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Many of these conditions are of interest to psychologists as they have neurological implications. |
Many of these conditions are of interest to psychologists as they have neurological implications. |
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⚫ | A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as [[paraneoplastic neurological disorder]]s (PNDs).<ref>{{cite journal |author=Rees JH |title=PARANEOPLASTIC SYNDROMES: WHEN TO SUSPECT, HOW TO CONFIRM, AND HOW TO MANAGE |journal=J. Neurol. Neurosurg. Psychiatr. |volume=75 Suppl 2 |issue= Suppl 2|pages=ii43–50 |year=2004 |pmid=15146039 |doi=10.1136/jnnp.2004.040378 |pmc=1765657}}</ref> These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative,<ref>{{cite journal |author=Darnell RB, Posner JB |title=Paraneoplastic syndromes affecting the nervous system |journal=Semin Oncol |volume=33 |issue=3 |pages=270–98 |year=2006 |pmid= 16769417 |doi=10.1053/j.seminoncol.2006.03.008}}</ref> though others (such as [[Lambert-Eaton myasthenic syndrome|LEMS]]) may improve with treatment of the condition or the tumour. Symptoms of paraneoplastic neurological disorders may include [[ataxia]] (difficulty with walking and balance), [[dizziness]], [[pathologic nystagmus|nystagmus]] (rapid uncontrolled eye movements), difficulty swallowing, loss of [[muscle tone]], loss of fine [[motor coordination]], slurred speech, memory loss, vision problems, sleep disturbances, [[dementia]], [[seizure]]s, sensory loss in the limbs. |
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+ | examples include: |
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+ | * [[Paraneoplastic cerebellar degeneration]] |
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+ | * [[Paraneoplastic limbic encephalitis]] |
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⚫ | # Therapies to reduce or slow neurological degeneration. Rapid diagnosis and treatment are critical for the patient to have the best chance of recovery. Since these disorders are relatively rare, few doctors have seen or treated PNDs. Therefore, it is important that PND patients consult with a specialist with experience in diagnosing and treating paraneoplastic neurological disorders. |
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==Classification== |
==Classification== |
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⚫ | A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as [[paraneoplastic neurological disorder]]s (PNDs).<ref>{{cite journal |author=Rees JH |title=PARANEOPLASTIC SYNDROMES: WHEN TO SUSPECT, HOW TO CONFIRM, AND HOW TO MANAGE |journal=J. Neurol. Neurosurg. Psychiatr. |volume=75 Suppl 2 |issue= Suppl 2|pages=ii43–50 |year=2004 |pmid=15146039 |doi=10.1136/jnnp.2004.040378 |pmc=1765657}}</ref> These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative,<ref>{{cite journal |author=Darnell RB, Posner JB |title=Paraneoplastic syndromes affecting the nervous system |journal=Semin Oncol |volume=33 |issue=3 |pages=270–98 |year=2006 |pmid= 16769417 |doi=10.1053/j.seminoncol.2006.03.008}}</ref> though others (such as [[Lambert-Eaton myasthenic syndrome|LEMS]]) may improve with treatment of the condition or the tumour. Symptoms of paraneoplastic neurological disorders may include [[ataxia]] (difficulty with walking and balance), [[dizziness]], [[pathologic nystagmus|nystagmus]] (rapid uncontrolled eye movements), difficulty swallowing, loss of [[muscle tone]], loss of fine [[motor coordination]], slurred speech, memory loss, vision problems, sleep disturbances, [[dementia]], [[seizure]]s, sensory loss in the limbs. |
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⚫ | # Therapies to reduce or slow neurological degeneration. Rapid diagnosis and treatment are critical for the patient to have the best chance of recovery. Since these disorders are relatively rare, few doctors have seen or treated PNDs. Therefore, it is important that PND patients consult with a specialist with experience in diagnosing and treating paraneoplastic neurological disorders. |
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==References== |
==References== |
Latest revision as of 11:16, 3 July 2013
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Paraneoplastic syndrome | |
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DiseasesDB | 2064 |
eMedicine | med/1747 |
MeSH | D010257 |
A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells.[1] These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor. Paraneoplastic syndromes are typical among middle aged to older patients, and they most commonly present with cancers of the lung, breast, ovaries or lymphatic system (a lymphoma).[2] Sometimes the symptoms of paraneoplastic syndromes show before the diagnosis of a malignancy, which has been hypothesized to relate to the disease pathogenesis. In this paradigm, tumor cells express tissue-restricted antigens (such as neuronal proteins), triggering an anti-tumor immune response which may be partially or, rarely, completely effective[3] in suppressing tumor growth and symptoms.[4][5] Patients then come to clinical attention when this tumor immune response breaks immune tolerance and begins to attack the normal tissue expressing that (e.g. neuronal) protein.
