Materiały źródłowe

• #1 Symptoms & Phases | Diagnosis & Treatment of Autoimmune Encephalitis

  https://autoimmune-encephalitis.org/symptoms-phases/

  Autoimmune encephalitis (AE) can produce an extensive range of neuropsychiatric symptoms. A major challenge in diagnosis is that different symptoms may appear at different times and different levels of intensity, so the disease may mimic many other disorders. […] The signs and symptoms of most antibody-mediated neurologic diseases start suddenly and progress rapidly over several weeks or months. […] In Autoimmune Encephalitis, the prodromal symptoms, if they occur, are flu-like symptoms which include: headache, fever, nausea, vomiting, diarrhea, or upper respiratory tract symptoms. […] Frequently the dominant feature that is seen in children following this first phase is seizure activity. Young children typically present with insomnia, seizures, abnormal movements, or a change in behavior such as irritability, temper tantrums, agitation, and reduction of verbal output.

• #1

  https://link.springer.com/article/10.1007/s00415-023-11685-3

  In NMDAR-AbE, neuropsychiatric symptoms are often preceded by viral prodromal symptoms, such as headache, fever, myalgia, and coryza, which usual occur around 4-14 days prior to disease onset. This is followed by an acute neuropsychiatric presentation with subsequent seizures and cognitive impairment. Around 14 weeks later, patients commonly develop a characteristic movement disorder, dysautonomia, and coma, which often precipitate intensive-care unit admission. […] Understanding longer term issues which patients encounter continues to be an area of active research with important everyday implications. For example, in NMDAR-AbE, it is widely accepted that treatments are generally highly effective. However, detailed neuropsychological assessments at long-term follow-up have demonstrated that ~80% of patients had moderate-to-severe cognitive impairment, with prominent deficits in executive function and memory. This challenges the current perception of this disease as showing a complete response to immunotherapy.

• #1 Autoimmune Encephalitis Symptoms | AE Alliance

  https://aealliance.org/patient-support/symptoms/

  Neurologic symptoms: Problems with memory and the process of thinking (also known as cognition), Abnormal movements, Seizures, Problems with balance or coordination (or ataxia), Having trouble speaking, Changes in vision, Loss of consciousness or coma. […] Psychiatric symptoms: Hallucinations, delusions, or paranoia (or psychosis), Aggressive behavior, Inappropriate sexual behaviors, Anxiety or panic attacks, Compulsive behaviors, Agitation, fear, Difficulty sleeping. […] One of the hallmarks of AE is a rapid development of symptoms and progression over time for most causes of AE. However, other neurological and psychiatric conditions may present with a sudden onset of symptoms or quickly worsening symptoms.

• #1 Symptoms & Phases | Diagnosis & Treatment of Autoimmune Encephalitis

  https://autoimmune-encephalitis.org/symptoms-phases/

  While teenagers and adults more often present with psychiatric symptoms, including agitation, aggression, inappropriate sexual behaviors, panic attacks, compulsive behaviors, euphoria, or fear, hallucinations, delusions, and psychomotor slowing, which may lead to hospital admission for psychosis. […] The disease progresses in a period of days or weeks to include reduction of speech, memory deficit, orofacial and limb dyskinesias, seizures, decreased level of consciousness, and autonomic instability manifested as excess salivation, hyperthermia, fluctuations in blood pressure, tachycardia, or central hyperventilation. […] Psychiatric Symptoms may fluctuate rapidly coming on and receding in severity and length of time. […] There is a progressive decrease in the level of consciousness which can progress to coma.

• #1 The Diagnosis and Treatment of Autoimmune Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4712273/

  Autoimmune encephalitis causes subacute deficits of memory and cognition, often followed by suppressed level of consciousness or coma. […] Patients generally have impaired memory and cognition over a period of days or weeks. […] The classical presentation of encephalitis consists of a subacute (days to a few weeks) progressive decrease in the level of consciousness, often with fluctuations, and altered cognition. Memory, especially retention of new information, may be impaired early in the clinical course. Patients may progress to coma. […] Psychiatric manifestations are common early in the course of autoimmune encephalitis. These may include psychosis, aggression, inappropriate sexual behaviors, panic attacks, compulsive behaviors, euphoria or fear. Symptoms may fluctuate rapidly. […] Abnormal movements may be the presenting symptom in several types of autoimmune encephalitis.

• #1 Autoimmune Encephalitis: A Physician’s Guide to the Clinical Spectrum Diagnosis and Management

  https://www.mdpi.com/2076-3425/12/9/1130

  Movement disorders are common in autoimmune encephalitis, particularly at a younger age. Their phenotypes are broad and include orofacial dyskinesia, paroxysmal dyskinesia, chorea, dystonia, myoclonic jerks, tremor, cerebellar ataxia, and parkinsonian symptoms. […] Some degree of cognitive impairment is the single most consistent finding in autoimmune encephalitis, such as those associated with autoantibodies targeting NMDAR, LGI-1, and GAD, among others. However, mild forms of cognitive dysfunction can be overlooked in the presence of dramatic neuropsychiatric features such as psychosis, among others. Cognitive impairment encompasses deficits in memory, language, executive functions, sustained attention, and apraxia. […] Although sleep disorders are not listed as a diagnostic criterion in clinical diagnosis of autoimmune encephalitis, sleep disturbances are frequently observed, although at times overlooked, in individuals with autoimmune encephalitis. A recent prospective study of 26 autoimmune encephalitis patients showed that 73% patients had new-onset sleep disruptions. Common sleep disturbances include rapid eye movement (REM), sleep behavior disorder and dream enactment behaviors, hypersomnia and fragmented sleep, insomnia and periodic limb movements, and REM sleep behavior disorders with stridor and disordered breathing.

