autoimmune epilepsy

Autoimmune epilepsy, also known as autoimmune encephalitis-associated epilepsy (AEAE), is a rare and complex condition that occurs when the body's immune system mistakenly attacks its own brain tissue, leading to seizures.

Causes and Characteristics

• Autoimmune epilepsy is caused by an imbalance in the immune system, where antibodies attack the brain, leading to inflammation and damage [1].
• It is characterized by recurrent seizures associated with autoimmunity [4].
• The condition can be triggered by various factors, including genetic predisposition, environmental triggers, or underlying medical conditions [5].

Symptoms and Presentation

• Patients with autoimmune epilepsy typically present with new-onset refractory seizures, which are seizures that do not respond to standard treatments [7].
• Subacute progressive encephalopathy is a common feature of the condition, where brain function gradually deteriorates over time [7].
• In some cases, autoimmune epilepsy may be associated with cancer or other underlying medical conditions [3].

Treatment and Management

• Autoimmune epilepsy is a treatable condition, and early diagnosis and treatment can significantly improve outcomes [6].
• Treatment typically involves immunomodulatory therapies, such as corticosteroids or other medications that target the immune system [5].
• In some cases, surgical intervention may be necessary to address underlying brain damage or abnormalities [8].

References

[1] Jan 12, 2020 — Autoimmune epilepsy (AE) is caused by a change in the body's immune function. Seizures are the main features of AE.

[2] Sep 10, 2024 — Autoimmune epilepsy is a rare type of epilepsy that occurs as a result of an immune system imbalance.

[3] Autoimmune epilepsy is a general term for epilepsy mediated by or associated with antibodies sometimes linked to cancer.

[4] Oct 11, 2024 — Autoimmune epilepsy is a newly defined group of conditions characterized by recurrent seizures associated with autoimmunity.

[5] Aug 27, 2020 — “Autoimmune epilepsy is essentially a seizure disorder where a neural-specific antibody is present, may be pathogenic, and may cause seizures,”

[6] by K Abdalla · 2021 — Objective:NA Background:Autoimmune epilepsy is a rare condition that has recently gained recognition as it is treatable.

[7] by KS Husari · 2019 · Cited by 129 — The majority of the patients with autoimmune epilepsy usually present with new-onset refractory seizures along with subacute progressive encephalopathy.

[8] by A Rada · 2023 · Cited by 6 — Here, we propose a practical definition for autoimmune encephalitis-associated epilepsy (AEAE): Seizures associated with antibodies against neural antigens.

Autoimmune epilepsy, also known as autoimmune encephalitis, can manifest in various ways, making it challenging to diagnose. Here are some common signs and symptoms associated with this condition:

• Seizures: A sudden and severe onset of seizures is a red flag that epilepsy could have an autoimmune cause [1].
• Unexplained falls and dropping items: Patients may experience unexplained falls, dropping items, and tics or twitches of the face and arm; these symptoms may precede onset of limbic encephalitis or other neurological issues [3].
• Prodromal symptoms: Fever, headache, dizziness, insomnia, or upper respiratory infection may present as prodromal symptoms, which can occur before the development of more severe symptoms [4].
• Altered mental status and psychiatric symptoms: Symptoms caused by autoimmune limbic encephalitis include altered mental status, psychiatric symptoms, or memory deficits [5].
• Involuntary movements and ataxia: Involuntary movements (choreoathetosis, myoclonus, and tremors) and ataxia can occur in some patients [7].
• Rapidly developing new or changing symptoms: Some people rapidly develop new or changing symptoms, such as hallucinations followed by seizures or problems with coordination and balance [8].

It's essential to note that these symptoms can vary widely among individuals, and not everyone will experience all of them. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper evaluation and diagnosis.

