Showing papers on "Myelitis published in 2014"
TL;DR: Overall, the risk of a demyelinating CNS disease following vaccination, although non-negligible, is relatively low and the benefits of vaccinations surpass the potential risks of CNS inflammation.
161 citations
TL;DR: Awareness of the expanding spectrum of neurological complications caused by VZV reactivation with and without rash will improve diagnosis and treatment.
Abstract: Purpose of review Varicella zoster virus (VZV) reactivation results in zoster, which may be complicated by postherpetic neuralgia, myelitis, meningoencephalitis, and VZV vasculopathy. This review highlights the clinical features, laboratory abnormalities, imaging changes, and optimal treatment of each of those conditions. Because all of these neurological disorders produced by VZV reactivation can occur in the absence of rash, the virological tests proving that VZV caused disease are discussed. Recent findings After primary infection, VZV becomes latent in ganglionic neurons along the entire neuraxis. With a decline in VZV-specific cell-mediated immunity, VZV reactivates from ganglia and travels anterograde to the skin to cause zoster, which is often complicated by postherpetic neuralgia. VZV can also travel retrograde to produce meningoencephalitis, myelitis, and stroke. When these complications occur without rash, VZV-induced disease can be diagnosed by detection of VZV DNA or anti-VZV antibody in cerebrospinal fluid and treated with intravenous acyclovir. Summary Awareness of the expanding spectrum of neurological complications caused by VZV reactivation with and without rash will improve diagnosis and treatment.
136 citations
TL;DR: Knowledge regarding the various neurological complications helps in looking for the warning signs and early diagnosis thereby improving patient outcome and a high degree of suspicion in endemic areas can help in picking up more cases thereby helping in understanding the true extent of neurological complications in dengue fever.
106 citations
Journal Article•
TL;DR: Investigation did not identify poliovirus infection as a possible cause for the observed cases, although EV-D68 was identified in upper respiratory tract specimens of two patients, and EV infection should be considered in the differential diagnosis in cases of AFP with anterior myelitis.
Abstract: In August 2012, the California Department of Public Health (CDPH) was contacted by a San Francisco Bay area clinician who requested poliovirus testing for an unvaccinated man aged 29 years with acute flaccid paralysis (AFP) associated with anterior myelitis (i.e., evidence of inflammation of the spinal cord involving the grey matter including anterior horn cell bodies) and no history of international travel during the month before symptom onset. Within 2 weeks, CDPH had received reports of two additional cases of AFP with anterior myelitis of unknown etiology. Testing at CDPH's Viral and Rickettsial Disease Laboratory for stool, nasopharyngeal swab, and cerebrospinal fluid (CSF) did not detect the presence of an enterovirus (EV), the genus of the family Picornaviridae that includes poliovirus. Additional laboratory testing for infectious diseases conducted at the CDPH Viral and Rickettsial Disease Laboratory did not identify a causative agent to explain the observed clinical syndrome reported among the patients. To identify other cases of AFP with anterior myelitis and elucidate possible common etiologies, CDPH posted alerts in official communications for California local health departments during December 2012, July 2013, and February 2014. Reports of cases of neurologic illness received by CDPH were investigated throughout this period, and clinicians were encouraged to submit clinical samples for testing. A total of 23 cases of AFP with anterior myelitis of unknown etiology were identified. Epidemiologic and laboratory investigation did not identify poliovirus infection as a possible cause for the observed cases. No common etiology was identified to explain the reported cases, although EV-D68 was identified in upper respiratory tract specimens of two patients. EV infection, including poliovirus infection, should be considered in the differential diagnosis in cases of AFP with anterior myelitis and testing performed per CDC guidelines.
86 citations
TL;DR: It is demonstrated that the brain and spinal cord exhibit distinct sensitivities to cellular mediators of tissue damage, and that IL-17 and IFN-γ differentially regulate recruitment of these mediators to each microenvironment.
