Longitudinal Extensive Myelitis Research Articles

Neuromyelitis optica spectrum disorders relapses and seasonal influence in an equatorial country cohort.

Information about seasonal distribution of Neuromyelitis optica spectrum disorders (NMOSD) attacks, particularly in tropical countries, has rarely been described and the reported data are diverse. To evaluate influence of seasonal variation in NMOSD relapses in an equatorial country. Exploratory observational, retrospective ecological study in a cohort of patients with NMOSD followed from January 2008 to December 2019. Data of demographic, clinical information, characteristics of relapses and seasonal temporal variation were recorded. Also, the annual, monthly and intra-annual seasonal variation of relapses was quantified. A negative binomial regression was used to estimate the associations between the number of relapses and climatic and temporal variables. One hundred thirteen patients were included, most of them were female (89.38%), with a mean age at NMOSD diagnosis was 44.97 (±13.98) and the median of relapses per patient were 2 relapses (IQR 1-3). The patients presented 237 relapses, most of these in AQP4 seropositive patients (87.76%) and longitudinal extensive myelitis was the most frequent type of relapse (53.59%). According to the temporal variation, relapses were more common in the second rainy season (28.69%) during November and December. However, there weren't significant differences in the number of relapses between seasons and climatic variables in the multivariable model. The number of NMOSD relapses in this equatorial country cohort did not exhibit any significant associations with climatic variations, including changes in rainy or dry seasons.

Longitudinally extensive transverse myelitis after epidural anaesthesia in childbirth

Introduction. Neurological complications related to epidural anesthesia are rare, but it is necessary to recognize and diagnose them as early as possible, in order to start appropriate therapy and prevent further neurological damage. One of the rare complications of regional anesthesia described in this paper is longitudinal extensive transversal myelitis (LETM). Case report. A 32-year-old patient, who gave birth by Caesarean section in due term, developed LETM the very same day. Considering the neuroradiological findings that indicated to the long central lesion in the thoracic and lumbar spine, and an expected reaction to the applied immunotherapy (immunosuppressive therapy and therapeutic plasma exchange), a diagnosis of LETM was made. Even with all the therapy and regression of the lesions, the patient could not stand up without support. During the subsequent treatment, according to the instructions of the physiatrist, physical therapy was carried out, to which the patient responded slowly but favorably, with a gradual return of the function of the lower extremities. Conclusion. Early diagnosis and timely treatment of LETM are crucial for the prognosis of the dis-ease and the early recovery of the patient.

Paraneoplastic encephalomyeloradiculits with multiple autoantibodies against ITPR-1, GFAP and MOG: case report and literature review

BackgroundRecently, antibodies against the alpha isoform of the glial-fibrillary-acidic-protein (GFAPα) were identified in a small series of patients with encephalomyelitis. Coexisting autoantibodies (NMDA receptor, GAD65 antibodies) have been described in a few of these patients. We describe a patient with rapidly progressive encephalomyeloradiculitis and a combination of anti-ITPR1, anti-GFAP and anti-MOG antibodies.Case presentation and literature reviewA 44-year old caucasian woman with a flu-like prodrome presented with meningism, progressive cerebellar signs and autonomic symptoms, areflexia, quadriplegia and respiratory insufficiency. MRI showed diffuse bilateral T2w-hyperintense brain lesions in the cortex, white matter, the corpus callosum as well as a longitudinal lesion of the medulla oblongata and the entire spinal cord. Anti-ITPR1, anti-GFAP and anti-MOG antibodies were detected in cerebrospinal fluid along with lymphocytic pleocytosis. Borderline tumor of the ovary was diagnosed. Thus, the disease of the patient was deemed to be paraneoplastic. The patient was treated by surgical removal of tumor, steroids, immunoglobulins, plasma exchange and rituximab. Four months after presentation, the patient was still tetraplegic, reacted with mimic expressions to pain or touch and could phonate solitary vowels. An extensive literature research was performed.ConclusionOur case and the literature review illustrate that multiple glial and neuronal autoantibodies can co-occur, that points to a paraneoplastic etiology, above all ovarian teratoma or thymoma. Clinical manifestation can be a mixture of typically associated syndromes, e.g. ataxia associated with anti-ITPR1 antibodies, encephalomyelitis with anti-GFAPα antibodies and longitudinal extensive myelitis with anti-MOG antibodies.

