Treatment strategies for autoimmune encephalitis

Shin, Yong-Won; Park, Kyung‐Il; Jung, Keun‐Hwa; Jung, Ki-Young; Lee, Sang Kun; Chu, Kon

doi:10.1177/1756285617722347

Cited by 204 publications

(229 citation statements)

References 153 publications

(250 reference statements)

Supporting

Mentioning

216

Contrasting

Unclassified

Order By: Relevance

“…Autoimmune encephalitis (AE) is an emerging type of immune‐mediated neurologic disorder. The clinical manifestations of AE are variable and depend on the presence of specific types of autoantibodies . Although most patients are responsive to immunotherapeutic agents, a substantial number of patients are intractable to first‐ and second‐line immunotherapies .…”

mentioning

confidence: 99%

Development of the clinical assessment scale in autoimmune encephalitis

Lim

Moon

Jun

et al. 2019

Annals of Neurology

Self Cite

140

128

View full text Add to dashboard Cite

We read with great interest the study by Lim et al, 1 which aimed to develop an easily applicable scale to grade the severity of autoimmune encephalitis (AE). Nine key clinical features were identified and included in the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). CASE showed good interobserver and intraobserver reliability, and internal consistency, as well as a high correlation with the modified Rankin Scale (mRS).We applied CASE retrospectively to a cohort of 60 patients with AE, admitted to our institution between 2012 and 2018, and prospectively in 3 additional patients. Median age at onset was 55 years (range = 3 months-88 years), in 13 of 63 (21%) onset was before 18 years of age, and 34 of 63 (54%) were female. All patients fulfilled the clinical diagnostic criteria for AE 2 : definite AE was diagnosed in 16 of 63 patients (25%), definite anti-NMDA receptor encephalitis in 11 of 63 (17%), definite autoimmune limbic encephalitis in 6 of 63 (10%), antibody-negative probable AE in 4 of 63 (6 %), possible AE in 25 of 63 (40%), and Hashimoto encephalopathy in 1 of 63 (2%). At the disease nadir, median mRS score was 4 (range = 3-5). We applied CASE at maximum clinical severity (median CASE score = 9, range = 3-24). In our cohort, CASE significantly correlated with mRS (p < 0.0001, r 2 = 0.5025; Fig), although the correlation strength was weaker than that reported by Lim et al. However, we encountered several issues when CASE was applied to our patients that need to be clarified. Severe clinical conditions, such as coma and status epilepticus, make other items hardly assessable (eg, gait instability, language). In such cases, it is not specified what score should be given to items that are not directly assessable (we gave the maximum score). Postictal state after seizures also interferes with the evaluation of most items. The authors should clarify how to apply the scale in these situations and whether this score is meant to be used only in clinically stable patients. Furthermore, when applying CASE to a pediatric population, some items, such as memory and language, were not easily assessable. We think that the scale is more suitable for adult patients. Moreover, it would be appropriate to develop a different scale for patients with altered consciousness.CASE is potentially a useful tool and addresses the unmet need of clinical scales to grade AE severity, as mRS is quite coarse in evaluating this condition. However, CASE needs further improvement and validation to be employed in clinical trials.

show abstract

mentioning

confidence: 99%

Development of the clinical assessment scale in autoimmune encephalitis

Lim

Moon

Jun

et al. 2019

Annals of Neurology

Self Cite

140

128

View full text Add to dashboard Cite

show abstract

“…If patients do not show a response in the first 2 weeks, the second line of treatment is rituximab, alone or combined with cyclophosphamide . Rituximab depletes B cells; however, its effectiveness over cyclophosphamide (a strong immunosuppressant) is not yet clear .…”

Section: Resultsmentioning

confidence: 99%

“…24,70 If patients do not show a response in the first 2 weeks, the second line of treatment is rituximab, alone or combined with cyclophosphamide. 71,72 Rituximab depletes B cells; however, its effectiveness over cyclophosphamide (a strong immunosuppressant) is not yet clear. 73 Lee et al reported on the efficacy and safety of rituximab as a second-line immunotherapy for AIE in 161 patients in which rituximab was associated with improvement on functional outcomes measured on the modified Rankin Scale.…”

Section: Advances In Treatmentmentioning

confidence: 99%

Autoimmune encephalitis: What has changed during the past 15 years?

