Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman
Background: Paraneoplastic stiff-limb syndrome (SLS) is a rare manifestation of underlying malignancy and could have distinctive features different from the classic stiff-person syndrome (SPS).Case Description: We present a case of anti-amphiphysin antibody (Ab)-associated paraneoplastic SLS, in an...
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Frontiers Media S.A.
2021-10-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2021.735895/full |
| _version_ | 1818719253525692416 |
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| author | Bhanu Gogia Elena Shanina Xiang Fang Jing He Xiangping Li |
| author_facet | Bhanu Gogia Elena Shanina Xiang Fang Jing He Xiangping Li |
| author_sort | Bhanu Gogia |
| collection | DOAJ |
| description | Background: Paraneoplastic stiff-limb syndrome (SLS) is a rare manifestation of underlying malignancy and could have distinctive features different from the classic stiff-person syndrome (SPS).Case Description: We present a case of anti-amphiphysin antibody (Ab)-associated paraneoplastic SLS, in an 83-year-old woman with invasive ductal carcinoma of the breast. She presented with stiffness, painful spasms of the distal legs, and asymmetrical fixed posturing of the foot. There are coexisting long-tract disturbance and lower-extremity weakness. Treatment with diazepam provided symptomatic relief while plasma exchange (PLEX) did not lead to significant clinical improvement. The patient was bedridden within 3 months and passed away within 6 months from symptom onset.Conclusion: This case highlights the importance of recognition of uncommon presentation of SPS and its oncological significance. This entity requires a high degree of suspicion for initiation of the proper workup. The rapid identification and treatment of the underlying tumor might offer the best chance for recovery. |
| first_indexed | 2024-12-17T20:04:00Z |
| format | Article |
| id | doaj.art-393e91ada6e7414eadbfac87be1b7e74 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-17T20:04:00Z |
| publishDate | 2021-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-393e91ada6e7414eadbfac87be1b7e742022-12-21T21:34:22ZengFrontiers Media S.A.Frontiers in Neurology1664-22952021-10-011210.3389/fneur.2021.735895735895Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly WomanBhanu Gogia0Elena Shanina1Xiang Fang2Jing He3Xiangping Li4Department of Neurology, Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, MA, United StatesDepartment of Neurology, University of Texas Medical Branch, Galveston, TX, United StatesDepartment of Neurology, University of Texas Medical Branch, Galveston, TX, United StatesDepartment of Pathology, University of Texas Medical Branch, Galveston, TX, United StatesDepartment of Neurology, University of Texas Medical Branch, Galveston, TX, United StatesBackground: Paraneoplastic stiff-limb syndrome (SLS) is a rare manifestation of underlying malignancy and could have distinctive features different from the classic stiff-person syndrome (SPS).Case Description: We present a case of anti-amphiphysin antibody (Ab)-associated paraneoplastic SLS, in an 83-year-old woman with invasive ductal carcinoma of the breast. She presented with stiffness, painful spasms of the distal legs, and asymmetrical fixed posturing of the foot. There are coexisting long-tract disturbance and lower-extremity weakness. Treatment with diazepam provided symptomatic relief while plasma exchange (PLEX) did not lead to significant clinical improvement. The patient was bedridden within 3 months and passed away within 6 months from symptom onset.Conclusion: This case highlights the importance of recognition of uncommon presentation of SPS and its oncological significance. This entity requires a high degree of suspicion for initiation of the proper workup. The rapid identification and treatment of the underlying tumor might offer the best chance for recovery.https://www.frontiersin.org/articles/10.3389/fneur.2021.735895/fullstiff person syndromestiff limb syndromeamphiphysin antibodyneurologic paraneoplastic syndromesparaneoplastic myelopathy |
| spellingShingle | Bhanu Gogia Elena Shanina Xiang Fang Jing He Xiangping Li Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman Frontiers in Neurology stiff person syndrome stiff limb syndrome amphiphysin antibody neurologic paraneoplastic syndromes paraneoplastic myelopathy |
| title | Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman |
| title_full | Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman |
| title_fullStr | Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman |
| title_full_unstemmed | Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman |
| title_short | Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman |
| title_sort | case report amphiphysin antibody associated stiff limb syndrome and myelopathy an unusual presentation of breast cancer in an elderly woman |
| topic | stiff person syndrome stiff limb syndrome amphiphysin antibody neurologic paraneoplastic syndromes paraneoplastic myelopathy |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2021.735895/full |
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