ECTRIMS 2021 – ePoster

Introduction: COVID-19 induced longitudinal extensive myelitis (LEM) is an extremely rare clinical manifestation. Aims and Methods: In this case study, we report a woman of LEM which appeared fifteen days after COVID-19 infection. Results: A 39-year-old woman, suffering from gradually increased weakness and severe pain at lower limbs and urinary retention was admitted to the emergency department. She was diagnosed with COVID-19 infection 15 days ago and fully recovered from respiratory symptoms. Her initial neurological examination showed loss of muscular strength in right lower limb 1/5 and left lower limb 2/5, hyperreflexia in the lower limbs and generalized allodynia. All modalities of sensation were diminished at the T10 segmental level. Her nasopharyngeal and oropharyngeal swab for polymerase chain reaction (PCR) to detect SARS-CoV-2 was positive. Cerebrospinal fluid (CSF) showed pleocytosis (whitecell count 110/mm3 ) with mostly polymorphonuclear leukocytes (90%), elevated protein (67 mg/dl) and normal glucose (78 mg/dl;concurrent serum glucose level was 96 mg/dl). Serology for SARS-CoV2 in CSF demonstrated infection (anti-SARS-CoV-2 IgG: 5.47 (negative<1). The other serum and CSF tests were negative for differential diagnosis of acute transverse myelitis. Electromyography (EMG) showed normal motor and sensory nerve conduction velocity in the upper and lower limbs. The somatosensory evoked potention test (SEP) determined prolonged latency of bilateral tibial nerve cortical responses with low-amplitudes. Cervical and thoracic MRI demonstrated a long segment of T2 hyperintensities within the central cord extending from C2-C3 level to T10 level. The patient was treated with methylprednisolone 1 g/d IV for five days. Because of her progressed neurological condition, the patient received five sessions of plasma exchange and additionally methylprednisolone 1 g IV was given for another ten days. The patient was discharged to an inpatient rehabilitation facility without any clinical improvement. Conclusions: Acute transverse myelitis have been reported during respiratory diseases due to SARS-CoV-2. Otherwise, the clinical manifestation of LEM induced by COVID-19 seen during infection is very rare. To the best of our knowledge, our case is the second one of LEM induced by COVID-19 confirmed by CSF antibody. This case remarks the importance of recognition and early treatment of LEM during COVID-19 infection.

Etiological and Radiological Spectrum of Longitudinal Myelitis: A Hospital-Based Study in North East India

Introduction An inflammatory lesion of the spinal cord where three or more than three vertebral segments of the cord is involved is called longitudinal extensive myelitis (LETM). It has several varied causes out of which neuromyelitis optica (NMO) and its spectrum disorder have received a distinct entity. Various radiological and clinical features help us to suspect an etiology which then further guides us into the treatment protocol and prognosis of the patients. Materials and Methods A retrospective study performed in a referral center in North East India in 15 months. Thirty-two patients of LETM were enrolled based on clinical and radiological available data. An attempt was made to classify the various etiologies and correlate with their radiological findings. Results The most common etiology noted was NMO seen in 7 patients (21.8%) followed by tuberculosis (TB) (18.7%) and post-infection myelitis (18.7%). Other etiology seen was acute disseminated encephalomyelitis (6.24%), spinal cord infarct (3.12%), radiation myelitis (6.24%), Japanese encephalitis sequalae (3.12%), systemic lupus erythematosus (3.12%), and remained undiagnosed in six patients (18.7%). Radiologically, cervico-dorsal spine was most common location in NMO (71%) whereas dorsolumbar in TB (50%). The lesion was predominantly central in both NMO (100%) and TB (80%) as compared with the other causes of LETM. It was noted that more than 50% of the transverse area of the cord was involved in both NMO (71%) and TB (50%), but < 50% involvement were more common in the post-infectious and others causes of LETM. Conclusion LETM has a various differential diagnosis, infection need to be kept in mind while ruling out NMO. Radiological features can suggest or help differentiate the various etiologies of LETM but NMO and infection like TB almost has the same features except for a different cord site predilection.

NMOSD-like and longitudinal extensive HTLV1-associated myelitis are extremes that flank an overlooked continuum.

