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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} Synonym 1; Synonym 2; Synonym 3
{{SK}} Cerebellar ataxia due to neoplasia;  
   
   
==Overview==
==Overview==
 
'''Paraneoplastic cerebellar degeneration''' (PCD) is a [[paraneoplastic syndrome]] associated with [[lung]], [[ovarian]], [[breast]], [[Hodgkin’s lymphoma]]<ref name=terrance>{{cite journal|last=O'Brien|first=Terrence J.|author2=Pasaliaris, Bill |author3=D'Apince, Anthony |author4= Byrne, Edward |title=Anti-Yo positive paraneoplastic cerebellar degeneration: a report of three cases and review of the literature|journal=Journal of Clinical Neuroscience|year=1995|volume=2|issue=4|pages=316–320|doi=10.1016/0967-5868(95)90052-7}}</ref>  and other [[cancer]]s. PCD is a rare condition that occurs in less than 1% of cancer patients<ref name=terrance /><ref name=Abdul>{{cite journal|last=Rana|first=Abdul, Oayyu|author2=Ranna, A.N. |author3=Adul, Ashfique |title=Acute ataxia due to anti-Yo antibody paraneoplastic cerebellar degeneration 4 months prior to diagnosis of uterine carcinoma|journal=Acta Neurologica Belgica|year=2012}}</ref><ref name=Finsterer>{{cite journal|last=Finsterer|first=Josef|author2=Voigtlander, Till |author3=Grisold, Wolfgang |title=Deterioration of anti-Yo-associated paraneoplastic cerebellar degeneration|journal=Journal of the Neurological Sciences|year=2011|volume=308|pages=139–141|doi=10.1016/j.jns.2011.06.051}}</ref> and usually occurs in middle-aged women.


==Historical Perspective==
==Historical Perspective==
*Paraneoplastic cerebellar degeneration was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Paraneoplastic cerebellar degeneration was first described in early 1980.  
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
   
   
==Classification==
==Classification==
*Paraneoplastic cerebellar degeneration may be classified according to [classification method] into [number] subtypes/groups:
Paraneoplastic cerebellar degeneration according to the presence or absence of an antibody, into several categories.  
:*[group1]
 
:*[group2]
:*[group3]
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The pathogenesis of paraneoplastic cerebellar degeneration is characterized by the presence of anti-Purkinje cell antibodies
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*The antibodies that have been associated with the development of paraneoplastic cerebellar degeneration, include:
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
:*Anti-P/Q type calcium channel antibodies
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
:*Anti-Tr antibodies
:*Anti-Ri (ANNA-2)
:*Anti-CV2
:*Antibodies to Ma proteins
:*Antibodies to the Zic4
*On gross pathology, characteristic findings of paraneoplastic cerebellar degeneration, include:
:*
:*
:*
*On microscopic histopathological analysis,findings of paraneoplastic cerebellar degeneration, include:
:*
:*
:*
   
   
==Causes==
==Causes==
* Paraneoplastic cerebellar degeneration may be caused by either [cause1], [cause2], or [cause3].
* Causes of paraneoplastic cerebellar degeneration, include:
* Paraneoplastic cerebellar degeneration is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
 
* There are no established causes for [disease name].
 
==Differentiating Paraneoplastic Cerebellar Degeneration from other Diseases==
==Differentiating [disease name] from other Diseases==
*Paraneoplastic cerebellar degeneration must be differentiated from other diseases that cause ataxia, dizziness, and nausea such as:
*Paraneoplastic cerebellar degeneration must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
 
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* The prevalence of paraneoplastic cerebellar degeneration is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
 
===Age===
===Age===
*Patients of all age groups may develop [disease name].
*Patients of all age groups may develop [disease name].

Revision as of 15:33, 13 April 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Cerebellar ataxia due to neoplasia;

Overview

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic syndrome associated with lung, ovarian, breast, Hodgkin’s lymphoma[1] and other cancers. PCD is a rare condition that occurs in less than 1% of cancer patients[1][2][3] and usually occurs in middle-aged women.

Historical Perspective

  • Paraneoplastic cerebellar degeneration was first described in early 1980.

Classification

Paraneoplastic cerebellar degeneration according to the presence or absence of an antibody, into several categories.

Pathophysiology

  • The pathogenesis of paraneoplastic cerebellar degeneration is characterized by the presence of anti-Purkinje cell antibodies
  • The antibodies that have been associated with the development of paraneoplastic cerebellar degeneration, include:
  • Anti-P/Q type calcium channel antibodies
  • Anti-Tr antibodies
  • Anti-Ri (ANNA-2)
  • Anti-CV2
  • Antibodies to Ma proteins
  • Antibodies to the Zic4
  • On gross pathology, characteristic findings of paraneoplastic cerebellar degeneration, include:
  • On microscopic histopathological analysis,findings of paraneoplastic cerebellar degeneration, include:

Causes

  • Causes of paraneoplastic cerebellar degeneration, include:


Differentiating Paraneoplastic Cerebellar Degeneration from other Diseases

  • Paraneoplastic cerebellar degeneration must be differentiated from other diseases that cause ataxia, dizziness, and nausea such as:

Epidemiology and Demographics

  • The prevalence of paraneoplastic cerebellar degeneration is approximately [number or range] per 100,000 individuals worldwide.

Age

  • Patients of all age groups may develop [disease name].
  • Paraneoplastic cerebellar degeneration is more commonly observed among patients aged [age range] years old.
  • Paraneoplastic cerebellar degeneration is more commonly observed among [elderly patients/young patients/children].

Gender

  • Paraneoplastic cerebellar degeneration affects men and women equally.
  • [Gender 1] are more commonly affected with [disease name] than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for [disease name].
  • Paraneoplastic cerebellar degeneration usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop [disease name].

Risk Factors

  • Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Paraneoplastic cerebellar degeneration is usually asymptomatic.
  • Symptoms of [disease name] may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with [disease name].
  • A [positive/negative] [test name] is diagnostic of [disease name].
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
  • Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with [disease name].
  • [Imaging study 1] is the imaging modality of choice for [disease name].
  • On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
  • [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

  • Paraneoplastic cerebellar degeneration may also be diagnosed using [diagnostic study name].
  • Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

  • There is no treatment for [disease name]; the mainstay of therapy is supportive care.
  • The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for [disease name].
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
  • [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

  • There are no primary preventive measures available for [disease name].
  • Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
  • Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

  1. 1.0 1.1 O'Brien, Terrence J.; Pasaliaris, Bill; D'Apince, Anthony; Byrne, Edward (1995). "Anti-Yo positive paraneoplastic cerebellar degeneration: a report of three cases and review of the literature". Journal of Clinical Neuroscience. 2 (4): 316–320. doi:10.1016/0967-5868(95)90052-7.
  2. Rana, Abdul, Oayyu; Ranna, A.N.; Adul, Ashfique (2012). "Acute ataxia due to anti-Yo antibody paraneoplastic cerebellar degeneration 4 months prior to diagnosis of uterine carcinoma". Acta Neurologica Belgica.
  3. Finsterer, Josef; Voigtlander, Till; Grisold, Wolfgang (2011). "Deterioration of anti-Yo-associated paraneoplastic cerebellar degeneration". Journal of the Neurological Sciences. 308: 139–141. doi:10.1016/j.jns.2011.06.051.