Autoimmunologiczna padaczka
Leczenie

Autoimmunologiczna padaczka to jednostka chorobowa charakteryzująca się napadami padaczkowymi wywołanymi przez nieprawidłową odpowiedź immunologiczną skierowaną przeciwko komórkom mózgu. Standardowe leki przeciwpadaczkowe (AED) wykazują ograniczoną skuteczność, dlatego podstawą terapii jest immunoterapia. Leczenie pierwszego rzutu obejmuje wysokodawkowe kortykosteroidy (metyloprednizolon 1 g/dobę IV przez 3-5 dni), dożylne immunoglobuliny (IVIg 0,4 g/kg/dobę przez 3-5 dni) oraz plazmaferezę, stosowaną w przypadku przeciwwskazań lub braku odpowiedzi na wcześniejsze metody. W przypadku braku poprawy po 2 tygodniach lub pogorszenia stanu klinicznego wdraża się leki drugiego rzutu, takie jak rytuksymab (1 g IV dwukrotnie w odstępie 2 tygodni), cyklofosfamid (750 mg/m² miesięcznie do 6 miesięcy), tocilizumab czy anakinra. Leczenie podtrzymujące może obejmować mykofenolan mofetylu, azatioprynę, rytuksymab lub cyklofosfamid, a terapia AED, zwłaszcza blokery kanałów sodowych (karbamazepina, fenytoina, okskarbazepina, lakozamid), pełni rolę wspomagającą kontrolę napadów.

  1. Autoimmunologiczna padaczka – leczenie
    1. Immunoterapia pierwszego rzutu
    2. Immunoterapia drugiego rzutu
    3. Leczenie skojarzone i fazy terapii
    4. Leczenie autoimmunologicznej padaczki związanej z nowotworem
    5. Skuteczność leczenia i rokowanie
    6. Leczenie specyficznych typów autoimmunologicznej padaczki
  2. Leczenie chirurgiczne
  3. Postępowanie wielodyscyplinarne i leczenie wspomagające
  4. Monitorowanie i leczenie długoterminowe
  5. Nowe kierunki w leczeniu
  6. Podsumowanie kluczowych zasad leczenia
    1. Kolejne rozdziały

Autoimmunologiczna padaczka – leczenie

Autoimmunologiczna padaczka (ang. autoimmune epilepsy) to stosunkowo nowo opisana jednostka chorobowa, charakteryzująca się napadami padaczkowymi wywołanymi przez nieprawidłową odpowiedź układu immunologicznego skierowaną przeciwko komórkom mózgu. Leczenie tej postaci padaczki różni się znacząco od podejścia terapeutycznego w innych rodzajach padaczki, ponieważ standardowe leki przeciwpadaczkowe (AED) zazwyczaj nie wykazują wystarczającej skuteczności. Podstawą terapii jest immunoterapia, której celem jest zmniejszenie aktywności układu odpornościowego i wyeliminowanie stanów zapalnych w mózgu.123

Immunoterapia pierwszego rzutu

Leczenie pierwszego rzutu w autoimmunologicznej padaczce obejmuje następujące opcje terapeutyczne:123

  • Kortykosteroidy w wysokich dawkach – najczęściej stosowany jest metyloprednizolon dożylnie (IVMP) w dawce 1 g/dobę przez 3-5 dni. Po tej fazie pacjenci często przechodzą na leczenie podtrzymujące z cotygodniowym podawaniem IVMP, które może być kontynuowane przez 4-6 tygodni, a następnie wydłużane są odstępy między dawkami przez kolejne 4-6 miesięcy.12
  • Dożylne immunoglobuliny (IVIg) – podawane w dawce 0,4 g/kg/dobę przez 3-5 dni. Ten rodzaj terapii jest szczególnie preferowany jako pierwsza opcja w przypadku autoimmunologicznego zapalenia mózgu, zwłaszcza gdy podejrzewa się etiologię wirusową, ponieważ IVIg jest skuteczny w autoimmunologicznej padaczce i bezpieczny w zapaleniu mózgu o podłożu wirusowym.12
  • Plazmafereza (PLEX) – procedura polegająca na usunięciu płynnej części krwi i oddzieleniu jej od komórek krwi. Następnie komórki krwi są ponownie wprowadzane do organizmu, a organizm wytwarza nowe osocze. Ta terapia pomaga usunąć przeciwciała powodujące atak układu odpornościowego na komórki mózgu. Plazmafereza jest zazwyczaj zarezerwowana dla pacjentów, którzy mają przeciwwskazania do IVMP lub IVIg, nie zareagowali na te terapie lub mają stan padaczkowy lub ciężkie objawy.12

Wybór konkretnej metody immunoterapii pierwszego rzutu zależy od indywidualnych cech pacjenta, współistniejących chorób oraz typu autoimmunologicznej padaczki. W przypadku braku odpowiedzi na pierwszy zastosowany lek, uzasadnione jest wypróbowanie drugiego. Wczesne rozpoczęcie immunoterapii ma kluczowe znaczenie dla poprawy rokowania i zmniejszenia ryzyka długotrwałych powikłań.12

Immunoterapia drugiego rzutu

Jeśli pacjent nie odpowiada na leczenie pierwszego rzutu w ciągu 2 tygodni lub następuje pogorszenie stanu klinicznego, należy rozważyć wprowadzenie leków drugiego rzutu:123

  • Rytuksymab – przeciwciało monoklonalne skierowane przeciwko komórkom B, podawane w dawce 1g dożylnie, dwa razy w odstępie 2 tygodni. Jest szczególnie skuteczny w leczeniu autoimmunologicznego zapalenia mózgu związanego z przeciwciałami, przyczyniając się do deplecji komórek B, które są prekursorami komórek plazmatycznych wydzielających patogenne przeciwciała.12
  • Cyklofosfamid – stosowany w dawce 750 mg/m² miesięcznie przez okres do 6 miesięcy.1
  • Tocilizumab – przeciwciało monoklonalne przeciwko receptorowi interleukiny-6.1
  • Anakinra – antagonista receptora interleukiny-1.1

W przypadku korzystnej odpowiedzi na leczenie należy rozważyć długotrwałą immunosupresję z zastosowaniem mykofenolanu mofetylu, azatiopryny, rytuksymabu lub cyklofosfamidu w celu zapobiegania nawrotom choroby.12

Leczenie skojarzone i fazy terapii

Leczenie autoimmunologicznej padaczki można podzielić na dwie główne fazy:12

  1. Faza ostra – intensywne leczenie immunomodulujące mające na celu szybkie opanowanie aktywności choroby i napadów padaczkowych.
  2. Faza podtrzymująca – długoterminowe leczenie immunosupresyjne, często trwające kilka lat, mające na celu zapobieganie nawrotom.

