MATERIALS AND METHODS: Spinal MRIs of 77 patients (mean age, 36.3 ⫾ 20 years) diagnosed with acutetransversemyelitis were evaluated retrospectively. Only the patients for whom an underlying cause of myelitis could not be identiﬁed within 3 months of symptom onset were included. Initial spinal MR images of patients were examined in terms of lesion extent, location and distribution, brain stem extension, cord expansion, T1 signal, contrast enhancement, and the presence of bright spotty lesions and the owl’s eyes sign. The relapse rates and Kurtzke Expanded Disability Status Scale scores at least 1 year (range, 1–14 years) after a myelitis attack were also recorded. Associations of MR imaging ﬁndings with clinical variables were studied with univariate associations and binary log-linear regression. Differences were considered signiﬁcant for P values ⬍ .05.
Neuromyelitis optica (NMO), as a rare autoimmune demyelinateting disorder, arouses inflammatory lesions in the optic nerves and spinal cord and causes some ser- ious clinical symptoms such as blindness and paralysis. In NMO, the inflammatory profile primarily involves eosino- phils/neutrophils and autoantibody reaction. At present, an autoantibody (NMO IgG) against aquaporin-4 (AQP4), a water channel expressed on astrocytes, has been incrimi- nated as a causative factor. Similar to other autoimmune diseases, Th17 cells and their effective cytokines (such as interleukin 6) participate in the pathogenesis of NMO [1,2]. Acutetransversemyelitis (ATM) is characterized as
Abstract: Transversemyelitis is a rare inflammatory myelopathy characterized by loss of motor and sensory function below the affected level of the spinal cord, and causes neurogenic bowel and bladder. Occasionally, it also causes neuropathic pain with spasticity. Traditional therapies for neu- ropathic pain are multiple, including multimodal analgesic regimens, antiepileptic or antidepres- sant medications, opioids, sympathetic blocks, and spinal cord stimulation. Persistent neuropathic pain can cause emotional distress by affecting sleep, work, recreation, and emotional well-being. Here we report the case of a patient suffering from intractable neuropathic pain following acutetransversemyelitis that was not relieved by combinations of nonsteroidal anti-inflammatory, anti- epileptic, antidepressant, and opioid medications, or by acupuncture. Implantation of an intrathecal morphine pump controlled the pain successfully without side effects, and enabled the patient to embark on intensive rehabilitation. The patient’s muscle strength has improved significantly and the patient may soon be able to use a walker with minimal assistance.
Objective: The objective of this study was to observe urodynamic profile of acutetransversemyelitis (ATM) patients and its correlation with neurological outcome. Patients and Methods: This prospective study was conducted in the neurorehabilitation unit of a tertiary university research hospital from July 2012 to June 2014. Forty‑three patients (19 men) with ATM with bladder dysfunction, admitted in the rehabilitation unit, were included in this study. Urodynamic study (UDS) was performed in all the patients. Their neurological status was assessed using ASIA impairment scale and functional status was assessed using spinal cord independence measure. Bladder management was based on UDS findings. Results: In total, 17 patients had tetraplegia and 26 had paraplegia. Thirty‑six patients (83.7%) had complaints of increased frequency and urgency of urine with 26 patients reported at least one episode of urge incontinence. Seven patients reported obstructive urinary complaints in the form of straining to void with 13 patients reported both urgency and straining to void and 3 also had stress incontinence. Thirty‑seven (86.1%) patients had neurogenic overactive detrusor with or without sphincter dyssynergia and five patients had acontractile detrusor on UDS. No definitive pattern was observed between neurological status and bladder characteristics. All patients showed significant neurological and functional recovery with inpatient rehabilitation (P < 0.05 and P < 0.001, respectively). Conclusions: The problem of neurogenic bladder dysfunction is integral to ATM. Bladder management in these patients should be based on UDS findings. Bladder characteristics have no definitive pattern consistent with the neurological status.
Several clinical factors associated with a poor outcome have been described in patients with acutetransversemyelitis, including back pain, severe functional deficit and motor involvement at onset of symptoms, symptoms progression within 24 hours, relapse occurrence, and spinal shock [3,14,21,22,24]. As was mentioned above, among the para-clinical factors, LETM has been associ- ated with a poor prognosis in these patients [18,19]. The multivariate analysis in the present study showed that urinary sphincter dysfunction and LETM were inde- pendently associated with a poor prognosis in IATM.
