Anti-Kelch-like protein 11 (KLHL11) antibody associated encephalitis -Two case reports and review of the literature.

Léon Betancourt, Alejandro; Schwarzwald, Anina; Millonig, Alban; Oberholzer, Michael; Sabater, Lidia; Hammer, Helly; Kamber, Nicole; Diem, Lara; Chan, Andrew; Hoepner, Robert; Salmen, Anke; Friedli, Christoph (2023). Anti-Kelch-like protein 11 (KLHL11) antibody associated encephalitis -Two case reports and review of the literature. European journal of neurology, 30(6), pp. 1801-1814. Wiley 10.1111/ene.15758

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INTRODUCTION

Kelch-like protein 11 antibodies (KLHL11-IgG) were first described in 2019 as a marker of paraneoplastic neurological syndromes (PNS). They have mostly been associated with testicular germ cell tumours (tGCT).

METHODS

Report of two patients with KLHL11-IgG encephalitis and comprehensive review of literature.

RESULTS

Patient 1 had been in remission from a tGCT 10 years prior. He developed episodic vertigo and diplopia progressing over a few days. Treatment with corticosteroids (CS) was started few days after symptom-onset. Patient 2 had transient diplopia which resolved spontaneously. Visual problems persisted for 7 months, when he additionally developed a progressive cerebellar syndrome. 1 year after onset, CS treatment was started. Initial MRIs were unremarkable in both patients, but analysis of cerebrospinal fluid (CSF) revealed chronic inflammation. KLHL11-IgG was positive in both patients (Patient 1 only in CSF, patient 2 in serum). Neoplastic screening has so far not revealed any signs of active underlying malignancy. We found 15 publications of 112 patients in total with KLHL11-IgG encephalitis. Most patients (n=82) had a cerebellar syndrome with or without signs of rhombencephalitis. The most common symptoms were ataxia (n=82) and vertigo (n=47), followed by oculomotor disturbances (n=35) and hearing disorders (n=31). 80 of 84 patients had a GCT as an underlying tumour.

CONCLUSION

Our cases demonstrate classical symptoms of KLHL11-IgG encephalitis. An early diagnosis and therapy is imperative. As with other PNS, clinical awareness is needed and further studies are required especially in regard to therapeutic management.

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