Autoantibodies to NMDA receptor in patients with chronic forms of epilepsia partialis continua

Abstract

The presence of autoantibodies against NMDA GluR epsilon2 suggests autoimmune pathologic mechanisms but is not a hallmark of Rasmussen's encephalitis. Patients with Rasmussen's encephalitis may have autoantibodies against several neural molecules, and these autoantibodies may be produced in the CNS after cytotoxic T cell-mediated neuronal damage.

Autoimmune Epilepsy: Some Epilepsy Patients Harbor Autoantibodies to Glutamate Receptors and dsDNA on both Sides of the Blood‐brain Barrier, which may Kill Neurons and Decrease in Brain Fluids after Hemispherotomy

“…These observations and suggestions are in line with the findings of other antiself Ab's, such as anti-Munc 18 Ab's (Yang et al, 2000) and anti-NMDA receptor Ab's, in the serum and CSF of patients with epilepsia partialis continua (EPC)/RE (Takahashi et al, 2003).…”

“…At present, we can only speculate that this autoimmunity might be a consequence of either a specific or a nonspecific "irritation" of the immune system, for example by certain viruses, bacteria, vaccinations in childhood, food poisoning, injury, trauma, stress or any other condition. Such a speculation is strengthened to some extent by a recent study (Takahashi et al, 2003) claiming that the "causative or underlying factors" of chronic EPC in 15 patients were infection in five, vaccination in three, encephalitis in two, and head trauma in one.…”

“…Despite all that, the direct evidence that this occurs in vivo in epilepsy patients is still misssing and a great deal remains to be explored for reaching an understanding of the role of anti-GluR3 Ab's in epilepsy. Among the issues not studied thus far are whether the cerebrospinal fluid (CSF) levels of anti-GluR3B Ab's correlate with seizure frequency, whether human anti-GluR3B rich serum or CSF have the ability to kill neurons, and whether RE patients and potentially other epilepsy patients harbor in addition to the anti-GluR3 Ab's and other anti-glutamate receptor Ab's (Takahashi et al, 2003), also elevated levels of other autoimmune Ab's of known pathogenic activity in the periphery and CNS. These latter Ab's could include: anti-double-stranded (ds) DNA Ab's, found primarily in systemic lupus erythematosus (SLE); anti-glutamic acid decarboxylase (GAD) Ab's, found primarily in insulindependent diabetes mellitus (IDDM) and in stiff person syndrome; anti-cardiolipin and anti-b2 glycoprotein I (b2GPI) Ab's, found primarily in anti-phospholipid syndrome (APS); and anti-nuclear Ab's found primarily in SLE and Sjögren's syndrome.…”

Anaesthesia for a patient with paraneoplastic limbic encephalitis with ovarian teratoma: relationship to anti‐N‐methyl‐d‐aspartate receptor antibodies

“…In Japan, young women with acute non-herpetic limbic encephalitis have been studied as acute juvenile female non-herpetic encephalitis (AJFNHE). Although antibodies against GluRe2 have been frequently found in the sera and CSF of patients with AJFNHE [2], they have been also detected in patients with Rasmussen's encephalitis and epilepsia partialis continua [7]. Of these diagnoses, AJFNHE fits well with the presentation of our patient.…”

Non-Herpetic Acute Limbic Encephalitis: A New Subgroup of Limbic Encephalitis?

“…Antibodies against each domain of GluRε2 (NR2B) and GluR1 (NR1) were examined by ELISA with synthesized peptides. NMDA=N-methyl-D-asparate, GluR= glutamate receptor On the other hand, the anti-GluR ε2 antibodies are also detected in Rasmussen encephalitis, acute encephalitis with refractory repetitive partial seizures (AERRPS), mild encephalopathy with a reversible splenial lesion (MERS), or HSE (Takahashi et al, 2003;Sakuma et al, 2009;Kai et al, 2009). Anti-GluRε2 antibody encephalitis may form a wider spectrum of encephalitis and encephalopathy.…”