ABSTRACT OBJECTIVE: To determine the frequency of longitudinally extensive transverse myelitis and Causes of longitudinally extensive transverse myelitis METHODOLOGY: A Cross-Sectional, retrospective study was conducted at Neurology Department, Mayo Hospital, Lahore from 1st January 2015 to 31st December 2015. Patients were included from inpatient with history of weakness of lower limbs/all four limbs with or without visual disturbances. History and clinical examination was consistent with transverse myelitis. Neuroimaging (MRI brain, (orbit in case of optic neuritis) and spinal cord) was done followed by LP/CSF, Vasculitis screening

Bano, Safia

Javed, Muhammad Athar

Numan, Ahsan

English

eCommons@AKU

Pakistan Journal of Neurological Sciences (PJNS) Volume 15 Issue 4 Article 8 12-2020 Longitudinally Extensive Transverse Myelitis and Neuromyelitis Optica spectrum Disorder: A Tertiary Care Centre Experience Safia Bano KEMU /Mayo hospital, Lahore Pakistan Muhammad Athar Javed KEMU /Mayo hospital, Lahore Pakistan Ahsan Numan KEMU /Mayo hospital, Lahore Pakistan Follow this and additional works at: https://ecommons.aku.edu/pjns  Part of the Neurology Commons Recommended Citation Bano, Safia; Javed, Muhammad Athar; and Numan, Ahsan (2020) "Longitudinally Extensive Transverse Myelitis and Neuromyelitis Optica spectrum Disorder: A Tertiary Care Centre Experience," Pakistan Journal of Neurological Sciences (PJNS): Vol. 15 : Iss. 4 , Article 8. Available at: https://ecommons.aku.edu/pjns/vol15/iss4/8 LONGITUDINALLY EXTENSIVE TRANSVERSE MYELITIS AND NEUROMYELITIS OPTICA SPECTRUM DISORDER:A TERTIARY CARE CENTRE EXPERIENCESaa bano1, Muhammad Athar Javed2, Ahsan Numan3 1Assistant professor Neurology, KEMU /Mayo hospital, Lahore 2Professor Neurology (retired.), KEMU /Mayo hospital, Lahore3Professor Neurology, KEMU /Mayo hospital, LahoreSafia Bano, Assistant Professor Neurology, KEMU/Mayo Hospital ,Lahore safiabano207@gmail.comDate of submission: May 12, 2020 Date of revision: September 15, 2020 Date of acceptance: September 25, 2020ABSTRACT OBJECTIVE: To determine the frequency of longitudinally extensive transverse myelitis and Causes of longitudinally extensive transverse myelitisMETHODOLOGY: A Cross-Sectional, retrospective study was conducted at Neurology Department, Mayo Hospital, Lahore from 1st January 2015 to 31st December 2015. Patients were included from inpatient with history of weakness of lower limbs/all four limbs with or without visual disturbances. History and clinical examination was consistent with transverse myelitis. Neuroimaging (MRI brain, (orbit in case of optic neuritis) and spinal cord) was done followed by LP/CSF, Vasculitis screening.RESULTS: Total 37 patients of transverse myelitis (TM) (1287 patients were admitted in neurology department with various neurological disorders in study year) were enrolled in this study. Demographic data showed age distribution between 15-50 years. Out of 37patients, LETM was present in 16 patients. They were predominantly females (62.5% versus 37.5% male) patients. With majority has normal MRI brain (93.8% versus 6.2% abnormal). Most common cause of LETM was Neuro Myelitis Optica (NMOSD) 93.8% versus 6.2% Multiple Sclerosis (MS).CONCLUSION: Longitudinally extensive transverse myelitis (LETM) is not an uncommon disease, however, it remains under-diagnosed in our clinical practice due to either lack or cost of diagnostic facilities. LETM is common presentation of Neuromyelitis Optica Spectrum disorder (NMOSD) followed by multiple sclerosis, ADEM and other autoimmune diseases etc. Thus clinically important as it results in severe morbidity and patients are at risk of further attacks. Early diagnosis and confirmation of the underlying cause is vital in order to initiate appropriate therapy and favorable outcomesKEYWORDS: Neuro Myelitis Optica Spectrum disorder (NMOSD), Longitudinally Extensive Transverse Myelitis (LETM), Multiple Sclerosis (MS), Sarcoidosis, lupus.INTRODUCTION Transverse myelitis is a clinical heterogeneous syndrome, causes inflammation of spinal cord that results in varying degree of residual disability.1 The etiology of transverse myelitis is broad, it can be infectious (tuberculosis), inflammatory, vascular, neoplastic and