Objective:

To review details of a rare presentation of autoimmune encephalitis with antibody to GRAF1, which is rare and with little information about its origin or associated clinical syndrome, and educate clinicians to identify similar presentations early to allow effective treatment and prognostic counseling for patients and their family.

Background:

This case details presentation of a rare cause of autoimmune encephalitis with antibodies to GTPase regulator associated with focal adhesion kinase-1 (GRAF1). Reports in the literature may group this with other anti-Ca channel antibodies, and often associate it with an ataxic clinical syndrome. Little is known about the origin of these antibodies, as it is not always associated with malignancy or specific systemic conditions.

Design/Methods:

N/A

Results:

A 68 year old woman with a history of breast and cervical cancer, as well as Powassan encephalitis had recurrent episodes of encephalitis with prominent confusion, and was found to have a high titer of antibody to GRAF1 in her CSF with each clinical episode. She unfortunately did not respond well to immunosuppressive medications or IVIG, and passed away on hospice. The details of this case report highlight the clinical features associated with this case of GRAF1 antibody encephalitis and poses questions about whether cancer or prior CNS infection may be the origin of these antibodies, as our patient had a history or Powassan encephalitis. It is also not clear whether serial CSF testing can be used as maintenance to guide management.

Conclusions:

GRAF1 antibody encephalitis is a poorly understood cause of autoimmune encephalitis, without a consistent associated clinical syndrome, or associated condition that may be a source of the increased antibody titer. We pose the question whether this can be a post-infectious entity, and highlight a case of encephalitis which had recurrent episodes before becoming progressive and refractory to treatment.