BackgroundHTLV1-associated myelitis (HAM) is a slowly progressive myelopathy in which spinal cord MRI demonstrates no lesion or atrophy.ObjectiveWe examined the overlap between NMOSD features and HTLV1 infection.MethodsWe included all HTLV1-infected patients recruited in French West Indies (FWI) or referred from different centers, and suffering from at least one NMOSD feature. Literature connecting HTLV1-infection and NMOSD was reviewed.ResultsWe included six NMOSD-like HAM with acute onset, seronegative against AQP4 and MOG-Abs. All displayed extensive longitudinal myelitis, and the optic nerve was involved in three. We gathered 39 cases of NMOSD-like HAM patients from the literature. Atypical signs of HAM were relapses (15.4%), sensory level (50%), upper limb symptoms (35.9%), optic neuritis (10.2%). Typical lesions involved lateral funiculi and featured a double rope sign (56.3%).ConclusionWe propose that acute onset of NMOSD-like HAM could be more frequent than expected and should be evoked in high-risk patients. Extensive but often transient cord lesions could be the hallmark of an excessive inflammation of the funiculi targeted by HTLV1 infection. Although usually minor, a few HAM cases demonstrate specific MRI lesions, and the most severe cases may mimic NMOSD attacks.

Neuromyelitis Optica Spectrum Disorder: A Case Report of Effective Combination Immunosuppressant, Corticosteroids, and Therapeutic Plasma Exchange.

BACKGROUND:Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. The symptoms can occur simultaneously or separated by a variable period. NMOSD is associated with serum aquaporin antibodies 4 immunoglobulin G (AQP4-IgG).CASE PRESENTATION:We report a case of a 22-year-old male with complaints of weakness of all four limbs, impaired vision, urinary incontinence, and dyspnea. The Expanded Disability Status Scale (EDSS) was nine. Spinal magnetic resonance imaging (MRI) showed longitudinal extensive transversal myelitis. The brain MRI showed a normal impression, whereas the brain magnetic resonance spectroscopy (MRS) examination showed a description of the mild demyelination process. The serum antibody AQP4 (AQP4-IgG) results were seronegative, the cerebrospinal fluid examination was normal, and the oligoclonal band was negative. The ophthalmoscopic examination found bilateral papillary atrophy but optical coherence tomography (OCT) was still normal. Somatosensory evoked potential and visual evoked potential examinations were abnormal. The patient was diagnosed with NMOSD and was given combination immunosuppressant therapy, corticosteroids, and therapeutic plasma exchange. The patient experienced significant improvement with EDSS decreased to six.CONCLUSION:In the case of relapsing NMOSD patient, combination therapy of immunosuppressant’s, corticosteroids, and TPE was used. There were significant improvements from EDSS nine to six.

A descriptive study of clinical and radiological profile of longitudinal extensive myelitis in a tertiary hospital in Rajasthan, India

ObjectivesTo explore the aetiological, clinical and radiological profiles of patients with LETM presenting to this tertiary care hospital in North India. Patients and methodsAll eligible LETM patients presenting to our hospital between April 2015 and Jan 2016 were prospectively studied. A total of 37 patients were included and after thorough history, examination, relevant laboratory and radiological investigation, were profiled according to the various aetiologies of LETM. ResultsOur study included 37 patients (62% males and 38% females). Most patients were young (21–30 years). Main aetiologies of LETMp were NMO (8), inflammatory (9), idiopathic (6), NMOSD (4), Vitamin B12 deficiency (4) and others (6). Most patients (62%) had acute onset of symptoms except NMOSD and B12 deficient patients who presented subacutely too. NMO group was most disabled (poor Rankin and MRC assessments) at presentation; prognostically no (87%) or minimal improvement (13%) at discharge was seen in this group compared to other aetiologies. Forty point five percent patients had thoracic sensory complaints. CSF (pleocytosis 62%) and raised protein (81%) and brain abnormalities on MRI were seen in 11% patients. Bladder and optic nerve involvement (75.7% and 35% patients respectively). Seventy five percent NMO and 67% probable inflammatory aetiology patients also had B12 deficiency. ConclusionThis study concludes that LETMp has varied aetiologies with NMO having more disability and poorer outcomes. Thoracic cord segment is most commonly involved. Vitamin B12 deficiency may predispose patients to inflammatory LETM including NMO syndromes.

