Longitudinally Extensive Transverse Myelitis Patients

aloofnesswise broad Transverse Myelitis PatientsCharacteristics of Longitudinally Extensive Transverse Myelitis patients a retrospective psycho synopsis of 40 consecutive cases at a tertiary commission hospital from North-West IndiaAbstractLongitudinally pan center transverse myelitis (LETM), characterised by spinal anaesthesia stack inflammation extending three or more vertebral plane sections gaberdinethorn be perk upd by multiple disorders most ballpark being neuromyelitis optica(NMO). much(prenominal) is the frequent association between these deuce entities that there is a exploitation concern to regard them as being practically synonymous with severally other. However, all potential etiologies should be considered in the assessment of LETM. We conducted retrospective analysis (2010-2014) of 40 patients of LETM for demographic features, clinical being, science lab investigations and neuro- resourcefulness. Result Majority of the patients presented aggressively with bladder dysfunction and paraparesis. Ten out of 40(25%) were assort as NMO among which that 4 were serum NMO antibody verificatory. Among the rest, there were 6 patients of MS, 3 patients of tubercular, 2 patients severally of ADEM, spinal AVM and postinfectious etiology, nonpareil patient severally of SACD and SLE. A base of 13 patients remained in whom no causative factor could be identified from the getable investigations. Conclusion LETM veritable(a) with optic neuritis does not universally represent a diagnosis of NMO and one needs to be cautious while devising a diagnosis of NMO without consideration of other etiologies as the intervention and outlook differs among different etiologies.Abbreviations MS (multiple sclerosis) AVM (arteriovenous malformation) SACD (subacute combined degeneration) ADEM (acute disseminated encephalomyelitis) TM (transverse myelitis).Key words Longitudinally drawn-out transverse myelitis Neuromyelitis opticaIntroductionLongitudi nally extensive transverse myelitis (LETM) is a relatively late term designating a transverse myelitis(TM) that extends three or more vertebral segments vertically. These lesions, which may occasionally span the entire length of the spinal cord, are much rarer and in general associated with greater morbidity than the typical lesions of idiopathic TM or multiple sclerosis (MS) associated TM(1).The make out discriminating feature of LETM lesions is their length. The most frequent thrust of LETM is neuromyelitis optica (NMO). In recent judgment of convictions the association between these two conditions has been so heavily emphatic that when LETM is en opineered, an erroneous diagnosis of NMO may be made prior to elaborated consideration of other potential etiologies of LETM(1).Thus, early recognition and establishment of the etiology of LETM from appropriate workup is essential for optimizing return and in some cases commencing appropriate treatment to prevent future attacks of central scatter sensationed system (CNS) inflammation.Studies comparing clinical, science lab and radiological profiles of the LETM patients are scarce, especially from the developing countries. Thus, this study was conducted to review the characteristics of introduction and etiological classification of LETM at SMS Medical College and Hospital, Jaipur, a tertiary care centre of North India.Patients and MethodsThe study is a retrospective analysis (2010-2014) of 40 patients classified as having LETM on the basis of clinical manifestations of myelitis and spinal magnetic resonance imaging finding of lesions typically extending three or more vertebral segments in length, admitted in SMS Medical College and Hospital, Jaipur with the look oning objectives-To study the clinical, radiological and CSF profile of patients with LETM.To instruct the aetiology of the myelitis.Patient demographics, presenting symptoms, clinical manifestations and investigations were recorded on data pre sentation forms.The investigations included routine blood profile along with markers of connective waver disorders (ESR, CRP, ANA, Anti dS DNA), magnetic resonance imaging (of involved spinal cord segments in T2- weighted images of spinal MRI and MRI virtuoso), CSF analysis (including oligoclonal bands) and serum NMO antibody (NMO Ab) which was through with(p) by corroboratory immunofluorescence method.Results40 patients were found to fulfil the LETM criteria. 