Fatal Autoimmune Anti-NMDA-Receptor Encephalitis with Poor Prognostication Score in a Young Kenyan Female

Rakiro, Joe; Sokhi, Dilraj

doi:10.2147/imcrj.s311071

Cited by 4 publications

(6 citation statements)

References 13 publications

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“…Brain MRI can be normal or show multiple abnormalities in cortical and subcortical regions in FLAIR with possible contrast enhancement ( 175 ). It must be noted that access to brain MRI and antibody testing might be limited in some African situations, hampering the achievement of a correct diagnosis ( 151 , 177 ).…”

Section: Well Established Post-infectious or Tumour-related Autoantib...mentioning

confidence: 99%

“…Again, it must be underlined that access to plasmapheresis and immunoglobulin might be difficult in some African regions ( 151 ). Moreover, ICU might not be always available and when it is, mortality risk in ICU is high, mainly in relation to sepsis and tracheostomy requirements ( 177 , 186 ).…”

Section: Well Established Post-infectious or Tumour-related Autoantib...mentioning

confidence: 99%

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Post-Infectious Autoimmunity in the Central (CNS) and Peripheral (PNS) Nervous Systems: An African Perspective

et al. 2022

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The direct impact and sequelae of infections in children and adults result in significant morbidity and mortality especially when they involve the central (CNS) or peripheral nervous system (PNS). The historical understanding of the pathophysiology has been mostly focused on the direct impact of the various pathogens through neural tissue invasion. However, with the better understanding of neuroimmunology, there is a rapidly growing realization of the contribution of the innate and adaptive host immune responses in the pathogenesis of many CNS and PNS diseases.The balance between the protective and pathologic sequelae of immunity is fragile and can easily be tipped towards harm for the host. The matter of immune privilege and surveillance of the CNS/PNS compartments and the role of the blood-brain barrier (BBB) and blood nerve barrier (BNB) makes this even more complex. Our understanding of the pathogenesis of many post-infectious manifestations of various microbial agents remains elusive, especially in the diverse African setting. Our exploration and better understanding of the neuroimmunology of some of the infectious diseases that we encounter in the continent will go a long way into helping us to improve their management and therefore lessen the burden.Africa is diverse and uniquely poised because of the mix of the classic, well described, autoimmune disease entities and the specifically “tropical” conditions. This review explores the current understanding of some of the para- and post-infectious autoimmune manifestations of CNS and PNS diseases in the African context. We highlight the clinical presentations, diagnosis and treatment of these neurological disorders and underscore the knowledge gaps and perspectives for future research using disease models of conditions that we see in the continent, some of which are not uniquely African and, where relevant, include discussion of the proposed mechanisms underlying pathogen-induced autoimmunity. This review covers the following conditions as models and highlight those in which a relationship with COVID-19 infection has been reported: a) Acute Necrotizing Encephalopathy; b) Measles-associated encephalopathies; c) Human Immunodeficiency Virus (HIV) neuroimmune disorders, and particularly the difficulties associated with classical post-infectious autoimmune disorders such as the Guillain-Barré syndrome in the context of HIV and other infections. Finally, we describe NMDA-R encephalitis, which can be post-HSV encephalitis, summarise other antibody-mediated CNS diseases and describe myasthenia gravis as the classic antibody-mediated disease but with special features in Africa.

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Section: Well Established Post-infectious or Tumour-related Autoantib...mentioning

confidence: 99%

Section: Well Established Post-infectious or Tumour-related Autoantib...mentioning

confidence: 99%

Post-Infectious Autoimmunity in the Central (CNS) and Peripheral (PNS) Nervous Systems: An African Perspective

et al. 2022

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“…2 Anti-N-Methyl-D-Aspartic Acid (anti-NMDA) encephalitis is the most frequent and best-de ned autoimmune encephalitis, considered potentially fatal. [3][4] It was rst diagnosed in 2007. 5 The pathophysiology of anti-NMDA autoimmune encephalitis is characterized by the presence of immunoglobulin G "IgG" autoantibodies against the GluN1 subunit of the NMDA receptor in the CNS.…”

Section: Introductionmentioning

confidence: 99%

“…8 Clinical presentation of patients with anti-NMDA autoimmune encephalitis can resemble other autoimmune encephalitis with clinical presentations ranging from mild-moderate to severe, with mortality between 5% and 11%. [3][4][5][6][7][8][9] Anti-NMDA autoimmune encephalitis is usually characterized by the presence of oro-facial dyskinesia and acute or subacute neuropsychiatric and behavioral manifestations. 10 International consensus recommendations for the treatment of pediatric NMDAR antibody encephalitis have been established to treat patients with autoimmune encephalitis.…”

Section: Introductionmentioning

confidence: 99%

Effectiveness of Intra-thecal Methotrexate in Refractory Anti-N-methyl-d-aspartate Receptor Encephalitis

Hommady,

Alsohaibani,

Alayed

et al. 2023

Preprint

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Background Anti-N-methyl-d-aspartate receptor encephalitis is one of the most common autoimmune encephalitis for which first- and second-line therapies have been recommended following international consensus. However, some refractory cases do not respond to the first- and second-line therapy and require further immune-modulatory therapies such as intra-thecal methotrexate. In this study, we reviewed six confirmed cases of refractory anti-N-methyl-d-aspartate receptor encephalitis from two tertiary centers in Saudi Arabia that required escalation of treatment and received a six-month course of intra-thecal methotrexate. The aim of this study was to evaluate the effectiveness of intra-thecal methotrexate as immunomodulatory therapy for refractory anti-N-methyl-d-aspartate receptor encephalitis. Methods: We retrospectively evaluated six confirmed cases of refractory anti N-methyl-d-aspartate receptor encephalitis who did not improve after first- and second-line therapy and received monthly intra-thecal methotrexate treatment course for six consecutive months. We reviewed patient demography, underlying etiologies, and compared their modified Rankin score prior to receiving intra-thecal methotrexate and six months after completing the treatment. Results: Three of the six patients showed a marked response to intra-thecal methotrexate with a modified Rankin scale of 0–1 at 6-month follow-up. None of the patients experienced any side effects during or after intra-thecal methotrexate treatment, and no flareups were observed. Conclusion: Intra-thecal methotrexate may be a potentially effective and relatively safe escalation option for immunomodulatory therapy of refractory anti- N-methyl-d-aspartate receptor encephalitis. Future studies on intra-thecal methotrexate -specific treatment regimens may further support its utility, efficacy, and safety in treating refractory anti- N-methyl-d-aspartate receptor encephalitis.

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“…Their increasing incidence in high-income countries is attributed to increased recognition of antibody-positive cases. There are several recent case reports from Africa, in particular associated with N-methyl-D-aspartate-receptor antibodies 17–24. In contrast to non-treatable causes, many of the infective, metabolic and nutritional causes can be diagnosed and treated in Africa.…”

mentioning

confidence: 99%

Rapidly progressive dementia: limitations in Africa

Howlett

2022

Pract Neurol

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Fatal Autoimmune Anti-NMDA-Receptor Encephalitis with Poor Prognostication Score in a Young Kenyan Female

Cited by 4 publications

References 13 publications

Post-Infectious Autoimmunity in the Central (CNS) and Peripheral (PNS) Nervous Systems: An African Perspective

Post-Infectious Autoimmunity in the Central (CNS) and Peripheral (PNS) Nervous Systems: An African Perspective

Effectiveness of Intra-thecal Methotrexate in Refractory Anti-N-methyl-d-aspartate Receptor Encephalitis

Rapidly progressive dementia: limitations in Africa

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