Holocord involvement with sparing of the peripheral white matter rim in longitudinally extensive spinal cord lesions of neuromyelitis optica

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.

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The Prevalence of Long Spinal Cord Lesions and Anti-Aquaporin 4 Antibodies in Neuromyelitis Optica Patients in Taiwan

European Neurology ◽

10.1159/000322740 ◽

2011 ◽

Vol 65 (2) ◽

pp. 99-104 ◽

Cited By ~ 9

Author(s):

Kai-Chen Wang ◽

Ching-Piao Tsai ◽

Chao-Lin Lee ◽

Shao-Yuan Chen ◽

Shyi-Jou Chen

Keyword(s):

Spinal Cord ◽

Neuromyelitis Optica ◽

Aquaporin 4 ◽

Spinal Cord Lesions

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Poor Responses to Interferon-Beta Treatment in Patients with Neuromyelitis Optica and Multiple Sclerosis with Long Spinal Cord Lesions

PLoS ONE ◽

10.1371/journal.pone.0098192 ◽

2014 ◽

Vol 9 (6) ◽

pp. e98192 ◽

Cited By ~ 11

Author(s):

Kai-Chen Wang ◽

Kuan-Hsiang Lin ◽

Tzu-Chi Lee ◽

Chao-Lin Lee ◽

Shao-Yuan Chen ◽

...

Keyword(s):

Multiple Sclerosis ◽

Spinal Cord ◽

Neuromyelitis Optica ◽

Interferon Beta ◽

Spinal Cord Lesions

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An autopsied case of neuromyelitis optica with a large cavitary cerebral lesion

Multiple Sclerosis Journal ◽

10.1191/1352458505ms1236cr ◽

2005 ◽

Vol 11 (6) ◽

pp. 735-738 ◽

Cited By ~ 26

Author(s):

M Nakamura ◽

M Endo ◽

K Murakami ◽

H Konno ◽

K Fujihara ◽

...

Keyword(s):

Spinal Cord ◽

White Matter ◽

Neuromyelitis Optica ◽

White Matter Lesion ◽

Transverse Myelitis ◽

Cerebral Lesion ◽

Tissue Destruction ◽

Consciousness Disturbance ◽

Frontal White Matter ◽

Inflammatory Changes

We report a case of neuromyelitis optica (NMO) with a large cerebral lesion. The patient had an episode of fever and consciousness disturbance with a tumefactive frontal white matter lesion at age 43, and then repeated bilateral optic neuritis and transverse myelitis until she died at age 63. Histopathological examinations revealed that marked tissue destruction, cavities and inflammatory changes typical of NMO were seen in the cerebrum as well as the optic nerves and spinal cord. This is the first autopsied case of NMO with a tumefactive cerebral lesion that later became cavitary.

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7 T imaging reveals a gradient in spinal cord lesion distribution in multiple sclerosis

Brain ◽

10.1093/brain/awaa249 ◽

2020 ◽

Vol 143 (10) ◽

pp. 2973-2987 ◽

Cited By ~ 1

Author(s):

Russell Ouellette ◽

Constantina A Treaba ◽

Tobias Granberg ◽

Elena Herranz ◽

Valeria Barletta ◽

...

Keyword(s):

Multiple Sclerosis ◽

Spinal Cord ◽

White Matter ◽

Cervical Spinal Cord ◽

White Matter Lesions ◽

Progressive Multiple Sclerosis ◽

Secondary Progressive Multiple Sclerosis ◽

Spinal Cord Lesions ◽

Secondary Progressive ◽

Relapsing Remitting

Abstract We used 7 T MRI to: (i) characterize the grey and white matter pathology in the cervical spinal cord of patients with early relapsing-remitting and secondary progressive multiple sclerosis; (ii) assess the spinal cord lesion spatial distribution and the hypothesis of an outside-in pathological process possibly driven by CSF-mediated immune cytotoxic factors; and (iii) evaluate the association of spinal cord pathology with brain burden and its contribution to neurological disability. We prospectively recruited 20 relapsing-remitting, 15 secondary progressive multiple sclerosis participants and 11 age-matched healthy control subjects to undergo 7 T imaging of the cervical spinal cord and brain as well as conventional 3 T brain acquisition. Cervical spinal cord imaging at 7 T was used to segment grey and white matter, including lesions therein. Brain imaging at 7 T was used to segment cortical and white matter lesions and 3 T imaging for cortical thickness estimation. Cervical spinal cord lesions were mapped voxel-wise as a function of distance from the inner central canal CSF pool to the outer subpial surface. Similarly, brain white matter lesions were mapped voxel-wise as a function of distance from the ventricular system. Subjects with relapsing-remitting multiple sclerosis showed a greater predominance of spinal cord lesions nearer the outer subpial surface compared to secondary progressive cases. Inversely, secondary progressive participants presented with more centrally located lesions. Within the brain, there was a strong gradient of lesion formation nearest the ventricular system that was most evident in participants with secondary progressive multiple sclerosis. Lesion fractions within the spinal cord grey and white matter were related to the lesion fraction in cerebral white matter. Cortical thinning was the primary determinant of the Expanded Disability Status Scale, white matter lesion fractions in the spinal cord and brain of the 9-Hole Peg Test and cortical thickness and spinal cord grey matter cross-sectional area of the Timed 25-Foot Walk. Spinal cord lesions were localized nearest the subpial surfaces for those with relapsing-remitting and the central canal CSF surface in progressive disease, possibly implying CSF-mediated pathogenic mechanisms in lesion development that may differ between multiple sclerosis subtypes. These findings show that spinal cord lesions involve both grey and white matter from the early multiple sclerosis stages and occur mostly independent from brain pathology. Despite the prevalence of cervical spinal cord lesions and atrophy, brain pathology seems more strongly related to physical disability as measured by the Expanded Disability Status Scale.