22 among the 40 patients were slight than 30 historic period age (55%). Overall Mean age was 28 categorys with male person female ratio of 1.81, suggestive of male preponderance. asunder from it no specific trend was observed among the study group in terms of their demographics.The majority of patients presented with bladder dysfunction, paraparesis and quadriparesis. Most of the patients had an acute presentation. Collectively, thoracic spinal cord segment was most normally involved. 12 patients had clinically eviden tiary vision impairment at the time of presentation of which 10 were classified as NMO according to Wingerchuck et al criteria, one case was classified as MS and another(prenominal) was a case of SACD (Table 1).Table 1 Presenting clinical featuresTable 2 Radiological FindingsCerebrospinal fluid (CSF) pleocytosis was seen in 55 %( 22 out of 40 patients) ranging from 10 to 250 cells/cumm. Among 10 NMO patients, 5 showed CSF pleocytosis of which 2 had neutrophilic predominance. The maximum cell count among NMO patients was 35 cells with lymphocytic predominance. isolated from it, 3 patients of tubercular, 2 patients severally of postinfectious etiology and ADEM , one patient of MS and 4 patients of undetermined etiology also showed CSF pleocytosis. The maximum CSF cell count was 250 cells in one of the tubercular patient with lymphocytic predominance. CSF proteins were elevated in 15 out of 40(37.5%) patients of which 3 patients were each(prenominal) of MS and tubercular etiology, 2 patients each of NMO and postinfectious etiology and 5 patients were of undetermined kinsperson. Out of 40 patients, 18 had extensive lesions involving 6 spinal cord segments .Among 38 patients in whom MRI encephalon was done, 10 showed abnormalities, of which 5 patients were of MS, two were each of ADEM and tubercular etiology and another one was NMO (NMO Ab positive) (Table 2).Among the MS patients, abnormalities were seen involving deep uninfected matter of bilateral intellectual hemispheres, corpus callosum, top dogstem and principal(a) ganglia.Based upon the above-mentioned clinical presentation and investigations, patients were classified according to their etiologies (Table 3).Table 3 Etiology of LETM patients10 patients were of NMO fulfilling revise diagnostic criteria for neuromyelitis optica by Wingerchuk et al (7). In 8 out of 10 patients NMO antibody was sent, 2 patients refused for it. 4 out of them were NMO Ab positive and remaining 4 were negative. Among 10 NMO p atients just one patient had lesions in MRI thinker typical of NMO ( learn 12)).Four of ten patients in whom serum NMO Ab was done showed positivity and one of the NMO Ab positive patient had brain MRI abnormality involving brainstem, shadow part of corpus callosum, left parietal periventricular white matter. Six patients were of MS fulfilling the revised McDonald criteria for diagnosis of MS. Three patients were of tubercular myelitis ,two patients each were of ADEM , postinfectious etiology, and spinal AVM. One was having nutritional cause in form of vitamin B12 need and one patient had SLE (ANA dsDNA positive). Rest of the patients could not be categorized to a expressed etiology from the useable investigations.DiscussionIn our series, common presenting symptoms were bladder dysfunction, paraparesis, quadriparesis, and visual impairment. Majority of the patients studied suffered from bladder dysfunction and paraparesis.It is a difficult task to determine the underlying etio logy of LETM and it is worth exploring each case for subtle clues that may point toward the correct underlying diagnosis as the prognosis and long-term treatment decision differs in each category. Till now there are no studies describing association of clinical features and demographic features with the etiology of the myelitis (2).In our series the maximum CSF cell count (250) was in tubercular patient. Maximum CSF cell count in NMO and MS patients was 35 and 30 respectively.It is now well established that LETM does not universally represent a diagnosis of NMO, even in the setting of optic neuritis. However, in our series demyelinating disorders were found to be the most common cause of LETM and NMO was the most common etiology among demyelinating disorders.All NMO patients fulfilled the criteria laid ware by Wingerchuk et al (3). Among ten NMO patients, four were NMO antibody positive. Three main laboratory techniques are utilized in identifying the antibodies. In our patients it was done by