The role of cerebellar abnormalities in neuromyelitis optica – a comparison with multiple sclerosis and healthy controls

2014 ◽  
Vol 21 (6) ◽  
pp. 757-766 ◽  
Author(s):  
Katrin Weier ◽  
Arman Eshaghi ◽  
Stefano Magon ◽  
Michaela Andelova ◽  
Ernst-Wilhelm Radue ◽  
...  

Background: In relapsing–remitting multiple sclerosis (RRMS), the cerebellum is a known predilection site for atrophy. Neuromyelitis optica (NMO) is characterized by extensive lesions in the spinal cord and optic nerve; however, cerebellar involvement has been less studied. Secondary degeneration of the spinocerebellar tract could impact the cerebellum in NMO. Objective: We aimed to investigate whether spinal cord and cerebellar volume measures differ between patients with NMO and RRMS. Methods: Volumetric analyses of the cerebellum (TCV), the upper cervical cord (UCV) as well as the whole brain (NBV) of age- and gender-matched patients with NMO ( n=30; 56% AQP4 +ve) and RRMS ( n=25) were performed on 3T brain magnetic resonance imaging (MRI) and compared with 34 healthy controls (HC). Results: UCV was significantly reduced in NMO patients (6.3 cm3) as compared with HC (6.7 cm3), while patients with MS had reduced brain volumes compared with HC (NBV=1482 cm3; p<0.001; TCV=188 cm3; p=0.042), but UCV close to normal values. Patients with RRMS and NMO differed in NBV ( p=0.001; lower in RRMS) and by trend (towards reduction in RRMS) in cerebellar volume ( p=0.06). Conclusions: While atrophy seems to be diffuse in MS patients, a rather focussed pattern with predominant involvement of the UCV was observed in NMO patients.

2014 ◽  
Vol 20 (14) ◽  
pp. 1860-1865 ◽  
Author(s):  
Marita Daams ◽  
Florian Weiler ◽  
Martijn D Steenwijk ◽  
Horst K Hahn ◽  
Jeroen JG Geurts ◽  
...  

Background: The majority of patients with multiple sclerosis (MS) present with spinal cord pathology. Spinal cord atrophy is thought to be a marker of disease severity, but in long-disease duration its relation to brain pathology and clinical disability is largely unknown. Objective: Our aim was to investigate mean upper cervical cord area (MUCCA) in patients with long-standing MS and assess its relation to brain magnetic resonance imaging (MRI) measures and clinical disability. Methods: MUCCA was measured in 196 MS patients and 55 healthy controls using 3DT1-weighted cervical images obtained at 3T MRI. Clinical disability was measured using the Expanded Disability Status Scale (EDSS), Nine-Hole-Peg test (9-HPT), and 25 feet Timed Walk Test (TWT). Stepwise linear regression was performed to assess the association between MUCCA and MRI measures, and between MUCCA and clinical disability. Results: MUCCA was smaller (mean 11.7%) in MS patients compared with healthy controls (72.56±9.82 and 82.24±7.80 mm2 respectively; p<0.001), most prominently in male patients. MUCCA was associated with normalized brain volume, and number of cervical cord lesions. MUCCA was independently associated with EDSS, TWT, and 9-HPT. Conclusion: MUCCA was reduced in MS patients compared with healthy controls. It provides a relevant marker for clinical disability in long-standing disease, independent of other MRI measures.


2014 ◽  
Vol 21 (7) ◽  
pp. 875-884 ◽  
Author(s):  
Viola Biberacher ◽  
Christine C Boucard ◽  
Paul Schmidt ◽  
Christina Engl ◽  
Dorothea Buck ◽  
...  

Background: Despite agreement about spinal cord atrophy in progressive forms of multiple sclerosis (MS), data on clinically isolated syndrome (CIS) and relapsing–remitting MS (RRMS) are conflicting. Objective: To determine the onset of spinal cord atrophy in the disease course of MS. Methods: Structural brain magnetic resonance imaging (MRI) was acquired from 267 patients with CIS (85) or RRMS (182) and 64 healthy controls (HCs). The upper cervical cord cross-sectional area (UCCA) was determined at the level of C2/C3 by a segmentation tool and adjusted for focal MS lesions. The coefficient of variation (CV) was calculated from all measurements between C2/C3 and 13 mm above as a measure of structural variability. Results: Compared to HCs (76.1±6.9 mm2), UCCA was significantly reduced in CIS patients (73.5±5.8 mm2, p=0.018) and RRMS patients (72.4±7.0 mm2, p<0.001). Structural variability was higher in patients than in HCs, particularly but not exclusively in case of focal lesions (mean CV HCs/patients without/with lesions: 2.13%/2.55%/3.32%, all p-values<0.007). UCCA and CV correlated with Expanded Disability Status Scale (EDSS) scores ( r =−0.131/0.192, p=0.044/<0.001) and disease duration ( r=−0.134/0.300, p=0.039/< 0.001). CV additionally correlated with hand and arm function ( r=0.180, p=0.014). Conclusion: In MS, cervical cord atrophy already occurs in CIS. In early stages, structural variability may be a more meaningful marker of spinal cord pathology than atrophy.


2012 ◽  
Vol 18 (9) ◽  
pp. 1259-1268 ◽  
Author(s):  
Eric C Klawiter ◽  
Junqian Xu ◽  
Robert T Naismith ◽  
Tammie LS Benzinger ◽  
Joshua S Shimony ◽  
...  

