Case of LGI-1 Associated Encephalitis Presenting as Isolated Asymmetric Chorea
Meenakshi Roy1, Christopher Goetz1
1Rush University Medical Center, Section of Movement Disorders, Department of Neurological Sciences
Objective:
NA
Background:

Chorea can be part of a wide spectrum of autoimmune, paraneoplastic, or para-infectious conditions. Antibodies against leucine-rich-glioma-inactivated-1 (LGI1), component of voltage-gated potassium channels (VGKC), typically produce a syndrome of autoimmune encephalitis (AE), hyponatremia, and facio-brachial dystonic seizures (FBDS). Movement disorders related to LGI-1 can include generalized chorea, typically occurring in the context of the above-cited syndromes. Isolated asymmetric chorea as a presenting feature is rare. We describe a patient with LGI1-associated-AE presenting with subacute asymmetric chorea. 

Design/Methods:
Case presentation with pre- and post-treatment videos
Results:
65-year-old man presented with subacute onset of continuous, unpredictable movements in extremities and trunk without face involvement. There were no changes in mood, behavior, or cognition. Neurologic exam showed asymmetric generalized chorea worse on the left, without other deficits. Labs showed mild hyponatremia (134 mEq/L). MRI brain showed subtle T2/FLAIR hyperintensities in bilateral mesial temporal lobes. Computed Tomography scans were without evidence of malignancy. Serum and CSF AE panel confirmed LGI-1-antibodies. CSF was otherwise unremarkable, without pleocytosis and marginally elevated protein 39 mg/dl (ref 7.0 - 35.0). Multiple electroencephalograms did not show epileptiform discharges or seizures.  The patient responded well to concurrent high-dose steroids and IVIG. Movements resolved at hospital discharge. 
Conclusions:

There are only a few case reports of chorea in LGI-1-autoimmune encephalitis, and typically in association with other classic features. The one other report we found of isolated LGI-1 chorea was by Ramdhani and Frucht (2014). Like theirs, our patient had isolated generalized chorea and mild hyponatremia, without classic features of AE. However, in our patient, the chorea was markedly asymmetric with occasional bilateral involvement. He continued to perform all activities of daily living independently. As is typical in LGI-1-AE, he did not have any underlying malignancy. He had a robust response to steroids and immunotherapy, with resolution of symptoms.

10.1212/WNL.0000000000204753