Idiopathic Transverse Myelitis Research Articles

The Role of Diffusion-Weighted MRI in Differentiation of Idiopathic Acute Transverse Myelitis and Acute Spinal Cord Infarction

The Role of Diffusion-Weighted MRI in Differentiation of Idiopathic Acute Transverse Myelitis and Acute Spinal Cord Infarction

Approach to acute or subacute myelopathy

Improved understanding of the differential diagnosis and improved investigative techniques, particularly neuroimaging and serologic testing, have facilitated the diagnosis of patients with acute and subacute myelopathy and reduced the proportion of patients who are labeled as having “idiopathic transverse myelitis.” Additionally, these advances have identified subgroups of patients in whom progression of deficit or future relapses are anticipated, allowing intervention and prophylaxis as appropriate. However, early management remains empiric and consists of high-dose corticosteroids for most patients. In the event of an inadequate response to corticosteroids or a subsequent atypical course, further investigations to detect diagnoses other than “transverse myelitis” should be considered and additional treatments, such as plasmapheresis, may be appropriate. Individualized diagnosis and treatment is more feasible now than in the past. Localizing an acute neurologic process to the spinal cord is often, but not always, straightforward (table 1). The ascending sensory symptoms of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) may confuse the diagnosis, as this complaint is also highly associated with acute myelopathy. Unequivocal upper motor neuron signs exclude AIDP, but are often not apparent in the early stages of a spinal cord insult. Although not invariably present, an unequivocal sensory level on the torso, sensory loss indicating involvement of spinal tracts (e.g., spinothalamic modality impairment contralateral to motor findings), or urinary retention localize to the spinal cord. Myopathy or neuromuscular junction disorders may be mistaken for myelopathy, particularly if the lower limbs are predominantly affected, but the absence of any sensory abnormality should suggest the correct localization. Bilateral mesial frontal lobe lesions (e.g., bilateral anterior cerebral artery distribution infarcts) could mimic a myelopathy, although abulia or other signs of frontal lobe dysfunction typically coexist. Autoimmune or paraneoplastic muscle stiffness syndromes, such as stiff-person syndrome associated with glutamic acid decarboxylase or amphiphysin autoantibodies, may be confused with spasticity …

POI04 Transverse myelitis—a single centre review

IntroductionInflammatory demyelinating transverse myelitis can be the first presentation of multiple sclerosis or neuromyelitis optica or remain an isolated event—idiopathic acute transverse myelitis (ATM). Data on relative proportions of these...

Idiopathic acute transverse myelitis in children: an analysis and discussion of MRI findings

Background:There is lack of reported magnetic resonance imaging (MRI) studies of idiopathic acute transverse myelitis (ATM) in children. Objective:To describe the imaging features of idiopathic ATM in children. Methods:We retrospectively analyzed the spinal MRI findings of children diagnosed with ATM. The anatomic regions, vertebral segmental length, gray or white matter involvement, cord expansion and gadolinium enhancement were examined. Results:A total of 27 children were diagnosed with isolated monophasic ATM with a mean follow-up of 5.2 years. Two children later diagnosed with neuromyelitis optica were excluded from the pediatric ATM cohort. None of the patients had a subsequent diagnosis of multiple sclerosis. The mean age of onset was 9.5 years (0.5–16.9 years). Spinal MRIs were abnormal in 21 (78%). The mean interval between symptom onset and the MRI was 1.7 days (0–19 days). Central cord hyperintensity involving gray matter was seen in all patients. A majority (67%) of the patients demonstrated long segment lesions with a mean segment length of 6.4. Conclusions:We conclude that central cord inflammation extending over three or more segments is the most common finding of idiopathic monophasic transverse myelitis in children. The risk of multiple sclerosis in children who experience isolated transverse myelitis as a first demyelinating event is low.

