Abstract Details

Title Exploring Rare Cases: Clinical Insights, Neoplasm Associations, and Therapeutic Approaches in Anti-KLHL11 Antibody-Associated Encephalitis: A Systematic Review of Case Reports

Topic Autoimmune Neurology

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 030

Objective

Anti-Kelch (KLHL)11-Associated Encephalitis - Clinical Insights, Neoplasm Associations, and Therapeutic Approaches.

Background

First identified in 2019, Anti-Kelch (KLHL)11 antibody encephalitis involves an antibody that targets the KLHL11 antigen intracellularly, detectable in serum and spinal fluid using specific assays. It mainly causes symptoms like ataxia, diplopia, and vertigo. This condition is often associated with tumors, especially testicular seminomas, followed by ovarian teratomas and other cancers.

Design/Methods

We conducted a literature review on PubMed, Web of Science covering the years 2014-2024 using the keywords "autoimmune encephalitis," "Anti-KLHL11 antibody," and "neurological paraneoplastic syndromes."

Results

We reviewed 1600 papers and selected 13 case reports. Of these, 10 were male (77%), aged 33-70, and 3 were female (23%)—62 years, 1 young and 1 middle-aged. 11 patients (84%) had tumors, while 2 did not (16%). The most frequent tumor type was testicular seminoma (54%), followed by other tumor types (27%). The MRI-brain, which showed abnormalities in 9 patients (69%) and normal results in 4 (31%). The most prevalent MRI findings were cerebellar and brainstem atrophy, particularly in the pons region. The symptoms, in descending order of occurrence, were ataxia (81%), vertigo (45%), hearing loss (45%), diplopia (36%), and other symptoms (18%). KLHL11 antibodies were tested in both CSF and serum in 8 patients (61%), only CSF in 3 (23%), and only serum in 2 (16%). The predominant treatment modalities were Rituximab and Methylprednisolone, used in 9 patients (69%).

Conclusions

This review of numerous case reports indicates the critical importance of suspecting Anti-KLHL11 antibody-associated encephalitis in individuals presenting with subacute, progressive brainstem and/or cerebellar symptoms. If this type of encephalitis is suspected, it is crucial to thoroughly investigate them for Anti KLHL11 antibodies and presence of concurrent neoplasms, particularly testicular seminomas in male patients. Further studies should focus on whether the stage of the associated neoplasms affects the prognosis of this disease.

Authors/Disclosures
Jayksh Chhabra, MBBS PRESENTER	Dr. Chhabra has nothing to disclose.
Akankshi Oberoi, MBBS	Dr. Oberoi has nothing to disclose.
Sharon A. Zachariah	Miss Zachariah has nothing to disclose.
Arjun Peddireddy, BA	Mr. Peddireddy has nothing to disclose.
Rajesh K. Gupta, MBBS (UTHealth)	Dr. Gupta has nothing to disclose.

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