A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report
Abstract Background Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. Case presentatio...
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| Format: | Article |
| Language: | English |
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BMC
2019-11-01
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| Series: | BMC Neurology |
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| Online Access: | https://doi.org/10.1186/s12883-019-1542-9 |
| _version_ | 1818460695722721280 |
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| author | Raffaele Longo Marc Wagner Benjamin Savenkoff Mathilde Chastenet de Castaing Guillaume Desiro Zead Tubail Laurent Hennequin Sinan Ben Mahmoud Nathalie Marcon Philippe Quetin Marco Campitiello Francesca Plastino |
| author_facet | Raffaele Longo Marc Wagner Benjamin Savenkoff Mathilde Chastenet de Castaing Guillaume Desiro Zead Tubail Laurent Hennequin Sinan Ben Mahmoud Nathalie Marcon Philippe Quetin Marco Campitiello Francesca Plastino |
| author_sort | Raffaele Longo |
| collection | DOAJ |
| description | Abstract Background Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. Case presentation A 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death. Conclusions This case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy. |
| first_indexed | 2024-12-14T23:34:20Z |
| format | Article |
| id | doaj.art-f231ad86522e43caa768700134fbb4e6 |
| institution | Directory Open Access Journal |
| issn | 1471-2377 |
| language | English |
| last_indexed | 2024-12-14T23:34:20Z |
| publishDate | 2019-11-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj.art-f231ad86522e43caa768700134fbb4e62022-12-21T22:43:39ZengBMCBMC Neurology1471-23772019-11-011911610.1186/s12883-019-1542-9A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case reportRaffaele Longo0Marc Wagner1Benjamin Savenkoff2Mathilde Chastenet de Castaing3Guillaume Desiro4Zead Tubail5Laurent Hennequin6Sinan Ben Mahmoud7Nathalie Marcon8Philippe Quetin9Marco Campitiello10Francesca Plastino11Division of Medical Oncology, “CHR Metz-Thionville”Division of Neurology, “CHR Metz-Thionville”Division of Nephrology, “CHR Metz-Thionville”Division of Medical Oncology, “CHR Metz-Thionville”Division of Medical Oncology, “CHR Metz-Thionville”Division of Nephrology, “CHR Metz-Thionville”Division of Radiology, “CHR Metz-Thionville”Division of Nuclear Medecine, “CHR Metz-Thionville”Division of Pathology, “CHR Metz-Thionville”Division of Radiotherapy, “CHR Metz-Thionville”Division of Medical Oncology, “CHR Metz-Thionville”Division of Medical Oncology, “CHR Metz-Thionville”Abstract Background Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. Case presentation A 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death. Conclusions This case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy.https://doi.org/10.1186/s12883-019-1542-9Limbic encephalitisParaneoplasticSmall cell carcinoma |
| spellingShingle | Raffaele Longo Marc Wagner Benjamin Savenkoff Mathilde Chastenet de Castaing Guillaume Desiro Zead Tubail Laurent Hennequin Sinan Ben Mahmoud Nathalie Marcon Philippe Quetin Marco Campitiello Francesca Plastino A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report BMC Neurology Limbic encephalitis Paraneoplastic Small cell carcinoma |
| title | A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report |
| title_full | A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report |
| title_fullStr | A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report |
| title_full_unstemmed | A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report |
| title_short | A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report |
| title_sort | paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma a case report |
| topic | Limbic encephalitis Paraneoplastic Small cell carcinoma |
| url | https://doi.org/10.1186/s12883-019-1542-9 |
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