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

2005 ◽  
Vol 11 (6) ◽  
pp. 735-738 ◽  
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.

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

2015 ◽  
Vol 6 (S1) ◽  
pp. 78-79
Author(s):  
Shotaro Hayashida ◽  
Katsuhisa Masaki ◽  
Takuya Matsushita ◽  
Mitsuru Watanabe ◽  
Ryo Yamasaki ◽  
...  
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Neuromyelitis Optica ◽  
Spinal Cord Lesions

Neuromyelitis Optica

2017 ◽  
Author(s):  
Teri L. Schreiner ◽  
Jeffrey L. Bennett
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Inflammatory Disorder ◽  
The Central Nervous System

Neuromyelitis optica (NMO), or Devic’s disease is an inflammatory disorder of the central nervous system that preferentially affects the optic nerves and spinal cord. Initially considered a variant of multiple sclerosis (MS), NMO is now clearly recognized to have distinct clinical, radiographic, and pathologic characteristics. Historically, the diagnosis of NMO required bilateral optic neuritis and transverse myelitis; however, the identification of a specific biomarker, NMO-IgG, an autoantibody against the aquaporin-4 (AQP4) water channel, has broadened NMO spectrum disease to include patients with diverse clinical and radiographic presentations. This chapter addresses the diagnosis, pathophysiology, and management of the disease.

scholarly journals Memantine ameliorates motor impairments and pathologies in a mouse model of neuromyelitis optica spectrum disorders.

2020 ◽  
Author(s):  
Leung-Wah Yick ◽  
Chi-Ho Tang ◽  
Oscar Ka-Fai Ma ◽  
Jason Shing-Cheong Kwan ◽  
Koon Ho CHAN
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Neurotrophic Factor ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Glutamate Excitotoxicity ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Motor Impairments ◽  
Spectrum Disorders

Abstract Background: Neuromyelitis optica spectrum disorders (NMOSD) are central nervous system (CNS) autoimmune inflammatory demyelinating diseases characterized by recurrent episodes of acute optic neuritis and transverse myelitis. Aquaporin-4 immunoglobulin G (AQP4-IgG) autoantibodies, which target the water channel aquaporin-4 (AQP4) on astrocytic membrane, are pathogenic in NMOSD. Glutamate excitotoxicity, which is triggered by internalization of AQP4-glutamate transporter complex after AQP4-IgG binding to astrocytes, is involved in early NMOSD pathophysiologies. We studied the effects of memantine, a N-methyl-D-aspartate (NMDA) receptor antagonist, on motor impairments and spinal cord pathologies in mice which received human AQP4-IgG. Methods: Purified IgG from AQP4-IgG-seropositive NMOSD patients were passively transferred to adult C57BL/6 mice with disrupted blood-brain barrier. Memantine was administered by oral gavage. Motor impairments of the mice were assessed by beam walking test. Spinal cords of the mice were assessed by immunofluorescence and ELISA. Results: Oral administration of memantine ameliorated the motor impairments induced by AQP4-IgG, no matter the treatment was initiated before (preventive) or after (therapeutic) disease flare. Memantine profoundly reduced AQP4 and astrocyte loss, and attenuated demyelination and axonal loss in the spinal cord of mice which had received AQP4-IgG. The protective effects of memantine were associated with inhibition of apoptosis and suppression of neuroinflammation, with decrease in microglia activation and neutrophil infiltration and reduction of increase in levels of proinflammatory cytokines including interleukin-1β (IL-1β), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF- α). In addition, memantine elevated growth factors including brain-derived neurotrophic factor (BDNF), glial cell line-derived neurotrophic factor (GDNF) and vascular endothelial growth factor (VEGF) in the spinal cord. Conclusions: Our findings support that glutamate excitotoxicity and neuroinflammation plays important roles in complement-independent pathophysiology during early development of NMOSD lesions, and highlight the potential of oral memantine as a therapeutic agent in NMOSD acute attacks.

