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Limbic encephalitis with LGI1 antibodies

Disease definition

Limbic encephalitis with LGI1 antibodies is a rare neuroimmunological disorder characterized by the onset of cognitive decline, psychiatric disturbances and seizures (distinctively faciobrachial dystonic seizures) in association with detection of LGI1 antibodies in serum or cerebrospinal fluid. Patients may present with confusion, hallucinations, vocalization, paranoia, tangentiality, aggressive outbursts and/or spatial disorientation, as well as obstinate hyponatremia. It is most often non-paraneoplastic, however comorbid tumors, such as small cell lung cancer and thymoma, have been reported.

ORPHA:163908

  • Synonym(s):
    • Limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: G04.8  G13.1
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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