Management of Voltage-Gated Potassium Channel Antibody Disorders

Merchut, M P

doi:10.1016/j.ncl.2010.03.024

Cited by 59 publications

(37 citation statements)

References 48 publications

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“…The last subtype is the limbic encephalitis characterised by the CNS involvement with encephalopathic seizures, hyponatremia and abnormal electroencephalographic abnormalities. The antibodies noticed in this immune disorder were pathogenic and were confirmed by the observations of electrophysiological changes in mice, upon administration of IgG derived from patients (Kleopas et al, 2006;Buckley & Vincent, 2005;Merchut, 2010). Recently, a novel pathology was attributed to limbic encephalitis in which auto antibodies were identified against leucine-rich glioma-inactivated 1 (LGI1) protein.…”

Section: Neuromyotonia (Nm)mentioning

confidence: 70%

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Recent Advances in the Treatment of Neurological Autoimmune Disorders

Kanwar¹,

Sriramoju²,

Kanwar³

2011

Autoimmune Disorders - Current Concepts and Advances From Bedside to Mechanistic Insights

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Section: Neuromyotonia (Nm)mentioning

confidence: 70%

“…Most of the patients with these disorders fairly respond when treated with immunosuppressive drugs like glucocorticosteroids. Also other recommendations include plasmapheresis and IVIg (Merchut, 2010).…”

Section: Neuromyotonia (Nm)mentioning

confidence: 99%

Recent Advances in the Treatment of Neurological Autoimmune Disorders

Kanwar¹,

Sriramoju²,

Kanwar³

2011

Autoimmune Disorders - Current Concepts and Advances From Bedside to Mechanistic Insights

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“…VGKC antibodies are seen in neuromyotonia, Morvan syndrome, epilepsy, and dysautonomic phenomena of hyperhidrosis and gastrointestinal dysmotility [1,2]. Interestingly, hyponatremia is not seen in these disorders [10]. Reports have postulated that the leucine-rich, glioma inactivated 1 (LGI-1) protein may be the target antigen of VGKC mediated LE whereas the contactin-associated protein 2 (CASPR-2) may be the antigen in Issaac's and Morvan's syndrome [9].…”

Section: Discussionmentioning

confidence: 99%

Hyponatremia and Voltage Gated Potassium Channel Antibody Associated Limbic Encephalitis

Cr¹,

Sarwal²

2014

J Neurol Neurophysiol

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Limbic encephalitis may occur as an infectious, paraneoplastic, or autoimmune phenomenon. One such cause of limbic encephalitis is voltage gated potassium channel antibodies (VKGC). Hyponatremia with new cognitive decline may be one of the presenting symptoms. The exact mechanism of hyponatremia is unknown though findings consistent with syndrome of inappropriate antidiuretic hormone (SIADH) are observed. We retrospectively reviewed all cases admitted to an academic medical center with a diagnosis of limbic encephalitis (848 adults between 2004 to 2010) and found six cases of VGKC antibody associated limbic encephalitis. Three of the six cases had SIADH that completely or partially resolved with a combination of water restriction and immunotherapy. The reversibility of hyponatremia and limbic encephalitis with immunomodulation suggests an antibody-mediated cause. We further review available literature for association of hyponatremia and VGKC limbic encephalitis and propose mechanisms of for the hyponatremia in autoimmune encephalitis.

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“…Although LE is frequently related to auto-immune etiology, a paraneoplastic syndrome is found in 30% of the cases 3 . When hyperhidrosis, autonomic dysfunction, psychiatric symptoms and neuromyotonic discharges are present, Morvan syndrome must be suspected 5 . Although often unrecognized, the neurological spectrum of VGKC antibody-associated LE includes seizures and neuropsychiatric symptoms, ranging from alterations in memory, behavior, and cognition…”

Section: Discussionmentioning

confidence: 99%

Subacute cognitive impairment, hyponatremia and mesial temporal lobe lesions: a typical presentation of voltage-gated potassium channel (VGKC) antibody-associated limbic encephalitis

Saccardi

Pedroso

Bernardino

et al. 2011

Arq. Neuro-Psiquiatr.

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For many years, several reports have called attention for non-viral limbic encephalitis (LE), particularly with paraneoplastic etiology. However, an increasing number of data have demonstrated that some of those disorders are immune-mediated with antibodies to voltage-gated potassium channels (VGKC)1 . Herein, we present a case of VGKC antibody-associated LE and discuss the main phenotypic and imaging features of this unusual disease. CASEA 50-year-old man presented to our hospital with three months history of progressive personality change, sleep disturbance, memory impairment, visual hallucinations and seizures. Neurological examination showed disorientation, cognitive impairment (Mini Mental State Examination: 13) and memory dysfunction. Blood tests showed hyponatremia -130 mmol/L (135-145 mmol/L). Thyroid function, antibodies and serologic tests were normal. Cerebrospinal fluid showed high level of proteins (558 mg/dL) and mild lymphocytic pleocytosis. Brain magnetic resonance imaging (MRI) disclosed bilateral T2 and FLAIR hyperintensity of mesial temporal lobes (Figure). Cancer investigation resulted negative. VGKC serum level was higher than 650 pmol/L (normal range less than 450 pmol/L). These findings confirmed the diagnosis LE with VGKC antibodies. Methylprednisolone was started, with partial improvement. Three weeks later, immunoglobulin 0.4 g/Kg daily for five consecutive days was initiated. One week after immunoglob-

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Management of Voltage-Gated Potassium Channel Antibody Disorders

Cited by 59 publications

References 48 publications

Recent Advances in the Treatment of Neurological Autoimmune Disorders

Recent Advances in the Treatment of Neurological Autoimmune Disorders

Hyponatremia and Voltage Gated Potassium Channel Antibody Associated Limbic Encephalitis

Subacute cognitive impairment, hyponatremia and mesial temporal lobe lesions: a typical presentation of voltage-gated potassium channel (VGKC) antibody-associated limbic encephalitis

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