• #1 Symptoms & Phases | Diagnosis & Treatment of Autoimmune Encephalitis

  https://autoimmune-encephalitis.org/symptoms-phases/

  Symptoms associated with AE can include: Memory disturbance, weakness or numbness of part of the body, loss of balance, slowed or blurred speech or loss of ability to speak, ataxia, autoimmune pain, involuntary movements, distorted vision, cognitive impairment, decreased level of consciousness – to the point of unresponsiveness, catatonia or coma, seizures – (either self-evident, or smaller seizures that show up on an EEG reading), behavior change, (aggression, panic attacks, compulsive behaviors, euphoria or fear), partial or complete loss of appetite for long periods, food and drink tasting inedible or triggering nausea, excessive eating without feeling sated, agitation, inability to sleep, loss of inhibition, inappropriate sexual behaviors, rapid, pressured, or involuntary speech, psychosis, hallucinations (visual or auditory) and delirium, paranoid thoughts, severe anxiety, depression.

• #1 Symptoms & Phases | Diagnosis & Treatment of Autoimmune Encephalitis

  https://autoimmune-encephalitis.org/symptoms-phases/

  The second phase is characterized by neurologic and psychiatric symptoms. Adult patients often show overt first time psychosis with different combinations of psychomotor agitation, delusions, catatonia, violence and aggression. […] When the disease progresses to this stage, almost half of these patients will require prolonged ICU stays and mechanical ventilation. Many develop autonomic dysregulation (disturbances, abnormalities) such as combinations of tachycardia, bradycardia, hypertension, hypotension, fever or hypothermia, or hyperthermia. Dyskinesia, hyper salivation that may interfere with airway management, no memory of events, also common is the development of seizure like motor activity, although EEG typically does not show changes consistent with epileptic activity.

• #1 Autoimmune encephalitis | Altru Health System

  https://www.altru.org/health-library/conditions/autoimmune-encephalitis

  Autoimmune encephalitis symptoms can vary but may include memory loss, changes in thinking, changes in behavior and seizures. […] Autoimmune encephalitis symptoms can occur in a pattern that is predictable depending on the type of autoimmune encephalitis. Many people have a headache, fever and other symptoms of an infection followed by psychiatric symptoms that may include anxiety, panic attacks, changes in behavior, agitation, hallucinations, delusions and trouble organizing thoughts. […] Autoimmune encephalitis symptoms can get worse over time. This disease course is known as progressive. Symptoms also might alternate between getting better and getting worse. This course is known as relapsing-remitting. […] Sometimes AE causes serious seizures that need emergency care, known as status epilepticus. These seizures last more than five minutes or occur one after another while the person is not conscious.

• #1 Autoimmune Encephalitis: What Psychiatrists Need to Know

  https://www.psychiatrictimes.com/view/autoimmune-encephalitis-what-psychiatrists-need-know

  Autoimmune encephalitis is an emerging and unique clinical entity that causes severe neuropsychiatric symptoms and results in significant morbidity and mortality. […] This progresses to prominent psychiatric symptoms, which can initially be indistinguishable from a psychotic break, including: agitation, disinhibited behavior, and most commonly florid psychosis and complex delusions. […] If left unchecked, this may progress into the middle and then late stages of the illness over weeks to months, which is characterized by steady decline in cognition and mental status leading to fulminant catatonia and then coma associated with extensor posturing. […] Clinical symptoms include a classic constellation of hyponatremia, refractory innumerate unilateral facial and upper arm twitching seizures known as fasciobrachial dystonic seizures, and indolent confusion and memory loss occurring over several months.

• #1

  https://link.springer.com/article/10.1007/s00415-023-11685-3

  Patients with LGI1-antibodies represent the commonest form of autoimmune encephalitis, which likely remains under-recognized due to its frequently insidious onset, the subtle focal seizures and its predilection for elderly males, a demographic not traditionally considered to have a primary autoimmune basis for their disease. These patients most commonly present with frequent, focal seizures […] Crucially, focal seizures precede limbic encephalitis (LE) in around 75% of cases, presenting an opportunity to alter the natural history of the disease. The natural history of LGI1-antibody encephalitis appears to be the invariable progression from seizures alone to an established LE, with prominent memory disturbance, frequent and ASM-resistant seizures and psychiatric disturbances. As patients progress clinically, their paraclinical investigations become increasingly abnormal, whereas patients with LGI1-antibodies and FBDS alone typically do not have abnormal investigations. Thereafter, increasing cognitive impairment parallels the accumulation of abnormal investigation findings, including hippocampal hyperintensities on T2-weighted MRI, ictal EEG abnormalities, and serum hyponatraemia, due to syndrome of inappropriate anti-diuretic hormone secretion (SIADH). Patients who develop LE are at risk of hippocampal atrophy associated with a fixed memory deficit with concomitant long-term disability. It is this, and a number of other residual cognitive deficits noted in these patients, which are potentially avoidable; it appears that early immunotherapy, particularly with corticosteroids, may prevent the progression from FBDS to LE. Overall, these findings emphasize the importance of neurologists being aware of the focal seizures as an early, treatable clinical presentation.

• #1 Autoimmune Encephalitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK578203/

  As mentioned above, usually, autoimmune encephalitis is treatment-responsive with immunosuppression and tumor removal, although staging of the cancer is independently associated with adverse outcomes. According to an observational cohort study, 94% received first-line therapy with immunosuppression or tumor removal that resulted in improvement within four weeks (53%), with most of them having good outcomes in the first 24 months. These outcomes continue to improve up to 18 months from symptom onset. Predictors of good outcomes were associated with early treatment and not requiring intensive care unit admission. […] Although autoimmune encephalitis is often treatment-responsive, it can lead to serious complications if left untreated or if there is a delay in treatment. This includes cognitive impairment, sleep disorders, hyperkinesia, autonomic dysfunction, persistent amnesia, coma, and status epilepticus.

