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A Young Woman Presenting with Encephalopathy: A Case Report

A Young Woman Presenting with Encephalopathy: A Case Report | AntiNMDA | Scoop.it
Introduction When cerebral white matter lesions are detected in young patients presenting with neurologic symptoms, the most likely diagnosis is multiple sclerosis (MS). Still, it is crucial to screen for important differential diagnoses which require sophisticated workup and sometimes immediate treatment, such as autoimmune encephalopathies, juvenile stroke, central nervous system (CNS) vasculitis, and, as in this case, Susac syndrome. Knowledge about key clinical and neuro-imaging features is therefore of utmost importance. Here, we report a case of a young woman presenting with encephalopathy, focal neurological deficits, and cerebral white matter lesions and discuss our diagnostic approach. Case A previously healthy 30-year-old woman presented to our emergency department due to a 1-week history of progressive confusion, personality change, vertigo, and stroke-like episodes with temporary aphasia. Clinical examination revealed fine motor impairment of the left hand, bilateral pyramidal signs, and deficits in memory, attention, and executive functions. As the patient was agitated, only a brief magnetic resonance imaging (MRI) protocol (diffusion-weighted, T2 and fluid attenuated inversion recovery [FLAIR] sequences) was performed, revealing multiple supra- and infratentorial punctate diffusion-restricted lesions as well as FLAIR-hyperintense white matter lesions without evidence of restricted diffusion. In addition, the central corpus callosum revealed round (snowball), linear (spoke), and “hanging” (icicles) T2 and FLAIR-hyperintense lesions (Fig. 1, Panel A). Due to these pathognomonic imaging findings, Susac Syndrome, an immune-mediated endotheliopathy of precapillary arterioles of the brain, retina, and inner ear [1], was considered as the most likely differential diagnosis. The patient was admitted to our neurological intensive care unit for observation and further diagnostic workup until potentially life-threatening differential diagnoses had been excluded. Sedation or mechanical ventilation was not necessary. Discussion of Differential Diagnoses Cerebrospinal fluid (CSF) analysis revealed a normal cell count, which made an infectious etiology unlikely. Still, she was treated with intravenous aciclovir (10 mg/kg bodyweight every 8 h) for 24 h until negative results for herpes virus polymerase chain reaction were obtained. CSF protein was mildly elevated (98 mg/dl, normal range < 60 mg/dl), indicating dysfunction of the blood–brain barrier, and intrathecal immunoglobulin synthesis was detected. Screening for antibodies against neuronal surface and intracellular antigens as well as for thyroid antibodies was negative, arguing against the differential diagnosis of autoimmune encephalitis or encephalopathy including steroid-responsive encephalopathy associated with autoimmune thyroiditis. Based on the clinical presentation and neuro-imaging findings (lesions in the central corpus callosum), as well as the pronounced dysfunction of the blood–brain barrier (indicated by the highly elevated CSF-to-serum albumin ratio > 47), the differential diagnosis of MS was unlikely. After proper treatment of the agitated state, an extended MRI protocol with susceptibility-weighted sequences and contrast-enhanced angiography was performed but did not reveal vascular abnormalities or signs of hemosiderin deposition. A thorough screening for other organ manifestations including systemic vasculitis was uneventful. Despite the lack of biopsy results, CNS vasculitis was therefore not considered as plausible differential diagnosis. Uneventful transesophageal echocardiography and laboratory screening for antiphospholipid syndrome and other coagulopathies made juvenile stroke unlikely. The diagnosis of Susac syndrome was finally confirmed by retinal fluorescein angiography (Fig. 1, Panel B), showing multiple branch retinal artery occlusions and arterial wall hyperfluorescence, and by pure-tone audiometry revealing hearing loss for low- and mid-tone frequencies. After high-dose intravenous methylprednisolone therapy (1 g/day for 3 days) and intravenous immunoglobulin administration (2 g/kg bodyweight over 2 days), neuropsychological and motor symptoms markedly improved. Treatment was continued with oral methylprednisolone (1 mg/kg bodyweight with slow tapering), mycophenolate mofetil (2 g/day), and intravenous immunoglobulins (1 g/kg bodyweight every other week). After 3 months (five cycles of intravenous immunoglobulins), the patient had fully recovered except for mild hearing deficits. Neuro-imaging did not reveal new lesions. Therefore, so far, no treatment intensification in terms of adding tacrolimus, cyclophosphamide, or rituximab was necessary. It is important to mention that the patient never suffered from headache and CSF examination revealed intrathecal immunoglobulin synthesis, which is both not typical for Susac syndrome and made a broad laboratory and neuro-imaging workup necessary. Conclusion Susac Syndrome is a rare but probably under-recognized condition typically manifesting with headache, encephalopathy, and focal neurological signs. It occurs primarily in young women (3–4 times more often than in men) at a median age of 30–35 years at onset [2] and affects the microvasculature of the brain, retina, and inner ear. Pathophysiologic mechanisms are incompletely understood; however, histopathologic findings and clinical improvement after initiation of immunosuppressive therapy strongly suggest an immune-mediated mechanism. The full diagnostic triad consists of (1) neurological manifestations and typical MRI findings (brain involvement), (2) branch retinal artery occlusions or arterial wall hyperfluorescence in retinal fluorescein angiography or signs of retinal branch ischemia in fundoscopy or optical coherence tomography (retinal involvement), and (3) new tinnitus, hearing loss or peripheral vertigo (cochlear involvement). Recommended treatment includes corticosteroids, intravenous immunoglobulins, and mycophenolate mofetil [3]. Depending on disease severity, tacrolimus, cyclophosphamide, or rituximab may be added. Treatment for at least 2 years is recommended [3]. Important differential diagnoses include MS, CNS vasculitis, autoimmune encephalopathies, and juvenile stroke.
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BREAKING NEWS! First Clinical Trial for Anti-NMDA Receptor Encephalitis

