Chronic pain as a manifestation of potassium channel-complex autoimmunity

Klein, Christopher J.; Lennon, Vanda A.; Aston, Paula A.; McKeon, Andrew; Pittock, Sean J.

doi:10.1212/wnl.0b013e3182698cab

Cited by 157 publications

(152 citation statements)

References 38 publications

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“…3,4 However, the clinical spectrum of Caspr2-associated autoimmunity is still being characterized and chronic pain has been shown to be a frequent accompaniment. 5 Our patient had a combination of OM with chronic neuropathic pain and mild cognitive deficits.…”

mentioning

confidence: 74%

Orthostatic myoclonus associated with Caspr2 antibodies

et al. 2016

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mentioning

confidence: 74%

Orthostatic myoclonus associated with Caspr2 antibodies

et al. 2016

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“…Autonomic involvement was noted in 29% of the cohort studied by Klein and colleagues [132], and in 3 (60%) of the patients described by Lahoria and coworkers [135]. Hypothermia was described in association with VGKC-complex antibody-associated LE in 4 patients [37], Patient 1 of whom had concomitant neuropathic patient, and in the absence thereof in the others who were conjectured to have otherwise disturbed hypothalamic thermoregulatory mechanisms as the cause for dysautonomia.…”

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 91%

“…Klein and coworkers [132] noted neuropathic pain associated with VGKC-complex autoimmunity in 4% patients evaluated neurologically at a tertiary referral center that was generally subacute in onset and regional or diffuse.…”

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

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Autoimmune encephalitis is a potentially severe disorder of the brain of diverse causes with a complex differential diagnosis. Recent advances in the past decade have led to the elucidation of new syndromes and biological markers transforming the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management. KeywordsAutoimmune, Encephalitis, Hashimoto, Encephalopathy Historical PerspectiveCorsellis and colleagues [1] coined the term limbic encephalitis (LE) in 1968, noting a relation to bronchial cancer in three patients in the sixth to eighth decade of life, and showing close clinicopathologic similarity to cases described by Brierley and colleagues [2] six years earlier. All three cases had subacute temporal lobe seizures, neuropsychiatric, and memory disturbances for two years before death. Postmortem examination showed inflammatory lesions in limbic grey matter sections of the brain, especially in medial temporal lobe structures of the uncus and amygdala nuclei, and hippocampal, cingulum and dentate gyri. Case 2 had an undifferentiated non-metastatic lung carcinoma removed six months after onset of neurological symptoms, while two others had a clinically unsus- The formulation of the relation of GAD antibodies to SPS has been especially instructive in understanding how far the science of autoimmune neurologic disorder has advanced. In 1988, Solimena and colleagues [28] investigated the existence of non-paraneoplastic CNS autoimmunity in a patient with SPS, epilepsy and type-1 diabetes (T1D), and increased titers of oligoclonal CSF IgG. Both the serum and CSF produced identical intense staining of all gray-matter regions. GAD65 was thence an important autoantigen in T1D, being highly expressed in the cytoplasm of pancreatic β cells. GAD-derived peptides were presentable by main histocompatibility complex (MHC) class I molecules and recognized by CD8+ cells on the surface of β cells [29]. Activation of CD8+ GAD-specific T-cells further differentiated into memory cells suggesting a pathogenic role in T1D [30]. However, only patients with very high titers of GAD were associated with LE [31], and they typically presented with recent-onset temporal lobe epilepsy (TLE) and intrathecal secretion, defining a form of non-paraneoplastic LE. Other patients within the SPS spectrum harbored antibodies agains...

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“…Neuropathic pain as a manifestation of VGKC-complex autoimmunity was noted in 316 (4%) of 1992 patients evaluated neurologically at a tertiary referral center [31] that was typically subacute in onset, nociceptive, regional, or diffuse. In those suspected of peripheral neuropathy, the abnormality detected most commonly in EDX studies was a minor reduction of sural SNAP with motor hyperexcitability, while structured examination revealed mild subjective loss of temperature and pain attributed to small fiber dysfunction.…”

Section: Laboratory Investigation Of Vgkc Complex Autoimmunitymentioning

confidence: 99%

“…Autonomic involvement, so noted in 29% of the cohort studied by Klein and colleagues [31] and in 3 (60%) of the patients described by Lahoria and coworkers [30], was ascertained by thermoregulatory sweating or axonal reflex testing (QSART). The symptoms of small fiber neuropathy (SFN), which arise from dysfunction in nociception, temperature and autonomic modalities [34], are most adequately assessed by assessment of ENF density [35] and a combination of cardiovagal, sudomotor and adrenergic functions tests [36].…”

Section: Dysautonomiamentioning

confidence: 99%

Limbic Encephalitis Associated with Voltage-Gated Potassium Channel-Complex Antibodies: Patient Report and Literature Review

Younger¹

2017

WJNS

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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.

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Chronic pain as a manifestation of potassium channel-complex autoimmunity

Cited by 157 publications

References 38 publications

Orthostatic myoclonus associated with Caspr2 antibodies

Orthostatic myoclonus associated with Caspr2 antibodies

Autoimmune Encephalitides

Limbic Encephalitis Associated with Voltage-Gated Potassium Channel-Complex Antibodies: Patient Report and Literature Review

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