Articles from Neurology

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Callen, D J.A. ; Shroff, M M. ; Branson, H M. ; Li, D K. ; Lotze, T ; Stephens, D; Banwell, B L.
Role of MRI in the differentiation of ADEM from MS in children.
Neurology.
Status
Publish Ahead of Print, POST AUTHOR CORRECTIONS, 26 November 2008
Abstract
Background: Acute disseminated encephalomyelitis (ADEM) is typically a monophasic demyelinating disorder. However, a clinical presentation consistent with ADEM can also be the first manifestation of multiple sclerosis (MS), particularly in children. Quantitative analyses of MRI images from children with monophasic ADEM have yet to be compared with those from children with MS, and MRI criteria capable of distinguishing ADEM from MS at onset have yet to be derived.Methods: A retrospective analysis of MRI scans obtained at first attack from 28 children subsequently diagnosed with MS and 20 children with ADEM was performed. T2/fluid-attenuated inversion recovery hyperintense lesions were quantified and categorized according to location, description, and size. T1-weighted images before and after administration of gadolinium were evaluated for the presence of black holes and for gadolinium enhancement. Mean lesion counts and qualitative features were compared between groups and analyzed to create a proposed diagnostic model.Results: Total lesion number did not differentiate ADEM from MS, but periventricular lesions were more frequent in children with MS. Combined quantitative and qualitative analyses led to the following criteria to distinguish MS from ADEM: any two of 1) absence of a diffuse bilateral lesion pattern, 2) presence of black holes, and 3) presence of two or more periventricular lesions. Using these criteria, MS patients at first attack could be distinguished from monophasic ADEM patients with an 81% sensitivity and a 95% specificity.Conclusions: MRI diagnostic criteria are proposed that may be useful in differentiating children experiencing the first attack of multiple sclerosis from those with monophasic acute disseminated encephalomyelitis.GLOSSARY: ADEM = acute disseminated encephalomyelitis; CIS = clinically isolated syndrome; FLAIR = fluid-attenuated inversion recovery; KIDMUS CC = lesions perpendicular to the long axis of the corpus callosum; KIDMUS discrete = sole presence of well-defined lesions; MS = multiple sclerosis; NA = not applicable; NPV = negative predictive value; ON = optic neuritis; OR = odds ratio; PPV = positive predictive value; RRMS = relapsing-remitting multiple sclerosis; TM = transverse myelitis

Callen, D J.A. MD, PhD; Shroff, M M. MD; Branson, H M. MD; Lotze, T MD; Li, D K. et al
MRI in the diagnosis of pediatric multiple sclerosis.
Neurology.
Status
Publish Ahead of Print, POST AUTHOR CORRECTIONS, 26 November 2008
Abstract
Background: MRI diagnostic criteria have not yet been adopted for pediatric multiple sclerosis (MS). MRI plays a pivotal role in supporting the diagnosis of MS in adults. We sought to quantitatively define the MRI features of pediatric MS, to determine features that distinguish MS from nondemyelinating relapsing childhood neurologic disorders, and to propose MRI criteria for lesion dissemination in space in children with MS.Methods: A retrospective analysis of MRI scans from 38 children with clinically definite MS and 45 children with nondemyelinating diseases with relapsing neurologic deficits (migraine, systemic lupus erythematosus) was performed. For each scan, T2/FLAIR hyperintense lesions were quantified and categorized according to location and size. Mean lesion counts in specific locations were compared between groups to derive diagnostic criteria. Validation of the proposed criteria was performed using MRI scans from a second independent MS cohort (n = 21).Results: MRI lesion location and size categories differed between children with MS and nondemyelinating controls with a medium to large effect size for most variables. The presence of at least two of the following-five or more lesions, two or more periventricular lesions, or one brainstem lesion-distinguished MS from other nondemyelinating disease controls with 85% sensitivity and 98% specificity.Conclusions: We propose modifications to the currently established McDonald MRI criteria for lesion dissemination in space that will enhance the diagnostic accuracy of these criteria for multiple sclerosis in children.GLOSSARY: ADEM = acute disseminated encephalomyelitis; CDMS = clinically definite MS; MS = multiple sclerosis; OND = other non-demyelinating neurologic diseases; SLE = systemic lupus erythematosus