Many of these conditions are of interest to psychologists as they have neurological implications.
Neurological
A particularly devastating form of paraneoplastic syndromes is a group of disorders classified as paraneoplastic neurological disorders (PNDs).[6] These paraneoplastic disorders affect the central or peripheral nervous system; some are degenerative,[7] though others (such as LEMS) may improve with treatment of the condition or the tumour. Symptoms of paraneoplastic neurological disorders may include ataxia (difficulty with walking and balance), dizziness, nystagmus (rapid uncontrolled eye movements), difficulty swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs.
examples include:
The most common cancers associated with paraneoplastic neurological disorders are breast, ovarian and lung cancer, but many other cancers can produce paraneoplastic symptoms as well.
Treatment options include:
- Therapies to eliminate the underlying cancer such as chemotherapy, radiation and surgery, and
- Therapies to reduce or slow neurological degeneration. Rapid diagnosis and treatment are critical for the patient to have the best chance of recovery. Since these disorders are relatively rare, few doctors have seen or treated PNDs. Therefore, it is important that PND patients consult with a specialist with experience in diagnosing and treating paraneoplastic neurological disorders.
Classification
Paraneoplastic syndromes can be divided into four main categories: Endocrine, neurological, mucocutaneous and hematological paraneoplastic syndromes, as well as others that may not fit into any of the above categories:
Syndrome class | Syndrome | Main causal cancers | Causal mechanism |
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Endocrine[8] | |||
Cushing syndrome | Ectopic ACTH and ACTH-like substance | ||
SIADH | antidiuretic hormone[9] | ||
Hypercalcemia |
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PTHrP (Parathyroid hormone-related protein), TGF-α, TNF, IL-1[9] | |
Hypoglycemia | Insulin or insulin-like substance[9] or "big" IGF-II | ||
Carcinoid syndrome | Serotonin, bradykinin[9] | ||
Polycythemia | See hematological paraneoplastic syndromes | ||
Hyperaldosteronism |
|
Aldosterone[10] | |
Neurological[11] | Lambert-Eaton myasthenic syndrome (LEMS) |
|
Immunologic |
Paraneoplastic cerebellar degeneration |
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Encephalomyelitis | inflammation of the brain and spinal cord | ||
Limbic encephalitis |
|
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Brainstem encephalitis | |||
Paraneoplastic Opsoclonus (involving eye movement)-Myoclonus |
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Autoimmune reaction against the RNA-binding protein Nova-1[12] | |
Anti-NMDA receptor encephalitis |
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Autoimmune reaction against NMDA-receptor subunits | |
Polymyositis |
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Mucocutaneous[14] | Acanthosis nigricans |
| |
Dermatomyositis | Immunologic[9] | ||
Leser-Trélat sign | |||
Necrolytic migratory erythema | Glucagonoma | ||
Sweet's syndrome | |||
Florid cutaneous papillomatosis | |||
Pyoderma gangrenosum | |||
Acquired generalized hypertrichosis | |||
Hematological[16] | Granulocytosis | G-CSF | |
Polycythemia | Erythropoietin[9] | ||
Trousseau sign | Mucins that activate clotting,[9] others | ||
Nonbacterial thrombotic endocarditis |
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Hypercoagulability[9] | |
Anemia |
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Unknown[9] | |
Others | Membranous glomerulonephritis |
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Tumor-induced osteomalacia |
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Stauffer syndrome |
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Neoplastic fever [18] |
References
- ↑ Paraneoplastic Syndromes, 2011, Darnell & Posner
- ↑ NINDS Paraneoplastic Syndromes Information Page National Institute of Neurological Disorders and Stroke
- ↑ Darnell,R.B., DeAngelis,L.M., Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies, [[{{{publisher}}}|{{{publisher}}}]], [[{{{date}}}|{{{date}}}]].
