1.
Heterogeneity of Multiple Sclerosis White Matter Lesions Detected With T2*-Weighted Imaging at 7.0 Tesla.
Yao, B, Ikonomidou, VN, Cantor, FK, Ohayon, JM, Duyn, J, Bagnato, F
Journal of neuroimaging : official journal of the American Society of Neuroimaging. 2015;(5):799-806
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Abstract
BACKGROUND AND PURPOSE Postmortem studies in multiple sclerosis (MS) indicate that in some white matter lesions (WM-Ls), iron is detectable with T2*-weighted (T2*-w), and its reciprocal R2* relaxation rate, magnetic resonance imaging (MRI) at 7.0 Tesla (7T). This iron appears as a hyperintense rim in R2* images surrounding a hypointense core. We describe how this observation relates to clinical/radiological characteristics of patients, in vivo. METHODS We imaged 16 MS patients using 3T and 7T scanners. WM-Ls were identified on T1-w / T2-w 3T-MRIs. Thereafter, WM-Ls with a rim of elevated R2* at 7T were counted and compared to their appearance on conventional MRIs. RESULTS We counted 36 WM-Ls presenting a rim of elevated R2* in 10 patients. Twenty-three (64%) lesions coincided with focal WM-Ls on T2-w MRIs; 13 (36%) coincided with only portions of larger lesions on T2-w images; and 20 (56%) corresponded to a hypointense chronic black hole. WM-Ls presenting a rim of elevated R2* were seen in both relapsing-remitting patients with low disability and in those with long-standing secondary progressive MS. CONCLUSIONS WM-Ls with a contour of high R2* are present at different MS stages, potentially representing differences in the contribution of iron in MS disease evolution.
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The physiological basis of conduction slowing in ALS patients homozygous for the D90A CuZn-SOD mutation.
Weber, M, Eisen, A, Stewart, HG, Andersen, PM, Hirota, N
Muscle & nerve. 2001;(1):89-97
Abstract
Familial amyotrophic lateral sclerosis (ALS) with the autosomal-recessively inherited D90A CuZn-superoxide dismutase (CuZn-SOD) mutation is characterized by a stereotypic slowly progressive, distinctive phenotype and very slow central motor conduction. To determine the basis of this slowing, we assessed corticomotoneuronal function using peristimulus time histograms (PSTHs) in 8 ALS patients homozygous for the D90A CuZn-SOD mutation. The results were compared with findings in 10 patients with multiple sclerosis (MS), in which slowing of central motor conduction is common, and 11 healthy subjects. PSTHs were constructed from 3-7 different, voluntarily recruited motor units recorded in each patient from the extensor digitorum communis muscle (EDC). In D90A and MS patients, the stimulus threshold, onset latency, number of excess bins, duration, amplitude, and synchrony of the primary peak differed significantly from controls (P < 0.0004). The mean onset latency of the primary peak in D90A patients was 35.3 ms, compared to 23.6 ms for MS patients and 19.3 ms for normal subjects (P < 0.0001). In the D90A patients, the onset latencies of the primary peak had a bimodal distribution, whereas in MS the distribution showed a continuum. Loss of synchrony was similar in D90A and MS patients, but the threshold, number of excess bins, and duration differed significantly (P < 0.0057), which suggests that either axonal loss or demyelination can result in delayed and desynchronized primary peaks. We propose that conduction slowing in the D90A homozygotes results from selective loss of fast-conducting large pyramidal cells with preservation of slow-conducting mono- or polysynaptic corticomotoneuronal connections.