Short segment myelitis as a first manifestation of neuromyelitis optica spectrum disorders

2017 ◽  
Vol 23 (3) ◽  
pp. 413-419 ◽  
Author(s):  
So-Young Huh ◽  
Su-Hyun Kim ◽  
Jae-Won Hyun ◽  
In Hye Jeong ◽  
Min Su Park ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Mri Findings ◽  
Initial Manifestation ◽  
Short Transverse ◽  
Imaging Characteristics ◽  
Referral Hospitals ◽  
Spectrum Disorders

Background: Some patients with neuromyelitis optica spectrum disorders (NMOSD) present with spinal cord lesions extending fewer than three vertebral segments (short transverse myelitis, STM), hindering an early diagnosis. Objective: We investigated the frequency and imaging characteristics of STM lesions in patients presenting with myelitis as an initial manifestation of NMOSD. Methods: Patients seen at three referral hospitals in Korea between June 2005 and March 2015 who met the following inclusion criteria were recruited for review: seropositivity for aquaporin-4 antibody, initial presentation with myelitis and spinal cord magnetic resonance imaging (MRI) performed within 1 month of initial myelitis onset. Results: Of the 76 enrolled patients, 65 (85.5%) collectively had 69 longitudinally extensive transverse myelitis lesions, while the remaining 11 (14.5%) had a total of 15 STM lesions. Of the 15 STM lesions, 5 spanned 2.5 vertebral segments, 6 were continuous over two segments, 3 showed a length of 1.5 segments and 1 was confined to a single segment. On axial imaging, all of the STM lesions involved the central grey matter. Conclusion: These MRI findings suggested that STM does not preclude the possibility of an NMOSD diagnosis.

scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody Associated Transverse Myelitis

2019 ◽  
Vol 62 (3) ◽  
pp. 123-126
Author(s):  
Iveta Chroustová ◽  
Miroslav Mareš ◽  
Leoš Ungermann ◽  
Edvard Ehler
Keyword(s):  
Spinal Cord ◽  
Cerebrospinal Fluid ◽  
Differential Diagnosis ◽  
White Matter ◽  
Transverse Myelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Igg Antibodies ◽  
Inflammatory Lesions ◽  
Important Differential Diagnosis ◽  
Central Myelin

Antibodies against myelin oligodendrocyte glycoprotein cause inflammatory lesions of central myelin – in optic nerves, of the brainstem, and spinal cord. There are characteristic changes of CNS white matter, protein-cytological association in cerebrospinal fluid, MOG IgG antibodies, a very important differential diagnosis and a relatively mild course.

Neuropathic pruritus (itch) in neuromyelitis optica

2012 ◽  
Vol 19 (4) ◽  
pp. 475-479 ◽  
Author(s):  
Liene Elsone ◽  
Tristan Townsend ◽  
Kerry Mutch ◽  
Kumar Das ◽  
Mike Boggild ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Dorsal Horn ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Neuropathic Pruritus ◽  
Aquaporin 4 Antibody

Background: Neuropathic pruritus (itch) is an uncommon, but well described, symptom in neurology. There are itch-specific neurons in the dorsal horn of the spinal cord. We noted excessive pruritus in patients with neuromyelitis optica (NMO). Objective: We aimed to explore the characteristics of pruritus in NMO patients. Methods: We reviewed case records of a well-defined cohort of 45 serial aquaporin-4 antibody-positive patients visiting the national NMO service. All patients were interviewed. Results: Of the 45 antibody-positive NMO patients, 44 had myelitis and 12 of those 44 (27.3%) patients reported pruritus within a week of other symptoms of transverse myelitis with central cord involvement. In three patients, pruritus was the first symptom of a relapse, while in one case, pruritus was the very first symptom of the index episode of NMO. Conclusion: Neuropathic pruritus seems to be a common, but under-recognised symptom of myelitis associated with NMO.

scholarly journals Simultaneous new onset of neuromyelitis optica spectrum disorder in identical twins

