Paraneoplastic Progressive Downbeat Nystagmus, Ataxia and Sensorineural Hearing Loss due to the ANTI-Kelch-11 Protein Antibody

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Title Paraneoplastic Progressive Downbeat Nystagmus, Ataxia and Sensorineural Hearing Loss due to the ANTI-Kelch-11 Protein Antibody
Creator Jorge C. Kattah; Scott D. Eggers; Sarah E. Bach; Divyanshu Dubey; Andrew B. McKeon
Affiliation Department of Neurology (JCK, SB), Illinois Neurologic Institute, Peoria, Illinois; Department of Neurology (JCK, SB), Saint Francis Medical Center and University of Illinois College of Medicine; Department of Neurosurgery (JCK, SB), University of Illinois College of Medicine, Peoria, Illinois; Department of Pathology (JCK, SB), Neuropathology Section, University of Illinois College of Medicine, Peoria; Departments of Neurology (SDE, DD, AM) and Laboratory Medicine and Pathology (DD, AM), Mayo Clinic, Rochester, Minnesota
Abstract A 45-year-old man with a history of testicular seminoma treated 8 years earlier presented with chronic progressive truncal and limb ataxia, progressive sensorineural hearing loss, and episodic vertigo. Eye movement and neuro-otology examinations showed localizing abnormalities to the bilateral cerebellar flocculus, vermis, and bilateral cerebellar hemispheres. Audiometric testing showed bilateral symmetric sensorineural hearing loss. There was a normal MRI of the brain. Cerebrospinal fluid (CSF) showed modest lymphocytic pleocytosis, and there was an elevated serum choriogonadotrophic hormone. An abdominal CT scan showed a solitary, large retroperitoneal lymph node, and histopathologic examination of the node biopsy showed granulomatous inflammation without microorganisms; eventually, immunohistochemical markers confirmed the diagnosis of metastatic seminoma. Although normal neuroimaging and inflammatory CSF reaction suggested a paraneoplastic etiology, the initial paraneoplastic antibody testing was negative. Subsequent investigation identified a positive kelch-11 protein antibody, thus confirming the paraneoplastic connection between the metastatic seminoma and the subacute neurologic-cochleovestibular syndrome.
Subject Ataxia; Autoantibodies; Tumor Biomarkers; Carrier Proteins; Eye Movements; Sensorineural Hearing Loss; Neoplasm Metastasis; Pathologic Nystagmus; Ocular Paraneoplastic Syndromes; Seminoma; Testicular Neoplasms; X-Ray Computed Tomography
OCR Text Show
Date 2021-06
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, June 2021, Volume 41, Issue 2
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s6b0pykz
Setname ehsl_novel_jno
ID 1996625
Reference URL https://collections.lib.utah.edu/ark:/87278/s6b0pykz
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