Okuda, D T. MD; Mowry, E M. MD; Beheshtian, A MD; Waubant, E ; Baranzini, S E. et al
Incidental MRI anomalies suggestive of multiple sclerosis: The radiologically isolated syndrome
Neurology.
Status
Publish Ahead of Print, POST AUTHOR CORRECTIONS, 10 December 2008
Abstract
Background: The discovery and broad application of MRI in medicine has led to an increased awareness in the number of patients with incidental white matter pathology in the CNS. Routinely encountered in clinical practice, the natural history or evolution of such individuals with respect to their risk of developing multiple sclerosis (MS) is unclear.Objective: To investigate the natural history of patients who exhibit incidental imaging findings highly suggestive of MS pathology.Methods: Detailed clinical and radiologic data were obtained from asymptomatic patients with MRI anomalies suggestive of MS.Results: The cohort consisted of 41 female and 3 male subjects (median age = 38.5, range: 16.2-67.1). Clinical evaluations were performed in 44 patients at the time of initial imaging; longitudinal clinical follow-up occurred for 30 patients, and longitudinal MRI data were acquired for 41 patients. Neurologic examination at the time of the initial MRI scans was normal in nearly all cases. While radiologic progression was identified in 59% of cases, only 10 patients converted to either clinically isolated syndrome or definite MS. The presence of contrast-enhancing lesions on the initial MRI was predictive of dissemination in time on repeat imaging of the brain (hazard ratio [HR] = 3.4, 95% confidence interval [1.3, 8.7], p = 0.01).Conclusion: Individuals with MRI anomalies highly suggestive of demyelinating pathology, not better accounted for by another disease process, are very likely to experience subsequent radiologic or clinical events related to multiple sclerosis. Additional studies will be necessary to fully define this risk.(C)2009AAN Enterprises, Inc.

Daumer, M PhD; Neuhaus, A MSc; Morrissey, S MD; Hintzen, R MD; Ebers, G C. MD;
MRI as an outcome in multiple sclerosis clinical trials.
Neurology.
Status
Publish Ahead of Print, POST AUTHOR CORRECTIONS, 10 December 2008
Abstract
Introduction: T2-weighted and gadolinium enhanced T1-weighted MRI scans measure plaque burden and breakdown of the blood-brain barrier, respectively, in multiple sclerosis (MS) lesions. These have become widely used outcome measures for monitoring disease activity in clinical trials and clinical practice. However, their use as surrogates or biomarkers for disability and relapses, key clinical outcome measures, has remained incompletely validated.Methods: In a clinical trial database comprised of 31 relapsing-remitting and secondary progressive MS trial placebo groups, we assessed relationships between 1) T2 lesion load (TLL) change and disability change and 2) gadolinium enhancement of MS lesions and on-study relapses with univariate and multivariate analyses.Results: In relapsing-remitting MS, TLL change (n = 223) made no independent contribution to predicting change in disability from baseline to trials' end. Similarly, inclusion of gadolinium enhancing lesions (n = 170) into multivariate models did not independently contribute to the predictive value for on-trial relapses. In secondary progressive MS, a small effect of TLL was found for disability change (n = 355) but in multivariate analysis this accounted for less than 5% of the variance in end-of-trial disability. Results were replicated in independent datasets, more than doubling effective sample sizes.Conclusions: MRI measures widely used in trials of relapsing-remitting and progressive multiple sclerosis add little if anything independently to the clinically relevant relapse and disability outcomes. These results reemphasize the importance of validating potential surrogate markers against clinical measures and highlight the need for better MRI markers of disease activity and progression.

Mlynash, M MD, MS; Olivot, J H. MD, PhD; Tong, D C. MD, FAHA; Lansberg, M G.
Yield of combined perfusion and diffusion MR imaging in hemispheric TIA.
Neurology.
Status
Publish Ahead of Print, POST AUTHOR CORRECTIONS, 17 December 2008
Abstract
Objective: Transient ischemic attacks (TIA) predict future stroke. However, there are no sensitive and specific diagnostic criteria for TIA and interobserver agreement regarding the diagnosis is poor. Diffusion-weighted MRI (DWI) demonstrates acute ischemic lesions in approximately 30% of TIA patients; the yield of perfusion-weighted MRI (PWI) is unclear.Methods: We prospectively performed both DWI and PWI within 48 hours of symptom onset in consecutive patients admitted with suspected hemispheric TIAs of <24 aca =" anterior" ci =" confidence" dwi =" diffusion-weighted" ica =" internal" mca =" middle" mra =" magnetic" mtt =" mean" or =" odds" pca =" posterior" pwi =" perfusion-weighted" rr =" risk" tia =" transient" toast =" Trial">

Fellgiebel, A MD, PhD; Keller, I MD; Marin, D; Muller, M J. MD, PhD; Schermuly, I et al
Diagnostic utility of different MRI and MR angiography measures in Fabry disease.
Source
Neurology. 72(1):63-68, January 6, 2009.
Abstract
Background: Neurologic hallmarks of Fabry disease (FD) include small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. To unravel the most promising imaging tool for the detection of CNS involvement in FD we compared the diagnostic utility of the different MR imaging findings.Methods: Twenty-five clinically affected patients with FD (age 36.5 +/- 11.0) and 20 age-matched controls were investigated by structural MRI, MR angiography, and diffusion tensor imaging (DTI). Individual WML volumes, global mean diffusivity (MD), and mean cerebral artery diameters were determined.Results: Using receiver operating characteristic analyses, enlarged diameters of the following cerebral arteries significantly separated patients with FD from controls: middle cerebral artery: area under curve (AUC) = 0.75, p = 0.005; posterior cerebral artery: AUC = 0.69, p = 0.041; carotid artery: 0.69, p = 0.041; basilar artery: AUC = 0.96,