First clinical inflammatory demyelinating events of the central nervous system in a population aged over 70 years: A multicentre study

BackgroundFew data are available regarding patients with very late-onset inflammatory demyelinating events. (VLO-IDE). ObjectivesThe aim of this study was to describe the clinical, biological, and radiological characteristics and aetiological diagnosis of very late first inflammatory demyelinating events of the central nervous system. MethodsWe conducted a national descriptive retrospective multicentre study on a case series of patients aged >70 years at the time of VLO-IDE. Patients were recruited from a national call on behalf of the 'Société Francophone de la Sclérose en Plaques' (French Multiple Sclerosis Society). ResultsTwenty-five patients were referred (F:M sex ratio 2.1:1). The most frequent clinical impairment was a spinal cord deficit (23/25), usually severe (disability score, median EDSS 4.5 [2–9.5]). Spinal cord lesions were usually extensive, spanning at least three segments (11/25), and large brain lesions were also observed (lesions >20 mm in 6/25). The final aetiological diagnoses comprised multiple sclerosis (9/25), neuromyelitis optica spectrum disorders (7/25), neurosystemic lupus erythematosus (2/25), transverse myelitis without aetiological diagnosis (6/25) and optic neuritis (1/25). ConclusionsThis study highlights a particular phenotype of first clinical inflammatory demyelinating events in predominantly female patients aged >70 years who have severe motor impairment with common longitudinal extensive myelitis and large and common very active radiological inflammatory lesions. Neuromyelitis optica spectrum disorders seem overrepresented.

Longitudinal extensive transverse myelitis (LETM) in children: A twenty-year study from Oman.

Objective:The data on children with diagnosis of idiopathic transverse myelitis (ITM) was searched to find the pattern of myelitis in Oman.Method:A retrospective study was carried out from January1995 to December 2014. Electronic medical records and patient medical files were seen to get the complete data of the children with ITM. This work was carried out at Sultan Qaboos University hospital, Muscat, Oman. The ethical committee of the hospital had approved the study. The diagnosis was based on the established criteria. Other causes of myelopathy were excluded.Results:19 children with idiopathic transverse myelitis were found. There were 18 out of 19 (94.6%) children with longitudinal extensive transverse myelitis (LETM).Conclusion:Longitudinal transverse extensive myelitis is the most common form of ITM in Oman.

Acute transverse myelitis in childhood: A single centre experience from North India.

Acute transvers myelitis (ATM) is a rare and disabling condition in childhood. There are only few reports of clinical profile, prognosis and predictors of ATM from developing countries. To study the clinical profile of children with ATM and predictors of its outcome. Retrospective analysis of children <12 years of age diagnosed with ATM over a period of 6 years from a tertiary care institute. Thirty six children (21 boys, median age-7.5 years) were diagnosed with ATM. Weakness was symmetrical at onset in 27 (75%) children with progression over a median of 2 days (IQR 1-5 days). Severe weakness at onset with lower limb power ≤ 1/5 on MRC scale was present in 27 (75%), a sensory level in 25(69.4%) and bladder dysfunction in 31(86.1%) children. MRI showed longitudinal extensive myelitis (LETM) in 27 (75%) children and the thoracic cord was most commonly affected [18 (50%)]. On a median follow up of 35 months (range IQR 11-57 months); 15 (41.7%) were non ambulatory or required assistance to walk. Severe weakness at onset with power ≤ 1 on MRC scale, spinal shock, respiratory muscle weakness, mechanical ventilation, greater mean time to diagnosis and treatment was associated with bad outcome. ATM was a monophasic illness in all, except in 3 children; all with neuromyelitis optica spectrum disorder. Progression to multiple sclerosis was not seen in any child in our cohort. In this series of childhood ATM from North India, the disease was severe, monophasic and involved long segments (≥ 3) of cord in majority. Nearly half the children remain dependent on follow up. Delayed diagnosis and delayed initiation of steroid therapy was associated with poor outcome.