paraneoplastic.2’17Transverse myelitis diagnosis is made on clinical history, examination findings, radiological, immunological and CSF analysis studies3 transverse myelitis causes florid inflammation of spinal cord and can lead to devastating neurological deficit within minutes to hours.4On imaging it shows involvement of one or more vertebral segments, if 3 or more vertebral segments are involved it is called longitudinal extensive transverse myelitis.5 Demyelinating disorders are the most common causes of acute transverse myelitis, main categories of acute inflammatory transverse myelitis includes multiple sclerosis, Neuromyelitis optica, Neuromyelitis optica spectrum disorders and idiopathic transverse myelitis.3 The differential diagnosis of LETM includes Multiple O R I G I N A L  A R T I C L E3 6P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 5  ( 4 )  O C T O B E R - D E C E M B E R  2 0 2 0Sana Younus1, Aisha S Chachar2, Ayesha I Mian3 sclerosis, Neuromyelitis optica spectrum disorder, vascuilitis esp. Lupus (SLE), and sarcoidosis, acute disseminated encephalomyelitis, idiopathic and others.6,16 A study from, E. Carnero Contentti, J.P. Hryb, et al, reported LETM being caused by NMOSD (37%), MS (3.7%) and other etiology as lupus (22.2%), idiopathic LETM (22.2%), tumor (11.1%) acute disseminated encephalomyelitis (7.4%), dural fistula (7.4%) etc.16.although several studies have been done worldwide but there is variability in the studies that are published. The results of this study will help us to understand the disease variability in our local cohort and it will open windows for newer research protocols and will set the priorities of the patient’s treatment.OBJECTIVES• o find out the frequency of longitudinally extensive   transverse myelitis.• To find out the causes of longitudinally extensive   transverse myelitisMETHODOLOGYA Cross-Sectional, Retrospective study was conducted at Neurology Department, Mayo Hospital, Lahore from 1st January 2015 to 31st December 2015. Inclusion criteria was; all cases of Transverse Myelitis, Both genders, Age 15-60 years, MRI cervical or dorsal spine consistent.Longitudinally extensive transverse myelitis (LETM) was defined as involvement of the spinal cord, with abnormal T2 signal on MRI with at least three or more vertebral body segments in length.The patients were excluded if; Cases of compressive Myelopathy due to any cause (n-16), MRI consistent with patchy demyelization (n-5) rather than LETM, Age below 15 or above 60 years and MRI could not be done.Patients were included from inpatient with history of weakness of lower limbs/all four limbs with or without visual disturbances. Clinically, history and examination was consistent with transverse myelitis and optic neuritis. Neuroimaging (MRI brain, (orbit in case of optic neuritis) and spinal cord) was done followed by LP/CSF (normal in all patients), vasculitis screening. Aquaporin -4 antibodies level could not be checked due to lack of availability and cost. For diagnosis of NMOSD/MS, Wingerchuk’s criteria18 and modified McDonald’s criteria19 was used.RESULTSTotal 37 patients of transverse myelitis (TM) (1287 patients were admitted in neurology department with various neurological disorders in study year) were enrolled in this study. Out of 37 patients, 16 patient’s neuroimaging was consistent with LETM. Demographical data showed age distribution between 15-50 years. Mean age of the patients was 27.3±8.5 years (range 15-50 years). Patients had longitudinally extensive transverse myelitis (LETM) i.e. 16. They were predominantly females 10(62.5%) versus 6(37.5%) males. Frequency distribution according to age is shown in table (Figure 1). With majority has normal MRI brain (93.8% versus 6.3% abnormal). Most common cause of LETM was Neuro Myelitis Optica spectrum disorder (NMOSD) 93.8% versus 6.2% Multiple Sclerosis (MS)(MRI brain was consistent with MS “dissemination in space criteria”) MS figure 2.According to episodes of myelitis distribution, 9(56.3%) had Myelitis and optic neuritis, while 4(25%), 2(12.5%) and 1(6.2%) had recurrent attack of myelitis(neuroimaging finding favor’s), single attack of myelitis (anti aquaporin-4 antibodies was positive) and recurrent attack of optic neuritis with myelitis figure 