CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis

Jarius, Sven; Hoffmann, Lutz; Clover, Linda; Vincent, Angela; Voltz, Raymond

doi:10.1016/j.jns.2007.11.004

Cited by 81 publications

(64 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A disturbance of the integrity of BBB was found in 6 (35%) patients based upon an abnormal CSF/ serum humoral immune response. Absence of CSF-specific OCB, considered a marker of autochthonous antibody synthesis within the CNS in all patients [152] suggested an extrathecal origin of VGKC-complex autoantibodies. Vincent and colleagues [33] reported the CSF findings in 10 patients, all with VGKC-complex antibody-associated LE, noting mild lymphocytosis and mild or moderately raised protein content in one-half.…”

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 93%

“…Both mesial temporal lobe hypometabolism on FDG brain PET [149] [150] and hypermetabolism have been described [151]. A patient with VGKC-complex LE [150] who did not definitively demonstrate structure abnormalities on serial brain MRI over time despite ongoing temporal lobe According to Jarius and colleagues [152], who performed 29 lumbar punctures in 17 patients with VGKC-complex LE, noting normal findings in up to 53% of CSF specimens. There were no significant differences between the CSF findings and the titers of serum VGKC-complex autoantibodies.…”

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 99%

See 1 more Smart Citation

Autoimmune Encephalitides

Younger¹

2017

WJNS

View full text Add to dashboard Cite

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...

show abstract

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 93%

Section: Anti-vgkc-complex Encephalitismentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

View full text Add to dashboard Cite

show abstract

“…A disturbance of the integrity of BBB was found in 6 (35%) patients based upon an abnormal CSF/serum humoral immune response. Absence of CSF-specific OCB, considered to be a marker of autochthonous antibody synthesis within the CNS in all patients [27] suggested an extrathecal origin of VGKC-complex autoantibodies. Vincent and colleagues [13] reported the CSF findings in 10 patients, all with VGKC-complex antibody-associated LE, noting mild lymphocytosis and mild or moderately raised protein content in one-half.…”

Section: Vgkc-complex Subtypingmentioning

confidence: 93%

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

Younger¹

2017

WJNS

View full text Add to dashboard Cite

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.

show abstract

“…VGKC limbic encephalitis is mostly non-paraneoplastic, although VGKC antibodies have been found in a small number of patients with tumors (Pozo-Rosich et al 2003). Jarius et al showed that even patients without CSF pathological findings or inflammatory changes can be positive for VGKC antibodies (Jarius et al 2008). Non-herpetic acute limbic encephalitis (NHALE) has been identified as a new subgroup of limbic encephalitis with a clinical presentation which is similar to HSE (Asaoka et al 2004;Ichiyama et al 2009;Kusuhara et al 1994;Shoji et al 2004).…”

Section: Limbic Encephalitismentioning

confidence: 99%

Biomarkers of Encephalitis

Bonneh‐Barkay¹

2011

Pathogenesis of Encephalitis

View full text Add to dashboard Cite

CSF findings in patients with voltage gated potassium channel antibody associated limbic encephalitis

Cited by 81 publications

References 18 publications

Autoimmune Encephalitides

Autoimmune Encephalitides

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

Biomarkers of Encephalitis

Contact Info

Product

Resources

About