paraneoplastic cerebellar degeneration

傍腫瘍性小脳変性症、腫瘍随伴性小脳変性症

WordNet

1. the process of declining from a higher to a lower level of effective power or vitality or essential quality (同)devolution
2. passing from a more complex to a simpler biological form (同)retrogression
3. relating to or associated with the cerebellum; "cerebellar artery"

PrepTutorEJDIC

1. 退歩,退化,墜落

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/03/31 10:24:00」(JST)

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Paraneoplastic cerebellar degeneration
Classification and external resources
ICD-10	G13.0
ICD-9	334.9
DiseasesDB	33977
eMedicine	neuro/299
MeSH	D020362

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic syndrome associated with lung, ovarian, breast, Hodgkin’s lymphoma and other cancers. PCD is a rare condition that occurs in less than 1% of cancer patients and usually occurs in middle-aged women.

As is the case with other paraneoplastic syndromes, PCD is believed to be due to an autoimmune reaction targeted against components of the central nervous system (in PCD, this is specifically Purkinje cells in the cerebellum, sometimes accompanied by a proliferation of Berg- mann astrocytes and microglia in the molecular layer of the cerebellum and a loss of granule cells ). It is thought to be triggered when tumor cells (in PCD, most commonly ovarian or breast cancer) express a protein normally expressed in the brain (in PCD, this is the Purkinje neuronal protein termed cdr2). This is believed to trigger an anti-tumor immune response that may be clinically significant, but also an anti-neuronal immune response. PCD patients harbor an anti-neuronal antibody known as anti-Yo (named after the first two letters of the index patient). PCD may be associated with onconeural antibodies directed against other intracellular antigens or against cell surface and with other tumors. When associated with small cell lung cancer, it is antibody termed "anti-Hu" (more commonly associated with paraneoplastic subacute sensory neuropathy and/or limbic encephalitis). The immune cells cross the blood–brain barrier, resulting in an autoimmune attack of Purkinje cells throughout the cerebellar cortex. Radiologic imaging occasionally reveals cerebellar atrophy. Other paraneoplastic antibodies may be associated with PCD symptoms, including anti-Tr and antibodies to glutamate receptor. Occasionally myoclonia and opsoclonus may occur.

Neurological symptoms present insidiously and progress rapidly for about 6 months to a severely disabled state followed by a variable plateau period that can last for months to years. The clinical cerebellar ataxia evident in patients with PCD are caused by Purkinje neuronal loss in the cerebellum. It is manifested by dysarthria, truncal, limb and gait ataxia, vertigo, nausea, vomiting, diplopia and nystagmus. Neurological symptoms precede the diagnosis of the underlying cancer in about 60% of cases. A cure of the cancer underlying PCD usually does not affect neurological symptoms, as cerebellar dysfunction stabilizes in the early stage after symptom onset before the cancer treatment has been initiated. There may be a role for high dose gammaglobulin therapy in the treatment PCD, but due to the rare occurrence of this disease, controlled trials of this therapy may be difficult.

Pathophysiology

The anti-Purkinje cell antibodies originally described in PCD led to the hypothesis that the antibody might be pathogenic, much as earlier studies had demonstrated pathogenicity of anti-acetylcholine receptor antibodies in myasthenia gravis. However, when the antibody was used to clone the cDNA encoding the cdr2 antigen, it was found to be an intracellular protein. This led to the suggestion that there might be a cell-mediated component (T cell) in disease pathogenesis. cdr2 antigen-specific CD8+ T cells were subsequently described in all PCD patients, making them a hallmark of the disease, and likely components in both the anti-tumor immune response and in the neuronal degeneration.

References

External links

• Antibody associated with cerebellar degeneration

UpToDate Contents

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• 1. 腫瘍随伴性小脳変性症 paraneoplastic cerebellar degeneration
• 2. 成人における小脳性運動失調の概要 overview of cerebellar ataxia in adults
• 3. 神経系の腫瘍随伴性症候群の概要 overview of paraneoplastic syndromes of the nervous system
• 4. 腫瘍随伴性および自己免疫性脳炎 paraneoplastic and autoimmune encephalitis
• 5. 末梢神経と筋肉に影響を与える腫瘍随伴性症候群 paraneoplastic syndromes affecting peripheral nerve and muscle

English Journal

• Brain 18F-FDG-PET characteristics in patients with paraneoplastic neurological syndrome and its correlation with clinical and MRI findings.

