Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes
Whipple's disease, caused by the gram-positive actinomycete Tropheryma whipplei, is a rare chronic systemic illness with significant diagnostic and therapeutic challenges, particularly when the CNS is involved. This case report details a 46-year-old man presenting with a constellation of sympto...
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Elsevier
2025-03-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S193004332401313X |
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| author | Moustafa A. Mansour, MD, MSc, MPhil Reem W. Malaeb, BS Islam E. Elnemr, MD Mohamed Abdel-Fattah El-Salamoni, MD Hamdi Nabawi Mostafa, MD Zarina Ahmadi, MD |
| author_facet | Moustafa A. Mansour, MD, MSc, MPhil Reem W. Malaeb, BS Islam E. Elnemr, MD Mohamed Abdel-Fattah El-Salamoni, MD Hamdi Nabawi Mostafa, MD Zarina Ahmadi, MD |
| author_sort | Moustafa A. Mansour, MD, MSc, MPhil |
| collection | DOAJ |
| description | Whipple's disease, caused by the gram-positive actinomycete Tropheryma whipplei, is a rare chronic systemic illness with significant diagnostic and therapeutic challenges, particularly when the CNS is involved. This case report details a 46-year-old man presenting with a constellation of symptoms including fatigue, hypersomnia, weight loss, bifrontal headaches, abdominal pain, treatment-unresponsive diarrhea, and skin hyperpigmentation. Neurological examination revealed oculomasticatory myorhythmia, and imaging studies showed nodular enhancement of the hypothalamus and basal ganglia, along with retroperitoneal lymphadenopathy. Diagnosis was confirmed through duodenal biopsy with PAS staining and PCR analysis. The patient was successfully treated with a combination of intravenous ceftriaxone followed by oral doxycycline and hydroxychloroquine, resulting in significant symptom improvement and full recovery of neurological function. This case underscores the importance of early, aggressive treatment and provides a comprehensive review of the current literature on Whipple's disease with CNS involvement, including a comparison of treatment regimens and outcomes. |
| format | Article |
| id | doaj-art-1d47eeedaf4b4338a490a7bef7e3db74 |
| institution | Kabale University |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-1d47eeedaf4b4338a490a7bef7e3db742025-01-18T05:03:53ZengElsevierRadiology Case Reports1930-04332025-03-0120314251430Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomesMoustafa A. Mansour, MD, MSc, MPhil0Reem W. Malaeb, BS1Islam E. Elnemr, MD2Mohamed Abdel-Fattah El-Salamoni, MD3Hamdi Nabawi Mostafa, MD4Zarina Ahmadi, MD5Department of Neurosurgery, Nasser Institute for Research and Treatment, Cairo, Egypt; Department of Neurology and Neurosurgery, Faculty of Medicine, Al-Azhar University, Cairo, Egypt; Corresponding author.Department of Health Professions, Faculty of Health Sciences, American University of Beirut, Beirut, LebanonDepartment of Neurosurgery, Nasser Institute for Research and Treatment, Cairo, EgyptDepartment of Human Physiology and Basic Medical Sciences, Faculty of Medicine, Al-Azhar University, Cairo, EgyptDepartment of Neurosurgery, Nasser Institute for Research and Treatment, Cairo, Egypt; Department of Neurosurgery, Misr University for Science and Technology, Giza, EgyptDepartment of Infectious Diseases and Tropical Medicine, Faculty of Medicine, Al-Azhar University, Cairo, EgyptWhipple's disease, caused by the gram-positive actinomycete Tropheryma whipplei, is a rare chronic systemic illness with significant diagnostic and therapeutic challenges, particularly when the CNS is involved. This case report details a 46-year-old man presenting with a constellation of symptoms including fatigue, hypersomnia, weight loss, bifrontal headaches, abdominal pain, treatment-unresponsive diarrhea, and skin hyperpigmentation. Neurological examination revealed oculomasticatory myorhythmia, and imaging studies showed nodular enhancement of the hypothalamus and basal ganglia, along with retroperitoneal lymphadenopathy. Diagnosis was confirmed through duodenal biopsy with PAS staining and PCR analysis. The patient was successfully treated with a combination of intravenous ceftriaxone followed by oral doxycycline and hydroxychloroquine, resulting in significant symptom improvement and full recovery of neurological function. This case underscores the importance of early, aggressive treatment and provides a comprehensive review of the current literature on Whipple's disease with CNS involvement, including a comparison of treatment regimens and outcomes.http://www.sciencedirect.com/science/article/pii/S193004332401313XWhipple's diseaseCNS bacterial infectionOculomasticatory myorhythmiaCase report |
| spellingShingle | Moustafa A. Mansour, MD, MSc, MPhil Reem W. Malaeb, BS Islam E. Elnemr, MD Mohamed Abdel-Fattah El-Salamoni, MD Hamdi Nabawi Mostafa, MD Zarina Ahmadi, MD Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes Radiology Case Reports Whipple's disease CNS bacterial infection Oculomasticatory myorhythmia Case report |
| title | Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| title_full | Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| title_fullStr | Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| title_full_unstemmed | Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| title_short | Central nervous system involvement in Whipple's disease: Report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| title_sort | central nervous system involvement in whipple s disease report of a rare pathological entity and comparative review of treatment strategies and outcomes |
| topic | Whipple's disease CNS bacterial infection Oculomasticatory myorhythmia Case report |
| url | http://www.sciencedirect.com/science/article/pii/S193004332401313X |
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