Abstract: Multiple sclerosis (MS) is an autoimmune disease in which inflammatory lesions lead to tissue injury in the brain and/or spinal cord. The specific sites of tissue injury are strong determinants of clinical outcome in MS, but the pathways that determine whether damage occurs in the brain or spinal cord are not understood. Previous studies in mouse models of MS demonstrated that IFN-γ and IL-17 regulate lesion localization within the brain; however, the mechanisms by which these cytokines mediate their effects have not been identified. In the present study, we show that IL-17 promoted, but IFN-γ inhibited, ELR(+) chemokine-mediated neutrophil recruitment to the brain, and that neutrophil infiltration was required for parenchymal tissue damage in the brain. In contrast, IFN-γ promoted ELR(+) chemokine expression and neutrophil recruitment to the spinal cord. Surprisingly, tissue injury in the spinal cord did not exhibit the same dependence on neutrophil recruitment that was observed for the brain. Our results demonstrate that the brain and spinal cord exhibit distinct sensitivities to cellular mediators of tissue damage, and that IL-17 and IFN-γ differentially regulate recruitment of these mediators to each microenvironment. These findings suggest an approach toward tailoring therapies for patients with distinct patterns of neuroinflammation.
76 citations
TL;DR: Several neurological conditions that can occur in Sjögren's syndrome are reviewed: sensory ganglionopathy, painful small fibre neuropathy, and transverse myelitis (independently or as part of neuromyelitis optica).
Abstract: Neurological symptoms occur in approximately 20% of patients with Sjogren's syndrome, and may be the presenting manifestations of the disease. Here, we review several neurological conditions that can occur in Sjogren's syndrome: sensory ganglionopathy, painful small fibre neuropathy, and transverse myelitis (independently or as part of neuromyelitis optica). We present the symptoms, signs, differential diagnoses, recommended diagnostic evaluation, and treatment of each of these, highlighting the features that should alert neurologists to consider Sjogren's syndrome.
68 citations
TL;DR: Clinicians should suspect PRES-SCI when patients with PRES have neurologic signs referable to the spinal cord, extreme elevation in blood pressure, MRI lesions that extend to the cervicomedullary junction, or grade IV hypertensive retinopathy, which, if diagnosed, would spare patients the morbidity of a standard myelitis workup and empiric treatment.
Abstract: Objective: To characterize a cohort of patients with the signs and symptoms of posterior reversible encephalopathy syndrome (PRES), but with clinical and radiologic involvement of the spinal cord. Methods: We report 2 cases of PRES with spinal cord involvement and identified an additional 6 cases in the Medline database using various search terms related to “spinal PRES,” “spinal reversible posterior leukoencephalopathy syndrome,” and “spinal hypertensive encephalopathy.” We analyzed the clinical and imaging characteristics of the 8 cases. Results: Average age was 31 years, with 5 male and 3 female patients. All patients had severe acute hypertension and a confluent, expansile central spinal cord T2 hyperintensity spanning at least 4 spinal segments, originating at the cervicomedullary junction. Of 8 patients, 7 had hypertensive retinopathy, a favorable clinical course with only antihypertensive treatment, and resolution of the spinal cord lesions on follow-up imaging. A total of 4 of 8 patients had symptoms referable to the spinal cord lesions and only 1 of 8 had a seizure. Conclusion: In light of the already wide definition of PRES, we propose a new syndrome named PRES with spinal cord involvement (PRES-SCI). Clinicians should suspect PRES-SCI when patients with PRES have neurologic signs referable to the spinal cord, extreme elevation in blood pressure, MRI lesions that extend to the cervicomedullary junction, or grade IV hypertensive retinopathy. These clinical scenarios should prompt a cervical spine MRI to help guide patient management decisions and prognostication. When clinicians evaluate longitudinally extensive spinal T2 hyperintensities, they should consider PRES-SCI, which, if diagnosed, would spare patients the morbidity of a standard myelitis workup and empiric treatment.
61 citations
TL;DR: The imaging and clinical features of idiopathic and disease‐associated transverse myelitis are described and its major differentials are discussed, with discussion of how MR imaging features assist in the identification of various sub‐types of transverseMyelitis.
Abstract: Transverse myelitis is an acute inflammatory disease of the spinal cord, characterized by rapid onset of bilateral neurological symptoms. Weakness, sensory disturbance, and autonomic dysfunction evolve over hours or days, most progressing to maximal clinical severity within 10 days of onset. At maximal clinical severity, half will have a paraparesis, and almost all patients have sensory disturbance and bladder dysfunction. Residual disability is divided equally between severe, moderate and minimal or none. The causes of transverse myelitis are diverse; etiologies implicated include demyelinating conditions, collagen vascular disease, and parainfectious causes, however, despite extensive diagnostic work-up many cases are considered idiopathic. Due to heterogeneity in pathogenesis, and the similarity of its clinical presentation with those of various noninflammatory myelopathies, transverse myelitis has frequently been viewed as a diagnostic dilemma. However, as targeted therapies to optimize patient outcome develop, timely identification of the underlying etiology is becoming increasingly important. In this review, we describe the imaging and clinical features of idiopathic and disease-associated transverse myelitis and its major differentials, with discussion of how MR imaging features assist in the identification of various sub-types of transverse myelitis. We will also discuss the potential for advanced MR techniques to contribute to diagnosis and prognostication.
