Different clinical characteristics of longitudinally extensive transverse myelitis with and without connective tissue di
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ORIGINAL ARTICLE
Different clinical characteristics of longitudinally extensive transverse myelitis with and without connective tissue disorders: a single-center retrospective study Qiu-Xia Zhang 1 & Chen-Na Huang 1 & Lin-Jie Zhang 1 & Ming Yi 1 & Nan Wang 1 & Shu-Min Jiang 1 & Li-Sha Chou 1 & Sheng-Hui Chang 1 & Ting Li 1 & Li Yang 1 Received: 23 December 2019 / Accepted: 13 April 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Background and objective Autoimmune longitudinal extensive transverse myelitis (LETM) is often combined with connective tissue disorders (CTD). The purpose of this study was to compare the clinical characteristics of autoimmune LETM with and without CTD. Methods Ninety-two patients diagnosed with autoimmune LETM were enrolled from our clinical database and divided into two groups depending on whether they had a concomitant diagnosis of CTD. Differences in clinical, serological, and imaging characteristics between the two groups were evaluated and compared. Results Fifty-nine LETM patients without CTD and 33 LETM patients with CTD were included. LETM patients with CTD had higher Kurtzke Expanded Disability Status Scale at nadir and more severe sensory dysfunction (p < 0.05) than those without CTD. It was also found that LETM patients with CTD, compared with those without CTD, had elevated levels of immune inflammation markers such as IgG, IgA, and globulins (p < 0.05). These abovementioned characteristics were more prominent in patients with aquaporin-4 antibodies (AQP4-ab) than in those without them. In addition, the most common type of CTD in LETM was Sjögren syndrome (SS), which was usually diagnosed at the time of LETM or later. Conclusion LETM patients with CTD, especially those with AQP4-ab, had greater sensory dysfunction and higher levels of inflammatory markers than did LETM patients without CTD. Multicenter cooperation and long-term follow-up are necessary to further study the inherent implications and prognosis of the disease. Keywords Longitudinally extensive transverse myelitis . Connective tissue disorders . Sjögren syndrome . Aquaporin-4 antibodies
Introduction Longitudinally extensive transverse myelitis (LETM) is defined as transverse myelitis in which the spinal lesion spans three or more vertebral segments [1, 2]. The typical clinical manifestation of LETM is dysfunction below the affected spinal cord segment; this dysfunction may include limb numbness or paresthesia, limb weakness, urinary retention, and rectal dysfunction. The underlying etiologies
* Li Yang [email protected] 1
Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, 154 Anshan Road, Heping District, Tianjin 300052, China
of LETM are diverse, with neuromyelitis optica spectrum disorders (NMOSD) being the common cause. Acute myelitis is listed as one of the six core clinical characteristics of NMOSD, and LETM is considered to be an additional magnetic resonance imaging (MRI) requirement in patients who are negative for aquaporin-4 antib
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