BREAKING NEWS! First Clinical Trial for Anti-NMDA Receptor Encephalitis | AntiNMDA | Scoop.it
In the spring of 2020, the Foundation expressed its strong support for the first-ever Clinical Trial for a promising new drug to treat anti-NMDA receptor encephalitis. Fast forward to the Fall of 2021, when we learned that the Trial was approved and will be funded by the National Institutes of Health (NIH).
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Autoimmune encephalitis with coexistent LGI1 and GABABR1 antibodies: case report | BMC Neurology | Full Text

Autoimmune encephalitis with coexistent LGI1 and GABABR1 antibodies: case report | BMC Neurology | Full Text | AntiNMDA | Scoop.it
Background Autoimmune encephalitis (AE) with multiple auto-antibodies is of great clinical significance because its complex clinical manifestations and atypical imaging increase the difficulty of diagnosis, differential diagnosis and treatment, which may aggravate the disease, increase the recurrence rate and mortality. The coexistence of anti-Leucinie-rich Glioma Inactivated 1 (LGI1) and anti-γ-aminobutyric acid-beta-receptor 1 (GABABR1) has not been published before. Case presentation We herein present the case of a 60-year-old man with slow response, behavioral changes, psychosis and sleep disorders. Laboratory test included serum hyponatremia, positive serum LGI1 and GABABR1 antibodies using transfected cell-based assays. Electroencephalogram exhibited moderate diffusion abnormality. The patient responded well to steroid impulse treatment and sodium supplement therapy, and did not recur during the follow-up. Conclusions Here we report the first AE characterized by positive LGI1 and GABABR1 antibodies, as well as summarizing AE with multiple auto-antibodies reported so far, hopefully to provide experience for clinical practice.
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EEG Contribution to the Diagnosis of Antibody-Negative Autoimmune Encephalitis: A Case Report - FullText - Case Reports in Neurology 2021, Vol. 13, No. 3 - Karger Publishers

Autoimmune encephalitis (AE) is a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms. Early therapeutic intervention is important for AE.Therefore, wit...
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Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis | Neurology

Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis | Neurology | AntiNMDA | Scoop.it
We appreciate the interest in our research.1 According to Pollak and colleagues,2 criteria of possible autoimmune psychosis (AP) are fulfilled if a patient has abrupt onset psychotic symptoms with at least one of the following: the presence of a tumor, movement disorder (dyskinesias, catatonia), adverse response to antipsychotics, “severe or disproportionate” cognitive dysfunction, decreased level of consciousness, unexplained seizures, and significant autonomic dysfunction.2 Fulfilment of these criteria should lead to additional tests such as EEG, MRI, and serum or CSF investigations. In our series of 105 patients with first episode of psychosis (FEP), 20% fulfilled these criteria but never developed AP.1 We confirm that 2 of 3 patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis presenting with FEP did not fulfill any of these criteria, including catatonia, which is a complex syndrome with its own set of 12 criteria that include echolalia.3 Thus, catatonia and echolalia should not be used as interchangeable terms.
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Autoantibody-associated psychiatric symptoms and syndromes in adults: A narrative review and proposed diagnostic approach