55 citations
TL;DR: Novel therapeutic strategies for LETM in the context of NMO include eculizumab, which could be considered in patients with active disease who have failed azathioprine and rituximab, and thorough investigation of patients with LETM who are negative for NMO-IgG may lead to an alternate cause for myelopathy.
Abstract: Purpose of reviewLongitudinally extensive transverse myelitis (LETM) is a frequently devastating clinical syndrome which has come into focus for its association with neuromyelitis optica (NMO). Recent advances in the diagnosis of NMO have led to very sensitive and specific tests and advances in ther
52 citations
TL;DR: Recombinant antigen-based assays significantly increase AQP4-IgG detection in patients with rLETM, and AQP 4-IGG-negative adults with rleTM are rare.
Abstract: Importance Studies focused on recurrent longitudinally extensive transverse myelitis (rLETM) are lacking. Objectives To determine the aquaporin 4 (AQP4) IgG detection rate using recombinant human APQ4-based assays in sequential serum specimens collected from patients with rLETM categorized as negative by first-generation tissue-based indirect immunofluorescence (IIF) assay and to define the clinical characteristics and motor disability outcomes in AQP4-IgG–positive rLETM. Design, Setting, and Participants A search of the Mayo Clinic computerized central diagnostic index (October 1, 2005, through November 30, 2011), cross-linked with the Neuroimmunology Laboratory database, identified 48 patients with rLETM, of whom 36 (75%) were positive and 12 (25%) negative for neuromyelitis optica (NMO) IgG (per IIF of serial serum specimens). Stored serum specimens from “seronegative” patients were retested with recombinant human AQP4-based assays, including enzyme-linked immunosorbent, transfected cell-based, and fluorescence-activated cell-sorting assays. Control patients included 140 AQP4-IgG–positive patients with NMO, of whom a subgroup of 20 initially presented with 2 attacks of transverse myelitis (rLETM-onset NMO). Main Outcomes and Measures AQP4-IgG serostatus, clinical characteristics, and Expanded Disability Status Scale score. Results Six patients with negative IIF results were reclassified as AQP4-IgG positive, yielding an overall AQP4-IgG seropositivity rate of 89%. Fluorescence-activated cell-sorting, cell-based, and enzyme-linked immunosorbent assays improved the detection rate to 89%, 85%, and 81%, respectively. The female to male ratio was 2:3 for AQP4-IgG–negative rLETM and 5:1 for AQP4-IgG–positive patients. The AQP4-IgG–positive patients with rLETM or rLETM-onset NMO were similar in age at onset, sex ratio, attack severity, relapse rate, and motor disability. From Kaplan-Meier analyses, 36% of AQP4-IgG–positive patients with rLETM are anticipated to need a cane to walk within 5 years after onset. For patients with rLETM-onset NMO, the median time from onset to first optic neuritis attack (54 months) was similar to the median disease duration for AQP4-IgG–positive patients with rLETM (59 months). The median number of attacks was 3 for AQP4-IgG–positive patients with rLETM (range, 2-22), and the first optic neuritis attack for those with rLETM-onset NMO followed a median of 3 myelitis attacks (range, 2-19). Immunosuppressant therapy reduced the relapse rate in both AQP4-IgG–positive and AQP4-IgG–negative patients with rLETM. Conclusions and Relevance Recombinant antigen–based assays significantly increase AQP4-IgG detection in patients with rLETM, and AQP4-IgG–negative adults with rLETM are rare. Evolution to NMO can be anticipated in AQP4-IgG–positive patients. Early initiation of immunotherapy may result in a more favorable motor outcome.
50 citations
TL;DR: Findings warrant further examinations in large cohort series, as sera samples from 48 consecutive Japanese patients with myelitis or optic neuritis, but negative for anti-aquaporin (AQP) 4 antibodies (Abs), and 14 anti-Aquaporin 4 Ab-positive patients were tested forAnti-MOG Abs using a cell-based immunofluorescence assay with full-length human MOG cDNA.
