scholarly journals Recurrent longitudinally extensive transversal myelitis in a patient with Sjögren’s syndrome

2013 ◽  
Vol 70 (11) ◽  
pp. 1056-1058 ◽  
Author(s):  
Jelena Sekaric ◽  
Zeljko Zivanovic ◽  
Aleksandra Lucic-Prokin ◽  
Timea Kokai-Zekic ◽  
Kosta Petrovic ◽  
...  

Introduction. Longitudinally extensive transverse myelitis (LETM) is a transversal myelitis that extends through three or more vertebral segments in length. Case report. A 52- year-old woman was hospitalized due to pain in the lumbar region, difficulty in walking, hypoesthesia of the anogenital area and urinary retention. In the past medical history, two years earlier, the patient had been diagnosed with transversal myelitis confirmed by MRI of the cervical spine and six months earlier, the patient was diagnosed with primary Sj?gren?s syndrome (SS). During the current hospitalization MRI of the spinal cord revealed extensive inflammatory lesions of almost the whole spinal cord. Lumbar puncture (LP) revealed mild pleocytosis and slightly increased protein level. Isoelectric focusing of cerebrospinal fluid (CSF) and serum proteins was normal. Visual evoked potentials were normal. Serological testing excluded acute viral infections. Corticosteroid therapy was applied with good therapeutic response. Control MRI revealed regression of pathological changes in the spinal cord. Conclusion. A wide range of disorders can cause LETM, but usually the first line diagnosis is neuromyelitis optica (NMO). Based on the detection of NMO immunoglobulin G in the serum of affected patients, a variety of allied disorders were grouped under the name of NMO spectrum disorders, including recurrent myelitis associated with LETM and myelitis associated with autoimmune disorders such as SS. There have been only a few cases reported in the literature with recurrent LETM associated with non-organ specific autoimmune disorder.

2021 ◽  
Vol 14 (1) ◽  
pp. e238992
Author(s):  
Katherine Thornley ◽  
Carlo Canepa

An 80-year-old, previously healthy patient presents with acute transverse myelitis with sensory level at T8. The MRI scan of the spinal cord showed longitudinal extensive transverse myelitis, and she tested positive for aquaporin 4 antibodies in serum. She received treatment with intravenous and oral steroids, with no improvement and then underwent plasma exchange. She was then started on azathioprine for prevention of relapses, while continuing physiotherapy and occupational therapy. Eventually, she was transferred to a specialised spinal cord centre for long-term rehabilitation.


2018 ◽  
pp. 201-204
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand

Primary CNS angiitis (PACNS) is distinguished from other vasculitides by its exclusive involvement of the CNS, generally in a multifocal manner. Lesions are typically, but not invariably, ischemic or hemorrhagic. Symptoms most frequently include headache, but also commonly involve cognitive impairment, focal neurological symptoms, and sometimes seizures. The disorder may involve medium or small-sized vessels. MRI of brain and spinal cord is usually abnormal, frequently showing cortical and subcortical infarcts. However, in the case of smaller vessel involvement, ischemic demyelination may be seen. Angiography frequently suggests the diagnosis by demonstration of “beading,” but biopsy is often necessary to firmly establish the diagnosis. Treatment is usually with high-dose pulse methylprednisolone followed by a prolonged course of oral prednisone, but the addition of other immunosuppressant medication is often necessary.


2020 ◽  
pp. 004947552095644
Author(s):  
Manjeet Kumar Goyal ◽  
Mahesh Lal

Transverse myelitis typically extends two or less spinal segments, whereas longitudinal extensive transverse myelitis (LETM) extends three or more spinal segments in length and may occasionally span all the segments of the spinal cord. We present a case of spinal tuberculosis presenting with LETM with true lower motor neuron-type flaccid paraplegia.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Kenya Nishioka ◽  
Ryota Tanaka ◽  
Satoshi Tsutsumi ◽  
Hideki Shimura ◽  
Yutaka Oji ◽  
...  

Background. Longitudinally extensive transverse myelitis (LETM) is characterized by spinal cord inflammation extending vertically through three or more vertebral segments. The widespread use of MRI revealed LETM more frequency than ever. We report the case of a patient with pathologically confirmed small-cell lung carcinoma metastasis into the spinal cord presenting as LETM.Case Presentation. A 74-year-old man developed rapidly progressive sensorimotor disturbance and vesicorectal dysfunction. T2-weighted magnetic resonance imaging of the spine revealed LETM at the level of from T3 to conus medullaris; gadolinium enhancement showed concurrent tumor in the thoracic spinal cord from T10 to T11. Systemic survey identified a nodular mass in the lung that was verified as small-cell carcinoma. Following initial failed treatment by high-dose steroid, the patient underwent an emergent microsurgical tumor resection. Histological examination was identical with the lung carcinoma. The patient died of tumor progression at the 47th day after admission. At autopsy, only changes of edema were found in the gray matter of the cord, while tumor cells were not noted in it.Conclusion. Metastasis may rarely present symptoms of LETM. Prompt identification of underlying etiology by contrast examination and systemic survey is crucial for the patient assumed as LETM.