W przypadku szybkiej progresji i opornego przebiegu konieczne jest bardziej agresywne podejście terapeutyczne, w tym połączenie zarówno terapii pierwszego, jak i drugiego rzutu.1

Ważnym elementem leczenia jest również zastosowanie leków przeciwpadaczkowych (AED), które mimo ograniczonej skuteczności w autoimmunologicznej padaczce, mogą odgrywać rolę wspomagającą w kontrolowaniu napadów. Badania wskazują, że AED działające poprzez blokowanie kanałów sodowych, takie jak karbamazepina, fenytoina, okskarbazepina i lakozamid, wykazują najlepsze efekty w kontrolowaniu aktywności napadowej.123

Leczenie autoimmunologicznej padaczki związanej z nowotworem

W przypadku gdy autoimmunologiczna padaczka jest związana z nowotworem (postać paraneoplastyczna), leczenie nowotworu stanowi istotny element terapii. Właściwe leczenie choroby nowotworowej jest niezbędne do osiągnięcia kontroli nad napadami padaczkowymi.123

Jeśli autoimmunologiczna padaczka rozwija się u pacjentów z nowotworem, leczenie przeciwnowotworowe powinno być podstawową interwencją, pod warunkiem że autoimmunologiczna padaczka jest odpowiednio kontrolowana. Zazwyczaj wymaga to współpracy między neurologiem a onkologiem w celu opracowania optymalnego planu leczenia.1

Pacjenci z autoimmunologiczną padaczką związaną z nowotworem mają na ogół gorsze rokowanie niż pacjenci, u których rozwija się autoimmunologiczna padaczka o innym podłożu.1

Skuteczność leczenia i rokowanie

Skuteczność leczenia autoimmunologicznej padaczki zależy od wielu czynników, w tym od typu przeciwciał, czasu od wystąpienia objawów do rozpoczęcia leczenia oraz obecności strukturalnych uszkodzeń mózgu.12

Badania wykazują, że:123

  • 67-81% pacjentów z podejrzeniem autoimmunologicznej padaczki leczonych immunoterapią doświadcza poprawy klinicznej
  • 67% pacjentów może osiągnąć całkowitą wolność od napadów po zastosowaniu immunoterapii
  • 75% pacjentów z przeciwciałami przeciwko receptorowi GABA-A ma częściowy lub całkowity powrót do zdrowia przy odpowiednim leczeniu
  • U pacjentów z zapaleniem mózgu z przeciwciałami anty-LGI1, anty-NMDAR i anty-GABABR brak wyładowań padaczkowych w EEG może sugerować możliwość odstawienia leków przeciwpadaczkowych w stabilnych klinicznie przypadkach bez napadów

Wczesne rozpoczęcie immunoterapii jest kluczowym czynnikiem prognostycznym. Opóźnienie w leczeniu może prowadzić do trwałych uszkodzeń mózgu i zmniejszenia szans na całkowitą kontrolę napadów. Na przykład przeciwciało w zapaleniu mózgu z przeciwciałami przeciwko LGI1 czasami powoduje utratę neuronów hipokampa, gdy leczenie jest opóźnione.12

Około 15% pacjentów nadal nie odpowiada dobrze na leczenie immunomodulujące, co wskazuje na potrzebę dalszych badań nad opornymi postaciami autoimmunologicznej padaczki.1

Leczenie specyficznych typów autoimmunologicznej padaczki

Podejście terapeutyczne może różnić się w zależności od konkretnego typu autoimmunologicznej padaczki i związanych z nią przeciwciał:123

  • Padaczka z przeciwciałami anty-NMDAR (receptora N-metylo-D-asparaginowego) – zazwyczaj dobrze reaguje na immunoterapię, zwłaszcza przy wczesnym rozpoczęciu leczenia
  • Padaczka z przeciwciałami anty-LGI1 – na ogół dobrze reaguje na kortykosteroidy, immunoglobuliny dożylne i rytuksymab
  • Padaczka z przeciwciałami anty-GAD65 – często trudniejsza w leczeniu, słabiej odpowiada na immunoterapię i może wymagać długotrwałego leczenia immunosupresyjnego
  • Zespół Rasmussena – zwykle nie reaguje na leki i często wymaga leczenia chirurgicznego, szczególnie u dzieci

Leczenie chirurgiczne

W niektórych przypadkach autoimmunologicznej padaczki, szczególnie w zespole Rasmussena, który dotyka głównie dzieci, leczenie chirurgiczne może być niezbędne, gdy choroba nie odpowiada na leki.12

Najbardziej skutecznym leczeniem w tej desperackiej sytuacji jest czynnościowa hemisferektomia u dzieci, najlepiej wykonana jak najwcześniej, ponieważ plastyczność mózgu niemowlęcego może zmniejszyć poważne następstwa choroby, a także samego leczenia chirurgicznego.1

Inne interwencje chirurgiczne mogą obejmować:12

  • Ablację laserową – używaną w przypadku zidentyfikowania wyraźnego ogniska padaczkowego
  • Neuromodulację – w tym stymulatory nerwów lub inwazyjne stymulatory neuronalne
  • Usunięcie fragmentu mózgu (resekcję) – stosowane w wybranych przypadkach, gdy ognisko padaczkowe jest zlokalizowane w strukturalnie uszkodzonym obszarze mózgu

Należy jednak zauważyć, że badania wskazują, iż wyniki leczenia chirurgicznego w autoimmunologicznej padaczce wydają się być gorsze w porównaniu do innych form padaczki lekoopornej.1

Postępowanie wielodyscyplinarne i leczenie wspomagające

Kompleksowe leczenie pacjentów z autoimmunologiczną padaczką często wymaga podejścia wielodyscyplinarnego, obejmującego różnych specjalistów:123

  • Neurolog epileptolog – koordynujący leczenie
  • Immunolog – pomagający w doborze optymalnej immunoterapii
  • Onkolog – w przypadkach związanych z nowotworem
  • Farmaceuta – zarządzający lekami
  • Pracownik socjalny – łączący rodziny z usługami wsparcia
  • Pielęgniarka koordynująca – pomagająca w koordynacji wizyt i wypełnianiu dokumentacji
  • Psychiatra – pomagający pacjentom i rodzinom w radzeniu sobie ze zmianami osobowości, problemami z pamięcią i zmianami nastroju lub zachowania związanymi z padaczką

Oprócz leczenia podstawowej choroby, ważne jest również leczenie wspomagające:123

  • Rehabilitacja – fizjoterapia, terapia zajęciowa i logopedyczna mogą być bardzo pomocne w poprawie powrotu do zdrowia
  • Leczenie objawów psychiatrycznych – leki przeciwdepresyjne, przeciwlękowe czy przeciwpsychotyczne mogą być potrzebne do kontrolowania objawów psychiatrycznych związanych z autoimmunologicznym zapaleniem mózgu
  • Modyfikacja diety – dieta ketogeniczna może mieć właściwości przeciwzapalne i być stosowana jako terapia wspomagająca
  • Powolne przywracanie do normalnych aktywności – stopniowe wprowadzanie do codziennych czynności i interakcji z bliską rodziną i przyjaciółmi przed powrotem do pracy lub szkoły

Monitorowanie i leczenie długoterminowe

Większość pacjentów z autoimmunologiczną padaczką wymaga długotrwałej opieki, z wizytami kontrolnymi co 2-4 miesiące i kontynuacją leczenia immunosupresyjnego.12

Decyzje dotyczące czasu trwania leczenia podtrzymującego są podejmowane indywidualnie, ale zazwyczaj obejmują:12

  • Początkowy okres leczenia podtrzymującego trwający około 3 lat, po którym następuje ponowna ocena i próba odstawienia immunosupresji
  • W przypadku nawrotu choroby zalecane jest długotrwałe leczenie immunosupresyjne
  • W ciężkich przypadkach, takich jak NMDAR, leczenie immunomodulujące jest często kontynuowane przez około 2 lata

Istotne jest, aby nie odstawiać jednocześnie leków przeciwpadaczkowych i immunosupresyjnych, ponieważ w przypadku wystąpienia napadu nie wiadomo, czy jest on spowodowany uszkodzeniem strukturalnym prowadzącym do padaczki, czy nawrotem autoimmunologicznego zapalenia mózgu wymagającym immunoterapii.12

