Abstract
Background: The family of disorders associated with antibodies against neuronal surface antigens is a rapidly expanding category of autoimmune neurologic disease. Methods: Clinical and laboratory findings of a patient with voltage-gated Kv1 potassium channel (VGKC)-complex autoantibodies are reported and the literature is reviewed. Results: New-onset faciobrachial dystonic seizures, distal large and painful small fiber peripheral neuropathy, dysautonomia, cognitive and memory disturbances were associated with hypometabolism of the left temporal lobe without evidence of systemic malignancy in whole-body positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography. Brain MRI and cerebrospinal fluid were normal with absent intrathecal VGKC-complex antibodies consistent with extrathecal production. Epidermal nerve fiber studies confirmed small fiber neuropathy in association with abnormal autonomic laboratory testing. Discussion: Neurologic involvement associated with VGKC complex autoimmunity is a precisely delineated disorder involving the central, peripheral, and autonomic nervous system.
Highlights
New-onset faciobrachial dystonic seizures, distal large and painful small fiber peripheral neuropathy, dysautonomia, cognitive and memory disturbances were associated with hypometabolism of the left temporal lobe without evidence of systemic malignancy in whole-body positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography
Autoantibodies against the voltage-gated Kv1 potassium channel-(VGKC)complex detected by radio immunoprecipitation assay (RIA) in the sera of patients with autoimmune encephalitis do not bind to VGKC-complex channel
The earliest affected patients with VGKC-complex encephalitis were described by Brierley and colleagues in 1960 [4]
Summary
Autoantibodies against the voltage-gated Kv1 potassium channel-(VGKC)complex detected by radio immunoprecipitation assay (RIA) in the sera of patients with autoimmune encephalitis do not bind to VGKC-complex channel. Attention has been directed toward identifying the principal autoantigens in the VGKC-complex and an expanding spectrum of corresponding phenotypes. The principal autoantigens most commonly defined are leucine-rich glioma-inactivated protein 1 (LGI1) in the central nervous system (CNS) and contactin-associated protein-like (CASPR2) which associates with transiently-expressed axonal glycoprotein (TAG1), post synaptic density Protein-Drosophila disc large tumor suppressor-zonula occludens-1 protein (PDZ), and the ankyrin-spectrin protein in both the peripheral nervous system (PNS) and CNS [1] [2]. The commonest presentation of VGKCcomplex autoantibodies is LE in the CNS and neuromyotonia or Morvan syndrome in the PNS [3]. We present the clinical and laboratory findings of a patient that manifested PNS, CNS and autonomic nervous system (ANS) involvement
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