Plasma exchange in inflammatory demyelinating disorders of the central nervous system: reasonable use in the clinical practice

Queiroz, André Luiz Guimarães de; Neto, Herval Ribeiro Soares; Kobayashi, Thiago Taya; Silva, Sônia Maria Cesar de Azevedo

doi:10.1055/s-0042-1758447

Cited by 4 publications

(5 citation statements)

References 56 publications

(169 reference statements)

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“…Therapeutic plasma exchange serves as an extracorporeal blood purification technique used in treating various medical conditions [ 2 , 6 ]. This process involves separating plasma from whole blood and replacing it with either plasma or albumin to eliminate undesirable plasma components.…”

Section: Discussionmentioning

confidence: 99%

“…Therapeutic plasma exchange removes pathogenic factors in the serum, resulting in improvements in muscle strength, accelerated recovery, and reduced mechanical ventilation requirements [ 6 ]. A meta-analysis of six trials, which included 649 GBS patients (some ≥10 years of age), revealed the superiority of TPE over supportive care [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

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Plasma Exchange in Pediatric Neurology Patients: A Single-Center Experience

Aljezani,

Althubaiti,

Alhamed

et al. 2024

Cureus

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Background: Therapeutic plasma exchange (TPE) is a procedure involving the filtration of a patient's plasma to eliminate pathogenic components or address deficiencies. This technique finds varied indications in the pediatric age group, particularly in neuroinflammatory diseases. Objectives: The objective of this study is to delve into our local experience with TPE, focusing on indications, outcomes, and complications among children with neurological diseases at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. Results: Conducted at the pediatric department of KAUH in Jeddah from November 2008 to July 2023, this retrospective cohort study examined 15 patients, revealing a notable male predominance with 12 male patients (80%) and three female patients (20%). About two-thirds of patients exhibited an average illness severity, with a Glasgow Coma Scale (GCS) score of 10.7 and an Expanded Disability Status Scale (EDSS) score of 4.8. The median length of hospital stay was 23 days, and in the pediatric intensive care unit (PICU), it was 8.5 days. Presenting symptoms included limb weakness (n = 6), loss of consciousness (n = 3), dysphagia (n = 3), photophobia (n = 1), and ascending paralysis (n = 1). The TPE was performed for Guillain-Barré syndrome (GBS) (n = 7), myasthenia gravis (MG) (n = 3), transverse myelitis (TM) (n = 2), neuromyelitis optica (NMO) (n = 2), and systemic lupus erythematosus (SLE) cerebritis (n = 1). Twelve patients were admitted to the PICU, and mechanical ventilation was required for 10 patients. In magnetic resonance imaging (MRI) findings, abnormalities were observed in 10 cases, while the remaining five either had normal results or did not undergo MRI. Most patients required five sessions of TPE (n = 7). The median age at the initiation of TPE was 13 years. Twelve patients improved with TPE treatment, while three did not. Complications observed during and following TPE included fever (n = 5), electrolyte disturbance (n = 5), hypotension (n = 3), hypocalcemia (n = 2), bradycardia (n = 2), vomiting (n = 1), tachycardia (n = 1), eye rash (n = 1), infection (n = 1), and bleeding originating from the TPE procedure site (n = 1). Conclusion: In conclusion, our study underscores the significance of TPE as a therapeutic modality, emphasizing the imperative for ongoing research to fully exploit its potential across diverse medical contexts for enhancing patient care. Our findings, consistent with prior research, reveal plasma exchange's (PLEX's) wide-ranging applications and complications in neurological disorders.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Plasma Exchange in Pediatric Neurology Patients: A Single-Center Experience

Aljezani,

Althubaiti,

Alhamed

et al. 2024

Cureus

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“…A typical PLEX cycle lasts two to three weeks and comprises five to seven cycles. However, this can vary as a meta-analysis by Queiroz et al mentioned in clinical practice that cycles can range from 5 to 15 for treatment response [ 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

