Pediatric Transverse Myelitis Research Articles

Comparison of acute flaccid myelitis and transverse myelitis in children and evaluation of diagnostic criteria.

Acute flaccid myelitis (AFM) and transverse myelitis (TM) are serious conditions that may be difficult to differentiate, especially at onset of disease. In this study, we compared clinical features of pediatric AFM and TM and evaluated current diagnostic criteria, aiming to improve early and accurate diagnosis. Two cohorts of children with enterovirus D68-associated AFM and clinically diagnosed TM were compared regarding presenting clinical features, additional investigations, and outcome. Current diagnostic criteria for AFM and TM were applied to evaluate their specificity. Children with AFM (n = 21) compared to those with TM (n = 36) were younger (median 3 vs. 10 years), more often had a prodromal illness (100% vs. 39%), predominant proximal weakness (69% vs. 17%), and hyporeflexia (100% vs. 44%), and less often had sensory deficits (0% vs. 81%), bowel and/or bladder dysfunction (12% vs. 69%), and hyperreflexia (0% vs. 44%). On magnetic resonance imaging, brainstem involvement was more common in AFM (74% vs. 21%), whereas supratentorial abnormalities were only seen in TM (0% vs. 40%). When omitting the criterion of a sensory level, 11 of 15 (73%) children with AFM fulfilled the diagnostic criteria for TM. Of children with TM, four of 33 (12%) fulfilled the diagnostic criteria for probable/definite AFM. Although there is considerable overlap between AFM and TM in children, we found important early differentiating clinical and diagnostic features. Meeting diagnostic criteria for AFM in children with TM and vice versa underlines the importance of thorough clinical examination and early and accurate diagnostic studies.

Clinical Approach to Pediatric Transverse Myelitis, Neuromyelitis Optica Spectrum Disorder and Acute Flaccid Myelitis.

Pediatric transverse myelitis (TM) is an acquired, immune-mediated disorder that leads to injury of the spinal cord and often manifests as weakness, numbness, bowel dysfunction, and/or bladder dysfunction. Multiple etiologies for myelitis can result in a similar clinical presentation, including idiopathic transverse myelitis (TM), multiple sclerosis (MS), neuromyeltis optica spectrum disorder (NMOSD) associated with anti-aquaporin 4 antibodies, MOG antibody-associated disease, and acute flaccid myelitis (AFM). Diagnosis relies on clinical recognition of the syndrome and confirming inflammation through imaging and/or laboratory studies. Acute treatment is targeted at decreasing immune-mediated injury, and chronic preventative therapy may be indicated if TM is determined to be a manifestation of a relapsing disorder (i.e., NMOSD). Timely recognition and treatment of acute transverse myelitis is essential, as it can be associated with significant morbidity and long-term disability.

Disability Scores After Idiopathic Pediatric Transverse Myelitis are Associated with Clinical Features at Onset of Illness (P4.6-040)

Disability Scores After Idiopathic Pediatric Transverse Myelitis are Associated with Clinical Features at Onset of Illness (P4.6-040)

Safety and efficacy of plasma exchange in pediatric transverse myelitis.

We sought to review safety and efficacy of therapeutic plasma exchange (TPE) in a cohort of pediatric patients with transverse myelitis. Billing data of all plasma exchanges performed at our tertiary care pediatric hospital between August 2010 and August 2016 were compared to electronic medical records to find all patients whose indication for apheresis was transverse myelitis. Patient outcomes were quantified on the modified Rankin Scale. Fifteen of 19 patients (79%) had major improvement in symptoms after a course of 4-7 therapeutic plasma exchanges. The majority required further inpatient (6, 32%) or outpatient (8, 42%) physical therapy. Four (21%) patients returned to baseline and over 75% regained their ability to ambulate as of last follow-up. Four adverse events were noted over 114 treatments. TPE can be a useful treatment for pediatric transverse myelitis. The retrospective nature of this study without a comparator group limits conclusions about efficacy. However, controlled trials would help to validate our results. This study provides Class IV evidence that plasma exchange is safe and effective in pediatric transverse myelitis.

