scholarly journals Role of Plasma Exchange in a Steroid- and IVIG-Refractory Patient with Acute Disseminated Encephalomyelitis: A Case Report

2020 ◽  
Vol 47 (5) ◽  
pp. 420-423 ◽  
Author(s):  
Parmatma Prasad Tripathi ◽  
Rekha Hans ◽  
Ratti Ram Sharma ◽  
Divjot Singh Lamba ◽  
Preeti Paul ◽  
...  

Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease usually affecting children and is treated with high-dose steroid therapy. Case Report: An 8-year boy presented with limbs weakness and complete loss of vision and was resistant to steroid therapy. He was further treated with plasma exchange and showed full recovery from the neurological deficit. Conclusion: Therapeutic plasma exchange appears to be effective in ADEM patients in reversing the neuropathological process especially refractory to steroids and intravenous immunoglobulin.

2014 ◽  
Vol 9 (4) ◽  
pp. 55-58
Author(s):  
R Adhikari ◽  
A Tayal ◽  
PK Chhetri ◽  
B Pokhrel

The involvement of central nervous system in children with typhoid fever is common. Acute disseminated encephalomyelitis is a rare immune mediated and demyelinating disease of the central nervous system that usually affects children. We report a 7-year-old child with typhoid fever who developed acute cerebellar syndrome due to acute disseminated encephalomyelitis.Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 55-58 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10237


Author(s):  
LJ Baxter ◽  
S Chen ◽  
JM Burton

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.


1991 ◽  
Vol 4 (6) ◽  
pp. xxxi
Author(s):  
E.Díez Tejedor ◽  
J. Tejada ◽  
A. Frank ◽  
M. Lara ◽  
P. Barreiro

2017 ◽  
Vol 46 (3) ◽  
pp. 695-701 ◽  
Author(s):  
Yukihiko Yasui ◽  
David A. Hart ◽  
Norihiko Sugita ◽  
Ryota Chijimatsu ◽  
Kota Koizumi ◽  
...  

Background: The use of mesenchymal stem cells from various tissue sources to repair injured tissues has been explored over the past decade in large preclinical models and is now moving into the clinic. Purpose: To report the case of a patient who exhibited compromised mesenchymal stem cell (MSC) function shortly after use of high-dose steroid to treat Bell’s palsy, who recovered 7 weeks after therapy. Study Design: Case report and controlled laboratory study. Methods: A patient enrolled in a first-in-human clinical trial for autologous implantation of a scaffold-free tissue engineered construct (TEC) derived from synovial MSCs for chondral lesion repair had a week of high-dose steroid therapy for Bell’s palsy. Synovial tissue was harvested for MSC preparation after a 3-week recovery period and again at 7 weeks after therapy. Results: The MSC proliferation rates and cell surface marker expression profiles from the 3-week sample met conditions for further processing. However, the cells failed to generate a functional TEC. In contrast, MSCs harvested at 7 weeks after steroid therapy were functional in this regard. Further in vitro studies with MSCs and steroids indicated that the effect of in vivo steroids was likely a direct effect of the drug on the MSCs. Conclusion: This case suggests that MSCs are transiently compromised after high-dose steroid therapy and that careful consideration regarding timing of MSC harvest is critical. Clinical Relevance: The drug profiles of MSC donors and recipients must be carefully monitored to optimize opportunities to successfully repair damaged tissues.


2020 ◽  
Vol 21 (4) ◽  
pp. 178-186
Author(s):  
Dariusz Dziubek ◽  
◽  
Mateusz Rajchel ◽  
Barbara Stańko-Kałamarz ◽  
Mirosław Dziki

The paper presents a case of a 61-year-old patient with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), along with a detailed description of the diagnostic and therapeutic process, as well as radiological records reflecting the evolution of lesions depending on the steroid therapy used. We also refer to the latest literature reports on the aetiology, pathogenesis, diagnosis and treatment of this clinical entity, including its potential relationship with lymphoproliferative and lymphohistiocytic processes. Furthermore, we present a spectrum of diseases that can clinically and radiologically mimic CLIPPERS (CLIPPERS-mimics), along with the proposed differential diagnosis. Finally, the paper presents the recommended treatment strategy for both acute disease and a maintenance therapy, with particular emphasis on the role of steroid therapy.


2021 ◽  
Vol 8 (10) ◽  
pp. 21-27
Author(s):  
Kenia` Izzawa ◽  
Putri Irsalina ◽  
Yudha Haryono

Background: Acute disseminated encephalomyelitis (ADEM) is a demielination disease of the central nervous system (CNS) in response to previous infection or immunizations that occur acute, monofasic. ADEM generally occurs in children and young adults, rarely in middle-aged or elderly. Case report: We present a woman 46 years old with chief complaint of headache since 7 months ago, weakness of both legs, sudden loss of vision in both eyes since 3 days after weakness of both legs. There was no history of infection or previous vaccination. Brain and whole spine MRI were performed. Initial therapy methylprednisolone does not show improvement, then continued with administration of intravenous immunoglobulin. After 6 months, clinical improvements were obtained and no new lesions of the imaging. Conclusion: In this case report it is presented a case of ADEM in adult without a history of infection who recovered after intravenous immunoglobulin. Then that is still the current question, whether ADEM in the adult can be occur without preceded infection history? Keywords: ADEM, adult woman, history of infection.


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