Category: Neuroimaging (Non-PD)
Objective: We present a case that will raise awareness about the disease with atypical clinical and rich imaging findings that has benefited greatly from intensive immunotherapy despite its poor prognosis.
Background: Anti-leucine-richglioma inactivated protein 1 (LGI1) encephalitis is of autoimmune origin, characterized by subacute cognitive impairment and faciobrachial dystonic seizures.
Method: Our case was a 77 years old female patient who was admitted to the emergency department with involuntary contraction of her right arm for two days. Dystonic contraction in both upper extremities, choreiform movements in the right lower extremity distal, and short-term leaps in the legs were observed. During the follow-up, the patient developed altered consciousness.
No pathology was detected in the diffusion MR and cranial MR images other than chronic ischemic changes at admission. However hyperintense appearance was observed in T1 and T2 weighted series at the level of bilateral basal ganglia on cranial MR imaging taken a week later.
Routine CSF analysis was normal but positive Anti LGI1 autoantibodies were detected.
She was diagnosed with Anti LGI-1 encephalitis and received high dose methylprednisolone. During the treatment the patient lost her consciousness and transmitted to the intensive care unit. She was started on 2 gr/kg intravenous immunoglobulin (IVIG) and continued to receive high dose intravenous methylprednisolone. Under this treatment patient’s condition deteriorated rapidly, went into a coma and intubated. Therefore plasmapheresis was started 7 days after the end of IVIG treatment. A total of seven cure of plasmapheresis were performed.
Results: After the completion of plasmapheresis, a gradual improvement in consciousness was observed. The general belief in terms of treatment is the administration of antiepileptic with immunotherapy (cortiocsteroids, IVIG, plasmapheresis, rituximab).
Conclusion: In our case, we prevented the mortal outcome of the disease by intensive immunotherapy in the early period. Our patient is still under low-dose oral corticosteroid treatment and her well-being continues.
To cite this abstract in AMA style:
O. Yilmaz Kusbeci, I. Keskin, M. Gedizlioglu. A Rare Disease with Atypical Clinical And Imaging Findings: Anti Lgi-1 Encephalitis [abstract]. Mov Disord. 2020; 35 (suppl 1). https://www.mdsabstracts.org/abstract/a-rare-disease-with-atypical-clinical-and-imaging-findings-anti-lgi-1-encephalitis/. Accessed June 15, 2025.« Back to MDS Virtual Congress 2020
MDS Abstracts - https://www.mdsabstracts.org/abstract/a-rare-disease-with-atypical-clinical-and-imaging-findings-anti-lgi-1-encephalitis/