A reversible cause of longitudinally extensive transverse myelopathy answer

D. Posterior reversible encephalopathy syndrome with spinal cord involvement Longitudinal transverse extensive myelitis (LETM) is defined as an inflammatory spinal cord lesion that appears on spinal MRI with high signal intensity on T2-weighted sequences and extends over three or more vertebral segments [[1]Trebst C. Raab P. Voss E.V. et al.Longitudinal extensive transverse myelitis–it’s not all neuromyelitis optica.Nat Rev Neurol. 2011; 7: 688-698Crossref PubMed Scopus (116) Google Scholar]. LETM arises secondary to a large number of etiologies, including neuromyelitis optica spectrum disorders and other demyelinating disorders, systemic inflammatory and autoimmune diseases, infectious diseases, neoplastic and paraneoplastic processes, and rarely, nutritional and vascular causes [[1]Trebst C. Raab P. Voss E.V. et al.Longitudinal extensive transverse myelitis–it’s not all neuromyelitis optica.Nat Rev Neurol. 2011; 7: 688-698Crossref PubMed Scopus (116) Google Scholar]. Posterior reversible encephalopathy syndrome (PRES) with spinal cord involvement can be present in several conditions, such as uncontrolled high blood pressure, renal failure and during the use of immunosuppressive agents [[2]Hinchey J. Chaves C. Appignani B. et al.A reversible posterior leukoencephalopathy syndrome.N Engl J Med. 1996; 334: 494-500Crossref PubMed Scopus (2706) Google Scholar]. Despite the usual association with parieto-occipital signal abnormalities, infratentorial and spine compromise have also been reported [2Hinchey J. Chaves C. Appignani B. et al.A reversible posterior leukoencephalopathy syndrome.N Engl J Med. 1996; 334: 494-500Crossref PubMed Scopus (2706) Google Scholar, 3de Havenon A. Joos Z. Longenecker L. et al.Posterior reversible encephalopathy syndrome with spinal cord involvement.Neurology. 2014; 83: 2002-2006Crossref PubMed Scopus (54) Google Scholar]. Spinal cord involvement in the context of PRES (PRES-SCI) is extremely rare, and gives rise to longitudinally extensive spinal lesions, frequently correlating with Grade IV hypertensive retinopathy [[3]de Havenon A. Joos Z. Longenecker L. et al.Posterior reversible encephalopathy syndrome with spinal cord involvement.Neurology. 2014; 83: 2002-2006Crossref PubMed Scopus (54) Google Scholar]. Therefore, PRES-SCI should be suspected in patients with longitudinally extensive transverse myelitis during hypertensive emergencies. The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. A reversible cause of longitudinally extensive transverse myelopathy: questionJournal of Clinical NeuroscienceVol. 22Issue 11PreviewA 27-year-old man presented with a 5 day history of progressive headache with nausea, vomiting, blurred vision, weakness in the lower limbs and urinary incontinence. His past medical and familial histories were unremarkable. An examination revealed blood pressure of 230/140 mmHg, bilateral hypertensive retinopathy with papilledema, and mild paraparesis with brisk tendon reflexes. His blood tests revealed elevated creatinine (3.4 mg/dL) and urea (145 mg/dL) levels. The MRI scan of the brain and spine showed a longitudinally extensive signal change from the brainstem down to the mid-thoracic spine level, without contrast enhancement (Fig. Full-Text PDF

Relapse of neuromyelitis optica during pregnancy—Treatment options and literature review

Neuromyelitis optica (NMO) is a rare demyelinating disorder haracterized by recurrent optic neuritis and longitudinal extensive ransverse myelitis (LETM). The pathogenesis of NMO is still under xtensive study with recent findings supporting the pathological ole for humoral immunity, with the production of anti-aquaporinautoantibodies of the IgG class combined with the activation of he complement cascade [1,2]. These data indicate a distinctive athology of NMO compared to MS and recognition of NMO as a eparate disease entity. The pathological role of anti-aquaporin-4 utoantibodies (anti-AQP4 Ab) depends on induction of astroyte dysfunction resulting in oligodendrocyte loss [3,4]. 68–91% of atients with NMO have circulating IgG1 Abs against aquaporin(AQP4), termed NMO-IgG [5] a water channel protein expressed ainly in the central nervous system (CNS) in the plasma memrane of astrocytes [6]. NMO is a rare disorder and only since it as been distinguished from multiple sclerosis are data regarding he course of disease and effects of treatment starting to appear. In

Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease

Objective: We aimed to report the frequency and implications of antibodies to myelin oligodendrocyte glycoprotein (MOG-ab) in adults with demyelinating syndromes suspicious for neuromyelitis optica (NMO). Methods: Samples from 174 patients (48 NMO, 84 longitudinally extensive myelitis (LETM), 39 optic neuritis (ON), and three acute disseminated encephalomyelitis (ADEM) who presented initially with isolated LETM) were retrospectively examined for AQP4-ab and MOG-ab using cell-based assays. Results: MOG-ab were found in 17 (9.8%) patients, AQP4-ab in 59 (34%), and both antibodies in two (1.1%). Among the 17 patients with MOG-ab alone, seven (41%) had ON, five (29%) LETM, four (24%) NMO, and one (6%) ADEM. Compared with patients with AQP4-ab, those with MOG-ab were significantly younger (median: 27 vs. 40.5 years), without female predominance (53% vs. 90%), and the clinical course was more frequently monophasic (41% vs. 7%) with a benign outcome (median Expanded Disability Status Scale: 1.5 vs. 4.0). In eight patients with paired serum-cerebrospinal fluid (CSF) samples, five had MOG-ab in both samples and three only in serum. Antibody titres did not differ among clinical phenotypes or disease course. MOG-ab remained detectable in 12/14 patients (median follow-up: 23 months) without correlation between titres’ evolution and outcome. Conclusion: MOG-ab identify a subgroup of adult patients with NMO, LETM and ON that have better outcome than those associated with AQP4-ab. MOG-ab are more frequently detected in serum than CSF and the follow-up of titres does not correlate with outcome.