• #1 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  Most people recover with treatment. The earlier you receive treatment, the more quickly you may recover. Early treatment also lowers the chances of having lasting symptoms due to AE or having another bout of autoimmune encephalitis. […] The long-term outlook can vary from person to person. Full recovery may take months or years. Many people continue to have symptoms related to thinking and behavior for longer than a year. But treatment continues to improve symptoms for 18 months to two years. […] Some people fully recover while others may have lasting symptoms that are mild or more serious. Getting treated early helps improve your long-term outlook. […] People who have recovered from certain types of autoimmune encephalitis, such as anti-NMDA receptor encephalitis and anti-LGI1 encephalitis, are at risk of symptoms coming back. Sometimes symptoms return after several years.

• #1 Autoimmune encephalitis: recovery, residual symptoms and predictors of long-term sequelae | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/early/2025/01/19/jnnp-2024-334957

  Of 172 patients, 138 (80%) achieved good recovery (modified Rankin Scale (mRS) 2) with a median recovery time of 4 months (95% CI: 2 to 6 months). […] Despite good recovery, several residual symptoms persisted 24 months post-AE, including cognitive deficits (53%), seizures (26%), depression (23%), sleep disorders (25%), brainstem/cerebellar symptoms (13%), other movement disorders (14%) and autonomic symptoms (12%). […] While the majority of patients achieved functional independence after AE, several residual symptoms persisted.

• #1 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2023.0242

  Autoimmune etiologies are a common cause for encephalitis. The clinical syndromes consistent with autoimmune encephalitis are both distinct and increasingly recognized, but less is known about persisting sequelae or outcomes. […] The mortality rates were 6%19% and the relapse risks were 10%62%. Most patients achieved a good outcome based on a score on the modified Rankin Scale (mRS) of 2. […] These more-detailed assessments revealed that most patients had persistent impairments, with frequent deficits in cognitive function, especially memory and attention. Depression and anxiety were also common. Many of these sequelae continued to improve over months or even years after the acute illness. […] While we found that lasting impairments were common among survivors of autoimmune encephalitis, additional research is needed to better understand the nature and impact of these sequelae.

• #1 Antibody-mediated autoimmune encephalitis: A practical approach | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/88/8/459

  As the disease progresses, autonomic dysfunction becomes more prominent and commonly necessitates monitoring in an intensive care unit. […] Seizures can occur at any time and are often not responsive to antiepileptic medications alone. […] Approximately 80% of patients require ICU admission. […] From 10% to 25% of patients have a clinical relapse, though symptoms are less severe than in the initial presentation. […] About 50% of patients also develop faciobrachial dystonic seizures characterized by focal or lateralized rapid coordinated movements of an arm or the face that may occur hundreds of times a day. […] Autonomic hyperactivity has also been reported. This includes hyperhidrosis, tachycardia, blood pressure lability, and urinary dysfunction. […] The initial workup for anti-LGI1 encephalitis includes MRI, EEG, and CSF analysis but is usually unrevealing aside from a mild hyponatremia on basic metabolic testing. […] This syndrome characteristically responds briskly to tier 1 treatments (corticosteroids, intravenous immunoglobulin, plasmapheresis), but 20% to 30% of patients may experience a relapse necessitating long-term immunosuppression.

• #1 Autoimmune Encephalitis Testing Recommendations

  https://testguide.labmed.uw.edu/guideline/autoimmune_encephalitis

  Acute and subacute encephalitis are a debilitating group of inflammatory disorders of the brain with a complex differential diagnosis. They often develop as a rapidly progressive encephalopathy, usually in less than 6 weeks, with memory deficits or altered mental status. […] The most common (40-60%) of these disorders is NMDA-receptor autoimmune encephalitis. Anti-NMDA receptor encephalitis is associated with CSF IgG antibodies against the GluN1 subunits of the glutamate NMDA receptor. The disease predominantly affects young individuals (95% younger than 45 years) with a female sex predominance of 4:1. Approximately half of women older than 18 years had an underlying tumor, usually an ovarian teratoma. […] There is little evidence to support monitoring titers of these antibodies over time. Antibody titers do not necessarily normalize with response to therapy.

• #1 NMDAR-Antibody Encephalitis | Encephalitis International

  https://www.encephalitis.info/types-of-encephalitis/autoimmune-encephalitis/nmdar-antibody-encephalitis/

  The disease often comes on over 1-4 weeks and mainly affects young people, with around 40% of cases under 18 years of age. Females are affected more often than males. At onset, the most distinctive features include prominent and mixed psychiatric symptoms, seizures, confusion and memory loss. The psychiatric features often include bizarre and rather disturbing behaviours with mood changes, and patients are often initially looked after in mental health hospitals. They may see things which arent there, develop strange beliefs or appear agitated. After this, often 10 to 20 days later, patients develop a movement disorder, variations in blood pressure, heart rate, temperature and loss of awareness. […] The movement disorder often consists of continuous writhing and twitching of face and limbs but can also be a generalised slowing-down of movements. Most patients develop several of these features, but very rarely individual patients may experience only a few of these features.

• #1 NMDAR-Antibody Encephalitis | Encephalitis International

  https://www.encephalitis.info/types-of-encephalitis/autoimmune-encephalitis/nmdar-antibody-encephalitis/

  NMDAR-antibody encephalitis is an autoimmune disease that causes psychiatric features, confusion, memory loss and seizures followed by a movement disorder and loss of consciousness. The disease can respond well to various therapies that dampen down the immune system and the removal of an underlying tumour if one is found, but improvement is often slow and persistence and patience are often required to achieve more impressive longer term improvements.