• Masangkay N1, Basu S, Moghbel M, Kwee T, Alavi A.
• Nuclear medicine communications.Nucl Med Commun.2014 Oct;35(10):1038-46. doi: 10.1097/MNM.0000000000000163.
• AIM: This study aimed to examine the imaging characteristics and clinical and MRI correlates of brain F-fluorodeoxyglucose (F-FDG)-PET imaging in patients with paraneoplatic neurological syndrome.MATERIALS AND METHODS: Data of patients diagnosed with paraneoplastic neurological syndrome were retrosp
• PMID 25023997

• Antibodies to Delta/notch-like epidermal growth factor-related receptor in patients with anti-Tr, paraneoplastic cerebellar degeneration, and Hodgkin lymphoma.

• Greene M1, Lai Y1, Baella N2, Dalmau J3, Lancaster E1.
• JAMA neurology.JAMA Neurol.2014 Aug;71(8):1003-8. doi: 10.1001/jamaneurol.2014.999.
• IMPORTANCE: The anti-Tr immune response is associated with paraneoplastic cerebellar degeneration and Hodgkin lymphoma (HL). One case series has reported that the Delta/notch-like epidermal growth factor-related receptor (DNER) is the actual target for anti-Tr antibodies, but this result has not bee
• PMID 24935874

• Carbonic anhydrase-related protein VIII antibodies and paraneoplastic cerebellar degeneration.

• Höftberger R1, Sabater L, Velasco F, Ciordia R, Dalmau J, Graus F.
• Neuropathology and applied neurobiology.Neuropathol Appl Neurobiol.2014 Aug;40(5):650-3. doi: 10.1111/nan.12118.
• PMID 24446689

Japanese Journal

• A Case of Follicular Lymphoma Associated with Paraneoplastic Cerebellar Degeneration

• Shimazu Yayoi,Minakawa Eiko N.,Nishikori Momoko,Ihara Masafumi,Hashi Yuichiro,Matsuyama Hirofumi,Hishizawa Masakatsu,Yoshida Sonoyo,Kitano Toshiyuki,Kondo Tadakazu,Ishikawa Takayuki,Takahashi Ryosuke,Takaori-Kondo Akifumi
• Internal Medicine 51(11), 1387-1392, 2012
• … Paraneoplastic neurological disorders (PND) are neurological effects of malignancy that are recognized as immune-mediated disorders caused by aberrant expression of a tumor antigen that is normally expressed in the nervous system. … We report a case of cerebellar ataxia which turned out to be paraneoplastic cerebellar degeneration, a subtype of PND that develops cerebellar symptoms, that was caused by follicular lymphoma. …
• NAID 130002062123

• 乳癌による傍腫瘍性小脳変性症の1例

• 道下 新太郎,玉木 康博,野口 眞三郎
• 日本臨床外科学会雑誌 = The journal of the Japan Surgical Association 72(8), 1933-1936, 2011-08-25
• NAID 10029613812

• 多彩な腫瘍随伴症状を呈した小細胞肺癌の1例

• 若槻 雅敏,松尾 潔,萱谷 紘枝,藤原 慶一,米井 敏郎,佐藤 利雄
• 日本呼吸器学会雑誌 = The journal of the Japanese Respiratory Society 49(7), 517-522, 2011-07-10
• NAID 10029372395

Related Pictures

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★リンクテーブル★

リンク元	「傍腫瘍性小脳変性症」「腫瘍随伴性小脳変性症」
関連記事	「degeneration」「cerebellar」「paraneoplastic」

「傍腫瘍性小脳変性症」

　　[★]

英

paraneoplastic cerebellar degeneration

関

腫瘍随伴性小脳変性症

「腫瘍随伴性小脳変性症」

　　[★]

英

paraneoplastic cerebellar degeneration

関

傍腫瘍性小脳変性症

「degeneration」

　　[★]

• n.

• 変性、変性症、逆行変性、縮退、退化

関

degeneracy、degenerate、degenerative、denaturation、denature、involution、retract、retrograde degeneration、retrograde degradation、unfolding

　 　 　

「cerebellar」

　　[★]

• adj.

• 小脳の、小脳性の

関

cerebelli、cerebellum

　

「paraneoplastic」

　　[★]

• adj.

• 腫瘍随伴の、傍腫瘍性の、傍腫瘍の