Neuropathological Changes in Nakalanga Syndrome—A Case Report
Nakalanga syndrome is a clinical manifestation of onchocerciasis-associated epilepsy characterized by stunting, delayed or absent secondary sexual development and skeletal deformities, and is often accompanied by epileptic seizures. The pathophysiology of Nakalanga syndrome is unknown. Here, we desc...
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MDPI AG
2021-01-01
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| Online Access: | https://www.mdpi.com/2076-0817/10/2/116 |
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| author | An Hotterbeekx Martin Lammens Sylvester Onzivua Robert Lukande Francis Olwa Samir Kumar-Singh Stijn Van Hees Richard Idro Robert Colebunders |
| author_facet | An Hotterbeekx Martin Lammens Sylvester Onzivua Robert Lukande Francis Olwa Samir Kumar-Singh Stijn Van Hees Richard Idro Robert Colebunders |
| author_sort | An Hotterbeekx |
| collection | DOAJ |
| description | Nakalanga syndrome is a clinical manifestation of onchocerciasis-associated epilepsy characterized by stunting, delayed or absent secondary sexual development and skeletal deformities, and is often accompanied by epileptic seizures. The pathophysiology of Nakalanga syndrome is unknown. Here, we describe the post-mortem findings of a 17-year-old female who died with Nakalanga syndrome in northern Uganda. Macroscopic and histopathological examination of all major organs (liver, lungs, kidney and heart), including the brain and the pituitary gland, was performed. The suspected cause of death was malaria, and all major organs and pituitary gland appeared normal, except the lungs, which were edematous consistent with the malaria. Neuropathological changes include signs of neuro-inflammation (gliosis and activated microglia), which co-localized with tau-reactive neurofibrillary tangles and threads. The pathology was most abundant in the frontal cortex, thalamic and hypothalamic regions, and mesencephalon. The choroid plexus showed psammoma bodies. These findings indicate accelerated aging, probably due to repeated seizures. The neuropathological findings were similar to other persons who died with onchocerciasis-associated epilepsy. Examination of the pituitary gland did not reveal new information concerning the underlying pathophysiological mechanism of Nakalanga syndrome. Therefore, more post-mortem studies should be performed. |
| first_indexed | 2024-03-09T03:51:39Z |
| format | Article |
| id | doaj.art-96770c2fa37e4aed98903f3fd62a134a |
| institution | Directory Open Access Journal |
| issn | 2076-0817 |
| language | English |
| last_indexed | 2024-03-09T03:51:39Z |
| publishDate | 2021-01-01 |
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| spelling | doaj.art-96770c2fa37e4aed98903f3fd62a134a2023-12-03T14:27:07ZengMDPI AGPathogens2076-08172021-01-0110211610.3390/pathogens10020116Neuropathological Changes in Nakalanga Syndrome—A Case ReportAn Hotterbeekx0Martin Lammens1Sylvester Onzivua2Robert Lukande3Francis Olwa4Samir Kumar-Singh5Stijn Van Hees6Richard Idro7Robert Colebunders8Global Health Institute, University of Antwerp, 2100 Antwerp, BelgiumDepartment of Pathology, Antwerp University Hospital, 2100 Antwerp, BelgiumDepartment of Pathology, Makerere University Medical School, Kampala P.O. Box 7072, UgandaDepartment of Pathology, Makerere University Medical School, Kampala P.O. Box 7072, UgandaDepartment of Diagnostics, Faculty of Health Sciences, Lira University, Lira P. O. Box 1035, UgandaMolecular Pathology Group, Laboratory of Cell Biology & Histology, Faculty of Medicine and Health Sciences, University of Antwerp, 2100 Antwerp, BelgiumGlobal Health Institute, University of Antwerp, 2100 Antwerp, BelgiumDepartment of Pediatrics, Makerere University Medical School, Kampala P.O. Box 7072, UgandaGlobal Health Institute, University of Antwerp, 2100 Antwerp, BelgiumNakalanga syndrome is a clinical manifestation of onchocerciasis-associated epilepsy characterized by stunting, delayed or absent secondary sexual development and skeletal deformities, and is often accompanied by epileptic seizures. The pathophysiology of Nakalanga syndrome is unknown. Here, we describe the post-mortem findings of a 17-year-old female who died with Nakalanga syndrome in northern Uganda. Macroscopic and histopathological examination of all major organs (liver, lungs, kidney and heart), including the brain and the pituitary gland, was performed. The suspected cause of death was malaria, and all major organs and pituitary gland appeared normal, except the lungs, which were edematous consistent with the malaria. Neuropathological changes include signs of neuro-inflammation (gliosis and activated microglia), which co-localized with tau-reactive neurofibrillary tangles and threads. The pathology was most abundant in the frontal cortex, thalamic and hypothalamic regions, and mesencephalon. The choroid plexus showed psammoma bodies. These findings indicate accelerated aging, probably due to repeated seizures. The neuropathological findings were similar to other persons who died with onchocerciasis-associated epilepsy. Examination of the pituitary gland did not reveal new information concerning the underlying pathophysiological mechanism of Nakalanga syndrome. Therefore, more post-mortem studies should be performed.https://www.mdpi.com/2076-0817/10/2/116Nakalanga syndromenodding syndromeepilepsypost-mortempituitary glandUganda |
| spellingShingle | An Hotterbeekx Martin Lammens Sylvester Onzivua Robert Lukande Francis Olwa Samir Kumar-Singh Stijn Van Hees Richard Idro Robert Colebunders Neuropathological Changes in Nakalanga Syndrome—A Case Report Pathogens Nakalanga syndrome nodding syndrome epilepsy post-mortem pituitary gland Uganda |
| title | Neuropathological Changes in Nakalanga Syndrome—A Case Report |
| title_full | Neuropathological Changes in Nakalanga Syndrome—A Case Report |
| title_fullStr | Neuropathological Changes in Nakalanga Syndrome—A Case Report |
| title_full_unstemmed | Neuropathological Changes in Nakalanga Syndrome—A Case Report |
| title_short | Neuropathological Changes in Nakalanga Syndrome—A Case Report |
| title_sort | neuropathological changes in nakalanga syndrome a case report |
| topic | Nakalanga syndrome nodding syndrome epilepsy post-mortem pituitary gland Uganda |
| url | https://www.mdpi.com/2076-0817/10/2/116 |
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