TL;DR: The case report supports a relationship between anti-MOG antibodies and longitudinally extensive transverse myelitis, which was triggered by influenza infection, and further studies are needed to establish the clinical significance of anti- MOG antibodies for diagnosis, treatment, and prognosis.
Abstract: Background
Myelin-oligodendrocyte glycoprotein antibody (MOG antibodies) was found in various demyelinated diseases. This is the first report of a patient with longitudinally extensive transverse myelitis with an extremely high titer of MOG antibodies after an influenza infection. This case supports the view that MOG antibodies are linked to longitudinally extensive transverse myelitis and that influenza infection might trigger the MOG antibodies.
TL;DR: Recurrence risk in female and African American patients appears driven by a greater likelihood of developing NMO or NMO spectrum disorder, and sex, race, and serologic biomarkers warrant consideration when assessing risk of TM recurrence.
Abstract: Objective: This study sought to identify factors that increased the risk of recurrence after an initial transverse myelitis (TM) presentation. Methods: Retrospective cohort study of 192 patients initially presenting with TM of unknown etiology. Patients diagnosed with multiple sclerosis during the first myelitis episode were excluded. Demographic and laboratory data were analyzed for associations with recurrence. Results: One hundred ten of 192 patients (57%) eventually developed recurrent symptoms: 69 (63%) neuromyelitis optica (NMO) or NMO spectrum disorder, 34 (31%) non-NMO recurrent TM, and 7 (6%) systemic autoimmune disease. Multiple independent risk factors for recurrence were identified: African American race (risk ratio 1.60, p p = 0.007, 1.19–2.98), longitudinally extensive myelitis at onset (1.34, p = 0.036, 1.01–1.78), Sjogren syndrome antigen A (1.89, p = 0.003, 1.44–2.48), vitamin D insufficiency (4.00, p p = 0.006, 1.23–2.32), and the presence of inflammatory markers (e.g., immunoglobulin G index) in the CSF (2.14, p Conclusions: Sex, race, and serologic biomarkers warrant consideration when assessing risk of TM recurrence. Male sex and Caucasian American race were independently associated with risk of monophasic idiopathic TM. Recurrence risk in female and African American patients appears driven by a greater likelihood of developing NMO or NMO spectrum disorder.
TL;DR: The increased expression of 2 inflammatory markers in the spinal cord at 1 day after painful joint injury suggests that spinal inflammation may contribute to the initiation of pain after cervical facet joint injury.
Abstract: Study design This study used immunohistochemistry and an enzyme immunoassay to quantify interleukin-1α (IL-1α) and prostaglandin E2 (PGE2) levels in the spinal cord of rats at 1 day after painful cervical facet joint injury. Objective The objective of this study was to determine to what extent spinal inflammation is initiated early after a painful loading-induced injury of the C6-C7 facet joint in a rat model. Summary of background data A common source of neck pain, the cervical facet joint is susceptible to loading-induced injury, which can lead to persistent pain. IL-1α and PGE2 are associated with joint inflammation and pain, both locally in the joint and centrally in the spinal cord. Joint inflammation has been shown to contribute to pain after facet joint injury. Although spinal neuronal hyperactivity is evident within 1 day of painful facet injury, it is unknown if inflammatory mediators, such as IL-1α and PGE2, are also induced early after painful injury. Methods Rats underwent either a painful C6-C7 facet joint distraction or sham procedure. Mechanical sensitivity was assessed, and immunohistochemical and enzyme immunoassay techniques were used to quantify IL-1α and PGE2 expression in the spinal cord at day 1. Results Both IL-1α and PGE2 were significantly elevated (P≤ 0.04) at day 1 after painful injury. Moreover, although both spinal IL-1α and PGE2 levels were correlated with the withdrawal threshold in response to mechanical stimulation of the forepaw, this correlation was only significant (P = 0.01) for PGE2. Conclusion The increased expression of 2 inflammatory markers in the spinal cord at 1 day after painful joint injury suggests that spinal inflammation may contribute to the initiation of pain after cervical facet joint injury. Further studies will help identify functional roles of both spinal IL-1α and PGE2 in loading-induced joint pain. Level of evidence N/A.
TL;DR: Although lupus myelitis often presented at an active phase of SLE, one-third of events happened in the presence of low disease activity, and early initiation of effective immunosuppressive therapy facilitated recovery.