• #1 Autoimmune Encephalitis | Encephalitis International

  https://www.encephalitis.info/types-of-encephalitis/autoimmune-encephalitis/

  In some cases, autoimmune encephalitis can present with a distinct clinical syndrome, such as limbic encephalitis. This condition is characterised by inflammation of the limbic system, leading to significant memory impairment and psychiatric symptoms. […] The long-term outlook for autoimmune encephalitis varies depending on the underlying cause and the effectiveness of treatment. In general, patients who receive prompt and aggressive treatment tend to have better outcomes. However, some patients may experience persistent cognitive impairment, psychiatric symptoms, or abnormal movements, which can impact their quality of life. […] Early recognition and treatment of autoimmune encephalitis are critical to improving outcomes and reducing morbidity and mortality.

• #1 Autoimmune Encephalitis – PsychDB

  https://www.psychdb.com/cl/0-autoimmune-encephalitis/home

  Autoimmune Encephalitis (AE) is a broad term that encompasses a group of autoimmune inflammatory brain disorders. AE is increasingly being recognized as a unique, unrecognized type of encephalitis that affects all age-ranges, often with significant neuropsychiatric symptoms. The course of AE is usually (but not always) subacute. The timeline of symptom onset to clinical attention is usually over the course of several weeks. […] The symptoms of Autoimmune Limbic Encephalitis (ALE) are due to dysfunction of the limbic structures in the brain. This can include short-term memory impairment, behavioural changes, anxiety, depression, psychosis, and seizures. ALE most often occurs in middle-aged adults, but it can individuals of all ages. […] Diagnosis can be made when all 4 of the following criteria have been met (Graus et al. 2016): Subacute onset (rapid progression of less than 3 months) of working memory deficits, seizures, or psychiatric symptoms suggesting involvement of the limbic system.

• #1 Autoimmune encephalitis in Children | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/neurosciences/conditions/autoimmune-encephalitis/

  Children of all ages can have autoimmune diseases that affect the nervous system. Autoimmune diseases, including autoimmune encephalitis, can occur following an infection. They also can be related to a systemic autoimmune disease or a dysfunctional immune system, or no clear health issue. […] Encephalitis is a disease that causes inflammation in the brain. Depending on the parts of the brain affected, symptoms can vary widely. Symptoms may include: Seizures, Behavior or personality changes, Abnormal movements, Difficulty maintaining balance, Trouble thinking or speaking, Weakness, Numbness, Difficulty sleeping. […] There are several different types of encephalitis. When encephalitis occurs without a clear reason, it is called autoimmune encephalitis. […] Anti-NMDA receptor encephalitis is the most common form of autoimmune encephalitis in children. It is caused by the presence of antibodies in a specific part of the nerve cells in the brain, called NMDA receptors. In children, this form of encephalitis can be associated with a tumor called an ovarian teratoma, but it is very rare.

• #1 Autoimmune encephalitis in Children | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/neurosciences/conditions/autoimmune-encephalitis/

  Treatment in the hospital is aimed at reducing the amount of inflammation within your child’s brain. Our team may use high doses of steroids, intravenous immunoglobulin and plasmapheresis. If needed, your child also may receive additional immunotherapies, such as rituximab or cyclophosphamide. […] Long-term follow-up care with our team is important to address possible consequences of encephalitis, such as seizures, mood and personality changes, and learning difficulties.

• #1 Autoimmune encephalitis and psychiatric disorders

  https://researchoutreach.org/articles/autoimmune-encephalitis-and-psychiatric-disorders/

  Psychosis is an altered mental state in which a person loses contact with reality and hallucinates they see, hear, or feel things that are not present or becomes delusional, when they believe in something that is not true. […] More recently, autoimmune encephalitis has been identified as another cause of psychosis. Autoimmune encephalitis describes a group of diseases that happen when the body’s immune system falsely attacks healthy brain nerve cells or neurons, causing inflammation of the brain and severe disruption of its functions. […] The disease often starts as a common viral infection, with flu-like symptoms such as fever and headaches. A few weeks later, psychiatric or neurological signs start to arise, including strange behaviour, loss of memory, impaired speech, seizures, and difficulty moving.

• #1 Autoimmune encephalitis and psychiatric disorders

  https://researchoutreach.org/articles/autoimmune-encephalitis-and-psychiatric-disorders/

  Reisz’s study focused on a pregnant woman whose symptoms initially led to her being admitted to a psychiatric hospital. […] Given the difficulty of diagnosis, it’s no surprise that this clinical presentation is often missed by doctors. […] Since an initial diagnosis of schizophrenia is considered in many cases of autoimmune encephalitis, as it was with this one, finding a way to tell these two conditions apart is of great importance, especially since their treatment is entirely different.

• #1 Autoimmune Encephalitis: Brain & Spinal Inflammation | HSS

  https://www.hss.edu/condition-list_autoimmune-encephalitis.asp

  Inflammation in the brain can cause a variety of symptoms, including neurological and psychiatric problems. Symptoms often vary from patient to patient. They can include an sudden decline in work or school performance, loss of the ability to speak, abnormal body movements or seizures, vision loss, weakness of the arms or legs, and sleep problems. Psychiatric manifestations can range from anxiety and mood changes to psychosis with hallucinations, delusions or catatonia. The symptoms typically develop quickly over weeks to a few months. Long-standing psychiatric issues (for many months or years) are not a sign of autoimmune encephalitis. […] Autoimmune encephalitis (AE) is a type of brain inflammation where the bodys immune system attacks healthy cells and tissues in the brain or spinal cord. It is a rare, complex disease that can cause rapid changes in both physical and mental health.

• #1 Autoimmune Encephalitis

  https://med.uth.edu/neurology/multiple-sclerosis/autoimmune-encephalitis/

  An autoimmune condition in which patients immune system makes antibodies that attack different brain proteins, causing a spectrum of symptoms, which typically progress over several weeks to several months. […] Symptoms depends on what part of the brain is affected. Common symptoms may include confusion, cognitive impairment, personality changes, unusual behavior, seizures and involuntary movements. […] Autoimmune encephalitis can be a severe disease if left untreated. Most patients recover with rapid diagnosis and appropriate treatment.