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Lymphocyte Subsets Are Associated with Disease Status in Neuromyelitis Optica Spectrum Disorders

NeuroImmunoModulation ◽

10.1159/000520745 ◽

2021 ◽

pp. 1-10

Author(s):

Hong Yang ◽

Wei Liu ◽

Yi-Fan Wu ◽

De-Sheng Zhu ◽

Xia-Feng Shen ◽

...

Keyword(s):

Spinal Cord ◽

B Cells ◽

Neuromyelitis Optica ◽

Peripheral Blood ◽

Lymphocyte Subsets ◽

Disease Status ◽

Neuromyelitis Optica Spectrum Disorders ◽

Spinal Cord Lesions ◽

Cross Sectional ◽

Spectrum Disorders

Objective: At present, studies on lymphocytes are mostly conducted on CD19+ B cells and CD27+ B cells in neuromyelitis optica spectrum disorders (NMOSDs), but the exact changes in lymphocyte subsets (CD19+ B cells, CD3+ T cells, CD4+ Th cells, CD8+ Ts cells, the CD4+/CD8+ ratio, and NK [CD56+ CD16] cells) have rarely been studied. This study aimed to assess lymphocyte subset changes in patients with NMOSD. Methods: We performed a cross-sectional study of consecutive patients with acute NMOSD (n = 41), chronic NMOSD (n = 21), and healthy individuals (n = 44). Peripheral blood samples were obtained upon admission, and lymphocyte subsets were analyzed by flow cytometry. Levels of lymphocyte subsets among 3 groups were compared and its correlation with the length of spinal cord lesions was analyzed. Results: The levels of peripheral blood CD19+ B cells were significantly higher in patients with acute and chronic NMOSD than in healthy controls (HCs) (17.91 ± 8.7%, 13.08 ± 7.562%, and 12.48 ± 3.575%, respectively; p < 0.001) and were positively correlated with the length of spinal cord lesions in acute NMOSD (r = 0.433, p < 0.05). The peripheral blood CD4+/CD8+ ratio was significantly lower in patients with acute NMOSD and chronic NMOSD than in HCs (1.497 ± 0.6387, 1.33 ± 0.5574, and 1.753 ± 0.659, respectively; p < 0.05), and the levels of peripheral blood NK (CD56+ CD16) cells were significantly lower in patients with acute and chronic NMOSD than in HCs (13.6 ± 10.13, 11.11 ± 7.057, and 14.7 [interquartile range = 9.28], respectively; p < 0.01). Conclusions: The levels of certain subsets of peripheral blood lymphocytes are associated with disease status in NMOSD.

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An acute onset of paraparesis in 65 years old Croatian woman – a case report

NATIONAL JOURNAL OF NEUROLOGY ◽

10.28942/nnj.v1i1(12).50 ◽

2018 ◽

Author(s):

V. Kosta

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Case Report ◽

Neuromyelitis Optica ◽

Demyelinating Disease ◽

Acute Onset ◽

Spinal Cord Lesions ◽

The Central Nervous System ◽

High Doses

Neuromyelitis optica (NMO, Devic`sdisease) is a rare inflammatory, demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. Seropositivity for NMO-IgG (aquaporin 4antybodies) and longitudinally extensive spinal cord lesions (3 or more segments) are characteristics of NMO. We described a 65-year old woman with an acute onset of paraparesis that was not recognized as NMO at the beginning. The diagnosis was made three months later when she was readmitted because of the relapse.Despite the treatment with high doses of methylprednisolone, plasmapheresis and immunoglobulins her condition stayed unchanged – she was paraplegic and incontinent.

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