Article

A case of voltage-gated potassium channel antibody-related limbic encephalitis.

Addenbrooke's Hospital, Cambridge, UK.
Nature Clinical Practice Neurology (Impact Factor: 7.64). 07/2006; 2(6):339-43; quiz following 343. DOI: 10.1038/ncpneuro0194
Source: PubMed

ABSTRACT A 56-year-old man presented to hospital with a 6-month history of recurrent episodes of altered behavior and 'odd' episodes. He had become apathetic and uninterested in his family. He had no relevant past medical or family history. General and physical neurological examinations were unremarkable, as was bedside cognitive testing.
Brain MRI scan, 24-h electroencephalogram, serum and cerebrospinal fluid testing for voltage-gated potassium channel antibodies, blood screening for tumors, CT scans of the chest, abdomen and pelvis, whole-body PET scan, neuropsychological examination, brain 18F-fluorodeoxyglucose-PET scan.
Voltage-gated potassium channel antibody-related limbic encephalitis.
Antiepileptic drugs, immunomodulatory therapy, oral steroids, plasma exchange.

0 Followers
 · 
127 Views
  • Source
    • "Imaging abnormalities typical of VGKC-LE have also Fig. 2. A. and D. Axial T2/FLAIR MRI at the level of the temporal lobes and insula, respectively, with possible mesial temporal and insular hyperintensities; C. and F. FDG-PET images at these levels showing bitemporal and bilateral insular hypometabolism; B. and E. Coregistered MRI and PET at these levels, showing where the areas of hypometabolism align with the detailed neuroanatomy. been reported in patients manifesting personality changes and memory deficits without evidence of seizures [12] [13]. Furthermore, patients with VGKC-LE have been reported to have a normal brain MRI but bitemporal hypometabolism on brain PET [14] "
    [Show abstract] [Hide abstract]
    ABSTRACT: Preclinical and clinical studies have demonstrated the significance of inflammation and autoantibodies in epilepsy, and the use of immunotherapies in certain situations has become an established practice. Temporal lobe epilepsy can follow paraneoplastic or nonparaneoplastic limbic encephalitis associated with antibodies directed against brain antigens. Here, we focus on a patient with worsening confusion and temporal lobe seizures despite treatment with antiepileptic medications. Serial brain MRIs did not conclusively reveal structural abnormalities, so the patient underwent brain PET/MRI to simultaneously evaluate brain structure and function, revealing bitemporal abnormalities. The patient was diagnosed with voltage-gated potassium channel antibody-related limbic encephalitis based on clinical presentation, imaging findings, and antibody testing. Treatment included the addition of a second antiepileptic agent and oral steroids. His seizures and cognitive deficits improved and stabilized.
    12/2015; 4. DOI:10.1016/j.ebcr.2015.02.002
  • Source
    • "Moreover, post-mortem study of a VGKC-Ab patient revealed neural loss restricted to the hippocampus, and amygdala to a lesser extent, but no damage has thus far been evident in other MTL regions or neocortex (Khan et al., 2009). Additional imaging studies have provided further support that the hyperintensity signal (in MRI FLAIR sequences) as a result of VGKC-Ab/ acute amnesic encephalopathy, predominantly affects the MTL, specifically the hippocampus (Ances et al., 2005; Harrower et al., 2006; Reid et al., 2009). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Some prominent studies have claimed that the medial temporal lobe is not involved in retention of information over brief intervals of just a few seconds. However, in the last decade several investigations have reported that patients with medial temporal lobe damage exhibit an abnormally large number of errors when required to remember visual information over brief intervals. But the nature of the deficit and the type of error associated with medial temporal lobe lesions remains to be fully established. Voltage-gated potassium channel complex antibody-associated limbic encephalitis has recently been recognized as a form of treatable autoimmune encephalitis, frequently associated with imaging changes in the medial temporal lobe. Here, we tested a group of these patients using two newly developed visual short-term memory tasks with a sensitive, continuous measure of report. These tests enabled us to study the nature of reporting errors, rather than only their frequency. On both paradigms, voltage-gated potassium channel complex antibody patients exhibited larger errors specifically when several items had to be remembered, but not for a single item. Crucially, their errors were strongly associated with an increased tendency to report the property of the wrong item stored in memory, rather than simple degradation of memory precision. Thus, memory for isolated aspects of items was normal, but patients were impaired at binding together the different properties belonging to an item, e.g. spatial location and object identity, or colour and orientation. This occurred regardless of whether objects were shown simultaneously or sequentially. Binding errors support the view that the medial temporal lobe is involved in linking together different types of information, potentially represented in different parts of the brain, regardless of memory duration. Our novel behavioural measures also have the potential to assist in monitoring response to treatment in patients with memory disorders, such as those with voltage-gated potassium channel complex antibody limbic encephalitis.
    Brain 06/2013; 136(8). DOI:10.1093/brain/awt129 · 10.23 Impact Factor
  • Source
    • "Moreover, post-mortem study of a VGKC-Ab patient revealed neural loss restricted to the hippocampus, and amygdala to a lesser extent, but no damage has thus far been evident in other MTL regions or neocortex (Khan et al., 2009). Additional imaging studies have provided further support that the hyperintensity signal (in MRI FLAIR sequences) as a result of VGKC-Ab/ acute amnesic encephalopathy, predominantly affects the MTL, specifically the hippocampus (Ances et al., 2005; Harrower et al., 2006; Reid et al., 2009). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Some prominent studies have claimed that the medial temporal lobe is not involved in retention of information over brief intervals of just a few seconds. However, in the last decade several investigations have reported that patients with medial temporal lobe damage exhibit an abnormally large number of errors when required to remember visual information over brief intervals. But the nature of the deficit and the type of error associated with medial temporal lobe lesions remains to be fully established. Voltage-gated potassium channel complex antibody-associated limbic encephalitis has recently been recognized as a form of treatable autoimmune encephalitis, frequently associated with imaging changes in the medial temporal lobe. Here, we tested a group of these patients using two newly developed visual short-term memory tasks with a sensitive, continuous measure of report. These tests enabled us to study the nature of reporting errors, rather than only their frequency. On both paradigms, voltage-gated po
    Brain 06/2013; · 10.23 Impact Factor
Show more