• #1 Recovery – Autoimmune Encephalitis | IAES

  https://autoimmune-encephalitis.org/recovery/

  Even in those patients classified as having a good neurological outcome in Autoimmune Encephalitis, (mRS 0–2), cognitive recovery was incomplete with deficits in executive function and memory common and more severe in those with delayed treatment. This would suggest that the initial part of the illness may be critical in terms of neuronal damage and long-term disability which is why it is so important to be aware of autoimmune encephalitis as a culprit during the disease’s earlier psychiatric presentation. As AE progresses into the later neurologic stages, the potential for a long-term deficit increases.

• #2 Autoimmune encephalitis | Altru Health System

  https://www.altru.org/health-library/conditions/autoimmune-encephalitis

  Autoimmune encephalitis symptoms can vary but may include memory loss, changes in thinking, changes in behavior and seizures. […] Autoimmune encephalitis symptoms can occur in a pattern that is predictable depending on the type of autoimmune encephalitis. Many people have a headache, fever and other symptoms of an infection followed by psychiatric symptoms that may include anxiety, panic attacks, changes in behavior, agitation, hallucinations, delusions and trouble organizing thoughts. […] Autoimmune encephalitis symptoms can get worse over time. This disease course is known as progressive. Symptoms also might alternate between getting better and getting worse. This course is known as relapsing-remitting. […] Sometimes AE causes serious seizures that need emergency care, known as status epilepticus. These seizures last more than five minutes or occur one after another while the person is not conscious.

• #2 Autoimmune Encephalitis Symptoms | AE Alliance

  https://aealliance.org/patient-support/symptoms/

  The early stages of autoimmune encephalitis (AE) vary from person to person. Some people rapidly develop new or changing symptoms. For example, a person with AE may start experiencing hallucinations and then develop seizures or problems with their memory. Symptoms may sometimes fluctuate (or alternate between improving and worsening) early in the disease process. During this stage, people with AE are often diagnosed with a more common diagnosis that would cause their symptom(s). For instance, a person with new seizures may be diagnosed with epilepsy and a person who has uncontrolled movements may be diagnosed with a tic disorder or Tourette syndrome. […] Many people require numerous visits with different health care providers as symptoms worsen or new symptoms develop before AE is considered and a work-up initiated. While this is frustrating, due to the nature of AE and the overlap in symptoms with many more common conditions, it is often not until a person has developed several symptoms of AE that this rare condition is considered as a possible cause of these changes. Many people in the early stages of the disease will need to stay overnight in hospital for tests and to be treated for severe symptoms.

• #2

  https://link.springer.com/article/10.1007/s00415-023-11685-3

  Patients with LGI1-antibodies represent the commonest form of autoimmune encephalitis, which likely remains under-recognized due to its frequently insidious onset, the subtle focal seizures and its predilection for elderly males, a demographic not traditionally considered to have a primary autoimmune basis for their disease. These patients most commonly present with frequent, focal seizures […] Crucially, focal seizures precede limbic encephalitis (LE) in around 75% of cases, presenting an opportunity to alter the natural history of the disease. The natural history of LGI1-antibody encephalitis appears to be the invariable progression from seizures alone to an established LE, with prominent memory disturbance, frequent and ASM-resistant seizures and psychiatric disturbances. As patients progress clinically, their paraclinical investigations become increasingly abnormal, whereas patients with LGI1-antibodies and FBDS alone typically do not have abnormal investigations. Thereafter, increasing cognitive impairment parallels the accumulation of abnormal investigation findings, including hippocampal hyperintensities on T2-weighted MRI, ictal EEG abnormalities, and serum hyponatraemia, due to syndrome of inappropriate anti-diuretic hormone secretion (SIADH). Patients who develop LE are at risk of hippocampal atrophy associated with a fixed memory deficit with concomitant long-term disability. It is this, and a number of other residual cognitive deficits noted in these patients, which are potentially avoidable; it appears that early immunotherapy, particularly with corticosteroids, may prevent the progression from FBDS to LE. Overall, these findings emphasize the importance of neurologists being aware of the focal seizures as an early, treatable clinical presentation.

• #2 The Diagnosis and Treatment of Autoimmune Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4712273/

  Autoimmune encephalitis causes subacute deficits of memory and cognition, often followed by suppressed level of consciousness or coma. […] Patients generally have impaired memory and cognition over a period of days or weeks. […] The classical presentation of encephalitis consists of a subacute (days to a few weeks) progressive decrease in the level of consciousness, often with fluctuations, and altered cognition. Memory, especially retention of new information, may be impaired early in the clinical course. Patients may progress to coma. […] Psychiatric manifestations are common early in the course of autoimmune encephalitis. These may include psychosis, aggression, inappropriate sexual behaviors, panic attacks, compulsive behaviors, euphoria or fear. Symptoms may fluctuate rapidly. […] Abnormal movements may be the presenting symptom in several types of autoimmune encephalitis.

• #2 Antibody-mediated autoimmune encephalitis: A practical approach | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/88/8/459

  As the disease progresses, autonomic dysfunction becomes more prominent and commonly necessitates monitoring in an intensive care unit. […] Seizures can occur at any time and are often not responsive to antiepileptic medications alone. […] Approximately 80% of patients require ICU admission. […] From 10% to 25% of patients have a clinical relapse, though symptoms are less severe than in the initial presentation. […] About 50% of patients also develop faciobrachial dystonic seizures characterized by focal or lateralized rapid coordinated movements of an arm or the face that may occur hundreds of times a day. […] Autonomic hyperactivity has also been reported. This includes hyperhidrosis, tachycardia, blood pressure lability, and urinary dysfunction. […] The initial workup for anti-LGI1 encephalitis includes MRI, EEG, and CSF analysis but is usually unrevealing aside from a mild hyponatremia on basic metabolic testing. […] This syndrome characteristically responds briskly to tier 1 treatments (corticosteroids, intravenous immunoglobulin, plasmapheresis), but 20% to 30% of patients may experience a relapse necessitating long-term immunosuppression.

