West Syndrome in an Infant with Complete Corpus Callosal Agenesis
West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2024-01-01
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| Series: | Archives of Medicine and Health Sciences |
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| Online Access: | https://journals.lww.com/10.4103/amhs.amhs_105_23 |
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| author | S. Sheetal Nikhil Gladson Rakesh John Yamuna P. Kurup |
| author_facet | S. Sheetal Nikhil Gladson Rakesh John Yamuna P. Kurup |
| author_sort | S. Sheetal |
| collection | DOAJ |
| description | West syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pattern of modified hypsarrhythmia, which denotes severe disease. Hereby, we describe the case of a female infant who presented with IS; EEG showed a modified hypsarrhythmia pattern in the form of burst suppression, and magnetic resonance imaging brain showed complete corpus callosal agenesis. She was initiated on injection of adrenocorticotropic hormone and showed marked improvement in epileptic spasms. |
| format | Article |
| id | doaj-art-4722dddcacf841d1966e39f5d49dfed7 |
| institution | Kabale University |
| issn | 2321-4848 2321-6085 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Archives of Medicine and Health Sciences |
| spelling | doaj-art-4722dddcacf841d1966e39f5d49dfed72025-01-23T08:51:59ZengWolters Kluwer Medknow PublicationsArchives of Medicine and Health Sciences2321-48482321-60852024-01-0112112012210.4103/amhs.amhs_105_23West Syndrome in an Infant with Complete Corpus Callosal AgenesisS. SheetalNikhil GladsonRakesh JohnYamuna P. KurupWest syndrome is a severe form of epilepsy with onset in infancy and early childhood. The characteristic triad described includes infantile spasms (IS), an abnormal electroencephalogram (EEG) pattern referred to as hypsarrhythmia, and developmental regression. It comprises 2% of childhood epilepsies. Etiologically, West syndrome can be classified into symptomatic with known etiology and cryptogenic with unknown etiology. Hypsarrhythmia is defined as chaotic, asynchronous, disorganized slow background activity with high-voltage spike activity. Burst-suppression pattern is a rarely described pattern of modified hypsarrhythmia, which denotes severe disease. Hereby, we describe the case of a female infant who presented with IS; EEG showed a modified hypsarrhythmia pattern in the form of burst suppression, and magnetic resonance imaging brain showed complete corpus callosal agenesis. She was initiated on injection of adrenocorticotropic hormone and showed marked improvement in epileptic spasms.https://journals.lww.com/10.4103/amhs.amhs_105_23adrenocorticotropic hormoneburst suppression patterncorpus callosal agenesismodified hypsarrhythmiawest syndrome |
| spellingShingle | S. Sheetal Nikhil Gladson Rakesh John Yamuna P. Kurup West Syndrome in an Infant with Complete Corpus Callosal Agenesis Archives of Medicine and Health Sciences adrenocorticotropic hormone burst suppression pattern corpus callosal agenesis modified hypsarrhythmia west syndrome |
| title | West Syndrome in an Infant with Complete Corpus Callosal Agenesis |
| title_full | West Syndrome in an Infant with Complete Corpus Callosal Agenesis |
| title_fullStr | West Syndrome in an Infant with Complete Corpus Callosal Agenesis |
| title_full_unstemmed | West Syndrome in an Infant with Complete Corpus Callosal Agenesis |
| title_short | West Syndrome in an Infant with Complete Corpus Callosal Agenesis |
| title_sort | west syndrome in an infant with complete corpus callosal agenesis |
| topic | adrenocorticotropic hormone burst suppression pattern corpus callosal agenesis modified hypsarrhythmia west syndrome |
| url | https://journals.lww.com/10.4103/amhs.amhs_105_23 |
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