Abstract
A 36-year-old man had progressive gait ataxia, diplopia, and hearing loss over 3 years. On examination, he had upper extremity ataxia, truncal ataxia, bradykinesia, and lower extremity spasticity. Brain magnetic resonance imaging revealed a “hot cross bun” sign (Figure). Kelch-like protein 11 (KLHL11) IgG was detected in serum and cerebrospinal fluid.1Dubey D. Wilson M.R. Clarkson B. et al.Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis [published correction appears in JAMA Neurol. 2020;77(11):1453].JAMA Neurol. 2020; 77: 1420-1429Crossref PubMed Scopus (40) Google Scholar Positron emission tomography revealed a hypermetabolic mediastinal mass. Subsequent biopsy revealed seminoma. The hot cross bun sign has been described in patients with multiple system atrophy as well as in a patient with KLHL11 encephalitis.2Massano J. Costa F. Nadais G. Teaching neuroImage: MRI in multiple system atrophy: “hot cross bun” sign and hyperintense rim bordering the putamina.Neurology. 2008; 71: e38Crossref PubMed Scopus (14) Google Scholar,3Ishikawa H. Mandel-Brehm C. Shindo A. et al.Long-term MRI changes in a patient with Kelch-like protein 11-associated paraneoplastic neurological syndrome.Eur J Neurol. 2021; 28: 4261-4266Crossref PubMed Scopus (3) Google Scholar Some KLHL11 rhombencephalitis cases have been misdiagnosed as multiple system atrophy.1Dubey D. Wilson M.R. Clarkson B. et al.Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis [published correction appears in JAMA Neurol. 2020;77(11):1453].JAMA Neurol. 2020; 77: 1420-1429Crossref PubMed Scopus (40) Google Scholar This case highlights the importance of considering KLHL11 IgG evaluation in men with progressive brainstem/cerebellar syndrome despite the radiological findings.1Dubey D. Wilson M.R. Clarkson B. et al.Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis [published correction appears in JAMA Neurol. 2020;77(11):1453].JAMA Neurol. 2020; 77: 1420-1429Crossref PubMed Scopus (40) Google Scholar
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