Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis

2018 ◽  
Vol 37 (10) ◽  
pp. 2899-2905
Author(s):  
Amanda Mary Scott ◽  
Janeth Yinh ◽  
Timothy McAlindon ◽  
Robert Kalish
2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1167.2-1168
Author(s):  
P. Korsten ◽  
M. Plüß ◽  
S. Glaubitz ◽  
A. Jambus ◽  
R. Vasko ◽  
...  

Background:Systemic lupus erythematosus (SLE) can affect almost any organ system. Nevertheless, Lupus nephritis and neuropsychiatric manifestations (NPSLE) are associated with increased mortality (1). Therapeutic options include glucocorticoids, often pulse methylprednisolone (MP), and other immunosuppressive therapies. In refractory cases, therapeutic plasma exchange, rituximab, or intravenous immunoglobulins are often used (2). However, an optimal therapeutic strategy has not been established because NPSLE is an exclusion criterion in most clinical trials. In addition, NPSLE can present with a broad spectrum of manifestations ranging from cognitive dysfunction to severe and life-threatening disease with choreoathetosis or transverse myelitis (TM). In primary Sjögren’s syndrome (pSS), neurological manifestations most often include peripheral neuropathies, but TM has also been reported.Objectives:To analyze the clinical presentation and outcomes after treatment in severe, life-threatening NPSLE.Methods:We retrospectively analyzed clinical, laboratory, and imaging features in severe NPSLE manifestations in SLE and pSS patients at two tertiary academic centers (University Medical Center Göttingen, Germany, and ASST Spedali Civili Brescia, Italy) with a high volume of SLE patients. Severe NPSLE was defined as either severe movement disorder or extensive tetra- or paraplegia secondary to (longitudinally extensive) transverse myelitis.Results:Our retrospective chart review resulted in seven patients fulfilling the inclusion criteria (six with SLE and 1 with pSS). Of these, five were females (71.4%). Median age was 26 (16-55) years. Three were of Asian origin, four were of European descent. Median disease duration was 15 (2-228) months. Three patients presented with severe choreoathetosis, all had positive ANA, anti-dsDNA antibodies (abs), and complement consumption. Of note, all three had at least one positive antiphospholipid antibody (APLA). All patients received IV MP 1g x 3 and mycophenolate mofetil and achieved complete remission. Of the four patients with longitudinally extensive TM, all were ANA positive, only two had anti-dsDNA abs. None of them had APLA, and only one tested positive for anti-aquaporine-4 abs. Of all patients, only one had positive ribosomal P-abs. Patients with TM received IV MP 1g x 5 and either RTX (4 cycles with 375 mg/m2 or IVIg 0.4 g/kg/d x 5). All four TM patients improved; two improved markedly, two only moderately with residual deficits as assessed by EDMUS-grading scale and functional independence measure.Conclusion:Severe NPSLE, defined as choreoathetosis or TM require intensive treatment. While the former patients achieved complete remission, two of four patients with TM only achieved partial remission. Our data support the use of early and aggressive immunosuppressive therapy. Nevertheless, therapy for TM in the context remains insufficient and should be assessed in a controlled clinical trial setting.References:[1]Monahan RC, et al. Mortality in patients with systemic lupus erythematosus and neuropsychiatric involvement: A retrospective analysis from a tertiary referral center in the Netherlands. Lupus. 2020 Dec;29(14):1892–901.[2]Papachristos DA, et al. Management of inflammatory neurologic and psychiatric manifestations of systemic lupus erythematosus: A systematic review. Semin Arthritis Rheum. 2020 Dec 17;51(1):49–71.Disclosure of Interests:PETER KORSTEN Consultant of: PK has received honoraria by Abbvie, Bristol-Myers-Squibb, Chugai, Gilead, Glaxo Smith Kline, Janssen-Cilag, Pfizer, and Sanofi-Aventis, all unrelated to this study., Grant/research support from: PK has received research grants from GSK, unrelated to this study., Marlene Plüß: None declared, Stefanie Glaubitz: None declared, Ala Jambus: None declared, Radovan Vasko: None declared, Bettina Meike Göricke: None declared, Silvia Piantoni: None declared


2021 ◽  
Vol 74 (7-8) ◽  
pp. 273-276
Author(s):  
Nazan Şimşek Erdem ◽  
Seden Demirci ◽  
Tuğba Özel ◽  
Khalida Mamadova ◽  
Kamil Karaali ◽  
...  

