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Published on: May 7, 2019
An Analysis of Clinico-radiological Features and Outcome in Patients with Longitudinally Extensive Transverse
Karthik Thamarai Kannan1, Madhavi Karri1, Balakrishnan Ramasamy1
1Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India.
Background:
Longitudinally extensive transverse myelitis (LETM) is an immune-mediated neurological disorder affecting the spinal cord. It may be associated with the demyelination of the brain and optic nerves. Clinical features, radiological picture, and treatment outcomes vary depending on the etiology.
Objectives:
To assess different etiologies of LETM and analyze their differences in clinicoradiological features and treatment outcome.
Materials And Methods:
It is a single-center retrospective cross-sectional observational study. A total of 42 patients presented with LETM to our center for over 10 years (2010-2019) were included in this study. Their clinical, radiological, CSF findings, treatment, and neurological status (at nadir and 6 months) assessed by the EDMUS scoring system were compared and presented in this study.
Results:
In this study, 80% of patients were females, with the highest female predominance noted in NMOSD (87%). Among various etiologies, 16 patients had NMOSD, seven were idiopathic, five had connective tissue disease, and four were due to infective etiology. Bladder involvement was seen predominantly in patients with infective and systemic causes, whereas respiratory muscle involvement was seen predominantly with infective etiology. More than six-segment involvement was seen predominantly in idiopathic cases. All Ro-52 positive patients had relapses. NMOSD had a better neurological outcome than other etiology.
Conclusions:
NMOSD is the most common cause of LETM, with a good neurological outcome at 6 months, while infective etiology had a poor neurological recovery. NMOSD with Ro-52 antibody positivity had relapses despite being on immunosuppressant therapy.
Insights
Longitudinally extensive transverse myelitis (LETM) is often caused by NMOSD, which typically shows good recovery. However, infective causes lead to poor neurological outcomes, and Ro-52 positivity indicates a risk of relapse.
Area of Science:
- Neurology
- Immunology
- Neuroimmunology
Background:
- Longitudinally extensive transverse myelitis (LETM) is an immune-mediated spinal cord disorder.
- LETM can involve brain and optic nerve demyelination, with varied clinical, radiological, and treatment outcomes based on cause.
- Understanding the diverse etiologies of LETM is crucial for effective management.
Purpose of the Study:
- To investigate the various causes of LETM.
- To compare clinicoradiological features and treatment outcomes across different LETM etiologies.
- To identify prognostic factors for neurological recovery in LETM patients.
Main Methods:
- Retrospective, single-center observational study of 42 LETM patients (2010-2019).
- Analysis of clinical, radiological, CSF findings, treatment, and neurological status (EDMUS scoring).
- Comparison of outcomes based on etiology, including Neuromyelitis Optica Spectrum Disorder (NMOSD), idiopathic, connective tissue disease, and infective causes.
Main Results:
- Female predominance observed (80%), highest in NMOSD (87%).
- NMOSD was the most frequent cause (16 patients), followed by idiopathic (7), connective tissue disease (5), and infective (4).
- Bladder/respiratory involvement linked to infective/systemic causes; extensive segment involvement to idiopathic cases. Ro-52 positivity correlated with relapses. NMOSD showed better outcomes.
Conclusions:
- NMOSD is the leading cause of LETM, generally associated with good 6-month neurological recovery.
- Infective etiologies of LETM are linked to poor neurological recovery.
- NMOSD patients with Ro-52 antibodies experienced relapses despite immunosuppression, highlighting a need for targeted therapies.