3. DISCUSSIONThe most common cause of longitudinal extensive transverse myelitis is Neuromyelitis optica spectrum disorder (NMOSD).7,16 To reach the correct diagnosis and exact etiology of LETM is quite a challenging task. In our study most frequent cause for LETM found to be Neuromyelitis optica Spectrum disorder (NMOSD) 93.8% with female predominance 62.5%.A study from, E. Carnero Contentti, J.P. Hryb, et al, reported LETM being caused by NMOSD (37%), MS (3.7%) and other etiology as lupus (22.2%), idiopathic LETM (22.2%), tumor (11.1%) acute disseminated encephalomyelitis (7.4%), dural fistula (7.4%) etc.16 results are consistant to our study as NMOSD is the most common cause followed by MS.Another study results showed frequent cause of LETM is NMOSD (46.8%), MS (1.6%), isolated LETM (16%), recurrent LETM (14%), infectious etiology (11%) etc.20 which also support our study results.Acute transverse myelitis has a varied clinical presentation, etiology and outcome.8 NMOSD is a recurrent disorder, continuous lesion on MRI affecting central cord,9 whereas multiple sclerosis involves more peripheral part of the spinal cord and is patchy in nature.9 NMOSD is a autoimmune disease  affecting optic 3 7P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 5  ( 4 )  O C T O B E R - D E C E M B E R  2 0 2 0nerves, unilateral or  bilateral at presentation- optic neuritis and spinal cord- myelitis and causes  severe functional impairment.10 It predominantly affects women than men in a 3:1 ratio.11 The median age of onset is late 30s, however cases in pediatric population has been described.11 A study which include 76 patients with diagnosis of LETM, conducted in the United Kingdom in which 53 patients of LETM were diagnosed with NMOSD, 5 had ADEM, 5 had idiopathic LETM, 4 had infectious causes, 3 had MS, 2 had other inflammatory diseases etc.as in our study which show NMOSD is common cause of LETM.21NMOSD should be suspected in every case when patient presents with unilateral, bilateral optic neuritis or vision of light perception or worse.12 Prevention of further attacks is a crucial importance in the management of this clinically varied inflammatory disease.13 Treatment with immunosuppressive drugs are recommended to prevent relapses.13Classic multiple sclerosis (CMS) is a chronic degenerative, inflammatory disease that also have female predominance like NMOSD. CMS can present with isolated transverse myelitis, spinal cord lesions extending less than 2 vertebral segments, abnormal imaging, and positive oligoclonal bands in CSF.14 Optica spinal multiple sclerosis is a variant of multiple sclerosis.15 In our study there was 6.3% subjects had optica-spinal multiple sclerosis.  A limitation of this study was few number of cases and lack of anti-aquaporin 4 antibodies testing for confirmation of diagnosis in every case due to non-affordability and only admitted /hospitalized patient with history of weakness were included.CONCLUSIONLongitudinally extensive transverse myelitis (LETM) is not an uncommon disease, however, it remains under-diagnosed in our clinical practice due to either lack or cost of diagnostic facilities. LETM is common presentation of Neuro Myelitis Optica Spectrum disorder (NMOSD) followed by multiple sclerosis, ADEM and other autoimmune diseases etc. Thus clinically important as it results in severe morbidity and patients are at risk of further attacks. Early diagnosis and confirmation of the underlying cause is vital in order to initiate appropriate therapy and favorable outcome. LIMITATIONS OF STUDYNo study from Pakistan about Longitudinally extensive transverse myelitis.No International Study about exact frequency and causes of longitudinally extensive transverse myelitis.Limited data available.REFERENCES1. Krishnan C, Kaplin AI, Deshpande DM, Pardo CA,   Kerr DA. Transverse myelitis: pathogenesis,    diagnosis and treatment. Front Biosci.    2004;9:1483-99.2. Besteiro B, Guimarães J. Review of the Etiological   Causes and Diagnosis of Myelitis and Its Medical   Orientation Protocol. J Neurol Neurophysiol    2013;8:4153. Jacob A, Weinshenker BG. An approach to the   diagnosis of acute transverse myelitis. Semin    Neurol. 