation therapy, the patient gained almost normal gait and upper limb function. She was found to carry an autosomal-dominant missense mutation in the RANBP2 gene predisposing for ANE. Gene segregation was confirmed in other family members that had been affected by other episodes of acute steroid-responsive encephalopathies. We propose that a redefined diagnostic workup of ATM might include ANE1, as the frequency of this rare disorder might be underestimated.Journal of Neurology 01/2013; · 3.58 Impact Factor