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

作者：Shuting Chai, Gang Liu, Yan Zhang · 发表于：BMC Neurology · 年份：2025 · DOI：10.1186/s12883-025-04308-z · 被引用次数：6 · 研究领域：Autoimmune Neurological Disorders and Treatments、Glycogen Storage Diseases and Myoclonus、Vestibular and auditory disorders

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. Patient 2 exhibited a relatively slow disease course, primarily characterized by gait disturbances, slurred speech, and memory decline. He received only symptomatic treatment without immunotherapy. Follow-up assessments indicated slight symptomatic improvement. Brain magnetic resonance imaging (MRI) showed multiple hyperintense lesions on T2-weighted fluid-attenuated inversion recovery (T2/FLAIR) in the bilateral subcortical cerebral hemispheres and pons in patient 1. However, dominant pontine and cerebellar atrophy were noted in patient 2. Initial serum cell-based assay (CBA) testing detected anti-KLHL11 antibody titers of 1:30 in patient 1 and 1:100 in patient 2. CONCLUSION: This study indicates that p...