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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]
  • These may present variously such as with unexplained delirium, cognitive decline, or depression. Autoimmune encephalitis may be diffuse or localized as in limbic, brainstem, or basal ganglia encephalitis.[ncbi.nlm.nih.gov]
  • The authors are not aware of this presentation being reported in children.[omicsonline.org]
  • The case is reported of a 51 year old woman presenting with subacute progressive dementia with evidence of hyperthyroidism. She had Graves' disease associated with high titres of thyroid related autoantibodies.[ncbi.nlm.nih.gov]
  • A discussion of the present diagnostic options and needed further improvements including imaging, CSF analysis, and neurophysiological methods will be presented.[frontiersin.org]
Fatigue
  • Other common symptoms of encephalitis include headache, drowsiness, fever and fatigue.[drnibber.com]
  • .- ) 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]
  • 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]
  • 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]
Pressured Speech
  • speech, verbal reduction, mutism) Seizures Movement disorder, dyskinesias, or rigidity/abnormal postures Decreased level of consciousness Autonomic dysfunction or central hypoventilation At least one of the following laboratory study results: Abnormal[ncbi.nlm.nih.gov]
Recurrent Respiratory Infections
  • The usual presentation is with recurrent respiratory infections. There is a strong association between CVID and autoimmune disorders. However, for CVID to present initially and solely with Autoimmune Encephalitis (AIE) is extremely rare.[omicsonline.org]
Heart Rate Irregular
  • The resulting symptoms include mood and personality changes, violent outbursts, paranoia, psychosis, memory loss, speech problems, numbness, seizures, involuntary movements, increased heart rate, irregular heart rhythm, slowed breathing and/or decreased[bottomlineinc.com]
Pupillary Abnormality
  • . • Additionally, patients frequently develop pupillary abnormalities, bilateral facial palsy, Babinski’s sign, and bulbar palsy. • Generalised limb weakness can occur, which overlaps with features of GBS. 31. • CSF pleocytosis occurs in 45% patients.[slideshare.net]
  • Additionally, patients frequently develop pupillary abnormalities, bilateral facial palsy, Babinski’s sign, and bulbar palsy.[ncbi.nlm.nih.gov]
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]
  • Patients with limbic encephalitis for instance suffer from memory impairments as the limbic regions of the brain (amygdala, hippocampus) are affected. Other common symptoms of encephalitis include headache, drowsiness, fever and fatigue.[drnibber.com]
Limb Weakness
  • . • Generalised limb weakness can occur, which overlaps with features of GBS. 31. • CSF pleocytosis occurs in 45% patients. • Brain MRI usually normal, but brainstem abnormalities on T2w FLAIR imaging present in 23% of patients. • IgG anti-GQ1b antibodies[slideshare.net]
  • Generalised limb weakness can occur, which overlaps with features of Guillain-Barré syndrome. 84 CSF pleocytosis occurs in 45% of patients.[ncbi.nlm.nih.gov]

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]
  • 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]
  • 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]
  • Comparison of clinical course, neuroimaging studies and results of the workup for immune-mediated and unidentified etiologies are summarized in Table 2.[bmcneurol.biomedcentral.com]

Treatment

  • 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]
  • Thyroidectomy can be considered as one of the treatment options especially in thyrotoxic HE patients with uncontrolled relapses. Our patient is the first reported HE case with a family history.[ncbi.nlm.nih.gov]
  • […] 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]

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]
  • […] antibodies most common small cell carcinoma of the lung in 75% of cases anti-Hu syndrome consists of paraneoplastic encephalomyelitis, paraneoplastic sub-acute sensory neuropathy, and paraneoplastic cerebellar degeneration anti-Ma/Ta antibodies better prognosis[radiopaedia.org]
  • […] 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.[doi.org]
  • Autoimmune encephalitis is a recently recognized etiology of encephalitis which is mediated by various autoantibodies targeting neural cells or synapses.[clinicaltrials.gov]
  • Etiologies remain largely unknown.[bmcneurol.biomedcentral.com]
  • 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

  • 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]
  • 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.[doi.org]
  • In a large epidemiological study (The California Encephalitis Project), NMDAR antibodies were positive in half of the children with idiopathic encephalitis and psychiatric symptoms, and the frequency was four times that of encephalitis caused by herpes[annalsofian.org]
  • […] 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • […] 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.[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]
  • 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]
  • But on this World Encephalitis Day, I’m going to throw a grenade into the rigidity and bias that plague too many autoimmune encephalitis doctors, impede scientific progress, and prevent everyone like me from receiving their help: You don’t know everything[thedreamingpanda.com]

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