Background: Multiple sclerosis (MS) and neuromyelitis optica (NMO) both affect spinal cord with notable differences in pathology. Objective: Determine the utility of diffusion tensor imaging (DTI) to differentiate the spinal cord lesions of NMO from MS within and outside T2 lesions. Methods: Subjects greater than or equal to 12 months from a clinical episode of transverse myelitis underwent a novel transaxial cervical spinal cord DTI sequence. Ten subjects with NMO, 10 with MS and 10 healthy controls were included. Results: Within T2 affected white matter regions, radial diffusivity was increased in both NMO and MS compared with healthy controls ( p<0.001, respectively), and to a greater extent in NMO than MS ( p<0.001). Axial diffusivity was decreased in T2 lesions in both NMO and MS compared with controls ( p<0.001, p=0.001), but did not differ between the two diseases. Radial diffusivity and fractional anisotropy within white matter regions upstream and downstream of T2 lesions were different from controls in each disease. Conclusions: Higher radial diffusivity within spinal cord white matter tracts derived from diffusion tensor imaging were appreciated in NMO compared with MS, consistent with the known greater tissue destruction seen in NMO. DTI also detected tissue alterations outside T2 lesions and may be a surrogate of anterograde and retrograde degeneration.


Neurology ◽  
2020 ◽  
Vol 94 (13) ◽  
pp. e1395-e1406 ◽  
Author(s):  
Rachel Brandstadter ◽  
Oluwasheyi Ayeni ◽  
Stephen C. Krieger ◽  
Noam Y. Harel ◽  
Miguel X. Escalon ◽  
...  

ObjectiveTo test the hypothesis that higher-challenge gait and balance tasks are more sensitive than traditional metrics to subtle patient-reported gait dysfunction and future fall risk in early multiple sclerosis (MS).MethodsPersons with early MS (n = 185; ≤5 years diagnosed) reported gait function (MS Walking Scale) and underwent traditional disability metrics (Expanded Disability Status Scale [EDSS], Timed 25 Foot Walk). Patients and healthy controls (n = 50) completed clinically feasible challenge tasks of gait endurance (2-Minute Walk Test), standing balance (NIH Toolbox), and dynamic balance (balance boards; tandem walk on 2 ten-foot boards of different widths, 4.5 and 1.5 in). MRI assessed global and regional brain volumes, total T2 lesion volume (T2LV), infratentorial T2LVs and counts, and cervical cord lesion counts. Falls, near falls, and fall-related injuries were assessed after 1 year. We examined links between all tasks and patient-reported gait, MRI markers, and fall data.ResultsPatients performed worse on higher challenge balance, but not gait, tasks compared with healthy controls. Worse patient-reported gait disturbance was associated with worse performance on all tasks, but only dynamic balance was sensitive to mild patient-reported gait difficulty. Balance tasks were more correlated with MRI metrics than were walking tasks or EDSS score. Thirty percent of patients reported either a fall or near fall after 1 year, with poor dynamic balance as the only task independently predicting falls.ConclusionsBalance plays a leading role in gait dysfunction early in MS. Clinically feasible higher-challenge balance tasks were most sensitive to patient-reported gait, MRI disease markers, and risk of future falls, highlighting potential to advance functional outcomes in clinical practice and trials.


2019 ◽  
Vol 18 (2) ◽  
pp. 185-197 ◽  
Author(s):  
Olga Ciccarelli ◽  
Jeffrey A Cohen ◽  
Stephen C Reingold ◽  
Brian G Weinshenker ◽  
Maria Pia Amato ◽  
...  

Lupus ◽  
2019 ◽  
Vol 28 (14) ◽  
pp. 1656-1662
Author(s):  
J N Williams ◽  
C B Speyer ◽  
D J Kreps ◽  
D J Kimbrough ◽  
K Costenbader ◽  
...  

Objective Non-infectious myelitis in systemic lupus erythematosus (SLE) may be due to SLE myelitis, comorbid multiple sclerosis (MS), or neuromyelitis optica (NMO). We compared characteristics of these three conditions in SLE patients at a large academic institution. Methods We searched for neurologic diagnoses of SLE myelitis, NMO myelitis, and MS myelitis among 2297 patients with at least four 1997 American College of Rheumatology revised criteria for SLE between 2000 and 2015. Each subject was reviewed by a neurologist to confirm the underlying neurologic diagnosis. Demographic, clinical, laboratory, and radiographic data were extracted and compared using Fisher's exact test, analysis of variance, and Wilcoxon rank-sum test. Results Fifteen of the 2297 subjects with SLE (0.7%) met criteria for a spinal cord syndrome: seven had SLE myelitis, three had AQP4 seropositive NMO, and five had MS. The median SLE Disease Activity Index 2000 score at time of neurologic syndrome presentation was higher in SLE myelitis subjects (8, interquartile range (IQR) 7–16) compared with subjects with NMO (6, IQR 0–14) or MS (2, IQR 0–4), p = 0.02. Subjects with SLE myelitis were also more likely to have elevated anti-dsDNA antibodies at presentation (86%) compared with subjects with NMO (33%) or MS (0%), p = 0.03. Conclusion Myelitis occurs rarely among patients with SLE. Compared with subjects with SLE + NMO and subjects with SLE + MS, subjects with SLE myelitis had higher SLE disease activity at presentation.


2012 ◽  
Vol 81 (10) ◽  
pp. 2697-2701 ◽  
Author(s):  
Fernanda Miraldi Clemente Pessôa ◽  
Fernanda Cristina Rueda Lopes ◽  
João Victor Altamiro Costa ◽  
Soniza Vieira Alves Leon ◽  
Romeu Côrtes Domingues ◽  
...  

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