Chiari I malformation in children with transverse myelitis

ABSTRACTPurpose: Transverse myelitis (TM) is an acute inflammatory spinal cord injury. Asymptomatic Chiari I malformation (CMI) management is highly controversial, particularly when associated with a spinal syrinx. Here, we assess the occurrence of CMI in the pediatric TM population and management outcomes. Methods: We performed a retrospective cohort study based on 61 consecutively identified pediatric TM cases over an –8-year period. We reviewed demographic characteristics, radiographic findings, presenting symptoms, and long-term outcomes. Results: Eight CMI cases were identified within the TM cohort; all presented with TM by 16 months of age and affecting the cervical spinal cord. In three cases, CMI developed 15 months to 6 years after the onset of TM. One in 10 children with TM had CMI. Conclusion: These findings demonstrate a high prevalence of CMI in patients with TM. Large prospective clinical studies are needed to further investigate the natural history and recovery strategies of CMI involved with neuroinflammatory disorders.

The Differential Diagnosis and Initial Management of Pediatric Transverse Myelitis

Purpose of Review This review provides a guide to the basic differential considerations, diagnostic testing, and initial treatment of transverse myelitis, as well as complications to watch for and a discussion of recent data on outcomes of transverse myelitis (TM) in children.

Cognitive functioning in pediatric transverse myelitis

Background: Transverse myelitis (TM) is an inflammatory disease of the spinal cord. In pediatric TM patients, cognitive and psychological problems have been described only anecdotally. Objectives: Study aims include describing cognitive dysfunction among a cohort of pediatric TM patients as well as qualitatively exploring the impact of depression, medication, and fatigue on cognitive functioning. Methods: Twenty-four consecutive TM patients referred to a pediatric demyelinating diseases clinic completed neuropsychological screening. Means, standard deviations (SD), and percentages of patients performing at or below 1.0, 1.5, and 2.0 SD from the mean on tests administered are presented. Results: Means were generally average across domains; however, scores ranged widely across subjects within each domain. The highest rate of deficits was observed in fine-motor speed/dexterity. Slightly higher frequencies of impairment were observed in attention and memory as compared to processing speed and verbal fluency. Results did not suggest a clear association between cognitive problems and depression or medication use but did suggest that fatigue may impact cognitive functioning. Conclusions: This study is the first to document cognitive deficits in pediatric TM and raises questions regarding our understanding of the central nervous system (CNS) injury associated with TM. Findings warrant further exploration of neuropsychological outcomes in TM to inform appropriate intervention.

Cognitive Deficits in Pediatric Transverse Myelitis Patients: An Unexpected Finding (P04.108)

Objective: The objective of this study was to quantify the prevalence and severity of cognitive dysfunction among pediatric transverse myelitis (TM) patients, controlling for concomitant medication use and depression. Background Idiopathic TM is diagnosed based on evidence of inflammation within the spinal cord, in the absence of cerebral involvement. This differentiates it from acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). In pediatric ADEM and MS, cognitive dysfunction is expected and well described within the literature. Up to 20% of transverse myelitis (TM) cases manifest before adulthood. Problems with cognitive and psychological functioning in TM patients have been observed anecdotally; however, no empirical data exists regarding cognitive and psychological outcomes in this population. Within our pediatric demyelinating disease center, we had observed some individual cases of cognitive dysfunction among our TM population and began a formal assessment of these patients. At the initiation of this project, any evidence of cognitive dysfunction was typically attributed to concomitant depression or medication use. This abstract provides the first ever evidence of unexplained cognitive dysfunction in pediatric TM patients. Design/Methods: TM patients seen at our multidisciplinary care clinic were routinely screened for evidence of depression and/or cognitive deficits. This data was retrospectively reviewed under an IRB approved protocol. Data about diagnosis, medication use, comorbid depression, MRI findings, and cognitive function were extracted from the clinical record into SPSS. Twenty-four patients were included in the analysis and the prevalence of cognitive deficits determined. Results: Forty-seven percent of pediatric TM patients were found to be cognitively impaired if screening tests included fine-motor components. Seventeen percent of pediatric TM had evidence of cognitive dysfunction when tasks requiring a fine-motor component were excluded. There was not a contribution from medication use or depression. Conclusions: This is the first study ever to document evidence of cognitive dysfunction among pediatric TM patients. Disclosure: Dr. Harder has nothing to disclose. Dr. Graves has nothing to disclose. Dr. Spurgin has nothing to disclose. Dr. Greenberg has received personal compensation for activities with DioGenix, Greater Good Foundation, Biogen Idec, Serono, Inc., Sanofi-Aventis Pharmaceuticals, Inc., the Multiple Sclerosis Association of America and Teva Neuroscience as a consultant and/or speaker. Dr. Greenberg holds stock and/or stock options in Diogeix. Dr. Greenberg has received research support from Guthy-Jackson Charitable Foundation, Amplimmune, Inc. and the Accelerated Cure Project.

