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Abstract

Hippocampal Sclerosis in LGI1 and CSPR2 Positive Limbic Encephalopathy: Case Report

Author(s): Ammar Taha Abdulaziz, Le Zhang, Dong Zhou, JinMei Li

Background:

Limbic encephalopathy (LE) is a sub-acute neuropsychiatric disorder characterized by the involvement of limbic structures. It was described to be associated with voltage-gated potassium channel (VGKC)–complex antibodies, particularly, leucine-rich glioma inactivated 1 (LGI1), but less frequently contactin associated protein 2 (CASPR2).

Case report:

We present a female patient with sub-acute memory impairment, followed by psychiatric symptoms and cognitive impairment. Further investigation reveals positive (VGKC)–complex antibodies. Her initial cranial MRI was normal and responded very well to the immunotherapy. Subsequent one year follow-up showed seizure control, psychiatric symptoms remission and some memory improvement. Despite both LGI1 and CASPR2 being negative, her MRI revealed wide cortex atrophy including bilateral hippocampal sclerosis.

Conclusion:

The clinical phenotype of patient with both LGI1 and CASPR2 positive is diverse, so early diagnosis and treatment is crucial, as they may improve the clinical outcome; however, the development of wide cortex atrophy including hippocampal sclerosis soon after acute phase is rare, and may indicate a bad prognosis in long-term. Further follow-up is needed to clarify the prognosis.


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