{"id":9559,"date":"2025-01-06T11:21:29","date_gmt":"2025-01-06T11:21:29","guid":{"rendered":"https:\/\/www.kauveryhospital.com\/ima-journal\/?p=9559"},"modified":"2025-04-09T10:03:40","modified_gmt":"2025-04-09T10:03:40","slug":"paraneoplastic-rhombencephalitis-a-rare-neurological-manifestation-of-lung-adenocarcinoma","status":"publish","type":"post","link":"https:\/\/www.kauveryhospital.com\/ima-journal\/ima-journal-january-2025\/paraneoplastic-rhombencephalitis-a-rare-neurological-manifestation-of-lung-adenocarcinoma\/","title":{"rendered":"Paraneoplastic Rhombencephalitis: A rare Neurological Manifestation of Lung Adenocarcinoma"},"content":{"rendered":"<p class=\"caps\">[vc_row][vc_column][vc_column_text]<\/p>\n<h2><strong>Introduction<\/strong><\/h2>\n<p style=\"text-align: justify;\">Paraneoplastic Rhombencephalitis is a rare and serious neurological condition often associated with underlying malignancy. It is a type of paraneoplastic syndrome, where the immune system generates an autoimmune response against tumor antigens that cross-react with normal neuronal tissues.\u00a0Paraneoplastic Rhombencephalitis affects the brainstem and cerebellum, regions involved in motor coordination and autonomic functions.<\/p>\n<h2><strong>Case:<\/strong><\/h2>\n<p style=\"text-align: justify;\">A 66 years old male presented with progressive imbalance while walking, letharginess hiccups and constipation.\u00a0CSF analysis was normal.<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-9560\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-1.jpg\" alt=\"\" width=\"800\" height=\"440\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-1.jpg 890w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-1-300x165.jpg 300w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-1-768x422.jpg 768w\" sizes=\"auto, (max-width: 800px) 100vw, 800px\" \/><\/p>\n<div class=\"row\">\n<div class=\"col-lg-6\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-9561\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-2.jpg\" alt=\"\" width=\"350\" height=\"418\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-2.jpg 446w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-2-251x300.jpg 251w\" sizes=\"auto, (max-width: 350px) 100vw, 350px\" \/><\/div>\n<div class=\"col-lg-6\">\n<p class=\"highlight\"><strong>Bilateral symmetric FLAIR hyperintensities in mid brain, pons, medulla and cerebellar peduncles<\/strong><\/p>\n<\/div>\n<\/div>\n<div class=\"row\">\n<div class=\"col-lg-6\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-9572\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-3.jpg\" alt=\"\" width=\"350\" height=\"392\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-3.jpg 446w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-25-3-268x300.jpg 268w\" sizes=\"auto, (max-width: 350px) 100vw, 350px\" \/><\/div>\n<div class=\"col-lg-6\">\n<p class=\"highlight\"><strong>Homogeneous enhancement along the ventral aspect of the midbrain and pons.<\/strong><\/p>\n<\/div>\n<\/div>\n<h2><strong><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-9579\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-24-4-1024x497.jpg\" alt=\"\" width=\"800\" height=\"388\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-24-4-1024x497.jpg 1024w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-24-4-300x146.jpg 300w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-24-4-768x373.jpg 768w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/img-24-4.jpg 1071w\" sizes=\"auto, (max-width: 800px) 100vw, 800px\" \/><\/strong><\/h2>\n<p class=\"highlight\"><strong>FDG avid lesion in superior segment of lower lobe of right lung, Biopsy showed adenocarcinoma<\/strong><\/p>\n<h2><strong>Conclusion:<\/strong><\/h2>\n<p style=\"text-align: justify;\">Paraneoplastic rhombencephalitis (PRE) is a rare but serious neurological manifestation associated with certain malignancies. It results from an immune-mediated response that targets the brainstem and cerebellum and occasionally the spinal cord, leading to a range of neurological deficits such as ataxia, dysarthria, and cranial nerve abnormalities. Advanced imaging, especially MRI and PET scans, is essential for diagnosing paraneoplastic Rhombencephalitis and distinguishing it from other types. It typically has slow progression of symptoms and show predominant involvement of the brainstem (especially the pons and medulla) and cerebellum, with hyperintensities on T2-weighted and Fluid-Attenuated Inversion Recovery (FLAIR) sequences, reflecting inflammatory changes or edema, and while contrast enhancement may be observed in some cases, it is not universally present; in more severe or chronic cases, cerebellar atrophy may develop, contributing to the progression of neurological symptoms, and these radiological findings, when correlated with the clinical presentation of the patient. Particularly a history of malignancy suggestive of a paraneoplastic syndrome, strongly support the diagnosis of paraneoplastic rhombencephalitis, with positive autoantibodies, such as anti-Yo, anti-Hu, or anti-Ri, further aiding the confirmation. This case highlights not only the importance of MRI in picking up brain stem abnormalities not identified in PET scan but also the awareness of primary tumor presenting initially as Rhombencephalitis.<\/p>\n<h2><strong>Differentials:<\/strong><\/h2>\n<ul>\n<li>Infectious Rhombencephalitis: May show focal abscess or nonspecific hyperintensities.<\/li>\n<li>Autoimmune Brainstem Encephalitis: Have different pattern of involvement.<\/li>\n<li>CLIPPERS: Shows characteristic punctate and curvilinear enhancement peppering the pons and adjacent structures.<\/li>\n<li>Neuromyelitis spectrum disorders (NMOSD)<\/li>\n<li>Myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD).<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<p><strong><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-8409 size-thumbnail\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish-150x150.jpg 150w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish-300x300.jpg 300w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish-1024x1024.jpg 1024w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish-768x769.jpg 768w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/05\/dr.manish.jpg 1500w\" sizes=\"auto, (max-width: 150px) 100vw, 150px\" \/>Dr. Manish Yadav<\/strong><br \/>\n<em>DnB Radiology Resident<\/em><br \/>\nKauvery Hospital, Chennai<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<p><strong><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-9577 size-thumbnail\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2025\/01\/dr.babu-peter-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" \/>Dr. Babu Peter S<\/strong><br \/>\n<em>Consultant Radiologist<\/em><br \/>\nKauvery Hospital, Chennai<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Mentor:<\/strong><\/p>\n<p><strong><img loading=\"lazy\" decoding=\"async\" class=\"wp-image-8219 size-thumbnail alignleft\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/04\/kanagasabai-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" \/>Dr. Kanagasabai Kamalasekar<\/strong><br \/>\n<em>Consultant Radiology<\/em><br \/>\nKauvery Hospital, Chennai[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column][vc_column_text]<\/p>\n<p>[\/vc_column_text][\/vc_column][\/vc_row]<\/p>\n","protected":false},"excerpt":{"rendered":"<p>[vc_row][vc_column][vc_column_text] Introduction Paraneoplastic Rhombencephalitis is a rare and serious neurological condition often associated with underlying malignancy. It is a type of paraneoplastic syndrome, where the immune system generates an autoimmune<\/p>\n","protected":false},"author":2,"featured_media":9570,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[83],"tags":[],"class_list":["post-9559","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-ima-journal-january-2025"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v24.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Paraneoplastic Rhombencephalitis: A rare Neurological Manifestation of Lung Adenocarcinoma<\/title>\n<meta name=\"description\" content=\"Paraneoplastic Rhombencephalitis is a rare and serious neurological condition often associated with underlying malignancy.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" 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