Spinal Cord Atrophy and Early Motor Recovery following Transverse Myelitis in Pediatric Patients

ObjectiveTo compare the motor recovery following transverse myelitis in pediatric patients with and without spinal cord atrophy.MethodFrom January 1995 through December 2009, twenty children (8 boys and 12 girls with an onset at 5.7±3.8 years) that were diagnosed with transverse myelitis at a Children's Hospital in Korea, and undertaken an initial and follow-up spine magnetic resonance image (MRI) were included. Medical records and spine MRI scans were reviewed retrospectively. An initial MRI was taken 5.1±8.7 days after the onset. The interval between an initial and follow-up MRIs was 33.4±23.0 days. The motor recovery differences between subjects with and without spinal cord atrophy on follow-up MRIs were determined. Motor recovery was defined as the elevation of one or more grades of manual muscle tests of the Medical Research Council.ResultsEight patients had developed spinal cord atrophies and 12 patients had not. Of the 8 patients with spinal cord atrophy, 7 showed no motor improvement. Among the 12 patients without atrophy, 11 had motor improvement. Spinal cord atrophy on follow-up MRIs were related to the risk of no motor improvement (odds ratio=77.0, 95% confidence interval [4.114-1441.049], p-value=0.001).ConclusionChildren with transverse myelitis who had developed spinal cord atrophy on follow-up MRIs had poor motor recovery than those who had not. The appearance of spinal cord atrophy on follow-up MRI could be an indicator of poor prognosis in pediatric transverse myelitis.

The long (-itudinally extensive) and the short of it

Transverse myelitis (TM) is a potentially devastating acute inflammatory syndrome of the spinal cord. Diagnostic criteria have been recently proposed by the Transverse Myelitis Consortium Working Group1 in an effort to distinguish TM from infectious, compressive, or degenerative myelopathies. Although TM is more commonly reported in adults, TM also occurs in children and infants. The incidence of TM, as quoted by Pidcock et al.2 (referencing3,4), is 1,400 cases per year in the United States, of whom 20% are younger than 18 years. If these estimates are accurate, then TM affects 280 children per year in the United States alone. Yet a Medline search for articles in the English literature from 1995 to 2006 that directly relate to pediatric TM results in information pertaining to fewer than 250 affected children.5–16 The article by Pidcock et al., delineating the clinical features, laboratory and MRI findings, and functional outcome of 47 patients with childhood-onset TM, adds significantly to the available literature. All 47 patients met consortium criteria for TM.1 Age at diagnosis revealed a bimodal distribution with 15 of 47 patients (38%) presenting before age 3 years, and 23 (49%) presenting in adolescence. At onset, over 80% of children were paraplegic and had bladder …

Neuromyelitis Optica Immunoglobulin G in a Child

Neuromyelitis optica or Devic's syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.

Inflammatory transverse myelitis: evolving concepts

Acute transverse myelitis is a pathogenetically heterogeneous inflammatory disorder of the spinal cord. Here we describe recent advances in inflammatory non-infectious transverse myelitis. Particular attention will be paid to the serum autoantibody marker NMO-IgG and its application to acute transverse myelitis. The recent identification of neuromyelitis optica-IgG, a novel marker of neuromyelitis optica spectrum disorders (including longitudinally extensive transverse myelitis), contributes to an evolving understanding of acute transverse myelitis. Other serological markers, such as collapsin response-mediator protein-5 -IgG and amphiphysin-IgG, predict specific cancers in the setting of a paraneoplastic acute transverse myelitis. Furthermore, novel inflammatory markers such as interleukin-6 or other proteins in their signaling pathways may represent markers of disease severity and potential therapeutic targets. Additional cerebrospinal fluid biomarkers, such as protein 14-3-3 and neuron-specific enolase, may be useful prognostic indicators in transverse myelitis. Acute transverse myelitis in children, in contrast to adults, is more likely to be longitudinally extensive, and has a better prognosis and lower likelihood of recurrence. Prognostic factors in pediatric transverse myelitis are reviewed. The recent identification of novel biomarkers associated with acute transverse myelitis has led to a better understanding of the spectrum of disorders associated with inflammatory transverse myelitis, as well as a greater appreciation of its diverse and complex pathogenetic basis.