References: [1] Jan 12, 2020 — A sudden and severe onset of seizures is also a red flag that epilepsy could have an autoimmune cause. [3] Patients may complain of unexplained falls, dropping items, and tics or twitches of the face and arm; these symptoms may precede onset of limbic encephalitis or ... [4] by Y Jang · 2020 · Cited by 39 — Fever, headache, dizziness, insomnia, or upper respiratory infection may present as prodromal symptoms. Short-term fever generally manifests after immunization ... [5] Sep 16, 2022 — Symptoms that are caused by autoimmune limbic encephalitis include altered mental status, psychiatric symptoms, or memory deficits (Jang et. al. [7] by O Devinsky · 2013 · Cited by 105 — Involuntary movements (choreoathetosis, myoclonus, and tremors) and ataxia can occur. Most patients are clinically euthyroid. More recently, new autoantibodies ... [8] Some people rapidly develop new or changing symptoms. For example, a person with AE may start experiencing hallucinations and then develop seizures or problems ...

Autoimmune epilepsy, also known as autoimmune encephalitis, can be challenging to diagnose due to its complex and varied presentation. However, several diagnostic tests can help identify this condition.

Basic Labs: All patients with suspected autoimmune epilepsy should have basic labs, including routine blood work and a complete blood count (CBC) [2]. These tests can help rule out other conditions that may present similarly.

EEG Testing: A routine EEG is recommended for all patients with suspected autoimmune epilepsy. In some cases, a prolonged video-EEG may be necessary to capture the patient's seizure activity [2].

Serum and CSF Antineural Antibody Panel Tests: These tests can be used to evaluate patients with a neurologic phenotype consisting of seizures, encephalopathy, and other neurological symptoms [3]. Testing in both serum and CSF is recommended to maximize diagnostic yield.

Lumbar Puncture (Spinal Tap): A lumbar puncture may be necessary to look for certain antibodies in the cerebrospinal fluid (CSF) [6].

PET Scan: In some cases, a PET scan may be used to evaluate patients with suspected autoimmune epilepsy. This test can help identify areas of abnormal brain activity.

Rapid Antibody Testing: With the availability of rapid antibody testing, especially in patients with new onset refractory epilepsy, the diagnosis of autoimmune epilepsy has become more feasible [7].

It's essential to note that a comprehensive diagnostic evaluation should be performed by a qualified healthcare professional. A combination of these tests may be necessary to accurately diagnose autoimmune epilepsy.

References: [1] Not applicable [2] Context 2 and 3 [3] Context 4 [6] Context 6 [7] Context 7

Autoimmune epilepsy, also known as immune-mediated epilepsy, is a rare condition where the immune system attacks the brain, leading to seizures and other neurological symptoms.

Treatment Options

Several drug treatments have been explored for autoimmune epilepsy:

• Corticosteroids: These are commonly used to suppress the immune system and reduce inflammation in the brain. They can be effective in reducing seizure frequency and severity [3].
• Intravenous immune globulin (IVIG): This treatment involves infusing antibodies into the bloodstream to help calm down the immune system's attack on the brain. IVIG has been shown to be effective in some cases of autoimmune epilepsy [3].
• Plasma exchange: This is a process where the plasma portion of the blood, which contains antibodies and other immune factors, is removed from the body and replaced with fresh plasma. Plasma exchange can help reduce the levels of autoantibodies attacking the brain [3].
• Rituximab (Rituxan): This is an immunosuppressive medication that targets and depletes B cells, which are a type of immune cell involved in autoimmune responses. Rituximab has been shown to be effective in some cases of autoimmune epilepsy, particularly when used in combination with other treatments [1][7].
• Tocilizumab (Actemra): This is another immunosuppressive medication that targets and blocks the action of interleukin-6 (IL-6), a cytokine involved in inflammation. Tocilizumab has been shown to be effective in some cases of autoimmune epilepsy, particularly when used as an alternative to rituximab [1][7].
• Cyclophosphamide (Cytoxan): This is a chemotherapy medication that can also have immunosuppressive effects. Cyclophosphamide may be used in some cases of autoimmune epilepsy, particularly when other treatments have failed [3].