2021 ◽  
pp. 78-83
Author(s):  
Shunya Fujiwara ◽  
Yasuhiro Manabe ◽  
Yumiko Nakano ◽  
Yoshio Omote ◽  
Taijun Yunoki ◽  
...  

We report 2 cases of probable neuro-Behçet’s disease (NBD) with longitudinally extensive transverse myelitis (LETM). In both cases, the patients presented paraplegia, as well as sensory, bladder, and rectal disturbances. Magnetic resonance imaging (MRI) of patient 1 showed continuous high signal intensity extending from the midbrain to the entire spinal cord in the central part of the cord on T2-weighted imaging (T2WI). Spinal MRI of patient 2 revealed high signal intensity extending from Th2 to Th10 in the central part of the cord on T2WI. Both patients received high-dose methylprednisolone. A continuous lesion from the midbrain to the entire spinal cord as in patient 1 has not been previously reported. Patient 2 dramatically improved by infliximab therapy. The present cases suggest that NBD should be considered as a differential diagnosis in patients with LETM.


2021 ◽  
pp. 194187442110473
Author(s):  
Lucas Horta ◽  
Deepti Virmani ◽  
K. H. Vincent Lau ◽  
Pria Anand

This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.


2017 ◽  
Vol 18 (2) ◽  
pp. 143-145 ◽  
Author(s):  
Yudy Llamas ◽  
Karl Hazel ◽  
Patrick Nicholson ◽  
Lisa Costelloe

Campylobacter jejuni infection is well-known to precipitate Guillain-Barré syndrome through an immune-mediated attack on the peripheral nervous system. Molecular mimicry between C. jejuni lipo-oligosaccharides on the surface of infectious agents and human gangliosides in the peripheral nerves induces cross-reactive immune responses. Although gangliosides also occur in the central nervous system (CNS), autoimmune CNS disorders rarely follow C. jejuni infections. However, longitudinally extensive transverse myelitis commonly has a parainfectious cause, triggered by a wide range of micro-organisms including viruses and bacteria. We report a patient who developed longitudinally extensive transverse myelitis after C. jejuni enteritis associated with antiganglioside antibodies.


2011 ◽  
Vol 18 (3) ◽  
pp. 271-285 ◽  
Author(s):  
JL Kitley ◽  
MI Leite ◽  
JS George ◽  
JA Palace

Longitudinally extensive transverse myelitis refers to florid and widespread inflammation of the spinal cord causing T2 hyperintensity on spinal magnetic resonance imaging that is seen to extend over three or more vertebral segments. Whilst rare, longitudinally extensive transverse myelitis is clinically important as it can lead to catastrophic morbidity, and a group of these patients are at risk of further attacks. Early identification and establishment of the underlying aetiology is vital in order to initiate appropriate therapy and optimize outcomes. Whilst longitudinally extensive transverse myelitis is classically associated with neuromyelitis optica, there are many other causes. These include other inflammatory aetiologies, infection, malignancy and metabolic disturbance. Some of these are readily treatable. Laboratory and radiological investigations can help to differentiate these causes. Treatment of longitudinally extensive transverse myelitis hinges on distinguishing inflammatory and non-inflammatory aetiologies and identifying patients who are at high risk of a recurrent course.


2021 ◽  
Author(s):  
Jean-Christophe Van Cutsem ◽  
Ann-Sophie Lamon ◽  
Vincent Van Belleghem ◽  
Evelien Vancaester

Abstract IntroductionSince the COVID-19 pandemic, a growing number of central nervous system (CNS) complications in patients with COVID-19 have been reported. Isolated, longitudinally extensive transverse myelitis (LETM), is a unique presentation of CNS involvement. The limited reports, its diverse clinical manifestations and the possible long-term consequences make the reporting crucial to further our understanding of those syndromes occurring in COVID-19 positive patients.Case PresentationA 63-year old male consulted the emergency department after a sudden onset of gait ataxia, a one-week history of paresthesia progressing from the feet to the midsternal area and urinary. He tested positive on a SARS-CoV-2 RNA RT-PCR nasopharyngeal swab two days prior to the onset of his symptoms. Neurological examination showed a sensory level at T7 with symmetrically reduced fine touch, vibration, proprioception and furthermore an ataxic gait was observed. Cerebrospinal fluid on day one of admission showed pleocytosis, predominantly neutrophils, elevated protein count and normal glucose level and IgG. MRI of the spinal cord revealed a diffusely increased signal intensity involving the near-complete spinal cord, from the brainstem to level T12, fitting the diagnosis of LETM. ConclusionThe few cases of transverse myelitis in association with COVID-infection are believed to have an immune-mediated postinfectious mechanism. In this case however, parainfectious direct viral invasion of the spinal cord is far more likely because of a neutrophilic predominance in CSF and a short timespan between infection and symptoms. It could provide more clues that the SARS-CoV-2 is acutally capable of causing direct neurotoxic effects.


2013 ◽  
Vol 20 (7) ◽  
pp. 882-888 ◽  
Author(s):  
M Ringelstein ◽  
I Metz ◽  
K Ruprecht ◽  
A Koch ◽  
J Rappold ◽  
...  

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.


Sign in / Sign up

Export Citation Format

Share Document