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|Year : 2021 | Volume
| Issue : 5 | Page : 1412-1413
Longitudinally Extensive Transverse Myelitis in Highly Active Relapsing-remitting Multiple Sclerosis
Chen Fei Ng, Rabani Remli, Hui Jan Tan
Department of Medicine, Neurology Unit, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Cheras, Kuala Lumpur, Malaysia
|Date of Submission||18-Dec-2019|
|Date of Decision||12-Mar-2020|
|Date of Acceptance||11-Oct-2020|
|Date of Web Publication||30-Oct-2021|
Chen Fei Ng
Department of Medicine, Neurology Unit, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Cheras, Kuala Lumpur
Source of Support: None, Conflict of Interest: None
Transverse myelitis in multiple sclerosis is typically a short cord lesion with patchy distribution. Rarely, longitudinally extensive transverse myelitis can be seen in those with highly active disease or frequent relapses. The recognition of this uncommon phenotype in multiple sclerosis is important as the treatment is largely different from other demyelinating diseases. We describe a patient with highly active relapsing-remitting multiple sclerosis on interferon beta-1a who developed LETM after multiple relapses.
Keywords: Longitudinally extensive transverse myelitis, multiple sclerosis, myelin oligodendrocyte glycoprotein antibody disease, neuromyelitis optica spectrum disorder
Key Message: Longitudinally extensive transverse myelitis can occasionally occur in multiple sclerosis. This possibility should always be considered in patients with seronegative neuromyelitis optica spectrum disorder who do not response to treatment.
|How to cite this article:|
Ng CF, Remli R, Tan HJ. Longitudinally Extensive Transverse Myelitis in Highly Active Relapsing-remitting Multiple Sclerosis. Neurol India 2021;69:1412-3
Multiple sclerosis (MS) is an immune-mediated inflammatory demyelinating disorder of the central nervous system. Transverse myelitis is one of the main presentations and the lesions are typically patchy, short and asymmetrical. Longitudinally extensive transverse myelitis (LETM) involving 3 or more spinal cord segments is commonly seen in neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOG-AD). Here, we describe a patient with highly active relapsing-remitting multiple sclerosis who developed LETM after multiple relapses.
| » Case Summary|| |
A 31-year-old woman with 10-year history of MS initially presented with multiple episodes of supratentorial syndrome, left optic neuritis, and brainstem syndrome within one year. The attacks occurred in isolation in a relapsing and remitting pattern. She was born to non-consanguineous parents of Malay ethnicity. Clinically, she had patchy sensory loss in the lower limbs and reduced visual acuity on the left side. Magnetic resonance imaging (MRI) of the brain showed multiple hyperintense T2-weighted and gadolinium-enhancing lesions in the periventricular, juxtacortical and left pontine regions. Visual evoked potential was abnormal with prolonged P100 on the left side. Oligoclonal bands were absent in the cerebrospinal fluid. The diagnosis of relapsing-remitting multiple sclerosis (RRMS) was made based on her typical presentation and characteristic neuroimaging fulfilling the McDonald criteria 2010. She was treated with subcutaneous interferon beta-1a thrice weekly. Her disease was quiescent until three years later, she developed bilateral upper limb numbness. MRI of the brain and spine revealed multiple new lesions [Figure 1] and [Figure 2]. Treatment escalation to other disease-modifying therapy was discussed; however, she opted to remain on interferon. Unfortunately, she suffered three further attacks of partial myelitis in the next two years requiring hospitalization for intravenous methylprednisolone. MRI spine was repeated and showed long segment extension of spinal cord lesions from C2 to T2 level [Figure 3]. Serum anti-aquaporin 4 (AQP4) antibody was negative. Treatment was subsequently switched over from interferon to fingolimod 0.5 mg daily. She was closely monitored and has remained without clinical or radiological relapse for the last six years. The Kurtzke Expanded Disability Status Scale (EDSS) was zero at the latest review.
|Figure 1: T2-weighted FLAIR sequence of MRI brain. There were multiple hyperintense lesions in the periventricular regions (a), with typical Dawson's finger appearance (b)|
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|Figure 2: T2-weighted sequence of MRI brain, sagittal (a) and axial (b) views. Multiple short segment hyperintensity within C2 and T1 spinal level|
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|Figure 3: T2-weighted FLAIR sequence of MRI brain, sagittal (a) and axial (b) views. Repeated imaging showed longitudinally extensive hyperintense lesions which span from C2 to T2 level with cord atrophy|
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| » Discussion|| |
The differential diagnosis of acute inflammatory transverse myelitis is broad and can be categorized into demyelination, infection and inflammatory disorders. The common causes for demyelinating transverse myelitis include MS, NMOSD and MOG-AD. MS-associated transverse myelitis is usually a partial myelitis. The lesions are short (less than three spinal segments), peripherally located with predilection for lateral and posterior funiculi. Whereas in NMOSD-associated LETM, it spans over three or more contiguous spinal segment and predominantly involves central gray matter due to the abundance of AQP4. The presence of LETM is often considered a sine qua non for NMOSD. However, it can also occur in MS at a frequency of 2.3%, and up to 17% in pediatric MS., Particularly in aggressive MS, multiple short cord lesions following recurrent relapses may coalesce to manifest as LETM.
The typical brain imaging and AQP4 antibody negativity in this patient rendered the diagnosis of NMOSD unlikely. Treatment escalation to oral fingolimod was decided on the basis of higher potency, easier route of administration and patient's preference. Fingolimod is a sphingosine-1-phosphate–receptor modulator that prevents lymphocyte egress from lymph nodes. It has been shown to be superior than intramuscular interferon beta-1a with regard to relapse rate and MRI outcomes in relapsing-remitting MS. Of note, first dose-related bradycardia and atrioventricular block, herpes virus infection, macular edema and skin neoplasm had been observed. Thus, detailed cardiovascular, dermatology and ophthalmology screening, and serology for varicella-zoster virus should be performed prior to initiation of therapy.
The clinical diagnosis of MS has greatly improved with 2017 revision of McDonald criteria. Nevertheless, atypical feature such as LETM may mimic NMOSD and lead to inaccurate treatment. Therefore, it is crucial to always consider the possibility of MS in patients with LETM especially in seronegative NMOSD.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]