the indirect immunofluorescence method which has the reported sensitivity and specificity of 86% and 91% respectively (4). At present, it is un sporting whether there is truly a subset of patients with clinical NMO that are NMO antibody negative, or if this is a result of inadequate sensitivity of existing immunoassays to detect the antibody, or inadequately sensitive and specific diagnostic criteria, or a gang of all of these factors.Although a regular come about up was not available in all NMO patients, a telephonic survey revealed that four NMO patients, of which two were seronegative and in two NMO antibody was not done, had no relapse and are doing well. Apart from this, one patient died from subsequent relapse and one could not be assessed in follow up. Among the four NMO antibody positive patients, who were under regular follow up, two (50%) presented with relapsing- remitting form of illness. One was 25 forms mature male who initially had 4 episodes of parap aresis with near complete recovery each time and developed vision impairment in the fifth episode and another was a 12 year former(a) girl who had four relapses with synchronic occurrence of vision impairment and paraparesis in the first episode. Thus, 4 patients had a monophasic disease course in 1 year follow up. However, a diagnosis of monophasic NMO should be considered with caution, because more than 90% of patients with NMO ultimately develop a relapsing course. One prospective Class I study found that the presence of aquaporin-4specific autoantibodies (AQP4) predicts recurrence of TM or conversion to NMO(5). So was our observation in the form that out of the 3 patients who had relapse, 2 were NMO antibody positive. Therefore, we suppose that AQP4 antibody status, particularly seropositivity, has some predictive value.Median age of onset of NMO is in the fourth decade. In our series all NMO patients were below 40 years of age except one who was a 52 years old female (figure 3).Interestingly this patient apart from late onset, also presented with slowly modern paraparesis of one year duration which has not been described earlier in the literature.In spinal NMO lesions, the central part of the cord is commonly affected, including both grayness and white matter with peripheral sparing (figure 3). These imaging features may therefore help to differentiate MS from NMO in patients who present with LETM(6).Among the NMO antibody positive patients, one had typical brain lesions of NMO (Figure 1). In patients with clinical and radiological features differently typical for NMO, 6085% of cases have been shown to have abnormal brain lesions. Lesions involving the diencephalon and brainstem distinctly atypical for MS have been commonly reported in NMO patients. These distinctive lesions predominately involve the hypothalamus and provoke extend to brain tissue surrounding the third and fourth ventricle and aqueduct of Sylvius and seem to be characteristic brain lesions of NMO(7).There were 13 patients in whom a definite etiological diagnosis could not be made from the feasible investigations. This group is usually described in the literature as idiopathic. However, the idiopathic nature is a diagnosis of exclusion. In our series whether these patients were belonging to the category of postinfectious or some other form of demyelinating etiology was not clear from the investigations that were done in these patients as these patients were also later lost to follow up.Among 3 patients who were classified as tubercular on the basis of reactive CSF and positive TB PCR, one had intramedullary spinal tuberculomas (Figure 4). Intramedullary tuberculomas (IMT) are seen in only 2 out of 100,000 cases of tuberculosis and 2 out of 1,000 cases of central nervous system tuberculosis, are even rare as a cause of LETM(8).Out of these three, one had complete recovery with ATT and remaining 2 had poor outcome with bedridden status.Among 40 LETM patients, w e were able to categorise only two patients as ADEM on clinical and radiological grounds (Figure 5).Both patients were of paediatric age (14 and 16 years) group, had complete and rapid recovery with no recurrence on 6 month follow up, which made the diagnosis even more certain, and agree with the fact that monophasic course is the hallmark of ADEM. MRI features of ADEM that are unusual in MS are symmetric bilateral disease, relative sparing of the periventricular white matter and deep grey matter function (9). However, 