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Spinal cord demyelination, cervical cord demyelination

Aurora Messina, Natasha Van Zyl, Michael Weymouth, Stephen Flood, Andrew Nunn, Catherine Cooper, Jodie Hahn, Mary P Galea
Loss of hand function after cervical spinal cord injury (SCI) impacts heavily on independence. Multiple nerve transfer surgery has been applied successfully after cervical SCI to restore critical arm and hand functions, and the outcome depends on nerve integrity. Nerve integrity is assessed indirectly using muscle strength testing and intramuscular electromyography, but these measures cannot show the manifestation that SCI has on the peripheral nerves. We directly assessed the morphology of nerves biopsied at the time of surgery, from three patients within 18 months post injury...
2016: Brain Sciences
T Duval, S Lévy, N Stikov, J Campbell, A Mezer, T Witzel, B Keil, V Smith, L L Wald, E Klawiter, J Cohen-Adad
The myelin g-ratio is defined as the ratio of the inner to the outer diameter of the myelin sheath. This ratio provides a measure of the myelin thickness that complements axon morphology (diameter and density) with high specificity for assessment of demyelination in diseases such as multiple sclerosis. Previous work has shown that an aggregate g-ratio map can be computed using a formula that combines axon and myelin density measured with quantitative MRI. In this work, we computed g-ratio weighted maps in the cervical spinal cord of nine healthy subjects...
September 21, 2016: NeuroImage
B Mark Keegan, Timothy J Kaufmann, Brian G Weinshenker, Orhun H Kantarci, William F Schmalstieg, M Mateo Paz Soldan, Eoin P Flanagan
OBJECTIVE: To report patients with progressive motor impairment resulting from an isolated CNS demyelinating lesion in cerebral, brainstem, or spinal cord white matter that we call progressive solitary sclerosis. METHODS: Thirty patients were identified with (1) progressive motor impairment for over 1 year with a single radiologically identified CNS demyelinating lesion along corticospinal tracts, (2) absence of other demyelinating CNS lesions, and (3) no history of relapses affecting other CNS pathways...
October 18, 2016: Neurology
Silvia de Pasqua, Sabina Cevoli, Fabio Calbucci, Rocco Liguori
Pseudoathetosis and dystonia are rare manifestations of spinal cord disease that have been already reported in lesions involving the posterior columns at the cervical level. We report two patients with a cervical demyelinating lesion at C3-C4 level presenting with hand dystonia and pseudoathetoid movements. The movement disorder disappeared after steroid treatment. The cases we described highlight the importance of identifying secondary causes of movement disorders that can be reversible with appropriate therapy...
September 15, 2016: Journal of the Neurological Sciences
Alfonso Lema, Courtney Bishop, Omar Malik, Miriam Mattoscio, Rehiana Ali, Richard Nicholas, Paolo A Muraro, Paul M Matthews, Adam D Waldman, Rexford D Newbould
BACKGROUND: Demyelination is a core pathological feature of multiple sclerosis (MS) and spontaneous remyelination appears to be an important mechanism for repair in the disease. Magnetization transfer ratio imaging (MTR) has been used extensively to evaluate demyelination, although limitations to its specificity are recognized. MT saturation imaging (MTsat) removes some of the T1 dependence of MTR. We have performed a comparative evaluation of MTR and MTsat imaging in a mixed group of subjects with active MS, to explore their relative sensitivity to pathology relevant to explaining clinical outcomes...
August 5, 2016: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
Seiji Okada, Charles Chang, Geraldine Chang, James J Yue
BACKGROUND CONTEXT: Venous hypertensive myelopathy (VHM) results from spinal vascular malformations of arteriovenous shunting that increases spinal venous pressure, leading to congestive edema and neurologic dysfunction. There has been no report of VHM associated with cervical spondylotic myelopathy (CSM). PURPOSE: The aim of this study was to report an extremely rare case of VHM likely due to CSM. STUDY DESIGN: This study is a case report and review of the literature...
June 9, 2016: Spine Journal: Official Journal of the North American Spine Society
Manuel Taso, Olivier M Girard, Guillaume Duhamel, Arnaud Le Troter, Thorsten Feiweier, Maxime Guye, Jean-Philippe Ranjeva, Virginie Callot
Being able to finely characterize the spinal cord (SC) microstructure and its alterations is a key point when investigating neural damage mechanisms encountered in different central nervous system (CNS) pathologies, such as multiple sclerosis, amyotrophic lateral sclerosis or myelopathy. Based on novel methods, including inhomogeneous magnetization transfer (ihMT) and dedicated SC probabilistic atlas post-processing, the present study focuses on the in vivo characterization of the healthy SC tissue in terms of regional microstructure differences between (i) upper and lower cervical vertebral levels and (ii) sensory and motor tracts, as well as differences attributed to normal aging...
