Potassium Channel Antibody-associated Limbic Encephalitis Research Articles

A Case of Antiglutamic Acid Decarboxylase and Voltage-Gated Potassium Channel Antibody-Associated Limbic Encephalitis With Temporal Lobe Epilepsy

A Case of Antiglutamic Acid Decarboxylase and Voltage-Gated Potassium Channel Antibody-Associated Limbic Encephalitis With Temporal Lobe Epilepsy

Voltage-Gated, Potassium-Channel Antibody-Associated Limbic Encephalitis Presenting as Acute Psychosis

Voltage-Gated, Potassium-Channel Antibody-Associated Limbic Encephalitis Presenting as Acute Psychosis

Voltage-Gated, Potassium-Channel Antibody-Associated Limbic Encephalitis Presenting as Acute Psychosis

Voltage-Gated, Potassium-Channel Antibody-Associated Limbic Encephalitis Presenting as Acute Psychosis

Brief Manic Episode After Rituximab Treatment of Limbic Encephalitis

Brief Manic Episode After Rituximab Treatment of Limbic Encephalitis

Disease-relevant autoantibodies in first episode schizophrenia

Disease-relevant autoantibodies in first episode schizophrenia

PATU1 Characteristic faciobrachial dystonic seizures as an immunotherapy-responsive prodrome to voltage-gated potassium channel antibody-associated limbic encephalitis

LE defines the subacute onset of amnesia and seizures. However, there are few reliable clinical features distinguishing VGKC-antibody associated LE from the many other forms of LE. Identification of such...

Improvement of non-paraneoplastic voltage-gated potassium channel antibody-associated limbic encephalitis without immunosuppressive therapy

We describe a 61-year-old patient with clinical evidence of limbic encephalitis who improved with anticonvulsant treatment only, that is, without the use of immunosuppressive agents. Three years following occurrence of anosmia, increasing memory deficits, and emotional disturbances, he presented with new-onset temporal lobe epilepsy, with antibodies binding to neuronal voltage-gated potassium channels and bitemporal hypometabolism on FDG-PET scan; the MRI scan was normal. This is most likely a case of spontaneous remission, illustrating that immunosuppressive therapy might be suspended in milder courses of limbic encephalitis. It remains open whether treatment with anticonvulsant drugs played an additional beneficiary role through the direct suppression of seizures or, additionally, through indirect immunomodulatory side effects.

Development of Isaacs' syndrome following complete recovery of voltage-gated potassium channel antibody-associated limbic encephalitis

Autoantibodies against voltage-gated potassium channels (VGKC-Abs) are associated with acquired neuromyotonia (Isaacs' syndrome) and related disorders such as Morvan's syndrome and some cases of limbic encephalitis. The mechanisms underlying the various phenotypes induced by VGKC-Abs are not fully understood. Recently, we reported a case of LE with VGKC-Abs accompanied by severe intestinal pseudo-obstruction and thymoma. Thymectomy and immunosuppressive therapy induced dramatic clinical improvement of LE symptoms, and VGKC-Abs titers decreased from 1254 pM to 549 pM (normal>100 pM). Seventeen months later, the patient developed progressive generalized muscle cramping, paresthesias in his lower extremities, excessive sweating, and severe constipation. There was no recurrence of the LE. Electromyography showed fasciculation potentials and myokymic discharges, and the plasma VGKC-Abs titer was again elevated to 879 pM. Here we report a case of Isaacs' syndrome after complete remission of LE with VGKC-Abs that may provide an insight into a possible link among VGKC-Abs associated syndromes.

Rapid eye movement sleep behavior disorder and potassium channel antibody–associated limbic encephalitis

Of six patients registered in our center with nonparaneoplastic limbic encephalitis associated with antibodies to voltage-gated potassium channels, the five men had rapid eye movement sleep behavior disorder (RBD) coincident with voltage-gated potassium channel antibody-associated limbic encephalitis onset. In three patients, immunosuppression resulted in resolution of RBD in parallel with remission of the limbic syndrome. RBD persisted in two patients with partial resolution of the limbic syndrome. Our findings suggest that RBD is frequent in the setting of voltage-gated potassium channel antibody-associated limbic encephalitis and can be related to autoimmune-mediated mechanisms. In addition, these observations suggest that impairment of the limbic system may play a role in the pathogenesis of RBD.