Anti-kelch-like protein 11 antibody-associated encephalitis: two case reports and literature review.

ABSTRACT:

Background

Anti-kelch-like protein 11 (KLHL11) antibody-associated encephalitis is a rare autoimmune neurological disorder, typically presenting with cerebellar syndrome and brainstem involvement. Diagnosis relies on the detection of anti-KLHL11 antibodies in serum and/or cerebrospinal fluid (CSF). Immunotherapy remains the cornerstone of treatment.

Case presentation

Patient 1 presented with an acute onset of dizziness, limb weakness, and slowed responses, with rapid symptom progression. Despite receiving immunotherapy-including intravenous corticosteroids, immunoglobulin, plasma exchange, and efgartigimod-which resulted in partial improvement, the patient experienced a rapid relapse and further clinical deterioration. He ultimately succumbed to central circulatory failure.

SUBMITTER: Chai S 

PROVIDER: S-EPMC12272960 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature