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Related Concept Videos

Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Type I Diabetes I: Introduction01:12

Type I Diabetes I: Introduction

Type 1 diabetes mellitus is a chronic metabolic disorder characterized by an absolute deficiency of insulin resulting from the autoimmune destruction of pancreatic β-cells. Although it can occur at any age, it is most commonly diagnosed in childhood, adolescence, or early adulthood. The loss of insulin production impairs cellular glucose uptake, resulting in persistent hyperglycemia and necessitating lifelong insulin therapy.Autoimmune Destruction of β-CellsThe hallmark of type 1 diabetes is an...
Type I Diabetes II: Pathophysiology01:26

Type I Diabetes II: Pathophysiology

Type 1 diabetes mellitus arises from an immune-mediated destruction of pancreatic β-cells, resulting in an absolute deficiency of insulin. This process develops in genetically susceptible individuals when autoimmunity, environmental exposures, and immunologic dysregulation converge to trigger a targeted attack on the insulin-producing cells of the pancreas. The β-cells are located within the islets of Langerhans and are essential for regulating blood glucose by facilitating cellular uptake of...
Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...

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Related Experiment Video

Updated: May 21, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
26:48

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Recurrent Autoimmune Encephalitis in a Patient With Autoimmune Polyendocrine Syndrome Type 1.

Jason T Stemple1, Kamal Abulebda1, Grace F Ryan1

  • 1Division of Pediatric Critical Care, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, Indiana, USA, indiana.edu.

Case Reports in Critical Care
|May 20, 2026
PubMed
Summary
This summary is machine-generated.

Autoimmune polyendocrine syndrome Type 1 (APS-1) patients can experience autoimmune encephalitis. Early immunotherapy, including plasma exchange and rituximab, led to significant recovery in a critically ill adolescent with recurrent autoimmune encephalitis and APS-1.

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Area of Science:

  • Neurology
  • Immunology
  • Genetics

Background:

  • Autoimmune polyendocrine syndrome Type 1 (APS-1/APECED) is a rare autoimmune disorder.
  • Neurologic manifestations are increasingly recognized in APS-1.
  • Autoimmune encephalitis presents diagnostic and management challenges, particularly in rare genetic conditions.

Purpose of the Study:

  • To report a case of recurrent autoimmune encephalitis in an adolescent with APS-1.
  • To highlight the diagnostic difficulties and treatment strategies for autoimmune encephalitis in APS-1 patients.
  • To emphasize the favorable response to early immunomodulatory therapy.

Main Methods:

  • Case report of a critically ill adolescent female with APS-1 and a history of autoimmune encephalitis.
  • Clinical presentation included acute encephalopathy and seizures.
  • Diagnostic workup involved electroencephalography (EEG), brain MRI, and antibody testing (GAD65).
  • Treatment comprised aggressive immunotherapy: plasma exchange, high-dose corticosteroids, and rituximab.

Main Results:

  • The patient presented with focal seizures and cytotoxic lesions on brain MRI.
  • High-titer glutamic acid Decarboxylase 65 (GAD65) antibodies confirmed autoimmune encephalitis relapse.
  • Marked clinical improvement was observed after aggressive immunotherapy.
  • This represents the first reported case of recurrent autoimmune encephalitis in APS-1 with a positive treatment outcome.

Conclusions:

  • Autoimmune encephalitis is a significant neurologic complication in APS-1.
  • Early and aggressive immunotherapy can lead to favorable outcomes.
  • This case underscores the importance of considering autoimmune encephalitis in APS-1 patients with neurologic symptoms.