W przypadku pomyślnej odpowiedzi na immunoterapię i ustąpienia napadów, lekarz prowadzący może zasugerować stopniowe zmniejszanie dawki leków przeciwpadaczkowych przez kilka miesięcy, aby sprawdzić, czy napady powrócą. Wielu pacjentów z autoimmunologicznym zapaleniem mózgu staje się wolnych od napadów po zastosowaniu immunoterapii, chociaż może minąć kilka miesięcy od rozpoczęcia leczenia do ustąpienia napadów.1

Nowe kierunki w leczeniu

W miarę pogłębiania wiedzy na temat patofizjologii autoimmunologicznej padaczki pojawiają się nowe potencjalne opcje terapeutyczne:12

  • Inhibitory dopełniacza – ukierunkowane na kaskadę dopełniacza, która może uczestniczyć w uszkodzeniu neuronalnym
  • Inhibitory cytokin – blokujące cząsteczki sygnałowe układu odpornościowego odpowiedzialne za stan zapalny
  • Bortezomib – inhibitor proteasomu, który może być skuteczny w przypadkach opornych na standardowe terapie
  • Personalizowane terapie oparte na przewidywaniu odpowiedzi na immunoterapię – wykorzystujące skale takie jak RITE (Response to Immunotherapy in Epilepsy), które pomagają przewidzieć, którzy pacjenci mogą lepiej reagować na immunoterapię

Prowadzone są również badania kliniczne nad nowymi terapiami, które mogą przynieść nadzieję w przypadkach opornych na leczenie. Badania te testują innowacyjne leki i podejścia terapeutyczne.1

Podsumowanie kluczowych zasad leczenia

Skuteczne leczenie autoimmunologicznej padaczki opiera się na kilku fundamentalnych zasadach:1234

  • Wczesne rozpoznanie i rozpoczęcie leczenia immunomodulującego ma kluczowe znaczenie dla poprawy wyników leczenia
  • Leczenie obejmuje dwa główne elementy: terapię immunomodulującą modyfikującą przebieg choroby oraz leki przeciwpadaczkowe jako leczenie objawowe
  • Pierwsza linia leczenia obejmuje kortykosteroidy, dożylne immunoglobuliny lub plazmaferezę
  • W przypadku braku odpowiedzi na leczenie pierwszego rzutu należy zastosować leki drugiego rzutu, takie jak rytuksymab lub cyklofosfamid
  • Jeśli przyczyną jest nowotwór, jego leczenie stanowi istotny element terapii
  • Leki przeciwpadaczkowe, szczególnie blokery kanałów sodowych, mogą być stosowane jako leczenie wspomagające
  • Leczenie chirurgiczne może być konieczne w wybranych przypadkach, szczególnie w zespole Rasmussena
  • Wielodyscyplinarne podejście do leczenia zapewnia optymalne wyniki

Długoterminowe monitorowanie i leczenie podtrzymujące są niezbędne do zapobiegania nawrotom, a decyzje terapeutyczne powinny być dostosowane do indywidualnych potrzeb pacjenta na podstawie typu autoimmunologicznej padaczki, obecności przeciwciał oraz odpowiedzi na wcześniejsze leczenie.12