• #2 Autoimmune Encephalitis: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/autoimmune-encephalitis

  Autoimmune encephalitis can cause a wide range of symptoms because of the various types. Symptoms typically develop and evolve over weeks to months. […] Possible symptoms of AE include: Progressive decline in thinking (cognitive) abilities, Memory issues, Confusion, Seizures (which often dont respond to antiseizure medication), Abnormal movements, like repetitive movements of your mouth and face, muscle twitches and an exaggerated startle response, Issues with using language (aphasia), Problems with balance or coordination (ataxia), Psychosis, which can involve hallucinations, delusions and paranoia. […] Its important to get medical help right away if you develop these symptoms. […] The effectiveness of treatment for AE varies from case to case. Some people with AE respond very quickly to the first medications they receive within days. Others will need treatment that lasts for weeks or months.

• #2 Autoimmune Encephalitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK578203/

  Autoimmune encephalitis (AIE) is an immune-mediated condition that induces brain inflammation and is one of the most common causes of non-infectious encephalitis. AIE commonly presents as a new onset of memory loss, psychosis, altered mental status, or seizures, with the presentation taking place over a few weeks to three months. […] Approximately 70% of patients have prodromal manifestations of headache, fever, and other symptoms resembling viral etiology. Then, it progresses with acute to subacute onset of memory deficits, altered mental status, behavior changes, psychosis, orofacial dyskinesia, hallucinations, agitation, delusions, catatonia, seizures, or abnormal movements. These patients may have a fluctuating clinical course with interval improvement. […] Patients might progress to a decreased level of consciousness, central hypoventilation requiring mechanical ventilation.

• #2

  https://link.springer.com/article/10.1007/s00702-020-02258-z

  The four criteria are: (1) subacute onset of symptoms characterized by working memory deficits, seizures, or psychiatric symptoms. […] The differential diagnosis of autoimmune encephalitis is often demanding, and other diseases must be carefully ruled out in every patient. […] Patients with autoimmune encephalitis associated with antibodies against membrane surface antigens are more often affected by psychiatric symptoms than are those with autoimmune encephalitis associated with autoantibodies against intracellular antigens. […] In NMDAR encephalitis, the psychiatric symptoms even precede the appearance of neurologic symptoms. […] A typical constellation is an abrupt onset of psychiatric symptoms such as delusions, amnesia or agitation with no prior psychiatric history. […] The most frequent symptoms were mood dysfunction in 70%, delusions in 74%, aggression in 57% and auditory or visual hallucinations in 43%.

• #2 Autoimmune Encephalitis Symptoms | AE Alliance

  https://aealliance.org/patient-support/symptoms/

  Neurologic symptoms: Problems with memory and the process of thinking (also known as cognition), Abnormal movements, Seizures, Problems with balance or coordination (or ataxia), Having trouble speaking, Changes in vision, Loss of consciousness or coma. […] Psychiatric symptoms: Hallucinations, delusions, or paranoia (or psychosis), Aggressive behavior, Inappropriate sexual behaviors, Anxiety or panic attacks, Compulsive behaviors, Agitation, fear, Difficulty sleeping. […] One of the hallmarks of AE is a rapid development of symptoms and progression over time for most causes of AE. However, other neurological and psychiatric conditions may present with a sudden onset of symptoms or quickly worsening symptoms.

• #2 Autoimmune encephalitis: clinical spectrum and management | Practical Neurology

  https://pn.bmj.com/content/21/5/412

  In the acute phase, many patients with encephalitis show disorientation, confusion, confabulation and amnesia, features that may relate to the dense expression of many autoantigens in limbic structures, particularly the hippocampus. […] Seizures occur in most autoimmune encephalitis syndromes and are a common factor that triggers neurological attention. […] The autoimmune encephalitis syndromes may show a diverse spectrum of movement disorder phenomenologies. […] Dysautonomia is a common feature to many of these disorders. These symptoms are typically progressive through the initial disease course and can be life-threatening, requiring close monitoring. […] In our experience, pain is under-recognised in the autoimmune encephalitis syndromes particularly in patients with autoantibodies to CASPR2.

• #2 Autoimmune Encephalitis | Encephalitis International

  https://www.encephalitis.info/types-of-encephalitis/autoimmune-encephalitis/

  The signs and symptoms of autoimmune encephalitis can vary widely depending on the individual and the underlying cause of the condition. Common symptoms include: […] Psychiatric symptoms: These can range from psychosis and depression to anxiety, significantly impacting the patients mental health. […] Seizures: These can be focal or generalised, adding to the complexity of the condition. […] Abnormal movements: Patients may experience tremors, rigidity, and chorea, which can interfere with daily activities. […] Cognitive impairment: Memory loss and difficulty with concentration are common, affecting the patients ability to function normally. […] Autonomic dysfunction: Changes in heart rate and blood pressure can occur, complicating the clinical picture. […] Sleep disturbances: Insomnia and hypersomnia are frequent, further affecting the patients quality of life.

• #2 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  Autoimmune encephalitis symptoms can vary but may include memory loss, changes in thinking, changes in behavior and seizures. […] Autoimmune encephalitis symptoms can occur in a pattern that is predictable depending on the type of autoimmune encephalitis. Many people have a headache, fever and other symptoms of an infection followed by psychiatric symptoms that may include anxiety, panic attacks, changes in behavior, agitation, hallucinations, delusions and trouble organizing thoughts. […] Autoimmune encephalitis symptoms can get worse over time. This disease course is known as progressive. Symptoms also might alternate between getting better and getting worse. This course is known as relapsing-remitting. […] Sometimes AE causes serious seizures that need emergency care, known as status epilepticus. These seizures last more than five minutes or occur one after another while the person is not conscious.

