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2.1
Autoimmune Encephalopathy
Autoimmune Encephalitis

Presentation

This presentation can be missed or mistaken for depression, but can be diagnosed with a detailed neuropsychological evaluation. [ncbi.nlm.nih.gov]

The authors are not aware of this presentation being reported in children. [omicsonline.org]

A discussion of the present diagnostic options and needed further improvements including imaging, CSF analysis, and neurophysiological methods will be presented. [frontiersin.org]

Tumor markers may be present, suggesting PNS. [intechopen.com]

Entire Body System

  • Fatigue

    ) G31.2 ) benign myalgic encephalomyelitis ( ICD-10-CM Diagnosis Code G93.3 Postviral fatigue syndrome 2016 2017 2018 2019 Billable/Specific Code Applicable To Benign myalgic encephalomyelitis Type 1 Excludes chronic fatigue syndrome NOS ( R53.82 ) [icd10data.com]

    Other common symptoms of encephalitis include headache, drowsiness, fever and fatigue. [drnibber.com]

    Confusion -Disorientation -Psychosis -Coma -Tremors -Convulsions -Concentration Problems -Attention Span Problems -Difficulty Retaining Information -Short Term Memory Problems -Seizure Activity -Monoclonal Jerks - Involuntary Muscle Jerks -Dementia -Fatigue [answers.google.com]

    Today, he suffers socially, and has significant mental and physical fatigue. We are trying a steroid treatment, and his doctor will consider other treatments next. This discussion is closed to comments. [inspire.com]

    Table 2 Clinical course & investigations Prodrome symptoms • Not clearly defined 19(36%) 8(32%) 2(8%) • Headache 1(2%) 2(8%) 5(20%) • Fever 6(11%) 7(28%) 1(4%) • Fever with associated symptoms 18(34%) 3(12%) 12(48%) • URI symptoms 6(11%) 1(4%) 2(8%) • Fatigue [bmcneurol.biomedcentral.com]

Neurologic

  • Memory Impairment

    Patients with N–methyl–D–aspartate (NMDA) receptor antibodies, which are the most common in autoimmune encephalopathy, often cause psychiatric manifestation, memory impairment, seizures, dyskinesia, catatonia, autonomic instability and respiratory failures [ci.nii.ac.jp]

    Limbic encephalitis is characterized by short-term memory impairment, complex partial temporal lobe seizures and psychiatric symptoms. Signal abnormalities in the mesial temporal lobes without contrast enhancement are the typical MRI findings. [ncbi.nlm.nih.gov]

  • Focal Neurological Deficit

    No focal neurologic deficits are seen in the latter type, but neuropsychologic testing reveals severe cognitive deficits. No specific diagnostic test exists for Hashimoto's encephalopathy. Thyrotropin and FT4 levels should be relatively normal. [answers.google.com]

    The pathogenesis of the unexpected and fulminant disorder characterized by altered sensorium, cognitive and behavioral impairment, focal neurological deficits, epileptic seizures, and status epilepticus had been an enigma. [annalsofian.org]

    Co-existence of anti-NMDAR and –Ri antibodies was found in one patient: an 18- year-old male who initially developed hemophagocytic syndrome after salmonella sepsis and was admitted to the ICU where he had seizures without focal neurological deficits. [bmcneurol.biomedcentral.com]

  • Mild Cognitive Impairment

    Patients with NMDAR encephalitis seem to have a stormier initial course and require more intensive immunomodulation, but often have a better long-term prognosis than the LGI1 patients who are frequently left with mild cognitive impairment. [neurologyindia.com]

Workup

A while back, I got a full autoimmune encephalitis workup just to be sure I hadn’t had a different form of AE for all that time. I hoped it could reveal why my case has been so persistent. [thedreamingpanda.com]

Should there be a standardised approach to the diagnostic workup of suspected adult encephalitis? A case series from Australia. BMC Infect Dis. 2010 Dec 15;10:353. PMC3018438. Irani SR, Vincent A. [lifeinthefastlane.com]

Workup should include a thorough history and physical exam to determine if the clinical course could be consistent with AE. [aealliance.org]

Other serological workup revealed negative titers of anti-smooth antibodies, anti-LKM, anti-endomesial, anti-tissue transglutaminase IgG and IgA, anti-cardiolipin IgM & IgG, rheumatoid factor, anti-nuclear antibodies, anti-myeloperoxidase, antiproteinase [omicsonline.org]

Depending on the patient’s presentation and what the clinical workup reveals, treatments possibly employed include immune-modulating drugs if autoimmune markers or signs of inflammatory disease are present, or antibiotics for repeated sinus or throat [stanmed.stanford.edu]