M.A. Mealy has received speaker honoraria from the Con- sortium of Multiple Sclerosis Centers and has received re- search support from the NIH. T.-S. Nam and S.J. Pardo report no disclosures. C.A. Pardo has served on the scientiﬁc advi- sory board of the TransverseMyelitis Association and has received research support from Acorda Pharmaceuticals, Chugai Pharmaceutical Co. LTD, MedImmune, the NIH, and the Bart McLean Fund for Neuroimmunology Research (Project Restore TransverseMyelitis Association). N.L. Sobreira receives research support from the NIH, the Guthy Jackson Charitable Foundation, Viropharma, Acorda, Sanoﬁ, NeuralStem, and Genentech and serves as a consultant Table Autoimmune conditions associated with a monogenetic mutation
have produced criteria for the diagnosis of ATM (Table 1). All of the inclusion criteria must be present, and all of the exclusion criteria absent, for a diagnosis of acutetransversemyelitis to be made. Once diagnosed, ATM is then classified as either idiopathic (in the absence of all disease associated features), or disease associated (if they are present). There are two key features of the criteria in relation to our patient’s case: firstly, they propose that ATM can be diagnosed in the absence of radio- logical evidence of spinal lesions if cerebral spinal fluid (CSF) analysis reveals signs of inflammation; and sec- ondly that magnetic resonance imaging (MRI) scan findings of multifocal inflammation within the brain in the presence of ATM results in the classification of
Almost a year Clinical improvement following the first 3 doses of CYC; myelitis after discontinuing it; no relapse after 6 months of CYC Abbreviations: RTX, rituximab; CYC, cyclophosphamide; MiTX, mitoxanthrone; AZA, azathioprine; MMF, mycophenolate mofetil; PLEX, plasma exchange; Ig, immunoglobulin; SLE, systemic lupus erythematosus; APS, Antiphospholipid syndrome; MRI, magnetic resonance imaging; LETM, longitudinally extensive transversemyelitis; ON, optic neuritis; NMO, neuromyelitis optica; EDSS, Expanded Disability Status Scale; ATM, acutetransversemyelitis.
With acute presentation, emergent spinal MRI should be performed to exclude compressive causes that may require urgent surgery, such as epidural hematoma or disc herniation. The differential diagnosis of myelopathy is extensive and rapidity of symptoms will help to narrow it down. For acutetransversemyelitis, criteria include demon- stration of inflammation within the spinal cord
Pediatric acutetransversemyelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be dif- ferentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the TransverseMyelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some. MRI lesions are often centrally located with high T2 signal intensity involving gray and neighboring white matter. Longitudinally extensive ATM occurs in the majority. Asymptomatic lesions on brain MRI are seen in more than one-third and predict MS or NMO. The role of antibodies such as myelin oligodendro- cyte glycoprotein in monophasic and relapsing ATM and their significance in therapeutic ap- proaches remain unclear. ATM is a potentially devastating condition with variable outcome and presents significant cumulative demands on health and social care resources. Children generally have a better outcome than adults, with one-half making a complete recovery by 2 years. There is need for standardization of clinical assessment and investigation protocols to enable interna- tional collaborative studies to delineate prognostic factors for disability and relapse. There are no robust controlled trials in children or adults to inform optimal treatment of ATM, with one study currently open to recruitment. This review provides an overview of current knowledge of clinical features, investigative workup, pathogenesis, and management of ATM and suggests future directions. Neurology ® 2016;87 (Suppl 2):S46 – S52
Transversemyelitis commonly involves the thoracic spinal cord; in 10% of patients only the cervical spinal cord is affected (2). Choi et al have reported predominant in- volvement of the thoracic spinal cord in all but one in whom cervical cord involvement correlated with the sen- sory level. The level of MR examination in their patients was determined by the clinical deficit, which might be responsible for the restriction of the signal changes to 3 to 4 segments only. Our observations on acutetransversemyelitis have revealed that the MR signal changes in the spinal cord are quite extensive in the acute stage and exceed the sensory level by at least three spinal segments. The signal changes in the cervical spinal cord were present in six of 10 patients, three with upper limb weakness and three with central motor conduction abnormalities in the upper limbs. The patients had a poor outcome if the signal changes exceeded 10 spinal segments (3).
Acutetransversemyelitis is a focal inflammatory disorder of the spinal cord with sensory, motor and autonomic dys- function . Cytomegalovirus has been described rarely as a aetiological agent in immunocompetent adults present- ing with transversemyelitis with only ten cases described previously [4-13]. Infact, any form of severe infection with cytomegalovirus is rare . Out of the ten, only five cases describe abnormalities in the magnetic reson- ance imaging (MRI) of spinal cord in CMV associated transversemyelitis (Table 1). Two reports describe con- firmation of CMV infection with detection of CMV de- oxyribonucleic acid (DNA) in the serum by polymerase
Methods: In total, 260 consecutive patients were screened and 203 were included from three hospitals in Korea. All were tested for AQP4-Ab by using a cell-based assay. Patients who met the criteria for definite neuromyelitis optica (NMO) or had a positive AQP4-Ab test result were defined as the NMO group. Among the others, patients were assessed if they had acute disseminated encephalomyelitis, multiple sclerosis (MS), acutetransversemyelitis, optic neuritis, or other demyelinating disease as a clinically isolated syndrome of the brain.