Kolejne rozdziały

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Autoimmune epilepsy – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/autoimmune-epilepsy/diagnosis-treatment/drc-20576912
    Autoimmune epilepsy treatment differs from the treatment used for other types of epilepsy. Healthcare professionals use immunotherapy to reduce the immune system activity and treat seizures. […] If cancer is the cause of autoimmune epilepsy, treating the cancer is an important part of treatment. […] Immunotherapy medicines for autoimmune epilepsy may include: […] Depending on your response to the initial treatments, your healthcare professional also may recommend longer acting medicines that work on the immune system. […] Your healthcare professional also may recommend plasma exchange. […] Although antiseizure medicines don’t work as well in people with autoimmune epilepsy, they may still play a role in your treatment. […] Many people whose seizures are due to autoimmune encephalitis become seizure free after taking immunotherapy. […] Rasmussen syndrome, which mainly affects children, usually doesn’t respond to medicines. Brain surgery often is needed to treat this type of autoimmune epilepsy.
  • #1 Autoimmune Epilepsy
    https://practicalneurology.com/articles/2018-oct/autoimmune-epilepsy
    A newly recognized category of epilepsy caused by or associated with antibodies. Treatment of immunologic epilepsy includes targeted immunotherapies in combination with antiepileptic drugs. Currently, no strict diagnostic guidelines exist for autoimmune epilepsy. This article seeks to provide a basic clinical approach to diagnosis and treatment. Recommendations for tumor screening are antibody specific and covered later in this article, although often a specific antibody is not detected. When no specific antibody is detected, the clinical features and other test results guide diagnosis of autoimmune epilepsy, and broad cancer screening is also performed. Treatment should not be delayed while waiting for results because early treatment results in better outcomes. First-line treatment consists of either intravenous methylprednisolone (IVMP) (1 g/day for 3 to 5 days) or intravenous immunoglobulin (IVIg) (0.4g/kg/day for 3-5 days). If there is no response to the first agent, it is reasonable to try the second. Plasmapheresis (PLEX) is often reserved for those who have a contraindication to IVMP or IVIg, have failed those treatments, and have status epilepticus or severe symptoms. If patients worsen or there is no response within 2 weeks of a first-line therapy, second-line therapies are rituximab (1g IV) given twice 2 weeks apart or cyclophosphamide (750mg/m2 monthly up to 6 months). If there is a favorable response to treatment, chronic immunosuppression with mycophenolate mofetil, azathioprine, rituximab or cyclophosphamide should be considered. Appropriate treatment of an underlying cancer or tumor is also essential to recovery. Recovery can often be gradual and protracted. Many patients are hospitalized for 3 to 4 months followed by months of rehabilitation. Relapses of the disease are also possible.
  • #1 Clinical Approach to Autoimmune Epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7541993/
    IVIg and corticosteroids can be administered as the initial step of immunotherapy. […] The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] Early empirical immunotherapy should be actively considered for these patients. […] The duration of immunotherapy maintenance should also be addressed. […] The considered treatment options can include immunotherapeutics targeting a broad spectrum of immune responses, such as high-dose corticosteroids, IVIg, cyclophosphamide, and methotrexate. […] The presence of a clinicoradiologic mismatch in which there is no or only a minimal MRI lesion but severe clinical deterioration is suggestive of antibody-mediated encephalitis. […] The sequential application of immunotherapies including IVIg, rituximab, tocilizumab, and anakinra should then be cautiously but promptly considered in an hourly and daily fashion.
  • #1 :: JCN :: Journal of Clinical Neurology
    https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519
    The next step is to apply a maximum diagnostic effort. […] Therefore, clinicians should fully discuss the diagnosis and treatment approaches with the patient and their caregivers before deciding whether they will apply immunotherapeutics. […] Early empirical immunotherapy should be actively considered for these patients. […] The duration of immunotherapy maintenance should also be addressed. […] The pathogenesis of autoimmune encephalitis caused by synaptic antibodies are mediated by B cells. […] Thus, therapeutics against B cells can be an efficient strategy for this disease, with IVIg, high-dose corticosteroids, and PLEX being the first treatment of choice for immunotherapy, and rituximab and tocilizumab being the next treatment choices. […] If autoimmune epilepsy develops in patients with malignancy, cancer treatment should be the primary intervention as long as the autoimmune epilepsy is managed adequately.
  • #1 :: JCN :: Journal of Clinical Neurology
    https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519
    Since NORSE is an emergency condition, immunotherapy should be administered as soon as possible, preferably within hours or (at worst) days. […] As empirical immunotherapy, IVIg can be the first treatment of choice because it is both effective in autoimmune epilepsy and safe in viral encephalitis. […] If a patient does not fully respond to the initial immunotherapy, clinicians should move to an alternative immunotherapy such as rituximab, tocilizumab, anakinra, or cyclophosphamide. […] The occurrence of only sporadic seizures in autoimmune epilepsy will give clinicians more time to complete the differential diagnosis, and treatment and immunotherapy can be applied over days and even weeks. […] The first step is to control seizures by the appropriate administration of AEDs. […] However, an AED is an adjuvant treatment in autoimmune epilepsy, and few cases of seizure are prevented by an AED alone.
  • #1 Clinical Approach to Autoimmune Epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7541993/
    Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] The treatment of autoimmune epilepsy can be categorized into two axes: 1) disease-modifying treatment by immunotherapy and 2) the administration of appropriate AEDs. […] For precise immunotherapy, careful history-taking, neurologic examinations, and pathomechanism analysis of autoimmune epilepsy are mandatory.
  • #1
    https://link.springer.com/article/10.1007/s13311-019-00750-3
    Second-line agents such as rituximab, cyclophosphamide, mycophenolate, azathioprine, bortezomib, or tocilizumab are used in refractory cases or as a maintenance therapy to prevent relapses. […] Treatment of autoimmune epilepsy should be based on the severity of the clinical course. […] In patients with rapid progression and refractory course, more aggressive immunotherapy is needed including a combination of both first- and second-line therapies. […] In all cases, cancer surveillance as discussed above should be pursued. […] Preferred first-line treatments for autoimmune epilepsy include high-dose intravenous corticosteroid therapy, PLEX, and IVIG. […] A treatment response can be ascertained using a seizure diary to assess seizure frequency and/or change in semiology and neurological examination including screening mental status examination after completion of immunotherapy trial.
  • #1 Autoimmune Epilepsy – The Defeating Epilepsy Foundation
    https://www.defeatingepilepsy.org/understanding-epilepsy/autoimmune-epilepsy/
    Autoimmune epilepsy is treated by immunotherapy, removal of an immunologic trigger such as a tumor (when one is present) and anti-epileptic medications (Husari and Dubey, 2019). […] Immunotherapy is divided into two treatment cycles, the acute phase, then the maintenance phase. Depending on the severity of the case will decide on how aggressive of action needs to be taken with treatment. […] Anticonvulsant medications help with the maintenance phase to prevent seizure activity. In the article, Autoimmune Epilepsy, the authors pointed out that sodium channel blockers such as carbamazepine, phenytoin, oxcarbazepine, and lacosamide had the best success in controlling seizure activity. […] Surgery has been tried in some cases of autoimmune epilepsy, but studies show that the outcomes seem to be worse compared to other forms of drug-resistant epilepsy (Husari and Dubey, 2019). […] Treatment options include immunotherapy, antiepileptic medication, and surgery.
  • #1 Autoimmune Epilepsy: Symptoms, Causes, Diagnosis, Treatment
    https://www.healthline.com/health/epilepsy/autoimmune-epilepsy
    An early initiation of immunotherapy is associated with a favorable outcome. […] People with cancer-related autoimmune epilepsy generally have worse outcomes than people who develop autoimmune epilepsy with other underlying causes. […] There are no reliable guidelines for preventing it. […] Autoimmune epilepsy is a group of conditions that cause recurrent seizures due to brain inflammation caused by an autoimmune reaction.
  • #1 Autoimmune Epilepsy Testing – Mayo Clinic LaboratoriesplayEpilepsyGABA-A receptor antibodies
    https://news.mayocliniclabs.com/neurology/autoimmune-neurology/epilepsy/
    Autoimmune epilepsy is increasingly recognized in the spectrum of immune-mediated neurological disorders, which can be characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. The advent of more sensitive and specific serological detection methods is increasingly revealing previously underappreciated autoimmune epilepsies. Neural autoantibodies specific for intracellular and plasma membrane antigens aid the diagnosis of autoimmune epilepsy, but no single antibody is specific for this diagnosis. […] 67% of 27 patients with suspected autoimmune epilepsy treated with immunotherapy became seizure free. […] 81% of patients with suspected autoimmune epilepsy treated with immunotherapy experienced clinical improvement. […] Identifying epilepsy as autoimmune-mediated is crucial because patients may benefit from immune suppression, while traditional antiepileptic therapy may not be effective.