Aquaporin‐4 Antibodies (NMO‐IgG) as a Serological Marker of Neuromyelitis Optica: A Critical Review of the Literature

Antibodies to aquaporin-4 (called NMO-IgG or AQP4-Ab) constitute a sensitive and highly specific serum marker of neuromyelitis optica (NMO) that can facilitate the differential diagnosis of NMO and classic multiple sclerosis. NMO-IgG/AQP4-Ab seropositive status has also important prognostic and therapeutic implications in patients with isolated longitudinally extensive myelitis (LETM) or optic neuritis (ON). In this article, we comprehensively review and critically appraise the existing literature on NMO-IgG/AQP4-Ab testing. All available immunoassays-including tissue-based (IHC), cell-based (ICC, FACS) and protein-based (RIPA, FIPA, ELISA, Western blotting) assays-and their differential advantages and disadvantages are discussed. Estimates for sensitivity, specificity, and positive and negative likelihood ratios are calculated for all published studies and accuracies of the various immunoassay techniques compared. Subgroup analyses are provided for NMO, LETM and ON, for relapsing vs. monophasic disease, and for various control groups (eg, MS vs. other controls). Numerous aspects of NMO-IgG/AQP4-Ab testing relevant for clinicians (eg, impact of antibody titers and longitudinal testing, indications for repeat testing, relevance of CSF testing and subclass analysis, NMO-IgG/AQP4-Ab in patients with rheumatic diseases) as well as technical aspects (eg, AQP4-M1 vs. AQP4-M23-based assays, intact AQP4 vs. peptide substrates, effect of storage conditions and freeze/thaw cycles) and pitfalls are discussed. Finally, recommendations for the clinical application of NMO-IgG/AQP4-Ab serology are given.