Abstract: BackgroundTransverse myelitis (TM) is a relatively infrequent but severe complication in systemic lupus erythematosus (SLE). Owing to its rarity and unfavorable outcome, we investigated its general features on MRI and incidence in the context of lupus activity, to facilitate early recognition and treatment.MethodsWe report a case of a young man with clinically inactive lupus nephritis but who presented with a sudden attack of myelitis. We performed systematic literature search in Medline to study the clinical features of SLE-related TM.ResultsFrom 1960 to April 2013, a total of 72 articles containing 194 cases of lupus myelitis were found. Among acquired articles, 93 patients fulfilled the inclusion criteria. The majority of the cases (88.8%) were female. Longitudinal myelitis was the predominant imaging finding on MRI (71.4%, 45/63). Nearly two-thirds (61/94) of lupus myelitis occurred in association with active lupus, and one-third (33/94) occurred in low disease activity.ConclusionsUpon literature revi...
TL;DR: The most common spinal infections, their pathophysiologic, clinical manifestation, and their imaging findings are reviewed.
Abstract: Spinal infections represent a group of rare conditions affecting vertebral bodies, intervertebral discs, paraspinal soft tissues, epidural space, meninges, and spinal cord. The causal factors, clinical presentations, and imaging features are a challenge because the difficulty to differentiate them from other conditions, such as degenerative and inflammatory disorders and spinal neoplasm. They require early recognition because delay diagnosis, imaging, and intervention may have devastating consequences especially in children and the elderly. This article reviews the most common spinal infections, their pathophysiologic, clinical manifestation, and their imaging findings.
TL;DR: The pathology observed in the spinal cord was similar to the previously described changes in the brain during the infection, providing the first detailed description of histopathological and immunological alterations due to experimental T. gondii induced myelitis in mice.
Abstract: Infection with the protozoan Toxoplasma (T.) gondii causes chronic infection of the central nervous system and can lead to life-threatening encephalomyelitis in immunocompromised patients. While infection with T. gondii has long time been considered asymptomatic in immunocompetent hosts, this view is challenged by recent reports describing links between seropositivity and behavioral alterations.
However, past and current researches are mainly focused on the brain during Toxoplasma encephalitis, neglecting the spinal cord as a key structure conveying brain signals into motion. Therefore, our study aimed to fill the gap and describes the spinal cord pathology in an experimental murine model of toxoplasmosis.
In the spinal cord, we found distinct histopathological changes, inflammatory foci and T. gondii cysts similar to the brain. Furthermore, the recruitment of immune cells from the periphery was detected. Moreover, resident microglia as well as recruited monocytes displayed an increased MHC classes I and II expression. Additionally, the expression of pro- and anti-inflammatory cytokines was enhanced in the brain as well as in the spinal cord. In summary, the pathology observed in the spinal cord was similar to the previously described changes in the brain during the infection.
This study provides the first detailed description of histopathological and immunological alterations due to experimental T. gondii induced myelitis in mice. Thus, our comparison raises awareness of the importance of the spinal cord in chronic T. gondii infection.
TL;DR: The case of a 58-year-old man with metastatic melanoma who commenced ipilimumab post spinal decompression and radiation is reported, with myelitis with consequent paraplegia as a potential neurological immune-related side effect of ipilometricab.
Abstract: Background: Ipilimumab has been shown to improve overall survival in patients with metastatic melanoma; however, complete responses (CRs) are uncommon. Immune-related side effects usually involve the skin or gastrointestinal tract. Neurologic events occur less frequently but are well described. Case Report: We report the case of a 58-year-old man with metastatic melanoma who commenced ipilimumab post spinal decompression and radiation. He developed a colitis post cycle 2 and ipilimumab was discontinued. Imaging, however, documented a radiological CR. 8 weeks later, he developed paraplegia and a myelitis despite an ongoing radiological CR. Steroid use resulted in some improvement radiologically, without clinical improvement. Conclusion: We report myelitis with consequent paraplegia as a potential neurological immune-related side effect of ipilimumab. We further describe a patient with a CR after 2 cycles of ipilimumab in the setting of radiation.
TL;DR: Varied presentations seen in NMO and NMOSD highlight the need for a high index of suspicion for NMO in demyelinating episodes not classical for MS, supporting the emerging trend of treating all patients with immunotherapeutic agents at an early stage.