- ↑ Roberts,W.K., Darnell,R.B., Neuroimmunology of the paraneoplastic neurological degenerations, [[{{{publisher}}}|{{{publisher}}}]], [[{{{date}}}|{{{date}}}]].
- ↑ Albert,M.A., Darnell,R.B., Paraneoplastic neurological degenerations: keys to tumour immunity, [[{{{publisher}}}|{{{publisher}}}]], [[{{{date}}}|{{{date}}}]].
- ↑ Rees JH (2004). PARANEOPLASTIC SYNDROMES: WHEN TO SUSPECT, HOW TO CONFIRM, AND HOW TO MANAGE. J. Neurol. Neurosurg. Psychiatr. 75 Suppl 2 (Suppl 2): ii43–50.
- ↑ Darnell RB, Posner JB (2006). Paraneoplastic syndromes affecting the nervous system. Semin Oncol 33 (3): 270–98.
- ↑ MeSH Paraneoplastic+endocrine+syndromes
- ↑ 9.00 9.01 9.02 9.03 9.04 9.05 9.06 9.07 9.08 9.09 9.10 9.11 9.12 9.13 9.14 9.15 9.16 9.17 9.18 9.19 9.20 9.21 9.22 9.23 9.24 9.25 9.26 9.27 9.28 9.29 9.30 9.31 9.32 9.33 9.34 9.35 9.36 9.37 9.38 9.39 9.40 Table 6-5 in: Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson (2007). Robbins Basic Pathology, Philadelphia: Saunders. 8th edition.
- ↑ Mulatero P, Rabbia F, Veglio F. "Paraneoplastic hyperaldosteronism associated with non-Hodgkin's lymphoma.", New England Journal of Medicine. 2001 May 17;344(20):1558-9. Retrieved on 29 August 2012.
- ↑ MeSH Nervous+system+paraneoplastic+syndromes
- ↑ Buckanovich RJ, Posner JB, Darnell RB (1993). Nova, the paraneoplastic Ri antigen, is homologous to an RNA-binding protein and is specifically expressed in the developing motor system. Neuron 11 (4): 657–72.
- ↑ Dalmau J (January 2007). Paraneoplastic Anti–N-methyl-D-aspartate Receptor Encephalitis Associated with Ovarian Teratoma. Ann. Neurol. 61 (1): 25–36.
- ↑ Cohen PR, Kurzrock R (1997). Mucocutaneous paraneoplastic syndromes. Semin. Oncol. 24 (3): 334–59.
- ↑ (2001). Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. The Lancet 357 (9250): 96.
- ↑ Staszewski H (1997). Hematological paraneoplastic syndromes. Semin. Oncol. 24 (3): 329–33.
- ↑ Zadik Y, Nitzan DW (October 2011). Tumor induced osteomalacia: A forgotten paraneoplastic syndrome?. Oral Oncol 48 (2): e9–10.
- ↑ Zell JA, Chang JC. Neoplastic fever: a neglected paraneoplastic syndrome. PMID 15864658
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Pathology: Tumors, neoplasia, and oncology (C00-D48, 140-239) | |
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