The clinical course of idiopathic acute transverse myelitis in patients from Rio de Janeiro

The aim of this study was to describe the demographic, clinical and laboratory features of idiopathic acute transverse myelitis (IATM). Patients with non-compressive ATM receiving care at Hospital da Lagoa, Rio de Janeiro (Brazil) between 2000 and 2008 were selected. Of the 70 cases of acute myelopathies, the idiopathic form was identified in 41 following exclusion of the cases associated with systemic lupus erythematosus (n = 1), Sjogren's syndrome (n = 1), herpes zoster (n = 1), cytomegalovirus in an HIV-positive patient (n = 1), Schistosoma mansoni (n = 1), actinic myelitis (n = 1), infectious myelitis of unknown etiology (n = 2) and those that, following the first attack of myelitis, converted to NMO (n = 19) or to clinically defined MS (n = 2). Of the 41 cases of IATM, the majority of patients were female (68.3%) and white (65.9%). Median age at first myelitis was 37.0 +/- 11.8 years. Over a median observation time of 36 months, 39.0% of patients remained monophasic, while recurrences occurred in 61.0% of cases. The number of ATM/patient ranged from one to seven. Among the recurrent cases the median time between the first and the second ATM was 12 months (range 1-150 months).The first myelitis was characterized mainly by partial myelitis with motor and sensorial dysfunction (63.4%). Complete and severe myelitis occurred more frequently among monophasic patients and partial myelitis with moderate dysfunction at onset in recurrent cases; however, over the long-term, dysfunction and disability were mild in both groups. Serial spine MRI confirmed spinal cord inflammation in 92.0% of cases and extensive spinal cord lesion was identified in 61.0%. Brain MRI was normal or not suggestive of MS in 94.4% of cases. CSF showed pleocytosis in 41.2%, increased IgG index in 24.0% and oligoclonal bands in 38.0% of 34 patients tested. Abnormal visual evoked potentials occurred in 11.5% of 26 patients. Positivity for anti-AQP4 was found in 23.5% of the 17 cases tested, suggesting limited forms of NMO. This study suggests some new aspects of the clinical course of IATM such as the high conversion rate to NMO, the predominance of women and a higher frequency of recurrent forms.

Pearls: Myelopathy

Both general neurologists and neurologists with a broad spectrum of subspecialty interests are often asked to evaluate patients with disorders of the spinal cord. Over the past decade, there have been significant advances in our understanding of a wide spectrum of immune-mediated, infectious, metabolic, hereditary, paraneoplastic, and compressive myelopathies. Advances have been made in the classification and management of spinal vascular malformations. Aortic reconstruction surgery has led to an increased incidence of spinal cord stroke. It is important to recognize a dural arteriovenous fistula as a cause of progressive myelopathy. In the past, noninfectious inflammatory myelopathies have frequently been categorized as idiopathic transverse myelitis. Advances in neuroimaging and discovery of a serum antibody marker, neuromyelitis optica-immunoglobulin G (NMO-IgG), have allowed more specific diagnoses, such as multiple sclerosis and neuromyelitis optica. Abnormalities suggestive of demyelinating disease on brain magnetic resonance imaging (MRI) are known to be highly predictive of conversion to multiple sclerosis in a patient who presents with a transverse myelitis ("clinically isolated syndrome"). Acquired copper deficiency can cause a clinical picture that mimics the subacute combined degeneration seen with vitamin B (12) deficiency. A history of bariatric surgery is commonly noted in patients with copper deficiency myelopathy. Genetics has advanced our understanding of the complex field of hereditary myelopathies. Three hereditary myelopathy phenotypes are recognized: predominantly cerebellar (e.g., Friedreich's ataxia), predominantly motor (e.g., hereditary spastic paraparesis), and a leukodystrophy phenotype (e.g., adrenomyeloneuropathy). Evaluation of myelopathies when no abnormalities are seen on spinal cord imaging is a commonly encountered diagnostic challenge. This article presents some "clinical pearls" in the evaluation and management of spinal cord diseases in context of these recent developments.

Anaesthetic management of an obstetric patient with idiopathic acute transverse myelitis

Idiopathic acute transverse myelitis is a rare focal inflammatory disorder of the spinal cord causing motor, sensory and autonomic dysfunction. We report the successful use of general anaesthesia for caesarean section in a patient with this disease. Potential anaesthetic concerns include autonomic dysreflexia and hyperkalaemia following the use of suxamethonium. Further complicating issues with this patient included psychotic depression and new-onset neuropathic pain on a background of chronic pain symptoms.