June 2016: NMR in Biomedicine
Massa Zantah, Timothy B Coyle, Debapriya Datta
Neuromyelitis Optica (NMO) is a demyelinating autoimmune disease involving the central nervous system. Acute respiratory failure from cervical myelitis due to NMO is known to occur but is uncommon in monophasic disease and is treated with high dose steroids. We report a case of a patient with NMO who developed acute respiratory failure related to cervical spinal cord involvement, refractory to pulse dose steroid therapy, which resolved with plasmapheresis.
2016: Case Reports in Pulmonology
C Lebrun
Even prior to the introduction of criteria defining the radiologically isolated syndrome (RIS), longitudinal clinical data from individuals with incidentally identified T2 lesions suggestive of multiple sclerosis (MS) were described. Healthy individuals who do not exhibit signs of neurological dysfunction may have a brain MRI performed for a reason other than suspicion of MS that reveals unexpected anomalies highly suggestive of demyelinating plaques given their size, location, and morphology. These healthy subjects lack a history or symptomatology suggestive of MS and fulfill formal criteria for RIS, a recently described MS subtype that shares the phenotype of at-risk individuals for future demyelinating events...
October 2015: Revue Neurologique
S Presas-Rodríguez, L Grau-López, J V Hervás-García, A Massuet-Vilamajó, C Ramo-Tello
BACKGROUND: Myelitis can appear as an initial symptom in the context of demyelinating diseases, systemic inflammatory diseases, and infectious diseases. We aim to analyse the differences between myelitis associated with multiple sclerosis (MS) and myelitis resulting from other aetiologies. METHODS: Single-centre, retrospective analysis of patients with initial myelitis (2000-2013). Demographic, aetiological, clinical, radiological and prognostic variables were analysed and compared between patients with myelitis from MS and those with myelitis due to other aetiologies...
March 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Lucy Matthews, Shannon Kolind, Alix Brazier, Maria Isabel Leite, Jonathan Brooks, Anthony Traboulsee, Mark Jenkinson, Heidi Johansen-Berg, Jacqueline Palace
Inflammatory demyelinating lesions of the central nervous system are a common feature of both neuromyelitis optica and multiple sclerosis. Despite this similarity, it is evident clinically that the accumulation of disability in patients with neuromyelitis optica is relapse related and that a progressive phase is very uncommon. This poses the question whether there is any pathological evidence of disease activity or neurodegeneration in neuromyelitis optica between relapses. To investigate this we conducted a longitudinal advanced MRI study of the brain and spinal cord in neuromyelitis optica patients, comparing to patients with multiple sclerosis and controls...
2015: PloS One
Lindsay Tetreault, Christina L Goldstein, Paul Arnold, James Harrop, Alan Hilibrand, Aria Nouri, Michael G Fehlings
Cervical spinal cord dysfunction can result from either traumatic or nontraumatic causes, including tumors, infections, and degenerative changes. In this article, we review the range of degenerative spinal disorders resulting in progressive cervical spinal cord compression and propose the adoption of a new term, degenerative cervical myelopathy (DCM). DCM comprises both osteoarthritic changes to the spine, including spondylosis, disk herniation, and facet arthropathy (collectively referred to as cervical spondylotic myelopathy), and ligamentous aberrations such as ossification of the posterior longitudinal ligament and hypertrophy of the ligamentum flavum...
October 2015: Neurosurgery
Jianrui Yin, Youming Long, Fulan Shan, Yongxiang Fan, Linzhan Wu, Rong Zhong, Cong Gao, Xiaohui Chen, Qingchun Gao, Ning Yang
Neuromyelitis optica (NMO) is a severe, idiopathic, immuno-mediated, inflammatory demyelinating disease of the central nervous system. We examined the clinical features between male and female NMO patients, for which not much data exist. One hundred and eight Chinese Han patients with NMO were analysed retrospectively, all had been detected for the aquaporin-4 (AQP4) antibody using a cell-based assay. Of 108 NMO patients, 92 were female and 16 were male (female/male = 5.75). Ninety-four (87%) were positive for the AQP4 antibody in their serum and/or cerebral spinal fluid...
