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transverse myelitis neuromyelitis optica

Lu-Mei Chi, Yu Gao, Guang-Xian Nan
RATIONALE: Neuromyelitis optica spectrum disorders (NMOSDs) represent recurrent autoimmune diseases, generally beginning with optic nerve neuritis or acute transverse myelitis. PATIENT CONCERNS: A 57-year-old male with long-term alcohol intake was hospitalized because of limb numbness. EMG examination showed the peripheral sensory nerve was in demyelination and an axonal injury was found. His symptoms could not be improved by vitamin B injection but were later significantly attenuated by dexamethasone treatment...
March 2018: Medicine (Baltimore)
Hongtao Hu, Xiaofan You, Jing Ye
BACKGROUND: Short transverse myelitis (STM) is considered uncommon in neuromyelitis optica spectrum disorders (NMOSD). Poor recognition of STM occurring in NMOSD may lead to increased delay in diagnosis and appropriate treatment. OBJECTIVES: The aim of this study was to assess the frequency and characteristics of STM in Chinese patients with NMOSD. METHODS: We enrolled 91 patients with NMOSD based on the 2015 International Consensus Diagnostic Criteria for NMOSD...
February 19, 2018: Multiple Sclerosis and related Disorders
Maureen A Mealy, Tai-Seung Nam, Santiago J Pardo, Carlos A Pardo, Nara L Sobreira, Dimitrios Avramopoulos, David Valle, Kathleen H Burns, Michael Levy
Objective: To identify genetic differences among siblings with a family history of idiopathic transverse myelitis (ITM). Methods: We compared whole-exome sequencing (WES) on germline samples from the 2 affected sisters with ITM with 3 of their healthy siblings. Results: The 2 sisters with ITM both had acute onset of sensory loss in the legs, weakness, and bowel/bladder dysfunction. The first developed ITM at age 15 years with a clinical nadir of complete paralysis, which slowly recovered over a few years...
February 2018: Neurology. Genetics
Daniel C Wiener, Tamara B Kaplan, Carlos E Bravo-Iñiguez, Jordan Miller, Aaron L Berkowitz, Michael T Jaklitsch
Neuromyelitis optica spectrum disorders are a group of relapsing, inflammatory, demyelinating neurologic syndromes involving the central nervous system associated with antibodies against aquaporin-4. Although most commonly an idiopathic autoimmune condition, neuromyelitis optica may occur as a paraneoplastic syndrome in rare instances. We report a case of transverse myelitis caused by paraneoplastic neuromyelitis optica as the presenting clinical syndrome in a patient with esophageal adenocarcinoma.
March 2018: Annals of Thoracic Surgery
C G Chee, K S Park, J W Lee, H W Ahn, E Lee, Y Kang, H S Kang
BACKGROUND AND PURPOSE: Longitudinally extensive transverse myelitis is a well-documented spinal manifestation of neuromyelitis optica spectrum disorders, however, other forms of nontumorous myelopathy can also manifest as longitudinally extensive transverse myelitis. Our aim was to evaluate the MR imaging features of aquaporin-4 antibody-positive longitudinally extensive transverse myelitis, which is strongly associated with neuromyelitis optica spectrum disorders. MATERIALS AND METHODS: We evaluated cervicomedullary junction involvement, cord expansion ratios, bright spotty lesions, the number of involved segments, skipped lesions, enhancement patterns, and axial distribution patterns using spinal MR imaging of 41 patients with longitudinally extensive transverse myelitis who underwent aquaporin-4 antibody testing...
February 15, 2018: AJNR. American Journal of Neuroradiology
Navneet K Singh, Alexander J Sweidan, Sarah Strube, Ignacio Carrillo-Nunez
Neuromyelitis optica spectrum disorders (NMOSDs) are a set of demyelinating disorders that primarily target the optic nerves and the spinal cord. Previously thought to be a subset of multiple sclerosis (MS), now is recognized as a distinct entity. We present a 59-year-old female patient who was admitted for acute upper and lower extremity weakness. The patient had woken up from sleep with sudden onset of weakness. Patient was initially diagnosed with a right hemispheric stroke; however, magnetic resonance imaging of the cervical spine later performed showed abnormal enhancement from C2-C4, representing transverse myelitis...