2008;28(1):105-20.4. Tosi L, Rigoli G, Beltramello A. Fibrocartilaginous   embolism of the spinal cord: a clinical and    pathogenetic reconsideration. J Neurol    Neuro-Surg & Psy. 2013;60(1):55-60.5. Young V, Quaghebeur G Transverse Myelitis and   Neuromyelitis Optica Spectrum Disorders. Semin   Ultrasound CT MR. 2016;37(5):384-956. Huang LK, Chung CC, Chen BZ, Chi NF, Hu CJ.   Systemic lupus erythematosus presented as    extensive longitudinal myelitis. Acta Neurol Taiwan   2013;22: 67-71.7. Trebst C, Raab P, Voss EV, Rommer P, Abu-Mughe isib M, Zettl UK, Stangel M. Longitudinal extensive  transverse myelitis—it's not all neuromyelitis    optica. Nature Reviews Neurol. 2011;7(12):688.8. Pandit L. Transverse myelitis spectrum disorders.   Neurol India. 2009;57(2):126-33.9. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinet  ti CF, Weinshenker BG. Revised diagnostic criteria   for neuromyelitis optica. Neurol.     2006;66:1485–1489.10. Sánchez AM, Posada LMG, Toscano CAO, López   AL.  Diagnostic approach to myelopathies. Rev   Colomb Radiol 2011;22:1-21.11. Trebst C, Jarius S, Berthele A, Paul F, Schippling   S, Wildemann B, Borisow N, Kleiter I, Aktas O,   Kümpfel T, Neuromyelitis Optica Study Group.   Update on the diagnosis and treatment of neuro  myelitis optica: recommendations of the Neuromy  elitis Optica Study Group (NEMOS). J Neurol.    2014;261(1):1-6.3 8P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 5  ( 4 )  O C T O B E R - D E C E M B E R  2 0 2 012. Lana-Peixoto MA. Devic's neuromyelitis optica: a   critical review. Arq Neuropsiquiatr.     2008;66(1):120-38.13. Nandhagopal R, Al-Asmi A, Gujjar AR. Neuromyeli  tis optica: an overview. Postgrad Med J.    2010;86(1013):153-9.14. Sigliti-Henrietta Pelidou, Sotirios Giannopoulos,   Sotiria Tzavidi, Georgios Lagos, and Athanassios P   Kyritsis Multiple sclerosis presented as clinically   isolated syndrome: the need for early diagnosis   and treatment. Ther Clin Risk Manag.    2008;4(3):627–630.15. Kira J. Neuromyelitis optica and optico spinal multiple sclerosis: Mechanisms and pathogenesis. Pathophysiol. 2011;18(1):69-79.16. E. Carnero Contentti, J.P. Hryb, F. Leguizamón, J.L. Di Pace, J. Celso, E. Knorre, M.B. Perassolo.Differ  ential diagnosis and prognosis for longitudinally   extensive myelitis in Buenos Aires, Argentina.Neu  rología,2017: 32(2) : 99-10517. Catalina COCLITUa, Athena MERGEANIa, Teodora   PARVUa, Octaviana RUSUa, Andrei CIOBOTARUa,   Ovidiu BAJENARUa,b, Florina ANTOCHIa. An    Uncommon Cause of Longitudinally Extensive    Transverse Myelitis. MAEDICA – a Journal of    Clinical Medicine 2016; 11(3):245-24918. Wingerchuk DM, Banwell B, Bennett JL et al.    International consensus diagnostic criteria for    neuromyelitis optica spectrum disorders.  Neurology. 201519. 2017 McDonald MS Diagnostic Criteria (Thomp  son et al., 2017)20. Samira Apostolos-Pereira, Paulo Marchiori,    Alessandra Dellavance, Leandro Lucato, Frederico   Jorge, Renata Simm, Douglas Sato, Dagoberto   Callegaro, Differential Diagnosis of Longitudinally   Extensive Transverse Myelitis, Neurology Feb    2013, 80 (7 Supplement) P02.10921. J. Kitley, M.I. Leite, W. Küker, G. Quaghebeur, J.   George, P. Waters, et al.Longitudinally extensive   transverse myelitis with and without aquaporin 4   antibodies ,JAMA Neurol, 70 (2013), pp.    1375-13813 9P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 5  ( 4 )  O C T O B E R - D E C E M B E R  2 0 2 0Conflict of interest: Author declares no conflict of interest.Funding disclosure: NilAuthor’s contribution:Safia Bano; concept, data collection, data analysis, manuscript writing, manuscript reviewMuhammad Ather Javed; data collection, data analysis, manuscript writing, manuscript reviewAhsan Numan; data collection, data analysis, manuscript writing, manuscript review4 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S V O L .  1 5  ( 4 )  O C T O B E R - D E C E M B E R  2 0 2 0

Longitudinally Extensive Transverse Myelitis and Neuromyelitis Optica spectrum Disorder: A Tertiary Care Centre Experience

https://ecommons.aku.edu/cgi/viewcontent.cgi?article=1501&amp;context=pjns

Longitudinally Extensive Transverse Myelitis and Neuromyelitis Optica spectrum Disorder: A Tertiary Care Centre Experience

Abstract

Similar works

Full text

Available Versions

eCommons@AKU