  • #1 :: JCN :: Journal of Clinical Neurology
    https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519
    The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] The autoantibody of LGI1-antibody encephalitis sometimes induces the loss of hippocampal neurons when treatment is delayed. […] Approximately 15% of patients still do not respond well to immunotherapy treatment, and so future research needs to focus on patients with refractory autoimmune epilepsy.
  • #1 Autoimmune epilepsy: Symptoms, causes, and treatment
    https://www.medicalnewstoday.com/articles/autoimmune-epilepsy
    This type of autoimmune epilepsy targets a particular receptor called NMDAR. […] A doctor makes a diagnosis when they find specific antibodies in the blood immunoglobulin G (IgG) that target NMDAR. […] A blood test for specific antibodies may confirm the diagnosis. […] Sometimes, surgical treatment is necessary. This may involve the removal of a portion of the brain. […] According to a 2023 case report, GAD65 antibody-associated epilepsy does not cause changes that are visible on an MRI scan or spinal tap and does not respond well to treatment with immunotherapy. […] However, a doctor may find anti-GAD65 antibodies in the blood. […] Early diagnosis of autoimmune epilepsy can help increase a person’s chances of stopping seizures long term. […] While autoimmune epilepsy typically does not respond well to antiseizure medications, effective treatments are available.
  • #1
    https://link.springer.com/article/10.1007/s40265-022-01826-9
    Epileptic syndromes with suspected immune aetiology without known autoantibodies respond well to immunomodulation while ASMs remain of limited use. […] The most effective treatment in this desperate situation consists of functional hemispherectomy in children as early as possible since the plasticity of the infant brain may lessen the severe sequelae of the disease as well as of the surgical treatment. […] Emerging novel therapeutic agents targeting different players in the immune cascade have arisen by progressively deciphering underlying pathomechanisms in AIE.
  • #1 Autoimmune Epilepsy Identification and Treatment
    https://www.cureepilepsy.org/webinars/identification-and-treatment-of-autoimmune-epilepsy/
    Autoimmune epilepsy is important to diagnose because one of the hallmarks of this condition is that it does not generally respond to typical anti-seizure medications. Immunotherapy is often used to treat people with this condition, by reducing inflammation in the brain. […] If immunotherapy and antiepileptic drug treatment does not work, there are lots of forms of immunosuppression. […] There are lots of different therapies. […] The ketogenic diet seems to have anti-inflammatory properties to it as well. […] If one area is structurally very damaged, you could consider surgery or neurostimulation too with various nerve stimulators or even invasive neural stimulators have been used in patients that are autoimmune as well. […] Immunotherapy and seizure meds to try to get your seizures under control long term. […] I never wean seizure meds and immunotherapy at the same time. […] If they were very severe like an NMDA, Ill often just continue immunotherapy and everything for about two years, thats what most people do.
  • #1 Uncovering the Mysteries and Multidisciplinary Care for Autoimmune Epilepsy Patients | UNM Health Blog | Albuquerque, New Mexico
    https://unmhealth.org/stories/2024/02/autoimmune-epilepsy-patients.html
    Autoimmune epilepsy is a disease in our bodys own immune system, which usually protect us, but instead attacks our brain, resulting in sudden seizures that often present as being refractory and associated with other neurological and psychiatric comorbidity. […] Autoimmune epilepsy can be triggered by a simple respiratory virus, another auto-immune disease like rheumatoid arthritis, or cancer somewhere in the body. So, it often doesnt respond well to typical epilepsy medications and often requires immunosuppressive therapy. […] In our epilepsy clinic, we also offer dedicated multidisciplinary care for patients with autoimmune epilepsy and support for families. […] Finding the root cause is the starting point for planning effective treatment. […] For many patients, steroid medications such as methylprednisolone and prednisone can help suppress their overactive immune system and reduce symptoms. Often, patients end up requiring short-term or long-term immune therapy including various monoclonal antibodies or chemo drugs.
  • #1 Autoimmune epilepsy | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy
    If you have symptoms other than seizures, such as trouble with memory or speech, you may need rehabilitation. Occupational and speech therapy can help. […] Rasmussen syndrome, which mainly affects children, usually doesn’t respond to medicines. Brain surgery often is needed to treat this type of autoimmune epilepsy.
  • #1 Uncovering the Mysteries and Multidisciplinary Care for Autoimmune Epilepsy Patients | UNM Health Blog | Albuquerque, New Mexico
    https://unmhealth.org/stories/2024/02/autoimmune-epilepsy-patients.html
    When cancer is the cause of autoimmune epilepsy (paraneoplastic subtypes of autoimmune epilepsy), we refer patients to our oncology colleagues to treat the tumor and continue helping with seizure management. […] Most patients with autoimmune epilepsy need ongoing care, with follow-up visits every two to four months with ongoing chemotherapy or immune suppression treatments. […] The team involved with this specialized care includes an autoimmune epilepsy faculty and a range of providers, such as: Pharmacist to manage medications, Social worker to connect families with support services, Nurse navigators to help coordinate appointments and complete paperwork. […] Psychiatrists to help patients and families manage epilepsy-associated personality changes, memory challenges, and variations in mood or behavior such as depression, paranoia, or hallucinations.
  • #1 Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management | Journal of Neurology, Neurosurgery & Psychiatry
    https://jnnp.bmj.com/content/92/8/897
    The optimal duration of maintenance therapy in relapsing forms of AE is unknown but published empiric approaches suggest initial maintenance period of 3 years followed by re-evaluation and attempt at withdrawal of immunosuppression. […] The best long-term preventive therapy for relapsing AE depends on the specific immunopathology of each AE subtype. […] The value of non-cell-depleting immunotherapies (eg, complement or cytokine inhibitors) is yet to be fully explored in the long-term management of AE.
  • #1 Treatment Considerations for Autoimmune Epilepsy
    https://www.cureepilepsy.org/webinars/autoimmune-epilepsy-treatment-considerations/
    This webinar provided information to help the audience understand more about autoimmune epilepsy and the different treatment options and considerations, including immunotherapy, for autoimmune-related seizures and epilepsies. […] It is important to diagnose autoimmune epilepsy because one of the hallmarks of this condition is that it does not generally respond to typical anti-seizure medications. Immunotherapy is often used to treat people with this condition, by reducing inflammation in the brain. […] When youre dealing with someone like NMDA receptor encephalitis that antibody is toxic. […] long term, often we use something called rituximab, which then is an antibody then directed at the bone marrow to stop making. […] I never wean seizure meds and immunosuppression at the same time, because then if they have a breakthrough seizure, you dont know is it because they have structural damage that now they have epilepsy or is it that theyre having a relapse of their autoimmune encephalitis and need immunotherapy. […] The reality is both. Ive seen it flare up from both. […] I took an NMDA receptor encephalitis patient off rituximab which had worked for her for years when she relapsed, I did give her steroids actually, but then I gave her rituximab to go back on it too.
  • #1 Autoimmune epilepsy | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy
    Your healthcare professional also may recommend plasma exchange. A healthcare professional removes the liquid part of your blood and separates it from your blood cells. Then the blood cells are put back into your body and your body makes more plasma. This therapy helps remove the antibodies that are causing the immune system to attack brain cells. […] Although antiseizure medicines don’t work as well in people with autoimmune epilepsy, they may still play a role in your treatment. However, the effectiveness of these medicines may be limited. If you take immunotherapy and become seizure free, your healthcare professional may suggest slowly reducing your dose of antiseizure medicines over several months to see if seizures return. […] Many people whose seizures are due to autoimmune encephalitis become seizure free after taking immunotherapy. It may take several months after the treatment for the seizures to stop. But seizures continue for some people even after immunotherapy. People with GAD65 antibodies are less likely to become seizure free.
  • #1 Autoimmune Epilepsy Diagnosis and Treatment | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/autoimmune-epilepsy-diagnosis-and-treatment/