2018: Clinical Medicine Insights. Case Reports
Matthias Baumann, Astrid Grams, Tanja Djurdjevic, Eva-Maria Wendel, Christian Lechner, Bettina Behring, Astrid Blaschek, Katharina Diepold, Astrid Eisenkölbl, Joel Fluss, Michael Karenfort, Johannes Koch, Bahadir Konuşkan, Steffen Leiz, Andreas Merkenschlager, Daniela Pohl, Mareike Schimmel, Charlotte Thiels, Barbara Kornek, Kathrin Schanda, Markus Reindl, Kevin Rostásy
Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution...
February 8, 2018: Journal of Neurology
Yin Liu, Michael Olek
Neuromyelitis optica is an inflammatory, demyelinating disease of the central nervous system that is characterized by severe relapsing attacks of optic neuritis and transverse myelitis. The current case describes a 29-year-old man with intractable epilepsy and diplegic spastic cerebral palsy who was given the diagnosis of neuromyelitis optica spectrum disorder after presenting with weakness, incontinence, and decreased visual acuity. His symptoms recurred 21 months after initial presentation. Magnetic resonance imaging of his spine revealed arachnoid cysts with regional mass effects...
February 1, 2018: Journal of the American Osteopathic Association
Shuchit Pandey, Ravindra K Garg, Hardeep S Malhotra, Amita Jain, Kiran P Malhotra, Neeraj Kumar, Rajesh Verma, Praveen K Sharma
INTRODUCTION: We evaluated the spectrum of acquired demyelinating and inflammatory disorders in patients presenting with an acute transverse myelopathy. We also studied differences between an acute idiopathic transverse myelitis and myelitis resulting from other etiologies. MATERIALS AND METHODS: Eighty consecutive patients with acute transverse myelopathy were included. At inclusion, clinical profile, serum and cerebrospinal fluid parameters, brain and spinal cord magnetic resonance imaging, and visual evoked potentials were obtained...
January 2018: Neurology India
Lisa M Deuel, Marjorie E Bunch
Neuromyelitis optic spectrum disorders are demyelinating conditions that are typically idiopathic, though various case reports have demonstrated an association with malignancy. We present the case of a 64year old woman with NMOSD in the setting of small cell lung cancer. She had longitudinally extensive transverse myelitis and left eye optic neuritis; aquaporin-4 antibodies were elevated. Biopsy of mediastinal adenopathy was positive for SCLC. Malignancy should be considered in any patient with an atypical presentation of NMOSD, or who does not respond to traditional therapies...
January 3, 2018: Journal of Neuroimmunology
Nicholas L Zalewski, Eoin P Flanagan, B Mark Keegan
OBJECTIVE: To evaluate specific myelopathy diagnoses made in patients with suspected idiopathic transverse myelitis (ITM). METHODS: A total of 226 patients 18 years and older were referred to Mayo Clinic Neurology for suspected ITM from December 1, 2010, to December 31, 2015. Electronic medical records were reviewed for detailed clinical presentation and course, laboratory and electrophysiologic investigations, and neuroimaging to determine the etiology. Current diagnostic criteria for ITM and alternative myelopathy diagnoses were applied...
January 9, 2018: Neurology
Gülsüm Alkan, Melike Emiroğlu, Ayşe Kartal, Harun Peru, Mustafa Koplay
Longitudinally extensive transverse myelitis (LETM) is defined as an inflammatory lesion of the spinal cord that extends to three or more segments. LETM is a commonly characteristic feature of neuromyelitis optica (NMO) or various autoimmune diseases. Manifestation of Mycobacterium tuberculosis (MTB) infection with LETM are rare and usually in the cervicothoracic spinal cord. Our patient presented with holocord LETM, so NMO was considered initially diagnosis. After in further research, MTB was diagnosed and treated successfully...
July 2017: Journal of Pediatric Neurosciences
Thomas Mathew, Kurian Thomas, Sonia Shivde, Shruthi Venkatesh, Smitha Mary Rockey
Neuromyelitis optica is a severe autoimmune demyelinating disease; however, the trigger for autoimmunity in an individual patient is unclear. Here, we report the case of a 48 year old female presenting with longitudinally extensive transverse myelitis in the cervical region of the spinal cord two weeks post herpes zoster infection in right C6 dermatome. Though post herpetic myelitis was provisionally diagnosed, serological evaluation revealed aquaporin-4 antibody positivity, confirming the diagnosis of neuromyelitis optica spectrum disorder...