November 2015: Neurological Research
Stefano Zanigni, Rossana Terlizzi, Caterina Tonon, Claudia Testa, David Neil Manners, Sabina Capellari, Roberto Gallassi, Roberto Poda, Laura Ludovica Gramegna, Giovanna Calandra-Buonaura, Luisa Sambati, Pietro Cortelli, Raffaele Lodi
INTRODUCTION: adult-onset autosomal dominant leukodystrophy (ADLD) is a rare inherited disorder due to a duplication of lamin-B1 (LMNB1) gene. The aim of this study was to investigate brain metabolic and microstructural alterations by using advanced MR techniques. METHODS: we performed brain MR scans including single-voxel proton-MR Spectroscopy ((1)H-MRS) of the lateral ventricles and parietal white matter and diffusion tensor imaging (DTI) in 4 subjects with LMNB1 gene duplication, 6 non-mutated relatives and 7 unrelated healthy controls...
August 2015: Brain Research Bulletin
E Kantorová, J Marcinek, K Zeleňák, K Kantor, J Michalik, Š Sivák, E Kurča, L Plank
STUDY DESIGN: Case report. OBJECTIVES: We report on a 52-year-old male patient with tumefactive demyelination of the spinal cord. SETTING: University Hospital and Jessenius Faculty of Medicine, Comenius University, Martin, Slovakia. BACKGROUND: In contrast to relatively frequent tumefactive fulminant lesions in the brain, cases affecting the spinal cord in isolation have been reported less frequently. METHODS: Description of the case report...
December 2015: Spinal Cord
Feng Wang, Ke Li, Arabinda Mishra, Daniel Gochberg, Li Min Chen, John C Gore
PURPOSE: This study aimed to evaluate the reproducibility and specificity of quantitative magnetization transfer (qMT) imaging for monitoring spinal cord injuries (SCIs). METHODS: MRI scans were performed in anesthetized monkeys at 9.4T, before and serially after a unilateral lesion of the cervical spinal cord. A two-pool fitting model was used to derive qMT parameters. RESULTS: qMT measures were reproducible across normal subjects, with an average pool size ratio (PSR) of 0...
April 2016: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
Sarah E Mondello, Michael D Sunshine, Amanda E Fischedick, Chet T Moritz, Philip J Horner
Cervical spinal cord contusion is the most common human spinal cord injury, yet few rodent models replicate the pathophysiological and functional sequela of this injury. Here, we modified an electromechanical injury device and characterized the behavioral and histological changes occurring in response to a lateralized C4 contusion injury in rats. A key feature of the model includes a non-injurious touch phase where the spinal cord surface is dimpled with a consistent starting force. Animals were either left intact as a control, received a non-injury-producing touch on the surface of the cord ("sham"), or received a 0...
December 15, 2015: Journal of Neurotrauma
Paweł Gogol, Anna Gogol, Andrzej Opuchlik, Dorota Dziewulska
Paraneoplastic neurologic syndromes are diagnosed when neurologic symptoms are associated with neoplasm and other causative factors are excluded. They may precede or be simultaneous to various types of neoplasms, mainly malignant. In men up to 45-50 years old the most common cancer causing the paraneoplastic syndrome is testicle tumor, manifesting usually as limbic/brain stem encephalitis and myelitis. Usually effective treatment of underlying neoplasm brings resolution of neurologic symptoms. But corticosteroids and intravenuous immunoglobulins are also used...
2015: Neurologia i Neurochirurgia Polska
Raed Abdullah Alroughani, Samar Farouk Ahmed, Riyadh Ahmed Khan, Jasem Yousef Al-Hashel
BACKGROUND: Unusual presentations of multiple sclerosis (MS) at onset may post a diagnostic dilemma to the treating neurologists. Spinal myoclonus is rare in MS and may lead to perform extensive investigations to rule out other etiologies affecting the spinal cord. CASE PRESENTATION: We described a 31-year-old male who presented with involuntary brief jerky movements of the left shoulder and arm with significant wasting of shoulder muscles. In retrospect, the patient had a progressive right leg weakness one year prior to his presentation...
2015: BMC Neurology
Khaled Abdel-Aziz, Torben Schneider, Bhavana S Solanky, Marios C Yiannakas, Dan R Altmann, Claudia A M Wheeler-Kingshott, Amy L Peters, Brian L Day, Alan J Thompson, Olga Ciccarelli
Spinal neurodegeneration is an important determinant of disability progression in patients with primary progressive multiple sclerosis. Advanced imaging techniques, such as single-voxel (1)H-magnetic resonance spectroscopy and q-space imaging, have increased pathological specificity for neurodegeneration, but are challenging to implement in the spinal cord and have yet to be applied in early primary progressive multiple sclerosis. By combining these imaging techniques with new clinical measures, which reflect spinal cord pathology more closely than conventional clinical tests, we explored the potential for spinal magnetic resonance spectroscopy and q-space imaging to detect early spinal neurodegeneration that may be responsible for clinical disability...
June 2015: Brain: a Journal of Neurology
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