Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia

Limbic encephalitis is associated with various tumours and antibodies. We describe a 33-year-old man with progressive cochleovestibular symptoms, cerebellar ataxia, myoclonus and antibodies against Kelch-like protein 11 (KLHL11). MR scan of the brain showed hippocampal T2 hyperintensities. FDG PET scan was normal. Biopsy of his enlarged left testes showed fibrosis without neoplasia. Immunotherapy so far has not helped significantly.

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Publication Details

Journal
Practical Neurology
Published
2025-02-20
DOI
https://doi.org/10.1136/pn-2024-004498
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00
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article

Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia

Iftikhar Ahmad, Divyanshu Dubey, Mohammad Razaq Dogar, A Goonetilleke et al.
Practical Neurology
Autoimmune Neurological Disorders and Treatments
article

Paraneoplastic KLHL11 encephalitis presenting with progressive tinnitus, hearing loss and ataxia

Iftikhar Ahmad, Divyanshu Dubey, Mohammad Razaq Dogar, A Goonetilleke, Suzi Elhabet, Anu Jacob, Gina Lee, A. Ben Mahmoud
article en

Abstract

Limbic encephalitis is associated with various tumours and antibodies. We describe a 33-year-old man with progressive cochleovestibular symptoms, cerebellar ataxia, myoclonus and antibodies against Kelch-like protein 11 (KLHL11). MR scan of the brain showed hippocampal T2 hyperintensities. FDG PET scan was normal. Biopsy of his enlarged left testes showed fibrosis without neoplasia. Immunotherapy so far has not helped significantly.

Practical NeurologyVol. 25(5)
Cleveland Clinic (US), Mayo Clinic (US), University of Liverpool (GB), Walton Centre (GB), Abu Dhabi Health Services (AE)
Good health and well-being
Openalex Percentile: Top 95%
Autoimmune Neurological Disorders and Treatments
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