An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia
Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizur...
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| Format: | Article |
| Language: | English |
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Korean Encephalitis and Neuroinflammation Society
2025-04-01
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| Series: | Encephalitis |
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| Online Access: | http://encephalitisjournal.org/upload/pdf/encephalitis-2024-00115.pdf |
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| author | Swati Parida Nikhil Pandey Anand Kumar Varun Kumar Singh Neetu Rani Dhiman Niraj Kumar Srivastava Deepika Joshi |
| author_facet | Swati Parida Nikhil Pandey Anand Kumar Varun Kumar Singh Neetu Rani Dhiman Niraj Kumar Srivastava Deepika Joshi |
| author_sort | Swati Parida |
| collection | DOAJ |
| description | Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizures ,myoclonus, spasticity, mutism and akinesia ultimately leading to a vegetative state. Balint’s syndrome, characterized by the triad of simultagnosia, optic ataxia, and oculomotor apraxia, as an initial presenting feature of SSPE is rare. Autonomic dysfunction in SSPE is attributed to central autonomic involvement, with decreased heart rate variability a predictor for arrhythmia and sudden cardiac death. We report an unusual case of a 22-year-old male presenting with features suggestive of Balint’s syndrome. Myoclonus and cognitive decline appeared 6 months after the first onset of symptoms, along with autonomic dysfunction. Thereafter, rapid progression of symptoms was noted. Cerebrospinal fluid and electroencephalography after the first symptom onset were consistent with a diagnosis of SSPE. The patient ultimately succumbed to his illness. Thus we highlight atypical presentation of SSPE with autonomic dysfunction. A high index of suspicion is needed for prompt and timely intervention. |
| format | Article |
| id | doaj-art-a3af4a96fdc745e9afbf60a4df06c25f |
| institution | OA Journals |
| issn | 2765-4559 2734-1461 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Korean Encephalitis and Neuroinflammation Society |
| record_format | Article |
| series | Encephalitis |
| spelling | doaj-art-a3af4a96fdc745e9afbf60a4df06c25f2025-08-20T02:11:26ZengKorean Encephalitis and Neuroinflammation SocietyEncephalitis2765-45592734-14612025-04-0152535610.47936/encephalitis.2024.0011582An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomiaSwati Parida0Nikhil Pandey1Anand Kumar2Varun Kumar Singh3Neetu Rani Dhiman4Niraj Kumar Srivastava5Deepika Joshi6 Department of Neurology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Department of Neurology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, IndiaSubacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizures ,myoclonus, spasticity, mutism and akinesia ultimately leading to a vegetative state. Balint’s syndrome, characterized by the triad of simultagnosia, optic ataxia, and oculomotor apraxia, as an initial presenting feature of SSPE is rare. Autonomic dysfunction in SSPE is attributed to central autonomic involvement, with decreased heart rate variability a predictor for arrhythmia and sudden cardiac death. We report an unusual case of a 22-year-old male presenting with features suggestive of Balint’s syndrome. Myoclonus and cognitive decline appeared 6 months after the first onset of symptoms, along with autonomic dysfunction. Thereafter, rapid progression of symptoms was noted. Cerebrospinal fluid and electroencephalography after the first symptom onset were consistent with a diagnosis of SSPE. The patient ultimately succumbed to his illness. Thus we highlight atypical presentation of SSPE with autonomic dysfunction. A high index of suspicion is needed for prompt and timely intervention.http://encephalitisjournal.org/upload/pdf/encephalitis-2024-00115.pdfsubacute sclerosing panencephalitisvisual agnosiadysautonomiasimultagnosia |
| spellingShingle | Swati Parida Nikhil Pandey Anand Kumar Varun Kumar Singh Neetu Rani Dhiman Niraj Kumar Srivastava Deepika Joshi An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia Encephalitis subacute sclerosing panencephalitis visual agnosia dysautonomia simultagnosia |
| title | An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia |
| title_full | An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia |
| title_fullStr | An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia |
| title_full_unstemmed | An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia |
| title_short | An interesting case of subacute sclerosing panencephalitis presenting with Balint’s syndrome and dysautonomia |
| title_sort | interesting case of subacute sclerosing panencephalitis presenting with balint s syndrome and dysautonomia |
| topic | subacute sclerosing panencephalitis visual agnosia dysautonomia simultagnosia |
| url | http://encephalitisjournal.org/upload/pdf/encephalitis-2024-00115.pdf |
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