November 2017: Multiple Sclerosis and related Disorders
Abdorreza Naser Moghadasi
In recent years, not only the incidence of brain demyelinating disease has increased, but also it seems that the world is facing new forms of autoimmune diseases of the brain. Distinguishing these diseases from each other is very important, since each requires a different treatment. The new criteria that were put forward in 2015 were meant to pave the way for better diagnosis of these diseases called neuromyelitis optica spectrum disease (NMOSD). However, too much emphasis on the criteria based on the convergence and association of radiological findings with the clinical symptoms actually causes confusion in the diagnosis...
November 2017: Multiple Sclerosis and related Disorders
Thomas Volkman, Cheryl Hemingway
Neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) is a rare relapsing autoimmune disease of the central nervous system constituting less than 1% of demyelinating diseases (Jeffery and Buncic, 1996). It preferentially affects the optic nerves and spinal cord, with the brain parenchyma generally spared. Demyelinating lesions are characterised by longitudinally extensive transverse myelitis (LETM) and often longitudinally extensive optic neuritis. Following the discovery of a novel pathogenic antibody, Aquaporin 4 in 2004 (Lennon et al...
November 2017: Multiple Sclerosis and related Disorders
Maciej Jurynczyk, Silvia Messina, Mark R Woodhall, Naheed Raza, Rosie Everett, Adriana Roca-Fernandez, George Tackley, Shahd Hamid, Angela Sheard, Gavin Reynolds, Saleel Chandratre, Cheryl Hemingway, Anu Jacob, Angela Vincent, M Isabel Leite, Patrick Waters, Jacqueline Palace
No abstract text is available yet for this article.
December 1, 2017: Brain: a Journal of Neurology
Ádám Annus, Krisztina Bencsik, Izabella Obál, Zsigmond Tamás Kincses, László Tiszlavicz, Romana Höftberger, László Vécsei
Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating, autoimmune diseases affecting the central nervous system. Typically, recurrent optic neuritis and longitudinal extensive transverse myelitis dominates the clinical picture. In most cases NMOSD are associated with autoantibodies targeting the water channel aquaporin-4 (AQP-4). NMOSD usually present in young adults. Clinical findings suggestive of NMOSD in elderly patients should raise the suspicion of a paraneoplastic etiology. To our knowledge, we report the first case of a 66 year-old female patient with paraneoplastic NMOSD that is associated with squamous cell lung carcinoma...
November 10, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Jinming Han, Meng-Ge Yang, Jie Zhu, Tao Jin
Neuromyelitis optica (NMO), considered to be mediated by autoantibodies, often cause severely disabling disorders of the central nervous system, and predominantly cause optic nerve damage and longitudinally extensive transverse myelitis. Remarkable progress has been made in deciphering NMO pathogenesis during the past decade. In 2015, the International Panel for NMO Diagnosis proposed the unifying term "NMO spectrum disorders" (NMOSD) and the updated NMOSD criteria reflects a wide range of disease and maintains reasonable specificity...
2017: Neuropsychiatric Disease and Treatment
Rinze Neuteboom, Colin Wilbur, Danielle Van Pelt, Moses Rodriguez, Ann Yeh
Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, whereas recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, magnetic resonance imaging features, outcomes, and serum biomarkers in these disorders...
August 2017: Seminars in Pediatric Neurology
P Vermersch, O Outteryck, D Ferriby, H Zéphir
BACKGROUND AND PURPOSE: Myelitis is common, related to multiple aetiologies and constitute in some cases a differential diagnosis for spinal cord tumors. Our objective was to review the clinical and paraclinical aspects of the main aetiologies of myelitis. METHODS: These aetiologies will be reviewed based on data not only from the scientific literature but also from our personal experience reported in different cohorts of patients. RESULTS: Multiple sclerosis is the main cause of partial myelitis in young adults...
November 2017: Neuro-Chirurgie
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