Common CYP7A1 promoter polymorphism associated with risk of neuromyelitis optica

Neuromyelitis optica (NMO) is a severe idiopathic inflammatory disease of the central nervous system primarily affecting the optic nerves and spinal cord. In this study, we generated genome-wide SNP data from NMO patients and normal controls (53 cases and 240 controls), and followed up on the association signals with samples from a larger number of inflammatory demyelinating diseases, including NMO ( n = 93), multiple sclerosis (MS, n = 71), idiopathic recurrent transverse myelitis (IRTM, n = 57), and normal controls ( n = 240). Statistical analyses revealed that a common promoter SNP in CYP7A1 has a protective/gene dose-dependent effect on the risk of NMO ( P = 0.0004). A stronger association between the variables and subsequently, a higher protective effect (lower OR) on the risk of NMO were observed among patients carrying the “G/G” genotype of rs3808607 than those with the “T/G” genotype (OR = 0.38/ P = 0.01 vs. OR = 0.12/ P = 0.0004, respectively). The associations which were only observed in patients with NMO suggest that there are differences in the genetic etiology of the inflammatory demyelinating diseases (NMO, classical MS, and IRTM).

Plasma exchange for acute attacks of CNS demyelination

Plasma exchange (PE) is used to treat severe episodes of CNS demyelination unresponsive to corticosteroids. Predictors of long-term response are not well known. We retrospectively reviewed the medical records of 41 patients consecutively treated by PE between January 1995 and July 2007. The primary outcome was improvement at 6 months after PE defined as decrease of >or=1 point in the Expanded Disability Status Scale (EDSS) score for patients with EDSS <or=7.5 or 1.5 points with EDSS >or=8.0 or improvement of more than 2 lines in the visual acuity chart for patients with optic neuritis (ON). Twenty-five patients (61%) were women, and the median age was 33 years (range 14-57 years). Twenty-three (56%) had multiple sclerosis, 2 (5%) had clinically isolated syndrome, 2 (5%) had Marburg disease, 7 (17%) had acute disseminated encephalomyelitis, 4 (10%) had neuromyelitis optica, 2 (5%) had idiopathic ON, and 1 (2%) had idiopathic transverse myelitis. The median EDSS score before the attack was 1.0 (range 0-6.5). At PE onset, the median EDSS score was 7.0 (range 3.0-9.5). Sixteen patients (39%) improved at discharge, and 26 (63%) improved at 6 months. In the multivariate analysis, early initiation of PE (odds ratio [OR] 6.29, 95% confidence interval [CI] 1.18-52.96) and improvement at discharge (OR 7.32, 95% CI 1.21-44.38) were significantly associated with response at 6 months. Plasma exchange (PE) was associated with clinical improvement in 63% of patients at 6 months. Early initiation of PE and improvement at discharge were predictors of this response. Twelve patients (48%) who did not improve early did so during follow-up.

Identifying Transverse Myelitis in the Emergency Care Arena

Transverse myelitis (TM) is a neurological syndrome involving destruction of the anterior and posterior (transverse) segments of the spinal cord. The objective of this article is to educate emergency nurses and nurse practitioners about how to recognize, diagnose, and treat TM. A comparison of the neurological findings of TM and Guillain-Barré syndrome is presented. In addition, this article will include an algorithm and specific diagnostic criteria to assist in the diagnosis of acute idiopathic TM.

Transverse myelitis as a presenting feature of late onset systemic lupus erythematosus

Transverse myelitis as a presenting feature of late onset systemic lupus erythematosus

Neuromyelitis optica‐IgG in idiopathic inflammatory demyelinating disorders amongst Hong Kong Chinese

Idiopathic inflammatory demyelinating disorders (IIDD) affect the central nervous system. In classical multiple sclerosis (CMS), brain, optic nerves [optic neuritis (ON)] and spinal cord [acute transverse myelitis (ATM)] are affected. In neuromyelitis optica (NMO), optic nerves and spinal cord are predominantly affected. NMO-IgG, an autoantibody targeting aquaporin-4, is a marker for NMO. We studied the frequency and clinical relevance of NMO-IgG seropositivity in IIDD patients. Neuromyelitis optica-IgG was detected by indirect immunofluorescence using primate cerebellum. Neuromyelitis optica-IgG was detected in six of 10 NMO patients (60%), six of 10 idiopathic relapsing transverse myelitis (IRTM) patients (60%), two of nine idiopathic relapsing ON patients (22%), one of 11 patients (9%) having single ON attack, one of 30 CMS patients (3%), and none of patients having single ATM attack or controls. Comparing NMO-IgG seropositive (n = 12) with NMO-IgG seronegative (n = 8) patients having NMO or IRTM, NMO-IgG seropositivity was associated with a higher relapse rate in first 2 years, 1.5 and 0.6 attacks/year for seropositive and seronegative groups respectively (P = 0.006), and non-significant trend towards more severe ON and myelitis with poorer clinical outcome. Neuromyelitis optica -IgG facilitates diagnosis of NMO spectrum disorders. NMO-IgG seropositivity is associated with higher relapse rate in first 2 years.