22% of ADEM patients had a periventricular lesion pattern indistinguishable from that seen in MS (10).Similar radiological features were observed by us in both patients (Figure 5 and 6). The radiological features of spinal cord involvement in ADEM in adults have not been well studied. In a small Dutch series, one-third of patients had lesions extending more than two vertebral segments on spinal MRI at presentation (11). Approximately 75% patients with ADEM have a pr eceding infection, and by definition there allow for be evidence of demyelination within the brain, as well as in the spinal cord, as seen in our patients (Figure 6). These features may help to differentiate ADEM from NMO spectrum disorders.Among the metabolic causes, subacute combined degeneration (SACD) of the cord due to vitamin B12 deficiency can produce longitudinally extensive hyperintense signal on MRI imaging as seen in one of our patient (Figure 7). Generally T2 weighted scans award focal high signal abnormalities in the white matter of the dorsal and posterior columns in SACD (12).In conclusion, the differential diagnosis of LETM is broad. Although characteristically associated with NMO, this diagnosis accounts for less than half of cases of isolated LETM in adults, and may be even less common in children. Patients presenting with LETM require a thorough work-up to obviate other treatable causes particularly infectious and insurgent.The management of LETM is dependen t on distinguishing inflammatory from non-inflammatory aetiologies and in identifying patients who are at high risk of further attacks.Figure LegendsFigure 1 MRI brain presentation hyperintensities (arrowheads) involving brainstem, left parietal periventricular white matter, posterior part of corpus callosum in seropositive patient of NMO (12 year old girl)Figure 2 MRI cervical spine T2 weighted sagittal and axial images of same patient showing predominant involvement of central grey matter (arrowheads).Figure 3 MRI cervical spine T2 weighted sagittal and axial images of 52 years old NMO antibody positive female showing predominant involvement of central grey matter.Figure 4T2 weighted sagittal and axial images showing hyperintensity (arrowheads) in cervical cord with contrast enhancement suggestive of tuberculoma.Figure 5 MRI cervicodorsal spine, T2 weighted sagittal and axial images of a 16 year old girl with ADEM.MRI brain of the same patient showed lesions (arrowheads) in basal ganglia, brainstem and cortex typical of ADEM.Figure 6 MRI brain and spine of a 14 years old male with ADEM showing hyperintensities in brain involving bilateral periventricular white matter and long segment hyperintensity in the cord extending upto conus.Figure 7 Hyperintense signal in posterior aspect (arrow) of cervicodorsal cord in T2 weighted axial and sagittal images of SACD patient.ReferencesEckstein C., Syc S. and Saidha S., differential coefficient diagnosis of longitudinall extensive transverse myelitis in adults ENJ 2011 3 1). July 2011.Scott T., Frohman E., De Seze J. et al., Evidence-based guideline clinical evaluation and treatment of transverse myelitis. clinical neurology December, 2011Wingerchuk et al. Revised diagnostic criteria for NMO. Neurology 2006661485-9Waters P, Vincent A. Detection of anti-aquaporin-4 antibodies in neuromyelitis optica Current status of the assays. Int MS J 2008 1599-105.Weinshenker B, Wingerchuk D, Vukusic S, et al. Neuromyelitis Optica IgG predicts relapse following longitudinally extensive transverse myelitis. Ann Neurol 2006 59566569Wingerchuk, DM. Neuromyelitis Optica Spectrum deranges. Continuum Lifelong Learning Neurol 2010 16(5) 105-121Brain Abnormalities in Neuromyelitis Optica Spectrum Disorder K. Woojun, K. Su-Hyun, H. So-Young and K. Ho Jin Multiple Sclerosis International Volume 2012.Imaging diagnosing of spinal anesthesia Intramedullary Tuberculoma Case Reports and Literature Review Lu Ming J Spinal Cord Med. Apr 201033(2)159 162Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain 1990113(Pt 2)291302Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000 123(Pt 12)24072422Ketelslegers I, Visser I, Neuteboom R, boon M, Catsman-Berrevoets C and Hintzen R. Disease course and outcom e of acute disseminated encephalomyelitis is more good in adults than in children. Mult Scler 2011 17(4) 441448Larner A, Zeman A, Allen C. MRI appearances in subacute combined degeneration of the spinal cord due to vitamin B12 deficiency. JNNP 19976299-101.