Diagnosis problems of paraneoplastic cerebellar degeneration
Paraneoplastic cerebellar degeneration (PCD) is a rare form of the paraneoplastic neurological syndrome (PNS). PCD is an autoimmune disease of the central nervous system (CNS) affecting the Purkinje cells and possibly other cells of the cerebellum. PCD is characterized by a rapid onset resulting in...
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| Format: | Article |
| Language: | Russian |
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IMA-PRESS LLC
2014-05-01
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| Series: | Неврология, нейропсихиатрия, психосоматика |
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| Online Access: | https://nnp.ima-press.net/nnp/article/view/380 |
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| author | N.A. Shnaider V.V. Ezhikova Yu.A. Dykhno D.V. Dmitrenko Yu.S. Panina |
| author_facet | N.A. Shnaider V.V. Ezhikova Yu.A. Dykhno D.V. Dmitrenko Yu.S. Panina |
| author_sort | N.A. Shnaider |
| collection | DOAJ |
| description | Paraneoplastic cerebellar degeneration (PCD) is a rare form of the paraneoplastic neurological syndrome (PNS). PCD is an autoimmune disease of the central nervous system (CNS) affecting the Purkinje cells and possibly other cells of the cerebellum. PCD is characterized by a rapid onset resulting in disability for a few days or weeks; a slow progredient increase in cerebellar symptomatology is observed less often. PCD develops in 1–3% of cancer patients; its fraction accounts for 25% of all forms of PNS. The mean incidence rate of PCD is about 2 cases per 1,000 cancer patients. PCD develops in patients with cancer of the ovary, uterus and fallopian tubes, with small cell lung cancer and Hodgkin's lymphoma. The incidence rate among females is 3 times higher than that among males. Females aged 50–65 years are most likely to suffer from PCD. PCDs are divided into four main subgroups that differ in terms of prognosis and range of associated antineuronal antibodies. In the last decade, different classes of anti-onconeuronal antibodies associated with PCD have been described; 9 of them have been best studied. Anti-Yo and anti-Hu antibodies are most frequently detected upon PCD. PCD can be also diagnosed without anti-onconeuronal antibodies associated with it or their titer in the blood can be low. Differential diagnosis of PCD is complex and is conducted for a wide range of CNS diseases. No single approach to treating PCD currently exists. Surgical removal of the tumor, the source of production of anti-onconeuronal antigens, followed by radiotherapy and/or chemotherapy, does not solve the problem completely, but may reduce severity of the clinical manifestations of PCD or stabilize the pathological process. This explains the need for rapid and profound search for a tumor in patients suspected with PCD. The authors described a clinical case of an acute debut of PCD in 47-year-old female, 6 months after which, the patient was diagnosed with breast cancer. The problems of PCD diagnosis by neurologists are discussed. The importance of the interdisciplinary approach to diagnosis and follow-up monitoring of patients with this nosology is noted. |
| first_indexed | 2024-04-10T02:03:59Z |
| format | Article |
| id | doaj.art-52500b6bbc8b4dbaaec5d3de6ae16e94 |
| institution | Directory Open Access Journal |
| issn | 2074-2711 2310-1342 |
| language | Russian |
| last_indexed | 2024-04-10T02:03:59Z |
| publishDate | 2014-05-01 |
| publisher | IMA-PRESS LLC |
| record_format | Article |
| series | Неврология, нейропсихиатрия, психосоматика |