Mycoplasma pneumoniae–Associated Transverse Myelitis and Rhabdomyolysis

Mycoplasma pneumoniae is a common cause of respiratory tract infection. Extrapulmonary manifestations of M. pneumoniae infection are also common. The present case is that of a previously healthy 4-year-old boy who displayed a novel simultaneous onset of both acute rhabdomyolysis and transverse myelitis associated with an infection of M. pneumoniae. He had no preceding symptoms or signs of respiratory tract infection. Intravenous immunoglobulin (1 g/kg per day) for 2 days was prescribed initially for the deterioration of neurologic condition. His rhabdomyolysis resolved without complication, but neurologic sequelae remained during 2 years of follow-up. Evaluation for M. pneumoniae infection is recommended in patients with idiopathic rhabdomyolysis and transverse myelitis, even if in the absence of antecedent respiratory symptoms.

Spinal cord stimulation for neuropathic pain following idiopathic transverse myelitis: A case report.

We present a patient with intractable neuropathic pain because of idiopathic transverse myelitis unresponsive to medical treatment. After a successful trial of spinal cord stimulation, a permanent stimulator was implanted. Improvement was noted in visual analogue scale, medication usage and daily function. Spinal cord stimulation may offer a therapeutic option for patients with neuropathic pain resulting from transverse myelitis and should be considered when other treatments are failed.

NMO-IgG-negative relapsing myelitis

Idiopathic transverse myelitis (I-TM) is typically monophasic, while relapsing forms are usually referred to spinal cord-restricted neuromyelitis optica (NMO), atypical multiple sclerosis (MS), or myelitis during the course of infections and connectivitis. Our objective was to evaluate the frequency of recurrent I-TM; to clarify the nosology of these forms through comparison with NMO and post-infectious TM (P-TM). Prospective cohort study on patients presenting with I-TM was carried out inpatients of Infectious and Neurologic Disease Clinics, Italy. Over an 8-year period, we recruited 13 patients with I-TM and 16 with P-TM. The patients were followed-up for at least 3 years with repeated brain and spinal cord magnetic resonance imaging (MRI) examinations, multimodal evoked potentials and serum screen for connectivitis. Relapses were defined on clinical and imaging criteria. Four patients with I-TM (31%) had a relapsing course . They were all males with age >50, and severe at-onset disability. The final outcome was poor in three out of four patients. Serum NMO-immunoglobulin G was undetectable in all patients. Longitudinally extensive myelitis was not predictive of relapses. I-TM and P-TM shared clinical, cerebrospinal fluid (CSF) and MRI features, as well as a similar rate (54 vs 38%) of peripheral nervous system involvement (polyradiculoneuritis), and an identical rate of relapses (31% for both forms). Our series support the existence of relapsing I-TM as a disease entity that does not appear related to NMO, nor to MS, cannot be further specified and shares many features with P-TM. The likelihood of relapses was unpredictable based on clinical, CSF and MRI findings.

Involvement of Acidic Fibroblast Growth Factor in Spinal Cord Injury Repair Processes Revealed by a Proteomics Approach

Acidic fibroblast growth factor (aFGF; also known as FGF-1) is a potent neurotrophic factor that affects neuronal survival in the injured spinal cord. However, the pathological changes that occur with spinal cord injury (SCI) and the attribution to aFGF of a neuroprotective effect during SCI are still elusive. In this study, we demonstrated that rat SCI, when treated with aFGF, showed significant functional recovery as indicated by the Basso, Beattie, and Bresnahan locomotor rating scale and the combined behavior score (p < 0.01-0.001). Furthermore proteomics and bioinformatics approaches were adapted to investigate changes in the global protein profile of the damaged spinal cord tissue when experimental rats were treated either with or without aFGF at 24 h after injury. We found that 51 protein spots, resolvable by two-dimensional PAGE, had significant differential expression. Using hierarchical clustering analysis, these proteins were categorized into five major expression patterns. Noticeably proteins involved in the process of secondary injury, such as astrocyte activation (glial fibrillary acidic protein), inflammation (S100B), and scar formation (keratan sulfate proteoglycan lumican), which lead to the blocking of injured spinal cord regeneration, were down-regulated in the contusive spinal cord after treatment with aFGF. We propose that aFGF might initiate a series of biological processes to prevent or attenuate secondary injury and that this, in turn, leads to an improvement in functional recovery. Moreover the quantitative expression level of these proteins was verified by quantitative real time PCR. Furthermore we identified various potential neuroprotective protein factors that are induced by aFGF and may be involved in the spinal cord repair processes of SCI rats. Thus, our results could have a remarkable impact on clinical developments in the area of spinal cord injury therapy.