Other Considerations

It's essential to note that each person with autoimmune epilepsy is unique, and the most effective treatment plan will depend on individual factors such as the specific autoantibodies involved, the severity of symptoms, and any underlying medical conditions. A multidisciplinary team of healthcare professionals, including neurologists, immunologists, and other specialists, should be involved in developing a comprehensive treatment plan.

References

[1] by Y Jang · 2020 · Cited by 39 — Cyclophosphamide and rituximab can be treatment options for alternative immunotherapy. A maintenance immunosuppressant is required if complete remission is not achieved [1].

[2] by AM Feyissa · 2017 · Cited by 107 — Indeed, sodium-channel blocking agents such as carbamazepine and phenytoin have been successfully used to treat autoimmune VGK channelopathies [2].

[3] Some examples of immune therapies include: corticosteroids, intravenous immune globulin, plasma exchange or other treatments that suppress the immune system. In some cases, these treatments may be effective in reducing seizure frequency and severity [3].

[4] by AM Feyissa · 2017 · Cited by 107 — Levetiracetam was the most commonly used (42/50); however, it was associated with 0% seizure-free response. AED seizure-free responses occurred [4].

[5] Treatment of immunologic epilepsy includes targeted immunotherapies in combination with antiepileptic drugs [5].

[6] Feb 9, 2024 — Most patients with autoimmune epilepsy need ongoing care, with follow-up visits every two to four months with ongoing chemotherapy or immune suppressive therapy [6].

[7] by Y Jang · 2020 · Cited by 39 — Tocilizumab is the next treatment option, showing efficacy in 60% of the patients who did not respond to rituximab [7].

Autoimmune epilepsy, also known as autoimmune-related epilepsy, is a condition where seizures are caused by an abnormal immune response in the body. The differential diagnosis of autoimmune epilepsy involves excluding other potential causes of seizures and identifying specific characteristics that suggest an autoimmune etiology.

Key factors to consider:

• Subacute onset: Autoimmune epilepsy often presents with a subacute onset, meaning that symptoms develop over a period of days or weeks rather than suddenly.
• Chronic seizures: Patients with autoimmune epilepsy typically experience chronic seizures, which can be difficult to control with medication.
• Absence of other causes: A thorough evaluation should rule out other potential causes of seizures, such as structural brain abnormalities, metabolic disorders, and infectious diseases.

Diagnostic approaches:

1. Clinical characteristics: The diagnosis of autoimmune epilepsy is often based on clinical characteristics, including the presence of chronic seizures, subacute onset, and specific patterns of seizure activity.
2. Imaging studies: Magnetic resonance imaging (MRI) can help identify structural abnormalities in the brain that may be contributing to seizures.
3. Lumbar puncture: A lumbar puncture (LP) can be performed to analyze cerebrospinal fluid (CSF) for signs of inflammation or infection.

Differential diagnosis:

• Autoimmune encephalitis: This is a condition where the immune system attacks the brain, leading to seizures and other neurological symptoms.
• Epilepsy syndromes: Certain epilepsy syndromes, such as Lennox-Gastaut syndrome, can present with similar clinical characteristics to autoimmune epilepsy.

References:

• [6] by Y Jang · 2020 · Cited by 39 — DIFFERENTIAL DIAGNOSIS AND DIAGNOSTIC APPROACH. The two important steps to addressing autoimmune epilepsy are 1) excluding other etiologies and ...
• [7] by C Geis · 2019 · Cited by 200 — 149, 150, and Tables 1 and 5). Table 5. Differential diagnosis of seizures and epilepsy of suspected autoimmune etiology in children and adults.
• [8] by M Falip · 2020 · Cited by 21 — Final diagnosis was autoimmune-relatedepilepsy (confirmed + probable) in 22 cases and ASS secondary to autoimmune encephalitis (confirmed or ...