Autoantibody-associated psychiatric symptoms and syndromes in adults: A narrative review and proposed diagnostic approach | AntiNMDA | Scoop.it
Autoimmune-mediated encephalitis is a disease that often encompasses psychiatric symptoms as its first clinical manifestation’s predominant and isolated characteristic. Novel guidelines even distinguish autoimmune psychosis from autoimmune encephalitis.
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Vennada's Story: Recovery from Anti-NMDA Receptor Encephalitis

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The grey zone between autoimmune encephalitis and autoimmune‐associated epilepsy - Morano - - Epilepsia Open

The grey zone between autoimmune encephalitis and autoimmune‐associated epilepsy - Morano - - Epilepsia Open | AntiNMDA | Scoop.it
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A Brain on Fire: Laura's Battle with Autoimmune Encephalitis

A Brain on Fire: Laura's Battle with Autoimmune Encephalitis | AntiNMDA | Scoop.it
Laura Martin, a strong student and standout goalie at Transylvania University, hit a sudden wall as things turned worse. Diagnosis: Autoimmune Encephalitis.
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Frontiers | Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis | Immunology

Frontiers | Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis | Immunology | AntiNMDA | Scoop.it
Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy.
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Frontiers | Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report | Neurology

Frontiers | Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report | Neurology | AntiNMDA | Scoop.it
Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis has been reported after SARS-CoV-2 infection, but not after SARS-CoV-2 vaccination. We report the first known case of anti-NMDAR encephalitis after SARS-CoV-2 immunization in a young female presenting with acute psychosis,...
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Lauren's Healing Story (Autoimmune Encephalitis) - Phoenix Helix

Lauren's Healing Story (Autoimmune Encephalitis) - Phoenix Helix | AntiNMDA | Scoop.it
When you have a disease only recently discovered and most doctors don't know it exists, it takes strong self-advocacy to get the help you need.
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CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis | AntiNMDA | Scoop.it
CSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated ...
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Physical Therapy Interventions and Outcome Measures for a Patient Diagnosed with Anti-NMDA Receptor Encephalitis

Physical Therapy Interventions and Outcome Measures for a Patient Diagnosed with Anti-NMDA Receptor Encephalitis | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common cause of
autoimmune encephalitis after acute demyelinating encephalitis. Patients usually present
with acute behavioral changes, psychosis, and abnormal limb movements and can also
...
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Meet Ambassador Jayden Liuzza

The Foundation is very proud to introduce our first and the world’s youngest Ambassador for Anti-NMDA receptor encephalitis, Jayden Liuzza. You may remember seeing Jayden ...Read More...
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Dysfunction of the Autonomic Nervous System and its Role in the Pathogenesis of Septic Critical Illness (Review)

Dysfunction of the Autonomic Nervous System and its Role in the Pathogenesis of Septic Critical Illness (Review) | AntiNMDA | Scoop.it
Dysfunction of the autonomic nervous system (ANS) of the brain in sepsis can cause severe systemic inflammation and even death. Numerous data confirmed the role of ANS dysfunction in the occurrence, course, and outcome of systemic sepsis.
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Frontiers | Risk Factors and Brain Metabolic Mechanism of Sleep Disorders in Autoimmune Encephalitis | Immunology

Frontiers | Risk Factors and Brain Metabolic Mechanism of Sleep Disorders in Autoimmune Encephalitis | Immunology | AntiNMDA | Scoop.it
BackgroundSleep disorders (SDs) in autoimmune encephalitis (AE) have received little attention and are poorly understood. We investigated the clinical characteristics, risk factors, and cerebral metabolic mechanism of SD in AE.MethodsClinical, laboratory, and imaging data were retrospectively...
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A peculiar case of psychosis: anti-NMDAr encephalitis | International Journal of Emergency Medicine | Full Text