    Immunotherapy is a key treatment for autoimmune epilepsy. It involves using medications to suppress the immune system. Steroids and immunoglobulin are common options. Steroids reduce inflammation in the brain. They can be given orally or intravenously. Intravenous immunoglobulin (IVIG) helps modulate the immune response. […] Doctors tailor treatment plans based on the type of antibody present in patients. Specific antibodies target different parts of the brain. The clinical approach varies accordingly. […] Each case is unique. Doctors adjust therapies based on patient response and needs. […] After initial treatment, patients enter a maintenance phase. This involves continuing lower doses of medications to prevent relapse. Regular follow-up is crucial during this time. […] Clinical trials offer new hope for refractory cases. These studies test innovative drugs and approaches.
  • #2 Clinical Approach to Autoimmune Epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7541993/
    Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] The treatment of autoimmune epilepsy can be categorized into two axes: 1) disease-modifying treatment by immunotherapy and 2) the administration of appropriate AEDs. […] For precise immunotherapy, careful history-taking, neurologic examinations, and pathomechanism analysis of autoimmune epilepsy are mandatory.
  • #2 Clinical Approach to Autoimmune Epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7541993/
    High-dose steroids and intravenous immunoglobulin (IVIg) have been the initial immunotherapies for autoimmune encephalitis, affecting a broad spectrum of autoimmune responses including humoral and cellular immune reactions. […] Immunotherapy was first applied to NORSE a few years ago. […] The available data indicate that a certain proportion of patients with FIRES and NORSE respond well to treatment with high-dose steroids (11% and 15%, respectively), IVIg (both 5%), and PLEX (2% and 6%). […] Since NORSE is an emergency condition, immunotherapy should be administered as soon as possible, preferably within hours or (at worst) days. […] Empirical immunotherapy is often required since it takes days or even weeks for the results to be obtained in the extensive testing required for a differential diagnosis.
  • #2 Autoimmune epilepsy | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy
    Autoimmune epilepsy treatment differs from the treatment used for other types of epilepsy. Healthcare professionals use immunotherapy to reduce the immune system activity and treat seizures. […] If cancer is the cause of autoimmune epilepsy, treating the cancer is an important part of treatment. […] Immunotherapy medicines for autoimmune epilepsy may include: High-dose corticosteroids. Your healthcare professional may treat you with methylprednisolone (Solu-Medrol) through an IV in your arm for up to five days. After this period, you may take methylprednisolone by IV regularly for weeks, gradually lengthening the time in between doses. […] Depending on your response to the initial treatments, your healthcare professional also may recommend longer acting medicines that work on the immune system. These may include rituximab (Rituxan, Truxima, others), cyclophosphamide, mycophenolate (Cellcept, Myhibbin), azathioprine (Azasan, Imuran), or tocilizumab (Actemra, Tofidence, Tyenne).
  • #2 :: JCN :: Journal of Clinical Neurology
    https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519
    Since NORSE is an emergency condition, immunotherapy should be administered as soon as possible, preferably within hours or (at worst) days. […] As empirical immunotherapy, IVIg can be the first treatment of choice because it is both effective in autoimmune epilepsy and safe in viral encephalitis. […] If a patient does not fully respond to the initial immunotherapy, clinicians should move to an alternative immunotherapy such as rituximab, tocilizumab, anakinra, or cyclophosphamide. […] The occurrence of only sporadic seizures in autoimmune epilepsy will give clinicians more time to complete the differential diagnosis, and treatment and immunotherapy can be applied over days and even weeks. […] The first step is to control seizures by the appropriate administration of AEDs. […] However, an AED is an adjuvant treatment in autoimmune epilepsy, and few cases of seizure are prevented by an AED alone.
  • #2 Autoimmune epilepsy | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy
    Your healthcare professional also may recommend plasma exchange. A healthcare professional removes the liquid part of your blood and separates it from your blood cells. Then the blood cells are put back into your body and your body makes more plasma. This therapy helps remove the antibodies that are causing the immune system to attack brain cells. […] Although antiseizure medicines don’t work as well in people with autoimmune epilepsy, they may still play a role in your treatment. However, the effectiveness of these medicines may be limited. If you take immunotherapy and become seizure free, your healthcare professional may suggest slowly reducing your dose of antiseizure medicines over several months to see if seizures return. […] Many people whose seizures are due to autoimmune encephalitis become seizure free after taking immunotherapy. It may take several months after the treatment for the seizures to stop. But seizures continue for some people even after immunotherapy. People with GAD65 antibodies are less likely to become seizure free.
  • #2 Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians
    https://www.mdpi.com/2227-9059/11/1/44
    The most recent International League Against Epilepsy (ILAE) classification has included “immune etiology” along with other well-known causes of epilepsy. […] The aim of this review is to provide a comprehensive and practical overview on diagnosis and management of immune-mediated seizures and epilepsy. […] When alternative etiologies are excluded and a strong suspicion of AE is present, first-line immunotherapies (including steroids, intravenous immunoglobulin (IVIG) or plasma exchange (PLEX)) should be considered early, before receiving Ab results. […] Treatment with intravenous methylprednisolone 1 g per day for 5 consecutive days is a common approach in patients with AE, and it is the preferred strategy in scenarios known to be highly steroid-responsive, such as anti-LGI1 encephalitis.
  • #2
    https://link.springer.com/article/10.1007/s40265-022-01826-9
    If corticosteroids are contraindicated or treatment response is not sufficient, therapy with IVIG or PLEX can be considered. […] Second-line agents such as rituximab (RTX) or cyclophosphamide (CTX) can be implemented if no clinical improvement is observed 2-3 weeks after the initiation of treatment. […] Rituximab is thought to be more effective in patients with antibody-mediated AIE. […] In paraneoplastic AIE, treatment of the underlying neoplasm is crucial. […] After high-dose immunotherapy, a subsequent bridging therapy should be initiated as prompt discontinuation of immunotherapy shows higher risk of relapse. […] Long-term immunosuppression is indicated once a relapse occurs. […] In a trial by Toledano et al., 62% of patients were responders, of whom 34% attained seizure freedom and 52% improved with the first agent.
  • #2
    https://aesnet.org/abstractslisting/rituximab-for-the-treatment-of-intractable-epilepsy-due-to-autoimmune-encephalitis–case-series
    Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged ICU admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. […] There is no accepted treatment for these disorders, but typical approaches involve chronic high-dose steroids (often for months or years), methotrexate or mycophenolate. Rituximab is a monoclonal antibody that depletes B-cells, which are precursors to plasma cells that secrete pathogenic antibodies. […] All patients demonstrated complete epilepsy control and improvement in symptoms with rituximab. […] These results suggest that empiric treatment with rituximab can be considered as add-on therapy for the treatment of recalcitrant status epilepticus.
  • #2 Autoimmune Epilepsy – The Defeating Epilepsy Foundation
    https://www.defeatingepilepsy.org/understanding-epilepsy/autoimmune-epilepsy/
    Autoimmune epilepsy is treated by immunotherapy, removal of an immunologic trigger such as a tumor (when one is present) and anti-epileptic medications (Husari and Dubey, 2019). […] Immunotherapy is divided into two treatment cycles, the acute phase, then the maintenance phase. Depending on the severity of the case will decide on how aggressive of action needs to be taken with treatment. […] Anticonvulsant medications help with the maintenance phase to prevent seizure activity. In the article, Autoimmune Epilepsy, the authors pointed out that sodium channel blockers such as carbamazepine, phenytoin, oxcarbazepine, and lacosamide had the best success in controlling seizure activity. […] Surgery has been tried in some cases of autoimmune epilepsy, but studies show that the outcomes seem to be worse compared to other forms of drug-resistant epilepsy (Husari and Dubey, 2019). […] Treatment options include immunotherapy, antiepileptic medication, and surgery.
  • #2 Antiepileptic drug therapy in patients with autoimmune epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5489139/
    We aimed to report the pattern of usage and efficacy of antiepileptic drugs (AEDs) in patients with autoimmune epilepsy (AE). […] The majority (n = 43, 86%) received at least 1 form of immunotherapy in combination with AEDs, while the remainder received AEDs alone. […] In select patients, AEDs alone were effective in controlling seizures. […] AEDs with sodium channel blocking properties resulted in seizure freedom in a few cases. […] The role of AEDs in these patients is also relevant as even after controlling the inflammatory response, some patients remain at risk of recurrent seizures, particularly if cerebral damage occurred during the acute encephalitic phase of the illness. […] Although intractability is a common feature in AE, some respond to AEDs, and they remain an important aspect of therapy.