Pantoja‐Ruiz

Santucci

Arenas

et al. 2019

Clinical & Exp Neuroim

View full text Add to dashboard Cite

Non‐infectious encephalitis has been a recognized entity for many years; however, its pathophysiology was not fully understood. Patients (usually women) presented with acute behavioral changes and were admitted to mental hospitals, without a correct diagnosis. With the new advances on antibody detection, the non‐infectious encephalitis panorama has been changing. Immune‐mediated diseases of the central nervous system encompass a variety of disorders, including the classic paraneoplastic encephalitis and the more recently discovered autoimmune encephalitis. Recognition of the new syndromes and antigens, in addition to the advances in diagnostic methods, such as antibody detection and imaging, and early implementation of immunotherapy, have changed the field of autoimmune encephalitis significantly over the past two decades. The best recognized form of autoimmune encephalitis is anti‐N‐methyl‐D‐aspartate receptor encephalitis, which usually presents with neuropsychiatric changes, seizures, autonomic dysfunction and usually a decreased level of consciousness. Nevertheless, the panorama for other types of receptors, such as anti‐α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid receptor, anti‐gamma‐aminobutyric acid and, most recently, anti‐glycine receptor, anti‐metabotropic glutamate receptor 5, dopamine receptor D2 and dipeptidyl‐peptidase‐like protein 6, which can present clinically as encephalitis, is currently changing, meaning that the panorama for diagnosis and treatment is broadening every day.

show abstract

“…Cyclophosphamide is considered a high-risk drug with various serious side effects, Rituximab could be a preferable agent. However, Cyclophosphamide is easily accessible and of lower cost than Rituximab [21]. It is not typically used in autoimmune neurological diseases and there are no accepted evidence based guidelines for dosing of Cyclophosphamide in anti-NMDAR encephalitis.…”

Section: Discussionmentioning

confidence: 99%

Non-paraneoplastic Anti-NMDAR Encephalitis: First Confirmed Adult Case Report in Latvia

Ziemele¹,

Литвиненко²,

Karelis³

et al. 2018

JPP

View full text Add to dashboard Cite

Anti-NMDAR (Anti-N-Methyl-D-aspartic acid) encephalitis is a rare autoimmune condition mainly affecting young women. It is associated with an underlying tumor in about 50% of reported cases. Antibodies directed against the GluN1 subunit of the NMDA receptor are responsible for the disease pathogenesis and their detection in the patient's serum and cerebrospinal fluid are required to make a definite diagnosis. Classical clinical presentation consists of flu-like symptoms, followed by psychiatric disturbances and impaired consciousness, epileptic seizures and movement disorders. During the past decade, it has become an emerging area of research and discussion as more than 1,000 cases have been reported since the first description of this specific disease entity in 2007. Despite a rather typical clinical course it is frequently diagnosed and treated with a delay up to many months. Overall prognosis tends to be favorable. However, it strongly depends on early diagnosis and rapid treatment initiation. While diagnostic criteria for probable and definite anti-NMDAR encephalitis have been proposed, there are no evidence based guidelines for specific treatment strategies. Glucocorticoids, plasma exchange and IVIG are generally used as 1st line treatment, in patients who do not respond, 2nd line treatment with Cyclophosphamide or Rituximab is used. We report a case of a confirmed non-paraneoplastic anti-NMDAR encephalitis with a rather classical manifestation in a Latvian woman who is first hospitalized in a psychiatric clinic then transferred to an ICU (intensive care unit), treated with glucocorticoids, plasma exchange and later Cyclophosphamide with a good outcome.

show abstract

Treatment strategies for autoimmune encephalitis

Cited by 204 publications

References 153 publications

Development of the clinical assessment scale in autoimmune encephalitis

Development of the clinical assessment scale in autoimmune encephalitis

Autoimmune encephalitis: What has changed during the past 15 years?

Non-paraneoplastic Anti-NMDAR Encephalitis: First Confirmed Adult Case Report in Latvia

Contact Info

Product

Resources

About