A Case of Longitudinally Extensive Transverse Myelitis

2018 ◽  
pp. 201-204
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Spinal Cord ◽  
Cognitive Impairment ◽  
Oral Prednisone ◽  
Transverse Myelitis ◽  
High Dose ◽  
Pulse Methylprednisolone ◽  
Brain And Spinal Cord ◽  
Extensive Transverse Myelitis ◽  
Vessel Involvement ◽  
Immunosuppressant Medication

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.

Neuromyelitis optica spectrum disorder (NMOSD) presenting as acute transverse myelitis with positive aquaporin 4 antibodies

2021 ◽  
Vol 14 (1) ◽  
pp. e238992
Author(s):  
Katherine Thornley ◽  
Carlo Canepa
Keyword(s):  
Spinal Cord ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Sensory Level ◽  
Mri Scan ◽  
Acute Transverse Myelitis ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Oral Steroids ◽  
Extensive Transverse Myelitis

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.

Evaluation of brain and spinal cord lesion distribution criteria at disease onset in distinguishing NMOSD from MS and MOG antibody-associated disorder

2020 ◽  
pp. 135245852093900 ◽  
Author(s):  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Chun-Hong Shen ◽  
Fan Yang ◽  
Wei Fang ◽  
...  
Keyword(s):  
Chinese Population ◽  
Diagnostic Performance ◽  
Disease Onset ◽  
Transverse Myelitis ◽  
Performance Accuracy ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Periventricular Lesions ◽  
Brain And Spinal Cord ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

Objective: To validate the recently proposed imaging criteria in distinguishing aquaporin-4 antibody (AQP4-ab)-seropositive neuromyelitis optica spectrum disorder (NMOSD) from multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein antibody-associated disorder (MOG-AD) at disease onset in a Chinese population. Methods: We enrolled 241 patients in this retrospective study, including 143 AQP4-ab-seropositive NMOSD, 73 MS, and 25 MOG-AD. Cacciaguerra’s criteria were described as fulfillment of at least 2/5 conditions including the absence of the combined juxtacortical/cortical lesions, the presence of longitudinal extensive transverse myelitis (LETM) lesions, the presence of periependymal-lateral ventricles lesions, the absence of Dawson’s fingers lesions, and the absence of periventricular lesions. Results: Fulfillment of at least 3/5 conditions was able to differentiate NMOSD from MS with a good diagnostic performance (accuracy = 0.92, sensitivity = 0.91, specificity = 0.93), yet failed to differentiate NMOSD from MOG-AD. LETM lesions showed the highest accuracy (0.78), sensitivity (0.70), and specificity (0.97) for NMSOD. Conclusion: Our research suggested the utility of Cacciaguerra’s criteria in a Chinese population at disease onset. A better diagnostic performance in NMOSD could be attained with at least 3/5 conditions fulfilled. Yet their utility in distinguishing NMOSD from MOG-AD was limited.

Tubercular longitudinally extensive transverse myelitis with lower motor neuron paralysis

2020 ◽  
pp. 004947552095644
Author(s):  
Manjeet Kumar Goyal ◽  
Mahesh Lal
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Spinal Tuberculosis ◽  
Transverse Myelitis ◽  
Lower Motor Neuron ◽  
Neuron Type ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Spinal Segments ◽  
Extensive Transverse Myelitis

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.

scholarly journals P.049 Medically refractory longitudinally extensive transverse myelitis successfully treated with cyclophosphamide induction

2016 ◽  
Vol 43 (S2) ◽  
pp. S32-S33
Author(s):  
LJ Baxter ◽  
S Chen ◽  
JM Burton
Keyword(s):  
Respiratory Failure ◽  
Plasma Exchange ◽  
Immune Therapy ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Intravenous Methylprednisolone ◽  
Intensive Care Admission ◽  
Extensive Transverse Myelitis

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.

A Woman With Subacute Numbness of the Trunk, Arms, and Legs

2021 ◽  
pp. 233-234
Author(s):  
Eoin P. Flanagan
Keyword(s):  
Spinal Cord ◽  
Magnetic Resonance ◽  
Magnetic Resonance Image ◽  
Oral Prednisone ◽  
Transverse Myelitis ◽  
Pulmonary Sarcoidosis ◽  
Response To Treatment ◽  
Gadolinium Enhancement ◽  
T2 Hyperintensity ◽  
Noncaseating Granulomas