  • #2 :: JCN :: Journal of Clinical Neurology
    https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519
    The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] The autoantibody of LGI1-antibody encephalitis sometimes induces the loss of hippocampal neurons when treatment is delayed. […] Approximately 15% of patients still do not respond well to immunotherapy treatment, and so future research needs to focus on patients with refractory autoimmune epilepsy.
  • #2 Autoimmune epilepsy due to N-methyl-d-aspartate receptor antibodies in a child: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-03117-5
    Initiation of immune therapy early for AEp (within 6 months of disease onset) is shown to result in favorable seizure control. […] Our case report highlights the importance of early diagnosis of AEp, early treatment with immunotherapy, long-term clinical follow-up, and timely escalation and continuation of immunotherapy in achieving a good patient outcome, retaining intellectual development, and quality of life.
  • #2 Autoimmune epilepsy: Symptoms, causes, and treatment
    https://www.medicalnewstoday.com/articles/autoimmune-epilepsy
    In particular, immunotherapy may be highly effective in treating inflammation in the brain and managing autoimmune epilepsy. […] Doctors may prescribe steroid medications, including prednisone and methylprednisolone, to people with this condition. […] Starting immunotherapy early is the best way to stop brain injury and prevent seizures in people with autoimmune epilepsy that does not link to cancer. […] To treat autoimmune epilepsy, doctors use steroid medications and immunotherapy to address the underlying autoimmune response.
  • #2 Autoimmune epilepsy | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy
    If you have symptoms other than seizures, such as trouble with memory or speech, you may need rehabilitation. Occupational and speech therapy can help. […] Rasmussen syndrome, which mainly affects children, usually doesn’t respond to medicines. Brain surgery often is needed to treat this type of autoimmune epilepsy.
  • #2 10 epilepsy treatments – from lowest to highest risk | Brain | UT Southwestern Medical Center
    https://utswmed.org/medblog/epilepsy-treatments/
    Occasionally, epilepsy is caused by the patients immune system attacking the brain. Autoimmune epilepsy is a relatively recently recognized cause of epilepsy that cannot be controlled with anti-seizure medications alone. […] Treatment options include: High-dose steroids, Administering a collection of human antibodies through the veins, Plasma exchange to filter the blood of disease-causing antibodies, Other medications that suppress the overly active immune system.
  • #2 Uncovering the Mysteries and Multidisciplinary Care for Autoimmune Epilepsy Patients | UNM Health Blog | Albuquerque, New Mexico
    https://unmhealth.org/stories/2024/02/autoimmune-epilepsy-patients.html
    When cancer is the cause of autoimmune epilepsy (paraneoplastic subtypes of autoimmune epilepsy), we refer patients to our oncology colleagues to treat the tumor and continue helping with seizure management. […] Most patients with autoimmune epilepsy need ongoing care, with follow-up visits every two to four months with ongoing chemotherapy or immune suppression treatments. […] The team involved with this specialized care includes an autoimmune epilepsy faculty and a range of providers, such as: Pharmacist to manage medications, Social worker to connect families with support services, Nurse navigators to help coordinate appointments and complete paperwork. […] Psychiatrists to help patients and families manage epilepsy-associated personality changes, memory challenges, and variations in mood or behavior such as depression, paranoia, or hallucinations.
  • #2 Autoimmune Encephalitis Treatment | AE Alliance
    https://aealliance.org/patient-support/treatment/
    Other people with AE will need more medications that act on the immune system in order to stop the brain inflammation. […] Physical therapy, occupational therapy and speech therapy may be very helpful to improve recovery and may be started in hospital and sometimes continued in rehabilitation centers. […] Starting treatment early seems to decrease long-term complications and the risk of relapse, as well as lead to faster recovery. […] Sometimes, medications that treat the symptoms of AE don’t work well when a person is first diagnosed, but doctors may recommend trying them again later after the brain inflammation has decreased because they may work better. […] Many patients need medications to help with behavioral and psychiatric symptoms of AE and they are an important part of AE treatment.
  • #2 Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management | Journal of Neurology, Neurosurgery & Psychiatry
    https://jnnp.bmj.com/content/92/8/897
    The optimal duration of maintenance therapy in relapsing forms of AE is unknown but published empiric approaches suggest initial maintenance period of 3 years followed by re-evaluation and attempt at withdrawal of immunosuppression. […] The best long-term preventive therapy for relapsing AE depends on the specific immunopathology of each AE subtype. […] The value of non-cell-depleting immunotherapies (eg, complement or cytokine inhibitors) is yet to be fully explored in the long-term management of AE.
  • #2 Autoimmune Epilepsy Identification and Treatment
    https://www.cureepilepsy.org/webinars/identification-and-treatment-of-autoimmune-epilepsy/
    Autoimmune epilepsy is important to diagnose because one of the hallmarks of this condition is that it does not generally respond to typical anti-seizure medications. Immunotherapy is often used to treat people with this condition, by reducing inflammation in the brain. […] If immunotherapy and antiepileptic drug treatment does not work, there are lots of forms of immunosuppression. […] There are lots of different therapies. […] The ketogenic diet seems to have anti-inflammatory properties to it as well. […] If one area is structurally very damaged, you could consider surgery or neurostimulation too with various nerve stimulators or even invasive neural stimulators have been used in patients that are autoimmune as well. […] Immunotherapy and seizure meds to try to get your seizures under control long term. […] I never wean seizure meds and immunotherapy at the same time. […] If they were very severe like an NMDA, Ill often just continue immunotherapy and everything for about two years, thats what most people do.
  • #2 Unraveling Autoimmune Epilepsy
    https://www.neurologylive.com/view/unraveling-autoimmune-epilepsy
    Higdon clarified that the kind of antibody present could influence response to immunotherapy. […] To predict which patients are more likely to respond to immunotherapy, Dubey and his team have also introduced and validated the RITE (Response to Immunotherapy in Epilepsy) score. […] While IVIG may potentially be more convenient and safer compared with steroids and plasmapheresis, well-designed comparative studies are currently lacking. […] Many patients with autoimmune epilepsy can be successfully managed if a proper algorithm is followed, such as the one suggested by Higdon. […] The ideal protocol for the duration of treatment and evaluation of response still needs to be developed, Higdon said. […] Most experts feel that a better understanding of the pathophysiology of autoimmune epilepsy is the way forward.
  • #2 Identification and Treatment of Autoimmune Epilepsy – Contemporary Advances in Epilepsy
    https://www.medpagetoday.com/resource-centers/contemporary-advances-epilepsy/identification-and-treatment-autoimmune-epilepsy/2088
    Among the one-third of adults with epilepsy of unknown etiology, an autoimmune cause appears to be the potential culprit in at least 20% of cases, according to new research published by Divyanshu Dubey, MD, and colleagues in JAMA Neurology. […] To aid clinicians in identifying individuals with autoimmune epilepsy, which does not respond to standard antiepileptic therapy, the study investigators devised an antibody prevalence in epilepsy (APE) score useful for predicting positive serologic findings. Moreover, the investigators showed that seropositive patients exhibited favorable responses to immunomodulatory therapy. […] Immunomodulatory therapy reduced seizure frequency in patients seropositive for autoantibodies. […] Patients with autoimmune-induced seizures tend to have recalcitrant disease that does not respond to antiepileptic drugs.
  • #2
    https://link.springer.com/article/10.1007/s13311-019-00750-3
    Even though seizures in autoimmune epilepsy are characteristically resistant to antiepileptic drugs (AEDs) alone, they continue to play an important role in symptomatic management. […] Epilepsy surgery has been tried in select cases of autoimmune epilepsy. […] Most patients with autoimmune epilepsy and encephalitis are responsive to either first- or second-line therapies. However, a subset of patients remains refractory to multiple immunotherapies. […] With better insight into the mechanisms of antibody-mediated and autoantigen-specific T-cell-mediated autoimmune epilepsy syndromes, utilization of AEDs and immunotherapy is likely to be further optimized.
  • #3 Immunotherapy for Autoimmune Epilepsy | Northwestern Medicine
    https://www.nm.org/conditions-and-care-areas/treatments/immunotherapy-for-autoimmune-epilepsy