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.


2015 ◽  
Vol 116 (3) ◽  
pp. 395-397 ◽  
Author(s):  
Giuliana Galassi ◽  
Alessandra Ariatti ◽  
Raffaella Rovati ◽  
Maurilio Genovese ◽  
Francesco Rivasi

2018 ◽  
Vol 21 (2) ◽  
pp. 161 ◽  
Author(s):  
Angamuthu Kanikannan ◽  
Yareeda Sireesha ◽  
MeghaS Uppin ◽  
Komal Bohra ◽  
Rajesh Alugolu ◽  
...  

The Clinician ◽  
2020 ◽  
Vol 13 (3-4) ◽  
pp. 78-83
Author(s):  
M. N. Guseynova ◽  
L. G. Zaslavskii ◽  
E. A. Skornyakova

Objective: description of a clinical case of the development of longitudinally spread transverse myelitis in a young woman suffering from nonHodgkin’s lymphoma (follicular, 1 degree, IIIA stage, complete remission from February 2017). This is a rare, significant case in clinical practice for both residents and neurologists, and the case is under discussion. It requires certain scientific studies in the search for specific antionkoneural antibodies or a more detailed study of the already known. It is necessary to introduce these studies into laboratory practice in Russia, which would help to accelerate the diagnosis of paraneoplastic myelitis.Materials and methods. Patient B., 30 years old, has been suffering non-Hodgkin’s lymphoma since February 2016 (follicular, I degree, IIIA stage, complete remission from February 2017), receives infusions of Rituximab with a dose of 600 mg once in 2 months, the subacute developed pronounced lower spastic paraparesis. In November 2018, suddenly, against the background of complete well-being, weakness and numbness of the legs appeared. The patient asked for help at the Center for Multiple Sclerosis on 11.17.2018, then she was hospitalized and examined. Patient B. received a pulse therapy with methylprednisolone 1000 mg intravenously № 5. Discharged with positive dynamics with a diagnosis of Demyelinating CNS disease, unspecified. Acute protracted transverse myelitis (LETM), EDSS = 3.5 points. On December 13, due to the increase in spacity and leg weakness, she was urgently hospitalized in the neurological department of the Clinical Hospital.Results. After the completion of laboratory and instrumental examination, the condition was regardedby us as paraneoplastic myelitis. Relapse of lymphoma was ruled out. The treatment was carried out: pulse therapy with methylprednisolone, Plasmapheresis and intravenous administration of IgG (Privigen) with a distinct positive dynamics.Conclusion. This clinical observation allowed us to become more familiar with paraneoplastic syndromes and, in particular, with paraneoplastic myelitis. In the clinical situation previously described, we encountered a number of features: longitudinally extensive transverse myelitis developed without relapse of lymphoma, progressed rapidly, there was no response to treatment (glucocorticosteroids, plasmapheresis, Rituximab). All these signs and MRI data of the cervical and thoracic spine (a symmetric hyper-intensive signal from level CII to level ThXI, mainly involving the central spinal cord T2 MRI mode) testified in favor of the paraneoplastic etiology of longitudinally extensive transverse myelitis.


2013 ◽  
Vol 263 (1-2) ◽  
pp. 145-147 ◽  
Author(s):  
Marina Frasquet ◽  
Luis Bataller ◽  
Estefanía Torres-Vega ◽  
María Durán-Moreno ◽  
José M. García-Verdugo ◽  
...  

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