Our patient presented several diagnostic challenges. Compressive causes had been excluded by early MRI. Acutetransversemyelitis (ATM) diagnosis was supported by acute paraparesis with bilateral sensory findings, im- paired sphincter function, the presence of spinal segmen- tal level of sensory disturbance and the evidence of inflammation within the spinal cord demonstrated by CSF pleocytosis. Given the wide range of possible causes for ATM and taking into account the particular details of our patient history we were confronted with a wide range of challenging hypothesis: are we facing post-infectious mye- litis secondary to acute gastroenteritis? Is this a toxin me- diated myelitis caused by spider or snake venom? What if the periumbilical skin lesion is a tick bite sequelae, and Borrelia or Rickettsia sp are implicated? What about viral myelitis, the most common aetiology for ATM? Shouldn’t we consider viruses that can cause myelitis worldwide (Herpes viruses, Enterovirus) but also those than can be present in Africa (Dengue, West Nile virus, HTLV1)? Fi- nally, how should we value a positive HIV screening test when no high risk exposure is identified? Could it be a
Out of the 100 patients with nontraumatic myelopathy, 59 patients received medical management and 41 patients underwent surgery. All those who underwent surgery had compressive myelopathy. 11 % of the nontraumatic myelopathy patients who had compressive myelopathy received conservative medical management due to their lack of fitness for surgery or their unwillingness to undergo surgical intervention. Twenty eight out of 48 patients were managed with steroids in the noncompressive myelopathy group. Demyelinating disorders like acutetransversemyelitis, multiple sclerosis and NMO spectrum disorders were the common etiologies requiring use of steroids.
ment may be diffuse, peripheral, or even punctate and speckled. These patterns are un- usual for tumors (18). Dural arteriovenous fis- tulas may cause myelopathy, but acute symp- toms were rarely present (7). The symptoms generally have a very insidious onset and sub- acute or chronic course and are marked by mild or moderate paraparesis or sensory dis- turbances. There are several possible patterns of presentation of this entity: nonspecific cord swelling, vague fuzziness or irregularity of cord margins caused by distention of small superfi- cial vessels, definite demonstration of patho- logically large vessels on noncontrast scans, or demonstration of abnormal vessels on post- contrast scans only (19, 20). Sometimes defi- nitely abnormal vessels are not seen and my- elography may be mandatory (21). Three of our patients with abnormal MR also underwent myelography, because they were middle-aged and their clinical syndromes initially seemed to progress despite steroid therapy. Myelo- graphic findings for all these patients were normal. Abnormal high signals on T2- weighted images, edema, and cord swelling may also occur after surgery (because of ob- struction of the extramedullary venous drain- age system) or cord contusion (22, 23). Both events can easily be excluded by the clinical history.
an increase in reports of children presenting similarly in other states, the Centers for Disease Control and Prevention (CDC) developed a case deﬁnition for acute ﬂaccid myelitis (AFM), which included persons #21 years of age with acute limb weakness and an MRI demonstrating predominantly gray matter lesions of the spinal cord to differentiate this condition from other forms of AFP, such as Guillain-Barré syndrome (GBS). 8 A total of 120 con ﬁ rmed cases were reported from August through December 2014, with a peak in late summer and early fall. Most cases had predominant anterior horn cell involvement, similar to poliomyelitis lesions. The CDC performed an extensive laboratory evaluation, including testing for poliovirus and other EVs, parechoviruses, arboviruses, and herpesviruses, to identify possible etiologies. Poliovirus was conclusively ruled out as a causative agent, and testing of hundreds of sterile-site specimens, including cerebrospinal ﬂ uid (CSF) and serum, provided limited insight into etiology. However, a concurrent nationwide outbreak of severe respiratory disease associated with EV-D68 in 2014 led to focused
previous LETM was seen in 5/23 (22%) patients, after 2-4 year duration of disease and with an EDSS score of 5-7. General spinal cord atrophy was observed in 7/23 (30%) patients after 2-4 years duration of disease in two and after 5-10 years in five with an EDDS score of 7-9. A strong correlation was observed (r = 0.88) between the occurrence of spinal cord atrophy and disability as analyzed by the polychoric correlation and the Fisher’ s exact test (p < 0.001). Normal appearance of the spinal cord was only observed in 3/23 (13%) patients and myelitis lesions shorter than LETM were found in 7/23 (30%) patients, after
and II, and a paraneoplastic panel (anti-NR1, anti-GAD65, anti-alpha 3AChR, anti-LGI1, anti-VGCC, anti-VGKC, anti-CASPR2, anti-amphiphysin, anti-CV2, anti-Hu, anti- Ma, anti-Ta, anti-recoverin, anti-Ri, anti-Yo, anti-Zic4) were negative. A serum IL-6 level was normal. A serum TNF-alpha level was not obtained. MRI of the brain dem- onstrated two new intracranial metastases. MRI of the spine showed progression of transversemyelitis from T3 to T11 (Fig. 3c), now clearly outside the radiation field. Body PET/CT revealed worsening osseous metastatic lesions; therefore the patient began dabrafenib and trame- tinib. Given his ascending transversemyelitis despite opti- mal therapy other options including tocilizumab and infliximab were considered. Based on the low IL-6 level, the patient was started on infliximab. Spinal MRI 3 weeks after the first dose of infliximab showed a dramatic reduc- tion of the level of the T2 cord signal abnormality back to