    Autoimmune epilepsy is a form of autoimmune encephalitis, encephalopathy or limbic encephalitis, in which seizures are the primary symptom. […] Autoimmune epilepsy is important to diagnose because patients with this condition usually have seizures that do not completely respond to standard anti-seizure medications, but often respond to immune therapies. Some examples of immune therapies include: corticosteroids, intravenous immune globulin, plasma exchange or other treatments that suppress the immune system.
  • #3
    https://link.springer.com/article/10.1007/s13311-019-00750-3
    In this review article, we provide an overview of autoimmune epilepsy with emphasis on the recent advances regarding pathophysiology, imaging studies, and therapeutic interventions. […] Treatment of patients with autoimmune epilepsy is comprised of immunotherapy, removal of an immunologic trigger such as a tumor (when applicable), and symptomatic therapy including antiseizure medications. […] Multiple studies have demonstrated favorable effects of early initiation of immunotherapy on seizure frequency and cognition. […] Immunotherapeutic agents are classically divided into first-line (acute phase) and second-line therapies (maintenance phase). […] First-line therapies include high-dose intravenous methylprednisolone (IVMP), intravenous immune globulin (IVIG), or plasma exchange (PLEX).
  • #3
    https://link.springer.com/article/10.1007/s13311-019-00750-3
    Second-line agents such as rituximab, cyclophosphamide, mycophenolate, azathioprine, bortezomib, or tocilizumab are used in refractory cases or as a maintenance therapy to prevent relapses. […] Treatment of autoimmune epilepsy should be based on the severity of the clinical course. […] In patients with rapid progression and refractory course, more aggressive immunotherapy is needed including a combination of both first- and second-line therapies. […] In all cases, cancer surveillance as discussed above should be pursued. […] Preferred first-line treatments for autoimmune epilepsy include high-dose intravenous corticosteroid therapy, PLEX, and IVIG. […] A treatment response can be ascertained using a seizure diary to assess seizure frequency and/or change in semiology and neurological examination including screening mental status examination after completion of immunotherapy trial.
  • #3 Antiepileptic drug therapy in patients with autoimmune epilepsy
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5489139/
    The observed response could also be due to the effectiveness of sodium channel blockers in focal onset epilepsy which is seen in 66% of patients in our cohort. […] Studies are needed to elucidate whether any differential efficacy is based on their effect on seizure mechanisms or immunomodulatory properties. […] Prospective studies are needed to clarify AED selection and to elucidate their immunomodulatory properties in AE.
  • #3 Uncovering the Mysteries and Multidisciplinary Care for Autoimmune Epilepsy Patients | UNM Health Blog | Albuquerque, New Mexico
    https://unmhealth.org/stories/2024/02/autoimmune-epilepsy-patients.html
    When cancer is the cause of autoimmune epilepsy (paraneoplastic subtypes of autoimmune epilepsy), we refer patients to our oncology colleagues to treat the tumor and continue helping with seizure management. […] Most patients with autoimmune epilepsy need ongoing care, with follow-up visits every two to four months with ongoing chemotherapy or immune suppression treatments. […] The team involved with this specialized care includes an autoimmune epilepsy faculty and a range of providers, such as: Pharmacist to manage medications, Social worker to connect families with support services, Nurse navigators to help coordinate appointments and complete paperwork. […] Psychiatrists to help patients and families manage epilepsy-associated personality changes, memory challenges, and variations in mood or behavior such as depression, paranoia, or hallucinations.
  • #3
    https://aesnet.org/abstractslisting/antiepileptic-drug-therapy-in-patients-with-suspected-autoimmune-epilepsy
    Intractability to antiepileptic drugs (AEDs) is a common feature of autoimmune epilepsy (AE) and immunotherapy is widely accepted as the first-line. […] AEDs remain the cornerstone of AE therapeutics, and some have documented anti-inflammatory properties. […] The majority of patients (n=43, 86%) received at least one form of immunotherapy in combination with AEDs while the reminder received AEDs alone. […] 27 patients (54%) became seizure free: 18 (36%) with immunotherapy, 5 (10%) with AEDs alone, and 4 (8%) with AEDs after failing immunotherapy. […] This retrospective study suggests that the selection of AED therapy in AE is arbitrary. […] In select patients, AEDs alone were effective in controlling seizures. […] Although levetiracetam was the most commonly used agent, there was no clear evidence to support its use based on documented efficacy. […] In contrast, carbamazepine, lacosamide and oxacarbazepine resulted in seizure freedom in a few cases. […] Prospective studies are needed to clarify AED selection and to elucidate their immunomodulatory properties in AE.
  • #3 Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians
    https://www.mdpi.com/2227-9059/11/1/44
    Data from the literature suggest that the overall efficacy of ASMs alone in AE is low. […] Finally, long-term use of ASMs in AE is a matter of debate. […] A minority of them will evolve to an autoimmune-associated epilepsy, especially those with onconeural (paraneoplastic) Abs, as well as those with GAD65 Abs. […] Our recommendation for minimizing seizure recurrence is to treat all AE patients with immunotherapy early over the course of the disease, and to consider ASM discontinuation in clinically stable, seizure-free cases with anti-LGI1, anti-NMDAR and anti-GABAbR lacking IEDs on EEG.
  • #3 Autoimmune Encephalitis Treatment | AE Alliance
    https://aealliance.org/patient-support/treatment/
    If the combination of symptoms and test results suggest that a person has “possible AE”, the current recommendations are to start first line medications that treat inflammation in the brain, such as steroids, IV immune globulin, and/or plasma exchange. […] In addition to the medications that act on the immune system to decrease brain inflammation, many people with AE will need supportive medications to treat the AE symptoms that cause suffering and disability. […] As the results return from the workup for AE, including anti-neuronal antibody testing, health care providers will be able to decide if a patient meets the criteria for “definite AE” or “probable AE” and further treatment decisions will be discussed. […] Some people with AE improve within days of receiving their first line treatments and don’t need additional medications that act on the immune system.
  • #3 Autoimmune Encephalitis Treatment | AE Alliance
    https://aealliance.org/patient-support/treatment/
    It may be helpful for patients with AE to have a psychiatrist on their treatment team to discuss the different reasoning, risks and benefits of available psychiatric medications. […] After initial diagnosis and treatments, both adults and children with AE enter the period of ongoing treatment and recovery. […] This is a challenging stage of disease for patients with slow recoveries and those with major behavioral changes or psychiatric symptoms. […] As patients improve and become more aware of their illness and their limitations, additional symptoms of frustration, anxiety, depression and aggression can develop. […] Slow re-introduction to activities and people is often preferred. Recovery often takes many months, and initial efforts should focus on daily activities and interacting with close family and friends before returning to work or school.
  • #3 Identification and Treatment of Autoimmune Epilepsy – Contemporary Advances in Epilepsy
    https://www.medpagetoday.com/resource-centers/contemporary-advances-epilepsy/identification-and-treatment-autoimmune-epilepsy/2088
    Accurately diagnosing these individuals not only helps avoid unnecessary therapy, but also offers the opportunity to treat patients with immunomodulatory therapies that have been shown to offer benefit, such as corticosteroids and intravenous immune globulin. […] Good seizure outcomes (ie, a >50% reduction in seizure frequency at the first follow-up visit) were strongly tied to the use of immunomodulatory therapy, particularly intravenous methylprednisolone or plasmapheresis. […] The concept that some of these patients that have medically intractable epilepsy actually have a treatable and potentially reversible condition is very important. […] If you diagnose autoimmune epilepsy in these patients early and treat them, you can actually cure them. […] Assessing this issue prospectively is really important, and that’s where this paper really has a unique position.
  • #4 Neuronal autoantibodies in a sample of Egyptian patients with drug-resistant epilepsy | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text
    https://ejnpn.springeropen.com/articles/10.1186/s41983-023-00685-9
    Although one of the features of this type of epilepsy is that it is usually resistant to ASMs, it generally responds better to targeted immunotherapies in combination with ASMs. […] The presence of antibody-negative AE was supported by the good response to immunotherapy trial in those patients, in the current study all but three patients received immunotherapy trial with first-line therapy, 92% of those patients became seizure free or had 50% reduction in seizure frequency after a mean of 19.826.14 days from start of immunotherapy. […] This suggests autoimmunity as the etiology or a responsible cause for ongoing epileptogenic process. […] When clinical, serological and radiological clues suggest an autoimmune etiology for intractable epilepsy, early initiation of immunotherapy is crucial as AE is a reversible condition with a rapid response to immunotherapy.

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