A 39-year-old woman had development of new-onset numbness in her left arm. This progressed over 2 to 3 weeks to involve the left axilla, trunk, lower extremities, and genital region. She had mild imbalance and left-sided weakness but remained ambulating without a gait aid. Repeated magnetic resonance imaging of the cervical spine showed the longitudinally extensive T2 lesion in a central location on axial sequences, with linear, dorsal, subpial gadolinium enhancement extending more than 2 vertebral segments. The magnetic resonance image findings were most suggestive of spinal cord neurosarcoidosis. Computed tomography of the chest showed bilateral hilar adenopathy. Serum levels of angiotensin-converting enzyme were normal. Transbronchial lung biopsy showed noncaseating granulomas. Noncaseating granulomas were confirmatory of pulmonary sarcoidosis, which led to a diagnosis of spinal cord neurosarcoidosis. Treatment with intravenous methylprednisolone for 5 days was repeated, followed by oral prednisone for 2 months. Her neurologic symptoms improved, and repeated magnetic resonance image showed a marked decrease in T2 hyperintensity and gadolinium enhancement consistent with interval response to treatment. A slow prednisone taper over 9 months was initiated. Spinal cord neurosarcoidosis often presents with an isolated myelopathy without symptoms of pulmonary sarcoidosis (eg, cough, dyspnea). The presentation can range from a subacute onset mimicking transverse myelitis (as in this case patient) to a more insidious progressive myelopathy over months to years.

scholarly journals Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids, with Cranial and Caudal Extension

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Mahmood Mubasher ◽  
Aseel Sukik ◽  
Ahmed Hassan El Beltagi ◽  
Ali Rahil
Keyword(s):  
Spinal Cord ◽  
Lower Limbs ◽  
High Dose ◽  
Oral Corticosteroids ◽  
Dose Oral ◽  
Multiple Levels ◽  
Romberg Test ◽  
Brain And Spinal Cord ◽  
The Brain

A 23-year-old lady presented with vertigo and imbalance in walking, blurring of vision, diplopia, and headache, in addition to numbness in the lower limbs over a period of six days. On examination patient had nystagmus, ataxia, positive Romberg test, and hyperreflexia. MRI examination of the brain and spinal cord showed evidence of faint bright signal intensity foci in T2/FLAIR involving bilateral cerebral hemispheres, subcortical deep white matter, bilateral thalami, posterior pons and left brachium pontis, and basal ganglia, with small nodular enhancement that aligned along curvilinear structures; those lesions also were apparent along the spinal cord at multiple levels. The clinical and radiological features suggested CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) syndrome. Symptoms improved dramatically with high dose oral corticosteroids. Our report addresses the radiological and clinical pattern of a case of CLIPPERS rhombencephalitis, with added superior and inferior extension to involve the brain and spinal cord, which is to emphasize the importance of raising the awareness of this disease and the combined role of radiologist and physicians for the diagnosis of this potentially treatable entity, responsive to glucocorticosteroid immunosuppression.

scholarly journals Longitudinally Extensive Transverse Myelitis with Intramedullary Metastasis of Small-Cell Lung Carcinoma: An Autopsy Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Kenya Nishioka ◽  
Ryota Tanaka ◽  
Satoshi Tsutsumi ◽  
Hideki Shimura ◽  
Yutaka Oji ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Transverse Myelitis ◽  
Small Cell ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Small Cell Lung ◽  
Thoracic Spinal Cord ◽  
Cell Lung Carcinoma ◽  
Extensive Transverse Myelitis

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.

Аcute transverse myelitis and Guillain – Barre overlap syndrome in a patient with СOVID-19

2022 ◽  
Vol 26 (6) ◽  
pp. 43-51
Author(s):  
E. A. Ruina ◽  
V. N. Grigoryeva ◽  
A. A. Lesnikova ◽  
K. M. Beliakov
Keyword(s):  
Spinal Cord ◽  
Transverse Myelitis ◽  
Bowel Dysfunction ◽  
Intravenous Immunoglobulins ◽  
Sensory Loss ◽  
Focal Lesion ◽  
High Dose ◽  
Bladder Bowel Dysfunction ◽  
Guillain Barré

We report a case of transverse myelitis and Guillain–Barre syndrome (GBS) overlap in the 42-year-old patient with moderate course of Coronavirus disease 2019 (СOVID-19). Nasopharyngeal SARS-CoV 2 RT-PCR was positive. Severe neck pain developed in this patient on the 5-th day of СOVID-19. A few hours later weakness in the feet arised and then spread to the thighs and arms. Quadriparesis, arefl exia in all limbs, sensory loss below the level of T4 and bladder/bowel dysfunction were present. Pyramidal signs were negative. There was no increase of COVID-19 severity at the time of neurological signs development. Magnetic resonance imaging of the spinal cord showed the focal lesion in the C2-T1 segments, which was consistent with the features of longitudinally extensive transverse myelitis. Along with the myelitis, acute motor axonal polyneuropathy was diagnosed. This diagnosis of GBS was supported by ascending weakness with arefl exia, albumin-cytological dissociation in cerebrospinal fl uid and the data of neuroelectrophysiological examination. We proposed that both myelitis and GBS had disimmune nature associated with COVID-19. The other possible causes of damage to the spinal cord and peripheral nervous system were excluded.Immunotherapy with high dose of intravenous immunoglobulins was administered. Steroids also were used taking into account the myelitis. At the follow up in 4 months the motor functions were found to be improved nonsignifi cantly, the patient was still severe disabled.

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 ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Viral Infections ◽  
Serum Proteins ◽  
Transverse Myelitis ◽  
Autoimmune Disorder ◽  
Lumbar Region ◽  
Wide Range ◽  
Organ Specific ◽  
Anogenital Area ◽  
Extensive Transverse Myelitis

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.

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