• #2

  https://link.springer.com/article/10.1007/s00415-023-11685-3

  In NMDAR-AbE, neuropsychiatric symptoms are often preceded by viral prodromal symptoms, such as headache, fever, myalgia, and coryza, which usual occur around 4-14 days prior to disease onset. This is followed by an acute neuropsychiatric presentation with subsequent seizures and cognitive impairment. Around 14 weeks later, patients commonly develop a characteristic movement disorder, dysautonomia, and coma, which often precipitate intensive-care unit admission. […] Understanding longer term issues which patients encounter continues to be an area of active research with important everyday implications. For example, in NMDAR-AbE, it is widely accepted that treatments are generally highly effective. However, detailed neuropsychological assessments at long-term follow-up have demonstrated that ~80% of patients had moderate-to-severe cognitive impairment, with prominent deficits in executive function and memory. This challenges the current perception of this disease as showing a complete response to immunotherapy.

• #2 Autoimmune encephalitis: clinical spectrum and management | Practical Neurology

  https://pn.bmj.com/content/21/5/412

  The importance of early recognition and diagnosis in autoimmune encephalitis is paramount to the ultimate goal of optimal immunotherapy. […] Early treatment with immunotherapy has shown great promise for preventing otherwise incipient cognitive impairment and functional disability. […] In NMDAR-antibody encephalitis, early treatment independently predicted good outcome (modified Rankin score 2) whereas delays in immunotherapy of 4 weeks were associated with poor functional outcomes at 1 year.

• #2 Autoimmune Encephalitis: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/autoimmune-encephalitis

  In general, getting treatment early in the course of autoimmune encephalitis tends to decrease your risk of long-term complications and relapse. […] Autoimmune encephalitis generally responds well to treatment and goes away. But this can take a long time for some people. AE can also relapse (come back), especially if you have cancer that’s not responding to treatment.

• #2 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2023.0242

  A recent large meta-analysis of 1,059 patients with a median follow-up of 12 months found that 71.5% had a good outcome, defined as an mRS score of 02, with mortality and relapse rates of 6% and 13%, respectively. […] Longitudinal studies using high-quality neurocognitive assessments are increasingly being performed, although most are limited by their retrospective designs and small samples. […] Most of those studies found persisting cognitive impairments despite patients improving to a good outcome according to mRS. […] The proportion with severe impairment had decreased significantly by the second study visit after a median of 2.1 years. […] Many of the included patients had psychiatric sequelae at their last follow-up, with studies finding at least some type of psychiatric or behavioral disturbance in 33%79%.

• #2 Diagnosis and Management of Autoimmune Encephalitis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/diagnosis-and-management-of-autoimmune-encephalitis

  Patients with AE can present with a host of different symptoms. […] A diagnosis of AE should be considered in patients presenting with progressive neurocognitive symptoms evolving over the course of weeks to months. Typical features include cognitive dysfunction (poor short-term memory, attention deficits), and seizures which are often refractory to antiseizure medication. […] Additional features include dysautonomia (labile blood pressure, tachycardia), extrapyramidal manifestations (cerebellar ataxia, dyskinesias, dystonia, choreiform movements), sleep disorders (insomnia, hypersomnolence, sleep-disordered breathing and narcolepsy) and neuropsychiatric symptoms (mania, psychosis, agitation, catatonia – predominantly seen in anti-NMDA-R encephalitis). […] Broadly speaking, AE associated with intracellular antigens portends a poor outcome and are less responsive to immune therapies. Conversely, patients with NAA targeting cell surface or synaptic antigens have a better response to immune therapy and if treated in a timely fashion show good long-term functional outcomes. […] There are several important clinical long-term sequelae arising from AE which impact quality of life. Neurocognitive symptoms (e.g. impaired concentration, memory problems, processing speed), mood disorders, sleeping difficulties, seizures, and fatigue are commonly seen.

• #2 Recovery – Autoimmune Encephalitis | IAES

  https://autoimmune-encephalitis.org/recovery/

  A longitudinal study for cognitive outcome has now been produced with 43 anti-NMDAr patients. All patients had cognitive deficits about 2 years after disease onset, mainly affecting memory and executive function. After 4 years, moderate or severe cognitive deficits persisted in 2/3 of patients despite good functional neurological outcome. Impaired cognitive outcome was predicted by delayed treatment and higher disease severity. However, continued improvement of cognitive function was observed for several years after disease onset in some patients. AE has a 7% mortality rate. AE-related deaths during the acute stage or follow up after discharge from the hospital have also been noted. Even if patients survive without immunotherapy, they may suffer a slower recovery requiring prolonged hospitalization.

• #2 The Diagnosis and Treatment of Autoimmune Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4712273/

  Seizures are common in autoimmune encephalitis and may be a presenting symptom. […] Cerebellitis may occur with infectious causes, but the presentation of a subacute cerebellar syndrome portends a good probability a specific autoimmune etiology and also a significant risk of tumors. […] Certain types of autoimmune encephalitis may precede or follow neuromuscular manifestations, particularly acquired neuromyotonia (Isaacs syndrome). […] In autoimmune encephalitis, relapse tends to follow a similar clinical course to the initial attack. In anti-NMDAR encephalitis, these relapse tend to be milder than the initial attack and manifest with confusion, worsening memory, personality change, hallucinations or new seizures.

• #2 Autoimmune Encephalitis: What Psychiatrists Need to Know

  https://www.psychiatrictimes.com/view/autoimmune-encephalitis-what-psychiatrists-need-know

  Clinical symptoms share characteristics with anti-NMDA receptor encephalitis, with early onset of confusion, psychosis, visual hallucinations, and personality change followed by frank and focal neurologic deficits such as hemiparesis, aphasia, or ataxia, fulminant confusion, catatonia, coma, posturing, and seizures. […] Neuropsychiatric symptoms often precede any overt evidence or diagnosis of tumor by several months, and commonly manifest as subacute depression, irritability, hallucinations, or memory loss occurring over weeks to months. […] Recovery is often a months-to-years long process, especially for patients with fulminant manifestations such as coma and catatonia.