Abstract: Background: There is insufficient data on the clinical and radiological features of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) from India. Objective: The objective of the following study is to examine the clinico-radiological features of NMO and NMOSD in an Indian cohort. Materials and Methods: This retrospective study included 44 consecutive patients who (1) satisfied the 2006 Wingerchuk criteria for NMO (16 seropositive and 7 seronegative); or (2) had isolated or recurrent optic neuritis (ON) with seropositivity (n = 4); or (3) had isolated or recurrent myelitis with seropositivity (n = 17). Results: The female:male ratio was 7.8:1 with median age of onset 26.5 (range 8-72). Annualized relapse rate (ARR) was comparable across all groups (F [3, 40] = 0.938 and P = 0.431). Various presentations other than ON and myelitis were noted. All 40 patients with myelitis had spinal cord lesions involving ≥3 vertebral segments during the course of the disease. Cervicomedullary involvement was seen in 32.5% (13/40) patients. Brain magnetic resonance imaging was available for 40 patients; eight of these (20%) had brain lesions in locations described in multiple sclerosis (MS), 27.5% (11/40) had lesions at sites unusual for MS and 52.5% (21/40) had normal brain imaging. Conclusion: NMO and NMOSD patients in this cohort have comparable ARR regardless of clinical presentation, supporting the emerging trend of treating all patients with immunotherapeutic agents at an early stage. Varied presentations seen in NMO and NMOSD highlight the need for a high index of suspicion for NMO in demyelinating episodes not classical for MS.
TL;DR: The diagnosis is hard to pinpoint, and a vast array of examinations are required to identify it, sometimes even posthumously, with more and more skin diseases being acknowledged as systemic ones, this viral infection is a more likely candidate for the same title.
TL;DR: Thyroid parameters from 243 serum samples revealed increased abnormalities of thyroid parameters in patients with NMO and TM than in MS patients, and abnormal TSH and TG-Ab were independently associated with myelitis occurrence in central nervous system demyelinating disorders.
Abstract: Transverse myelitis (TM) is associated with neuromyelitis optica (NMO) and multiple sclerosis (MS). Early recognition of useful parameters may be helpful to distinguish their difference. This retrospective study analyzed thyroid parameters from 243 serum samples (relapse = 128; remission = 115) of 178 patients with demyelinating diseases (NMO, n = 25; TM, n = 48; MS, n = 105). The relationship between thyroid and clinical parameters was analyzed. Patients with NMO and TM had a higher frequency of abnormal thyroid-stimulating hormone (TSH), anti-thyroglobulin antibodies (TG-Ab), and antithyroid peroxidase antibody (TPO-Ab) than MS patients (p<0.05). The level of TSH and TG-Ab returned to normal levels after administration of high-dose intravenous methylprednisolone (p<0.05). In 96 patients (NMO, n = 19; TM, n = 25; MS, n = 52) without treatment, serum levels of TSH, TG-Ab and TPO-Ab were significantly different between patients with and without myelitis (p<0.01). Patients positive for aquaporin-4 (AQP4) antibodies showed higher abnormalities of TSH (p = 0.001), TG-Ab (p = 0.004) and TPO-Ab (p<0.0001) levels than AQP4 antibodies negative patients. Logistic regression analyses revealed independent relationships between TSH (odds ratio [OR] = 33.994; p<0.0001), TG-Ab (OR = 7.703; p = 0.017) and myelitis occurrence in 96 patients at the active stage. In 52 MS patients experiencing their first attack, MS patients with myelitis were associated with TSH abnormalities (OR = 42.778; p<0.0001). This study showed increased abnormalities of thyroid parameters in patients with NMO and TM than in MS patients. MS patients with myelitis also had greater TSH abnormality than in MS patients without myelitis. Abnormal TSH and TG-Ab were independently associated with myelitis occurrence in central nervous system demyelinating disorders.
TL;DR: In this paper, the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSDs with antibodies against myelin oligodendrocyte glycoprotein.
Abstract: Neuromyelitis optica spectrum disorders (NMOSD) are characterized by severe optic neuritis and/or longitudinally extensive transverse myelitis, and some brain lesions are also unique to NMOSD. Serum autoantibodies against aquaporin-4 (AQP4) are detected in most cases of NMOSD. However, some patients with NMOSD remain seronegative despite repetitive testing during attacks with highly sensitive cell-based assays. The differential diagnosis of NMOSD is not restricted to multiple sclerosis and it includes many diseases that can produce longitudinally extensive myelitis and/or optic neuritis. We review the clinical features, imaging, and laboratory findings that can be helpful on the diagnostic work-up, discuss the differences between AQP4 antibody positive and negative patients with NMOSD, including features of NMOSD with antibodies against myelin oligodendrocyte glycoprotein.
