PURPOSE Autoimmune encephalitis is an inflammatory disorder of the brain that may be associated with different neuronal antibodies. Recently, an increasing number of valuable autoantibodies have been identified, including GABAAR antibodies, which appear to be associated with a severe form of encephalitis with refractory status epilepticus. We report here on a patient with encephalitis associate...

Anti-NMDAR encephalitis is a recently identified autoimmune disease, described by an immune-mediated loss of NMDA glutamate receptors, resulting in progressive mental deterioration. To date, literature on anti-NMDAR encephalitis has been largely clinically oriented, including descriptions of the clinical presentation and course, diagnostic methods, and potential clinical treatments. However, th...

anti n-methyl-d-aspartate receptor (nmdr) encephalitis has been recognized as the most frequent autoimmune encephalitis in children after acute demyelinating encephalomyelitis (adem).however due to the variable the variable clinical presentation, the paucity of specific finding on standard laboratory and radiological investigation remains under recognized. first discribed in 2005, most commonly...

Anti‐N‐methyl‐D‐aspartate receptor (anti‐NMDA‐R) encephalitis is a newly recognized neuro‐autoimmune disease. Patients usually present with a series of neurological and psychiatric syndromes including memory impairment, seizures, dyskinesia, autonomic nervous system dysfunction, etc. Anti‐NMDA‐R encephalitis usually affects young women with teratoma being a common co‐existing condition in nearl...

Background: Cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) is a helper T cell protein receptor that down regulates the immune system when bound to antigen presenting cells. Ipilimumab selectively binds to CTLA-4 inhibiting the immune tolerance to tumour cells and has recently been approved for the treatment of metastatic melanoma. Autoimmune sequelae are side-effects of such immunomodulat...

The development of encephalitis presents a dilemma to the clinician as during the early stages, when treatment would be most effective, the symptoms can be nonspecific with a broad differential. Imaging tests (e.g. magnetic resonance imaging and computed axial tomography scan), blood and urine tests as well as lumbar puncture are used to isolate and identify viruses, and together with careful a...

The autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) syndrome is a genetic disorder caused by a mutation in the autoimmune regulator (AIRE) gene. Immune deficiency, hypoparathyroidism and Addison's disease due to autoimmune dysfunction are the major clinical signs of APECED. We report on a 21-year-old female APECED patient with two inactivating mutations in the AIRE gene....

Autoimmune epilepsy is an isolated phenotype of autoimmune encephalitis, which may be suspected in patients with unexplained adult-onset seizure disorders or resistance to antiepileptic drugs (AEDs). Antibodies against leucine-rich glioma inactivated subunit 1 of the voltage-gated potassium channel (VGKC) complex, recently termed anti-LGI-1 antibodies, are one of the causes of autoimmune epilep...

objective: hashimoto encephalopathy (he) is known as a steroid-responsive encephalopathy associated with autoimmune thyroiditis or nonvascular inflammation-related autoimmune meningoencephalitis. the average age of onset of he is approximately 50 years; and it is more common in women. the onset of he may be acute or subacute. the course of most he cases is relapsing and remitting, which is simi...

PEARLS Limbic encephalitis is an inflammatory condition of the brain, often characterized by the subacute onset of short-term memory loss, disorientation, seizures, behavioral disturbance, and psychiatric symptoms. Besides paraneoplastic syndromes with onconeural antibodies targeted against intracellular neuronal antigens, limbic encephalitis may also arise from nonparaneoplastic mechanisms wit...