Treatment

[…] respond to treatment. [sciencedaily.com]

[…] subsequent plasma exchange [Figure 1]. [9] Second-line treatments include cyclophosphamide and rituximab. [10] Figure 1: Suggested treatment algorithm for autoimmune encephalitis. [neurologyindia.com]

The complexity of this disease illustrates how important understanding a disease process is in developing treatments. [drnibber.com]

Her encephalopathy was not improved by antithyroid drugs, but promptly responded to corticosteroid treatment, and stabilised with a gradual reduction of thyroid related autoantibody titres. [ncbi.nlm.nih.gov]

Prognosis

The concept of HE is becoming fragmented into a number of other types of autoimmune encephalitis which appear to have their own autoantibodies, prognosis and associated features. [encephalitis.info]

Patients with NMDAR encephalitis seem to have a stormier initial course and require more intensive immunomodulation, but often have a better long-term prognosis than the LGI1 patients who are frequently left with mild cognitive impairment. [neurologyindia.com]

[…] cortical atrophy with ex-vacuo ventricular dilatation 15 T2: hyperintense signal areas in the affected hemisphere 15 DWI/ADC: restricted diffusion may be seen in altered signal areas 15 T1 C+ (Gd): no significant post-contrast enhancement 15 Treatment and prognosis [radiopaedia.org]

Worse prognosis if: extremes of age ( 55y) comorbidities immunodeficiency virulent organism References and links LITFL Anti-NMDA Receptor Encephalitis (CCC) HSV encephalitis (CCC) Journal articles Armangue T, Leypoldt F, Dalmau J. [lifeinthefastlane.com]

Etiology

Significant inter- and intra-country differences in incidence and prevalence exist because multiple etiologic factors are implicated. Many past reviews have addressed sole etiologies. [dx.doi.org]

Etiologies remain largely unknown. [bmcneurol.biomedcentral.com]

Autoimmune encephalitis is a recently recognized etiology of encephalitis which is mediated by various autoantibodies targeting neural cells or synapses. [clinicaltrials.gov]

Fahad Muhamed Shareef AT Shaji Thomas John KA Salam Abstract Background:The etiology of encephalitis are numerous and extensive investigations for infectious agents and other causes are often negative. [babymhospital.org]

Epidemiology

We considered a comprehensive view of all etiologies (genetic/structural/metabolic) to be significant for both the developing and the developed world as well as routine clinical/epidemiology practice. [dx.doi.org]

The epidemiology of tumor-related autoimmune encephalitis mimics that of the underlying malignancy. Those with non-tumor related autoimmune encephalitis have a variable epidemiology but are mostly young patients with a female predilection 8. [radiopaedia.org]

Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177. [ Links ] 5. Zhao J, Guan H, Zhang J, Wang C, Xu X, Zhang Y, et al. [scielo.org.co]

[…] causes are numerous and may be infectious or non-infectious, many of which lack effective therapies Herpes simplex encephalitis is the commonest cause in Australasia and acyclovir should be started as soon as the diagnosis is suspected Always consider epidemiological [lifeinthefastlane.com]

Pathophysiology

Research areas Further investigation into the pathophysiology of the neurological conditions associated with harmful neuroinflammation or autoantibody against brain cells is warranted in order to advance the diagnostic testing, develop novel treatments [northwell.edu]

The following algorithms are available in Special Instructions: -Encephalopathy Autoimmune Evaluation Algorithm, Spinal Fluid -Meningitis/Encephalitis Panel Algorithm Clinical Information Discusses physiology, pathophysiology, and general clinical aspects [mayomedicallaboratories.com]

[…] soon as the diagnosis is suspected Always consider epidemiological factors that may indicate an unusual cause of encephalitis (e.g. arboviruses and zoonoses), especially in Australia Encephalitis is commonly fatal or results in significant morbidity PATHOPHYSIOLOGY [lifeinthefastlane.com]

Prevention

Epilepsy is a heterogeneous disorder, the symptoms of which are preventable and controllable to some extent. Significant inter- and intra-country differences in incidence and prevalence exist because multiple etiologic factors are implicated. [dx.doi.org]

Regarding treatment, one of the affected children received levetiracetam but no immunotherapy and showed “substantial recovery,” but three years later still requires antiseizure medication to prevent a recurrence of epilepsy. [mdedge.com]

Immune suppressing medications may be taken for prolonged periods of time (even after recovery) to prevent a relapse. [antinmdafoundation.org]

Prevention of these infections with natural immune support, may therefore mitigate the progression of the disease. The complexity of this disease illustrates how important understanding a disease process is in developing treatments. [drnibber.com]

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