Neuromyelitis Optica Spectrum Disorder

Cazzaniga,

Jara Silva,

Quinonez

et al. 2023

Cureus

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Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune condition characterized by recurrent episodes of optic neuritis (ON) and transverse myelitis. This case report aims to highlight the importance of considering atypical presentations of NMOSD when confronted with MRI-detected Wernicke's encephalopathy. The primary target in NMOSD is the aquaporin-4 (AQP4) protein, predominantly located on astrocyte surfaces. Antibodies binding to AQP4 can lead to astrocyte dysfunction and damage, contributing to NMOSD's distinctive pathology. The associated immune response and inflammation can cause secondary harm to various components of the central nervous system, including oligodendrocytes and neuronal axons. This inflammatory process results in perivascular demyelination and axonal injury, further aggravating neurological deficits in NMOSD. In this case, we present a 39-year-old female with no prior medical or surgical history who sought medical attention due to a three-week history of progressive eyelid heaviness and somnolence. NMOSD is an autoimmune condition primarily targeting the AQP4 protein, resulting in recurrent ON and transverse myelitis. The patient was initially misdiagnosed with myasthenia gravis due to somnolence and ptosis. Due to concerns about myasthenia gravis due to diffuse fatigue and bilateral ptosis, the patient was initially treated with intravenous immunoglobulin (IVIG) and admitted to the neurology service. On the first day of her hospitalization, MRI with and without contrast revealed extensive, non-enhancing T2-weighted-fluid-attenuated inversion recovery (T2-FLAIR) hyperintensities surrounding the third ventricle and affecting the periaqueductal grey, medial thalami, and mammillary bodies. There was also an interval increase in T2-FLAIR hyperintensity within the right medial temporal lobe, extending more posteriorly and inferiorly, abutting the temporal horn. Subsequent CSF encephalitis panel results showed positive West Nile virus (WNV) IgG but negative WNV IgM, and AQP4 antibodies were positive. Given the high specificity of AQP4 antibodies, the patient was diagnosed with neuromyelitis optica (NMO) encephalitis. This case underscores the importance of considering atypical presentations of NMO when confronted with MRI-detected Wernicke's encephalopathy. Since our patient primarily displayed somnolence and eye-related symptoms, neither NMO nor Wernicke's encephalopathy were initially considered in the differential diagnosis. Furthermore, despite MRI findings suggestive of Wernicke's encephalopathy, it was considered less likely due to the absence of thiamine deficiency and consistent denials by family members regarding alcohol use, gastrointestinal issues, or inadequate oral intake. This case underscores the importance of considering NMOSD in patients with atypical symptoms, even when initial presentations suggest other conditions. Timely diagnosis is crucial to prevent mismanagement and improve patient outcomes. Clinicians should maintain a high level of su...

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“…An important limitation in taking drugs with proven anti-inflammatory and antioxidant effects is their limited therapeutic range and safety profile. For example, corticosteroids are established as first-line drugs for the treatment of acute demyelinating attacks/exacerbations of MS [17,143,179,180]. Antioxidant effects have been established in MS patients, such as increased SOD activity and decreased ROS and MDA levels after methylprednisolone treatment [47,181,182].…”

Section: Discussionmentioning

confidence: 99%

“…Antioxidant effects have been established in MS patients, such as increased SOD activity and decreased ROS and MDA levels after methylprednisolone treatment [47,181,182]. However, if patients do not respond to corticosteroid therapy, which occurs in 20-25% of MS cases, a second corticosteroid pulse therapy in combination with TPE is recommended after an interval of 10-14 days [169,180,183], or switching to TPE entirely when the adverse effects of corticosteroids are presented. The latter would have its clinical rationale in terms of affecting OS as well.…”

Section: Discussionmentioning

confidence: 99%

Oxidative Stress and the Nuclear Factor Erythroid 2-Related Factor 2 (Nrf2) Pathway in Multiple Sclerosis: Focus on Certain Exogenous and Endogenous Nrf2 Activators and Therapeutic Plasma Exchange Modulation

Tonev,

Momchilova

2023

IJMS

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The pathogenesis of multiple sclerosis (MS) suggests that, in genetically susceptible subjects, T lymphocytes undergo activation in the peripheral compartment, pass through the BBB, and cause damage in the CNS. They produce pro-inflammatory cytokines; induce cytotoxic activities in microglia and astrocytes with the accumulation of reactive oxygen species, reactive nitrogen species, and other highly reactive radicals; activate B cells and macrophages and stimulate the complement system. Inflammation and neurodegeneration are involved from the very beginning of the disease. They can both be affected by oxidative stress (OS) with different emphases depending on the time course of MS. Thus, OS initiates and supports inflammatory processes in the active phase, while in the chronic phase it supports neurodegenerative processes. A still unresolved issue in overcoming OS-induced lesions in MS is the insufficient endogenous activation of the Nuclear Factor Erythroid 2-Related Factor 2 (Nrf2) pathway, which under normal conditions plays an essential role in mitochondria protection, OS, neuroinflammation, and degeneration. Thus, the search for approaches aiming to elevate endogenous Nrf2 activation is capable of protecting the brain against oxidative damage. However, exogenous Nrf2 activators themselves are not without drawbacks, necessitating the search for new non-pharmacological therapeutic approaches to modulate OS. The purpose of the present review is to provide some relevant preclinical and clinical examples, focusing on certain exogenous and endogenous Nrf2 activators and the modulation of therapeutic plasma exchange (TPE). The increased plasma levels of nerve growth factor (NGF) in response to TPE treatment of MS patients suggest their antioxidant potential for endogenous Nrf2 enhancement via NGF/TrkA/PI3K/Akt and NGF/p75NTR/ceramide-PKCζ/CK2 signaling pathways.

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Plasma exchange in inflammatory demyelinating disorders of the central nervous system: reasonable use in the clinical practice

Cited by 4 publications

References 56 publications

Plasma Exchange in Pediatric Neurology Patients: A Single-Center Experience

Plasma Exchange in Pediatric Neurology Patients: A Single-Center Experience

Neuromyelitis Optica Spectrum Disorder

Oxidative Stress and the Nuclear Factor Erythroid 2-Related Factor 2 (Nrf2) Pathway in Multiple Sclerosis: Focus on Certain Exogenous and Endogenous Nrf2 Activators and Therapeutic Plasma Exchange Modulation

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