TL;DR: Direct infection of the central nervous system by TB may mimic NMO in some respects, but whether NMO-like symptoms that develop during the course of TB should be considered and diagnosed as NMO is open to discussion.
Abstract: A number of reports have described the presence of tuberculosis (TB) in neuromyelitis optica (NMO) patients. However, a definite association between the two conditions has not been conclusively demonstrated. To investigate the association between NMO and TB in a Chinese population, we performed a retrospective review of hospital records of NMO patients, control patients and tuberculosis meningitis (TBM) patients from January 1, 1995 to December 31, 2011. The frequency of preceding/simultaneous active pulmonary TB (PTB) was not significantly different between NMO patients (1.1%) and control groups (2.3% in myasthenia gravis, 1.1% in polymyositis or dermatomyositis, zero in idiopathic facial palsy and viral meningitis/meningoencephalitis). NMO cases differed from TBM cases in terms of demographics, course (recurrent or monophasic), cerebrospinal fluid analysis and magnetic resonance images. Two TBM patients shared partial clinical features with NMO (one of the TBM patients had a longitudinal extensive spinal cord lesion involving the holocord, and the other had optic neuritis before anti-tuberculosis treatment). NMO antibodies were only detected in NMO patients and not in TBM patients with myelitis or optic neuritis. We could not confirm previous suggestions of the association between PTB and NMO. Direct infection of the central nervous system by TB may mimic NMO in some respects, but whether NMO-like symptoms that develop during the course of TB should be considered and diagnosed as NMO is open to discussion.
TL;DR: Four cases of spinal tuberculosis that presented with LETM were presented and none of them had a clinical profile fulfilling the diagnostic criteria for NMO, suggesting associations between NMO and active pulmonary tuberculosis.
Abstract: Tuberculosis of the central nervous system (CNS) accounts for approximately 1% of all cases of tuberculosis and half of these involve the spine. Intramedullary involvement is rare in tuberculosis and usually present in the form of radiculomyelitis, transverse myelitis, intraspinal granulomas, or thrombosis of anterior spinal artery. 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. LETM is most frequently associated with neuromyelitis optica (NMO). Moreover, associations between NMO and active pulmonary tuberculosis have been suggested by a number of case reports and case series. We present here four cases of spinal tuberculosis that presented with LETM and none of them had a clinical profile fulfilling the diagnostic criteria for NMO.
TL;DR: These patients had worse outcomes than typical patients with transverse myelitis/acute disseminated encephalomyelitis, and these observations build on previous work by other investigators that highlighted persistent flaccid paralysis and electrophysiological evidence of axonal loss portending a poorer prognosis.
Abstract: Importance Classically, transverse myelitis and acute disseminated encephalomyelitis are considered central nervous system demyelinating conditions. In both conditions, the spinal cord is involved to varying degrees, and there is a variety of presentations, usually involving some degree of progressive paralysis of the upper and/or lower extremities. Treatment usually consists of high-dose intravenous steroids in addition to plasma exchange and/or intravenous immunoglobulin. In some cases, immunosuppressive medications, such as intravenous cyclophosphamide, have been used with variable success. Cases with atypical features on examination, imaging, or with neurophysiological studies may be helpful in shedding light on the etiology and/or pathophysiology because many of these patients have permanent disabilities despite appropriate treatment. Observations This case series presents 5 pediatric cases observed from 2009-2012 at our medical center, Children’s Medical Center Dallas. These cases were notable because they provided evidence of autoimmune events affecting the central nervous system but with additional peripheral axonal pathology. Conclusions and Relevance We describe these cases with respect to findings that suggest a variant of these conditions that have concomitant nerve-root involvement. These patients had worse outcomes than typical patients with transverse myelitis/acute disseminated encephalomyelitis, and these observations build on previous work by other investigators that highlighted persistent flaccid paralysis and electrophysiological evidence of axonal loss portending a poorer prognosis. Furthermore, these cases suggest a potential role for approaching how we classify subtypes of transverse myelitis and acute disseminated encephalomyelitis.
TL;DR: T toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.
Abstract: Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.
TL;DR: Mapping diffusion abnormalities within the cervical spinal cord using a novel voxel-based approach can localize clinically relevant pathology in patients with MS.
