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: Clinically isolated syndromes (CIS) indicate the possibility of developing multiple sclerosis (MS) over time in approximately 20-85% of the cases. Thus, accurately identifying which patients will present a second demyelinating episode and determining the degree of disability they could develop over the mid- to long term is considered crucial for a more individualized treatment. For this reason, a number of prognostic markers have been studied in an attempt to identify those that could provide additional information about the disease course. This review focuses only on markers with proven predictive power in CIS patients in the everyday clinical practice. In general, markers of conversion to clinically definite MS (CDMS) are more robust than those available for disability progression. More specifically, magnetic resonance imaging is, to this day, the most powerful tool for predicting both conversion to CDMS and disability progression in the mid-term. Other useful markers include age of onset and presence of oligoclonal bands in cerebrospinal fluid. Identifying a practical marker that improves the prognostic value of the available tools remains an unmet need.Journal of the Neurological Sciences. 01/2014;
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ABSTRACT: The purpose of this study was to determine the incidence of clinically isolated syndrome (CIS), a potential precursor of multiple sclerosis (MS), and whether it varies by race/ethnicity in a multi-ethnic, population-based cohort. We conducted a retrospective cohort study of over 9 million person-years of observation from the multi-ethnic, community-dwelling members of Kaiser Permanente Southern California Health Plan from January 1, 2008 to December 31, 2010. Incidence of CIS and risk ratios comparing incidence rates between racial/ethnic groups were calculated using Poisson regression. We identified 468 newly diagnosed CIS cases that did not meet McDonald criteria for MS. The average age at diagnosis was 39.0 years (range 2.7-85.8) and 68.8 % were women. The female preponderance was more pronounced among black (75.7 %) and Hispanics (70.5 %) than in white and Asian individuals with CIS (66.5 and 54.5 %, respectively; P = 0.14). The most common presenting symptom in Hispanics was optic neuritis (P = 0.008), and in blacks, transverse myelitis (P = 0.07). Incidence of CIS was lower in Hispanics (3.8, 95 % CI 3.2-4.4, P < 0.0001) and Asians (2.4, 95 % CI 1.5-3.6, P < 0.0001) and similar in blacks (6.8, 95 % CI 5.3-8.5, P = 0.30) compared with whites (5.9, 95 % CI 5.1-6.7). The incidence of CIS varies by race/ethnicity and sex in a similar pattern to MS. In addition, the clinical presentation of CIS varies by race/ethnicity. These findings strengthen the probability that the old belief that blacks have a decreased risk of MS is no longer true. These findings highlight that studies that include minorities are likely to lead to important insights into the etiology and prognosis of CIS and MS.Journal of Neurology 04/2014; · 3.58 Impact Factor
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ABSTRACT: Background: The first episode of central nervous system (CNS) symptoms with a presumed inflammatory demyelinating cause is defined as clinically isolated syndrome (CIS) according to the 2007 consensus of the International Pediatric Multiple Sclerosis Study Group, which developed diagnostic criteria for CNS demyelination disease in children. Using this definition of CIS, we attempted to identify the natural course of pediatric patients with CIS in a single Korean institution and to determine the factors affecting their prognosis. Methods: We retrospectively reviewed the medical records of all pediatric patients (age <18 years old) who presented with clinical symptoms of CNS events between 1997 and 2008. Results: We identified 32 patients with CIS. Their mean age with standard deviation was 10.0 ± 4.1 years. The most common type of presentation of CIS was optic neuritis (ON). Sixteen (16/32, 50%) patients experienced a second demyelinating event. The mean interval between the first event and the recurrent episode was 21 ± 20 months. The mean follow-up was 6.1 ± 1.6 years. Eleven (34%) patients developed childhood onset multiple sclerosis (MS). In contrast to previous studies, asymptomatic brain lesions on magnetic resonance imaging (MRI) and the presence of cerebrospinal fluid (CSF) oligoclonal bands (OCBs) were not predictors of conversion to MS. Conclusion: In our study, a second relapse and initial presentation with brain stem, cerebellar, cerebral dysfunction, or multifocal CIS were strongly associated with the development of MS (p = 0.002). Despite clinical definitions and increased understanding of CIS in children, challenges remain in predicting its progression to a chronic demyelinating disease.Brain and Development. 01/2014;