Case-report: Scleroderma with longitudinally extensive myelitis

WCN 2013 No: 2685 Topic: 6 — MS & Demyelinating Diseases Case-report: Scleroderma with longitudinally extensive myelitis J.P.R.P.T. de Azevedo, G.D.C. Ferreira, F.K.M.S. Pinto, L.A.A. Reis, L.V. de Oliveira, D.S. Diniz. Neuroscience Nucleus, Clinical Hospital of Goias Federal University, Goiânia, Brazil; Faculty of Medicine, Federal University of Goias, Goiania, Brazil Background: There is an intrinsic relationship between systemic autoimmune diseases and myelitis. Most of them are reported with acute transverse myelitis, some of them with longitudinally extensive myelitis (LTEM) and by now in the Neuromyelitis optica spectrum disorders (NOSD) Researching in major scientific directories there are no cases reporting the association between acute myelitis and Scleroderma. Objective: Describe an unreported association with Scleroderma and LTEM. Patients and methods: A 53 year old man was admitted with progressive weakness in the last 24 h. Symptoms began with sudden pain and leg paresthesia and evolved to a complete medullary syndrome with paraplegia, bladder dysfunction and a sensory impairment by the level of T8. He was previously diagnosed with Scleroderma four years ago. He presented by Raynoud fenomena, sclerodactyly and telangiectasia in the face, chest, and upper and lower limbs. He presented a positive nucleolar pattern ANA. Lab tests have shown elevated proteins in CSF and elevated CRP and ESR. A full spinal MRI has shown a contiguous T2 increased signal beginning from the bottom of the medulla oblongata to the level of T8 confirmed in the transactional view. Results: The patient was submitted to a five-day course of Methylprednisolone followed by a course of Intravenous Human Hyperimmune Gammaglobulin. The patientwas dischargedwith prednisone, colchicin and chloroquine. He returned 27 days later fully walking, without bladder or sensory dysfunctions. Antiaquaporin-4 IgG autoantibody was negative. Conclusion: This case reported the association between Scleroderma and LTEM in the NOSD. doi:10.1016/j.jns.2013.07.1348 Abstract — WCN 2013 No: 2707 Topic: 6 — MS & Demyelinating Diseases Neurological symptoms in demyelinating disease mimicking an acute ischaemic stroke—Case report WCN 2013 No: 2707 Topic: 6 — MS & Demyelinating Diseases Neurological symptoms in demyelinating disease mimicking an acute ischaemic stroke—Case report K. Majstorovic, A. Arsovska, M. Perovska. Institute of Nephrology, Struga, The Former Yugoslav Republic of Macedonia; University Clinic of Neurology, Skopje, The Former Yugoslav Republic of Macedonia Background: The case presented here emphasizes the role of an accurate diagnosis for proper targeting of therapy. Objective: Demyelinating disease should be considered even in older patients with focal and fluctuating neurological symptoms. Patients and methods: In March 2013, a 50-year-old woman was admitted with acute left hemiplegia and severe dysarthria, which occurred three days ago. Ten days earlier the patient had intense vertigo, nausea, vomiting, diplopiae, speech difficulty and unstable gait. Neurological status on admission showed severe dysarthria, bilateral rhythmic nystagmus, left hemiplegia, vivid generalized muscle tendon reflexes with asymmetry in the right and positive Babinski sign on the right. In the following days MRI of the brain and lumbal puncture were performed. MRI of the brain showed hypersignal changes in T2 and FLAIR images. CSF electrophoresis showed mild immune activity in the central nervous system.VEP showed a prolonged latency in P100 on the right eye. Results: Therefore, disseminated demyelinating process was diagnosed and intravenous methylprednisolone treatment was initiated (1 g/day for 5 days) with a subsequent progressive improvement of the neurological symptoms. Discussion: Our case has shown that sudden, rapid and unusual neurological deficits can be observed in multiple sclerosis (MS). In such cases, there is a wide range of diseases in the differential diagnosis, including ischemic cerebrovascular events. The presented case demonstrates rare Multiple Sclerosis cases with late onset of the disease by the age of 50.The correct diagnosis of these two entities is essential for proper treatment. doi:10.1016/j.jns.2013.07.1349 Abstract — WCN 2013 No: 2349 Topic: 6 — MS & Demyelinating Diseases Memory impairment in patients with clinically isolated syndrome WCN 2013 No: 2349 Topic: 6 — MS & Demyelinating Diseases Memory impairment in patients with clinically isolated syndrome E.Hyncicova, E.Meluzinova,M. Vyhnalek, J. Vecanova, J. Libertinova, I. Kovařova, T. Nikolai, J. Hort, J. Laczo. Department of Neurology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic; International Clinical Research Center, St. Anne's University Hospital Brno, Brno, Czech Republic; Department of Neurology, 1st Faculty ofMedicine, Charles University andGeneral UniversityHospital in Prague, Prague, Czech Republic Abstracts / Journal of the Neurological Sciences 333 (2013) e358–e421 e369

Quantitative assays for anti-aquaporin-4 antibody with subclass analysis in neuromyelitis optica

Background: To clarify the clinical relevance of anti-aquaporin-4 (anti-AQP4) antibody titers and immunoglobulin (IgG) subclass. Methods: Using a bridging enzyme-linked immunosorbent assay (ELISA), a flow cytometric assay (FCMA) and an immunofluorescence assay (IFA) for anti-AQP4 antibodies, sera from 142 patients with multiple sclerosis (MS) as defined by the McDonald criteria (2005), 29 with neuromyelitis optica (NMO) who fulfilled the 1999 criteria, 19 with recurrent and/or longitudinally extensive myelitis (RM/LM), 86 with other non-inflammatory neurological diseases (OND) and 28 healthy controls (HC) were studied. Results: Anti-AQP4 antibody positivity rates by IFA, FCMA, and ELISA were 41.4%, 51.7% and 48.3%, respectively, in NMO (1999) patients, and 0% in the OND and HC groups. Twenty-six MS patients (18.3%) were positive for the antibody; 17 met the 2006 NMO criteria, including positivity for anti-AQP4 antibody, and five had longitudinally extensive myelitis (LM). Among the cases with anti-AQP4 antibody detected by FCMA, IgG1, 2, 3, and 4 anti-AQP4 antibodies were found in 97.8%, 37.0%, 6.5% and 6.5% respectively. There was no association of either antibody positivity or level of anti-AQP4 antibody IgG subclasses with clinical parameters after adjustment of p values for multiple comparisons. Conclusions: FCMA and bridging ELISA are useful for detecting and quantifying anti-AQP4 antibodies.