A peculiar case of psychosis: anti-NMDAr encephalitis | International Journal of Emergency Medicine | Full Text | AntiNMDA | Scoop.it
Background Psychosis in pregnancy is rare and could be life-threatening. It requires prompt evaluation and proper management accordingly. Anti-N-methyl-d-aspartate receptor (anti-NMDAr) encephalitis following herpes simplex virus (HSV) infection is a rare cause of psychosis during pregnancy.
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Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report - PMC

Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report - PMC | AntiNMDA | Scoop.it
Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis has been reported after SARS-CoV-2 infection, but not after SARS-CoV-2 vaccination. We report the first known case of anti-NMDAR encephalitis after SARS-CoV-2 immunization in a young ...
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Anti-NMDAR Autoantibodies Disrupt Ionotropic Receptor Signaling –

Anti-NMDAR Autoantibodies Disrupt Ionotropic Receptor Signaling – | AntiNMDA | Scoop.it
Vignesh Subramanian '24 Figure 1: The N-methyl-D-aspartate receptor (NMDAR) functions as an ion channel. N-methyl-D-aspartate receptors (NMDARs) are ligand-gated ion channels whose signaling enables higher-order functions, such as learning and memory, throughout the brain.
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Neural Antibody Testing for Autoimmune Encephalitis: A Canadian Single-Centre Experience | Canadian Journal of Neurological Sciences

Neural Antibody Testing for Autoimmune Encephalitis: A Canadian Single-Centre Experience - Volume 48 Issue 6
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The role of dendritic cells and their interactions in the pathogenesis of antibody-associated autoimmune encephalitis | Journal of Neuroinflammation | Full Text

The role of dendritic cells and their interactions in the pathogenesis of antibody-associated autoimmune encephalitis | Journal of Neuroinflammation | Full Text | AntiNMDA | Scoop.it
Autoimmune encephalitis (AE) is an inflammatory brain disease which is frequently associated with antibodies (Abs) against cell-surface, synaptic or intracellular neuronal proteins. There is increasing evidence that dendritic cells (DCs) are implicated as key modulators in keeping the balance...
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Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies | Read by QxMD

Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies | Read by QxMD | AntiNMDA | Scoop.it
RESULTS: Four hundred and fifty records were initially identified, of which 197 were included in the review. The selected studies were retrospective case-control (n=11), case series (n=83), and case reports (n=103) and overall described 1334 patients both of paediatric and adult age. Aetiology remains unexplained in about half of the cases, representing the so-called "cryptogenic NORSE". Among adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either non-paraneoplastic or paraneoplastic. Infections are the prevalent aetiology of paediatric non-cryptogenic NORSE. Genetic and congenital disorders can have a causative role in NORSE, and toxic, vascular, and degenerative conditions have been also described.
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Screening for pathogenic neuronal autoantibodies in serum and CSF of patients with first-episode psychosis

Screening for pathogenic neuronal autoantibodies in serum and CSF of patients with first-episode psychosis | AntiNMDA | Scoop.it
Patients with autoimmune encephalitides, especially those with antibodies to the N-methyl-d-aspartate receptor (NMDAR), often present with prominent psychosis and respond well to immunotherapies. Although most patients progress to develop various neurological symptoms, it has been hypothesised...
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Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis | Neurology Neuroimmunology & Neuroinflammation

Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
Immunotherapeutic strategies for GAD65-AE remain highly controversial.27 Most patients are considered to require immunotherapy, and early immunotherapy has been found to be associated with a better outcome.10,28 However, the different neurologic manifestations of SPS, CA, and LE appear to respond...
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Anti-NMDAR Encephalitis: Multidisciplinary Development of a Clinical Practice Guideline | American Academy of Pediatrics

Anti-NMDAR Encephalitis: Multidisciplinary Development of a Clinical Practice Guideline | American Academy of Pediatrics | AntiNMDA | Scoop.it
Knowledge about the diagnosis of autoimmune encephalitis (AE) is rapidly expanding. In the last 15 years, multiple new antibodies have been described. Anti-N-methyl-D-aspartate receptor (NMDAR)–antibody-mediated encephalitis, in particular, has been found to be common among teenagers and young adults1 and accounts for up to 86% of AE in patients aged <18 years.2 Other antibodies associated with AE (leucine-rich glioma-inactivated 1, contactin-associated protein-like 2, glutamic acid decarboxylase 65-kilodalton isoform, γ-aminobutyric acid A, and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) are reported in children as case reports or series and with less clear typical clinical syndromes.3–9
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