Diffusion Tensor Imaging in Idiopathic Acute Transverse Myelitis

Our study was based on our hypotheses that in idiopathic acute transverse myelitis (ATM), fractional anisotropy (FA) values would be abnormal not only in the T2-hyperintense lesion but also in the surrounding normal-appearing spinal cord and that the abnormal FA values in the spinal cord could be related to clinical outcome. Sagittal diffusion tensor imaging (DTI) was performed in 10 patients with idiopathic ATM (four men, six women; mean age, 45 years; age range, 20-66 years) and 10 sex- and age-matched normal volunteers. FA measurements were made in the spinal cord at three levels: lesion, proximal normal-appearing spinal cord, and distal normal-appearing spinal cord. The grade of FA decrease (mild, less than 10% decrease [(FA normal - FA pt) x 100 / FA normal]; moderate, 10-20%; severe, more than 20%) was related to the clinical outcome, which was determined by a neurologist using Paine's scale of normal, good, fair, or poor. Mean FA values in patients were significantly lower than those in normal volunteers in lesions (0.5328 vs 0.7125, p = 0.002) and distal normal-appearing spinal cord (0.6676 vs 0.7720, p = 0.0137). All three patients with a mild FA decrease or increase in distal normal-appearing spinal cord showed a normal or good outcome, but all three patients with a severe FA decrease in distal normal-appearing spinal cord showed a fair outcome, among the eight patients to whom steroid treatment was given. FA values in lesions and in distal normal-appearing spinal cord significantly decreased in patients with idiopathic ATM, and FA decrease in distal normal-appearing spinal cord might be related to clinical outcome.

INFECTIOUS AND INFLAMMATORY MYELOPATHIES

ABSTRACTInflammatory and infectious myelopathies are common and often treatable. Infectious causes include viral, bacterial, mycobacterial, fungal, and parasitic agents. Noninfectious inflammatory myelopathies were previously often categorized as idiopathic transverse myelitis, but advances in neuro

Idiopathic transverse myelitis presenting as the Brown-Sequard syndrome

Case report. To report an unusual case of Brown-Sequard syndrome (BSS) associated with idiopathic transverse myelitis (TM). Department of Neurosurgery, Chonnam National University Hospital, Gwangju, South Korea. A 38-year-old man presented with left leg weakness and right-sided decrease in sensation at the T11 level below. Magnetic resonance images (MRI) of the thoracic spine showed diffuse swelling of the spinal cord spanning the fifth to the eighth thoracic vertebra. The lesion had high signal intensity on T2-weighted images. Eccentric nodular enhancement within the left anterolateral aspect of the spinal cord was appreciated with gadolinium administration. The patient was treated with pulsed methylprednisone and showed marked improvement in neurological function within 3 days. An MRI at 5-month follow-up demonstrated complete resolution of the abnormalities. This case illustrates a rare case of BSS caused by idiopathic TM of the thoracic spinal cord. TM should be considered in the differential diagnosis of BSS.

606: Incidence, prognostic features and clinical outcomes of idiopathic acute transverse myelitis in Canterbury, New Zealand

Acute transverse myelitis (ATM) manifests as sensory, motor or autonomic dysfunction due to inflammation of the spinal cord. Common causes of inflammation include post radiation myelopathy, cord infarction, infections, multiple sclerosis or related to systemic disease. Recent diagnostic criteria for acute transverse myelitis by the Transverse Myelitis Consortium Working Group propose a definition for “idiopathic” ATM. Signs, symptoms, time course of progression and evidence for inflammation consistent with ATM are specified for inclusion criteria.