2021 ◽  
Vol 3 (2) ◽  
pp. e000174
Author(s):  
Ivo Bekavac ◽  
Matea Matejic ◽  
Riley J Woods ◽  
John I Halloran
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Neuromyelitis Optica ◽  
Cervical Spinal Cord ◽  
Identical Twins ◽  
Spectrum Disorder ◽  
Cerebral White Matter ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Csf Analysis ◽  
The Brain

ObjectiveTo present a case of two identical twins presenting concurrently with symptoms and subsequent initial diagnosis of neuromyelitis optica spectrum disorder (NMOSD).MethodsClinical, laboratory and MRI findings for both twins were reviewed and presented here.ResultsTwin A presented with right eye pain and subsequent blurred vision in right eye. MRI of the brain and spine demonstrated pre-chiasmal right optic nerve enhancement and T2 hyperintense lesions in the spinal cord at T7 and T9 levels. Cerebrospinal fluid (CSF) analysis was remarkable for NMO/aquaporin-4 (AQP4) fluorescence-activated cell sorting (FACS) titre of 1:32 and a serum NMO/AQP4-IgG positive titre of 1:10 000. Twin B presented with diplopia. MRI of the brain and spine demonstrated T2 hyperintense lesions in the periventricular cerebral white matter, in the periaqueductal white matter of the pons, in the midbrain and the cervical spinal cord. Neurological examination findings revealed incomplete right trochlear palsy, rotatory nystagmus, an incomplete left internuclear ophthalmoplegia and hyper-reflexia. CSF analysis was remarkable for NMO/AQP4 FACS titre of 1:256 and a serum NMO-IgG positive titre of 1:10 000. Both twins responded well to intravenous steroid therapy. There was no adverse environmental exposure present.ConclusionWe present an interesting and rare case of identical twins presenting concurrently and for the first time with NMOSD.

scholarly journals 3-Tesla Study of the Spinal Cord White Matter

2009 ◽  
Vol 22 (1_suppl) ◽  
pp. 85-93
Author(s):  
L. Albini Riccioli ◽  
A.F. Marliani ◽  
M. Leonardi
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
High Field ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Inhomogeneous Magnetic Field ◽  
Demyelinating Diseases ◽  
High Field Strength ◽  
Anatomical Area ◽  
3T Mr

Spinal cord as soon as brain, can be affected by dysmyelinating and demyelinating diseases, as Multiple Sclerosis (MS), Acute Disseminated Encephalomyelitis (ADEM), Neuromyelis Optica (NMO) and Transverse Myelitis. Investigation of the spinal cord with a high field strength MR system is hampered by the inhomogeneous magnetic field, physiological movements and the small size of the anatomical area. We describe normal and pathological neuroradiological findings in spinal cord white matter and the parameters of optimized sequences for use with the 3T MR systems.

Contribution of spinal cord biopsy to diagnosis of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder

2013 ◽  
Vol 20 (7) ◽  
pp. 882-888 ◽  
Author(s):  
M Ringelstein ◽  
I Metz ◽  
K Ruprecht ◽  
A Koch ◽  
J Rappold ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Diagnostic Process ◽  
Antibody Testing ◽  
Magnetic Resonance Imaging Mri ◽  
Extensive Transverse Myelitis ◽  
Aqp4 Antibody

Longitudinally extensive transverse myelitis is characteristic but not pathognomonic for neuromyelitis optica spectrum disorders (NMOSDs) and may mimic local tumors. In this retrospective study based on a cohort of 175 NMOSD patients we identified seven patients who initially presented with a longitudinally extensive spinal cord lesion and underwent spinal cord biopsy due to magnetic resonance imaging (MRI)-suspected malignancies. Remarkably, routine neuropathology was inconclusive and did not guide the diagnostic process to anti-aquaporin-4 (AQP4)-seropositive NMOSD. Serious postoperative complications occurred in 5/7 patients and persisted during follow-up in 2/7 patients (29%). Considering these sequelae, AQP4-antibody testing should be mandatory in patients with inconclusive longitudinally extensive spinal cord lesions prior to biopsy.

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