133 Aquaporin 4 antibody seropositivity and visual function in a UK cohort

ObjectivesTo identify clinical features of aquaporin 4 (AQP4) antibody positive optic neuritis (ON) in neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) in a UK cohort.MethodsWe performed a retrospective review...

Seroconversion of anti-aquaporin-4 antibody in NMO spectrum disorder: a case report

Dear Sirs,Anti-aquaporin-4 (AQP4) antibodies are known to be pres-ent both in patients with neuromyelitis optica (NMO) andNMO spectrum disorders (NMOsd), some limited forms ofNMO [1, 2]. The antibodies are considered to have animportant role in the pathogenesis of those disorders [3, 4].We had a patient with NMOsd who had acute longitudinallyextensive myelitis. Her serum anti-AQP4 antibody waspositive at the time myelitis developed, but was negative 8and 9 years before myelitis onset and seroconverted 3 yearsbefore the onset. Her case provides insight into when andhow anti-AQP4 antibody is produced.A 55-year-old Japanese woman developed numbnessand dysesthesia on April 10, 2010 which thereafter exten-ded symmetrically below the eighth cervical segment andcaused slight staggering gait in the dark. Spinal cord MRIT2-weighted images revealed longitudinally extensivemyelitis from the C4 to T2 vertebral segments with gado-linium-enhanced lesion from C7 to T1. She was referred tous, and admitted on May 18, 2010.Actually, she had visited us regularly for the follow-up ofanti-acetylcholine receptor (AChR) antibody-positivemyasthenia gravis (MG) since 1994. She had been treatedwith oral high-dose prednisolone from January 1995. InAugust 1995, she underwent extended thymectomy andhistological examination had revealed hyperplasia. Thosetreatmentshadrelievedallneurologicaldeficits by7 monthsafter surgery. From December 1998, the prednisolone dosewas maintained at 5 mg/day every second day with tempo-rary cessations. By May 2002, prednisolone therapy hadended, and she remained symptom-free.On her present admission, neurological examinationrevealed hypopallesthesia and hyperreflexia in her lowerlimbs, and mild weakness in her left adductor digiti miniand bilateral iliopsoas muscles. Cerebrospinal fluid exam-ination showed a normal cell count and protein levels witha negative oligoclonal band. Brain MRI detected noabnormality consistent with Barkhof’s criteria [5]. Anti-AQP4 antibodies, measured by enzyme-linked immuno-sorbent assays [6] in a serum sample obtained on May 18,2010 were positive. Our diagnosis was NMOsd.Serum samples had been stocked for MG researchbefore the onset of NMO. After receiving her informedconsent, we measured the presence of anti-AQP4 anti-bodies in her stocked serum samples. Figure 1 shows thelongitudinal change in the anti-AQP4 and anti-AChRantibodies before and after NMOsd onset. Anti-AQP4antibody remained negative 8 and 9 years before the onsetof longitudinally extensive myelitis and was seroconverted3 years before myelitis onset. Her anti-AQP4 antibody titrehad increased, and myelitis emerged.Recently, the case of a patient with NMO whose serumhad remained positive for anti-AQP4 antibody for morethan 10 years before onset was reported [7], but it is notclear whether all patients with NMO have anti-AQP4antibody ten or more years before onset. Our patient’s caseshows that seroconversion is possible and that elevation ofanti-AQP4 antibody does not cause NMO onset. Onset mayrequire long-term elevation of anti-AQP4 antibody or someother mechanism.We previously reported two patients with MG whopresented with NMO after thymectomy [8]. In that report,

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

Intravascular lymphoma is a rare disorder that commonly involves the central nervous system. Neurologic involvement may be the presenting and only manifestation. Identifying intravascular lymphoma as the cause of neurologic disease is diagnostically challenging. We report an elderly woman presenting with subacute onset paraparesis due to spinal cord involvement by an intravascular lymphoma. Progressive worsening was associated with extension of a longitudinally-extensive thoracic intramedullary spinal cord lesion. Extensive investigations failed to provide a diagnosis in life and repeated empiric therapeutic trials were unsuccessful. Diagnostic confirmation was postmortem. A longitudinally-extensive spinal cord lesion has a broad differential diagnosis. Intravascular lymphoma should be considered particularly in older individuals. The presence of coexisting hematologic abnormalities should prompt consideration of a bone marrow biopsy. Early diagnosis may direct therapy and lead to a more favorable prognosis.