• #2 Case report of anti-NMDA receptor encephalitis in a 24-year-old female: an uncommon presentation | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text

  https://ejnpn.springeropen.com/articles/10.1186/s41983-022-00512-7

  Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis. Due to the variability of the initial symptoms, anti-NMDAR encephalitis is not only underdiagnosed but also can be misdiagnosed as viral encephalitis or other pathologies. […] The clinical presentation of this disease is manifested by non-specific influenza symptoms, such as headache, fever, nausea and upper respiratory symptoms. Subsequently, acute psychiatric symptoms appear, such as agitation, visual and auditory hallucinations, anxiety, emotional lability, catatonia and disorganized thoughts. Neurological deterioration typically occurs 13 weeks after the onset of symptoms, including abnormal movements, seizures and autonomic nervous system disorders with tachycardia, bradycardia, hyperhidrosis, hypersalivation, tension instability.

• #2 Autoimmune Encephalitis – PsychDB

  https://www.psychdb.com/cl/0-autoimmune-encephalitis/home

  Autoimmune Encephalitis (AE) is a broad term that encompasses a group of autoimmune inflammatory brain disorders. AE is increasingly being recognized as a unique, unrecognized type of encephalitis that affects all age-ranges, often with significant neuropsychiatric symptoms. The course of AE is usually (but not always) subacute. The timeline of symptom onset to clinical attention is usually over the course of several weeks. […] The symptoms of Autoimmune Limbic Encephalitis (ALE) are due to dysfunction of the limbic structures in the brain. This can include short-term memory impairment, behavioural changes, anxiety, depression, psychosis, and seizures. ALE most often occurs in middle-aged adults, but it can individuals of all ages. […] Diagnosis can be made when all 4 of the following criteria have been met (Graus et al. 2016): Subacute onset (rapid progression of less than 3 months) of working memory deficits, seizures, or psychiatric symptoms suggesting involvement of the limbic system.

• #2 Autoimmune encephalitis in Children | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/neurosciences/conditions/autoimmune-encephalitis/

  Children of all ages can have autoimmune diseases that affect the nervous system. Autoimmune diseases, including autoimmune encephalitis, can occur following an infection. They also can be related to a systemic autoimmune disease or a dysfunctional immune system, or no clear health issue. […] Encephalitis is a disease that causes inflammation in the brain. Depending on the parts of the brain affected, symptoms can vary widely. Symptoms may include: Seizures, Behavior or personality changes, Abnormal movements, Difficulty maintaining balance, Trouble thinking or speaking, Weakness, Numbness, Difficulty sleeping. […] There are several different types of encephalitis. When encephalitis occurs without a clear reason, it is called autoimmune encephalitis. […] Anti-NMDA receptor encephalitis is the most common form of autoimmune encephalitis in children. It is caused by the presence of antibodies in a specific part of the nerve cells in the brain, called NMDA receptors. In children, this form of encephalitis can be associated with a tumor called an ovarian teratoma, but it is very rare.

• #2 Autoimmune encephalitis and psychiatric disorders

  https://researchoutreach.org/articles/autoimmune-encephalitis-and-psychiatric-disorders/

  Reisz’s study focused on a pregnant woman whose symptoms initially led to her being admitted to a psychiatric hospital. […] Given the difficulty of diagnosis, it’s no surprise that this clinical presentation is often missed by doctors. […] Since an initial diagnosis of schizophrenia is considered in many cases of autoimmune encephalitis, as it was with this one, finding a way to tell these two conditions apart is of great importance, especially since their treatment is entirely different.

• #2 Autoimmune (including paraneoplastic) encephalitis: Management – UpToDate

  https://www.uptodate.com/contents/autoimmune-including-paraneoplastic-encephalitis-management/print

  Autoimmune encephalitis is a heterogeneous entity that includes paraneoplastic, idiopathic, drug-induced, and postviral causes, often but not always in association with antibodies against either intracellular or cell-surface neuronal antigens. Patients can have severe and wide-ranging manifestations that include mental status changes, psychiatric symptoms, seizures, movement disorders, sleep disruption, dysautonomia, and hypoventilation. […] The initial and long-term management of autoimmune encephalitis, including paraneoplastic encephalitis, will be reviewed here. Clinical features and diagnosis are reviewed separately.

• #2 Mayo Clinic Health Library – Autoimmune encephalitis | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20117612

  Autoimmune encephalitis symptoms can vary but may include memory loss, changes in thinking, changes in behavior and seizures. […] Many people have a headache, fever and other symptoms of an infection followed by psychiatric symptoms that may include anxiety, panic attacks, changes in behavior, agitation, hallucinations, delusions and trouble organizing thoughts. […] Autoimmune encephalitis symptoms can get worse over time. This disease course is known as progressive. Symptoms also might alternate between getting better and getting worse. This course is known as relapsing-remitting. […] Sometimes AE causes serious seizures that need emergency care, known as status epilepticus. These seizures last more than five minutes or occur one after another while the person is not conscious. […] Most people recover with treatment. The earlier you receive treatment, the more quickly you may recover. Early treatment also lowers the chances of having lasting symptoms due to AE or having another bout of autoimmune encephalitis.

• #2 Autoimmune Encephalitis – PsychDB

  https://www.psychdb.com/cl/0-autoimmune-encephalitis/home

  Early identification of autoimmune encephalitis is important, so immunotherapy can begin quickly. Early treatment is associated with decreased seizure frequency, faster cognitive improvement, and improved survival. […] Diagnosis can be made when all 3 of the following criteria have been met (Graus et al. 2016): Subacute onset (rapid progression of less than 3 months) of working memory deficits (short-term memory loss), altered mental status, or psychiatric symptoms.

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