| spelling | doaj.art-52500b6bbc8b4dbaaec5d3de6ae16e942023-03-13T08:42:13ZrusIMA-PRESS LLCНеврология, нейропсихиатрия, психосоматика2074-27112310-13422014-05-0161354310.14412/2074-2711-2014-1-35-43371Diagnosis problems of paraneoplastic cerebellar degenerationN.A. Shnaider0V.V. Ezhikova1Yu.A. Dykhno2D.V. Dmitrenko3Yu.S. Panina4Voyno-Yasenetsky Krasnoyarsk State Medical University, the Ministry of Health of the Russian Federation, Neurological Center of Epileptology, Neurogenetics, and Brain Research of the University Hospital, Krasnoyarsk, RussiKryzhanovsky Krasnoyarsk Regional Cancer Center, Krasnoyarsk, Russiaoyno-Yasenetsky Krasnoyarsk State Medical University, the Ministry of Health of the Russian Federation, Neurological Center of Epileptology, Neurogenetics, and Brain Research of the University Hospital, Krasnoyarsk, RussiaVoyno-Yasenetsky Krasnoyarsk State Medical University, the Ministry of Health of the Russian Federation, Neurological Center of Epileptology, Neurogenetics, and Brain Research of the University Hospital, Krasnoyarsk, RussiaVoyno-Yasenetsky Krasnoyarsk State Medical University, the Ministry of Health of the Russian Federation, Neurological Center of Epileptology, Neurogenetics, and Brain Research of the University Hospital, Krasnoyarsk, RussiaParaneoplastic cerebellar degeneration (PCD) is a rare form of the paraneoplastic neurological syndrome (PNS). PCD is an autoimmune disease of the central nervous system (CNS) affecting the Purkinje cells and possibly other cells of the cerebellum. PCD is characterized by a rapid onset resulting in disability for a few days or weeks; a slow progredient increase in cerebellar symptomatology is observed less often. PCD develops in 1–3% of cancer patients; its fraction accounts for 25% of all forms of PNS. The mean incidence rate of PCD is about 2 cases per 1,000 cancer patients. PCD develops in patients with cancer of the ovary, uterus and fallopian tubes, with small cell lung cancer and Hodgkin's lymphoma. The incidence rate among females is 3 times higher than that among males. Females aged 50–65 years are most likely to suffer from PCD. PCDs are divided into four main subgroups that differ in terms of prognosis and range of associated antineuronal antibodies. In the last decade, different classes of anti-onconeuronal antibodies associated with PCD have been described; 9 of them have been best studied. Anti-Yo and anti-Hu antibodies are most frequently detected upon PCD. PCD can be also diagnosed without anti-onconeuronal antibodies associated with it or their titer in the blood can be low. Differential diagnosis of PCD is complex and is conducted for a wide range of CNS diseases. No single approach to treating PCD currently exists. Surgical removal of the tumor, the source of production of anti-onconeuronal antigens, followed by radiotherapy and/or chemotherapy, does not solve the problem completely, but may reduce severity of the clinical manifestations of PCD or stabilize the pathological process. This explains the need for rapid and profound search for a tumor in patients suspected with PCD. The authors described a clinical case of an acute debut of PCD in 47-year-old female, 6 months after which, the patient was diagnosed with breast cancer. The problems of PCD diagnosis by neurologists are discussed. The importance of the interdisciplinary approach to diagnosis and follow-up monitoring of patients with this nosology is noted.https://nnp.ima-press.net/nnp/article/view/380paraneoplastic cerebellar degenerationparaneoplastic neurological syndromediagnosiscanceranti-onconeuronal antibodies. |
| spellingShingle | N.A. Shnaider V.V. Ezhikova Yu.A. Dykhno D.V. Dmitrenko Yu.S. Panina Diagnosis problems of paraneoplastic cerebellar degeneration Неврология, нейропсихиатрия, психосоматика paraneoplastic cerebellar degeneration paraneoplastic neurological syndrome diagnosis cancer anti-onconeuronal antibodies. |
| title | Diagnosis problems of paraneoplastic cerebellar degeneration |
| title_full | Diagnosis problems of paraneoplastic cerebellar degeneration |
| title_fullStr | Diagnosis problems of paraneoplastic cerebellar degeneration |
| title_full_unstemmed | Diagnosis problems of paraneoplastic cerebellar degeneration |
| title_short | Diagnosis problems of paraneoplastic cerebellar degeneration |
| title_sort | diagnosis problems of paraneoplastic cerebellar degeneration |
| topic | paraneoplastic cerebellar degeneration paraneoplastic neurological syndrome diagnosis cancer anti-onconeuronal antibodies. |
| url | https://nnp.ima-press.net/nnp/article/view/380 |
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