Abstract: Objective: To apply a novel postprocessing voxel-based analysis for diffusion tensor imaging of the cervical spinal cord in multiple sclerosis (MS) in a prospective cross-sectional study. Methods: Fourteen patients with MS who were within 4 weeks of the onset of cervical myelitis (lesion C1-3) and 11 healthy controls underwent cervical spinal cord diffusion tensor imaging. Cervical spinal cord maps of fractional anisotropy (FA), mean diffusivity, radial diffusivity (RD), and axial diffusivity were registered and compared between patients and controls. Mean FA and RD values from significant thresholded clusters were regressed with clinical scores, after adjusting for cord area and age, to determine associations with physical disability. Results: Cord registrations for subjects were qualitatively assessed (scored out of 5) and those with low scores (1 or 2) were excluded from further analysis. Cord registration was considered good in 11 patients (6 females; mean age = 35.5 years) and 10 controls (6 females; mean age 44 years). Voxel-based comparisons showed patients with MS had lower FA and higher RD at C2-3 levels (left >right mainly in gray matter; p Conclusions: Mapping diffusion abnormalities within the cervical spinal cord using a novel voxel-based approach can localize clinically relevant pathology.
TL;DR: The cluster of transverse myelitis post-dengue with favorable clinical outcome here reported suggests an immune mediated mechanism for the spinal cord involvement in dengue.
Abstract: Background and Purpose: During the last two decades, clinical reports have begun to place increasing emphasis on the possible neurological complications related to dengue. However, reports of cases with myelitis post dengue are rare. This study describes an unprecedented cluster of transverse myelitis following a dengue virus infection.
Methods: 51 possible cases of neurological complications related to dengue were identified by the epidemiological surveillance of the State of Rondonia, Brazil and submitted to serial neurological examination, electromyography, vertebral MR and laboratory investigation to confirm the dengue diagnosis and rule out other arboviruses.
Results: The diagnosis of acute transverse myelitis post-dengue was established in 26 patients, the majority were women, young and white. Antibodies against virus IgM were present in all cases and DEN 3 virus was isolated by PCR in one patient. Treatment with IV steroids was useful.
Conclusions: The cluster of transverse myelitis post-dengue with favorable clinical outcome here reported suggests an immune mediated mechanism for the spinal cord involvement. Whereas dengue epidemics are frequent in tropical and subtropical countries, the dengue fever should be part in the differential diagnosis of the infectious and post-infectious myelitis.
TL;DR: The demyelinated lesions observed in this case were similar to lesions found in multiple sclerosis, and it is speculated that the lesions formed as a result of anti-NMDAR encephalitis.
Abstract: Objective: To describe an unusual case of a male patient with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis who presented with multiple white matter lesions. Brain biopsy of the patient was performed, and follow-up evaluation of the cerebrospinal fluid (CSF) NMDAR antibody titer was implemented. Design: Case report. Setting: University hospital. Patient: A 35-year-old man with anti-NMDAR encephalitis initially presented with fever and psychiatric symptoms. After an initial attack of anti-NMDAR encephalitis, 2 atypical relapses occurred, which presented with myelitis and multifocal white matter lesions; the lesions were open-ring-shaped and partially enhanced. Intervention: Analysis of the brain biopsy specimen revealed the presence of demyelinated lesions with discrete borders. Subsequent intravenous methylprednisolone therapy resulted in improvement in the brain lesions. Prednisolone and cyclophosphamide were orally administered thereafter. Clinical progression of the disease paralleled observed changes in the CSF NMDAR antibody titer.
TL;DR: This review focuses on inflammatory spinal cord injury and its relationships with multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyeliitis and systemic collagen vascular and paraneoplastic diseases.
Abstract: Inflammatory injury to the spinal cord causes a well-recognized clinical syndrome. Patients typically develop bilateral weakness, usually involving the legs, although the arms may also become affected, in association with a pattern of sensory changes that suggests a spinal cord dermatomal level. Bowel and bladder impairment is also common in many patients. Recognition of the clinical pattern of spinal cord injury should lead clinicians to perform imaging studies to evaluate for compressive etiologies. MRI of the spine is particularly useful in helping visualize intraparenchymal lesions and when these lesions enhance following contrast administration a diagnosis of myelitis is made. Cerebrospinal fluid analysis can also confirm a diagnosis of myelitis when a leukocytosis is present. There are many causes of non-compressive spinal cord injury including infectious, parainfectious, toxic, nutritional, vascular, systemic as well as idiopathic inflammatory etiologies. This review focuses on inflammatory spinal cord injury and its relationships with multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelitis and systemic collagen vascular and paraneoplastic diseases.