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Bilateral hearing loss preceding rhomboencephalitis - a hint for Kelch-like 11 syndrome.
- Source :
-
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology [Neurol Sci] 2023 Jan; Vol. 44 (1), pp. 369-372. Date of Electronic Publication: 2022 Sep 16. - Publication Year :
- 2023
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Abstract
- Background: Paraneoplastic neurological syndromes have diverse clinical presentations and offer an opportunity for early diagnosis of malignancy and treatment. Recently, a new paraneoplastic syndrome associated with seminoma was described, consisting of rhombencephalitis with antibodies targeting the Kelch-like protein 11 (KLHL11). Questions were raised as to the spectrum of clinical symptoms and strength of association to seminoma.<br />Methods: We present a 45-year-old man with bilateral sensorineural hearing loss, vertigo, and progressive ataxia. An extensive diagnostic workup led to the diagnosis of anti-KLHL11 paraneoplastic syndrome based on an immunofluorescence assay showing a typical pattern and a confirmatory serological assay. As a result, the patient underwent a meticulous search for an underlying seminoma.<br />Results: Although initially, all images were interpreted as negative, a revision of the positron emission tomography-CT (PET-CT) examination identified a small mediastinal suspicious mass. The mass was resected, and pathological examination confirmed it to be an extra-testicular seminoma.<br />Conclusions: Patients presenting with progressive sensorineural hearing loss, vertigo, and ataxia should be evaluated for KLHL11 paraneoplastic syndrome. Furthermore, we support a strong association between anti-KLH11 rhombencephalitis and an underlying seminoma and recommend a thorough search for an undiagnosed germ cell tumor in these patients.<br /> (© 2022. Fondazione Società Italiana di Neurologia.)
- Subjects :
- Male
Humans
Middle Aged
Positron Emission Tomography Computed Tomography
Hearing Loss, Bilateral complications
Vertigo complications
Ataxia complications
Seminoma complications
Seminoma pathology
Paraneoplastic Syndromes complications
Paraneoplastic Syndromes diagnosis
Testicular Neoplasms complications
Testicular Neoplasms diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1590-3478
- Volume :
- 44
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
- Publication Type :
- Report
- Accession number :
- 36112277
- Full Text :
- https://doi.org/10.1007/s10072-022-06369-1