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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} Cerebellar ataxia due to neoplasia;  
{{SK}} Synonym 1; Synonym 2; Synonym 3
   
   
==Overview==
==Overview==


'''Paraneoplastic cerebellar degeneration''' (PCD) is a rare [[paraneoplastic syndrome]] associated with [[lung]], [[ovarian]], [[breast]], [[Hodgkin’s lymphoma]], and other types of tumors. Paraneoplastic cerebellar degeneration occurs in less than 1 to 3% of cancer patients. The pathogenesis of paraneoplastic cerebellar degeneration is due to an autoimmune reaction targeted against components of the central nervous system.  The presence of anti-Purkinje cell is triggered by tumor cells, that normally express a Purkinje neuronal protein termed CDR2 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, and antibodies to the Zic4.  Paraneoplastic cerebellar degeneration is more commonly observed among patients between 40 to 60 years old. Paraneoplastic cerebellar degeneration affects females more commonly than males. The majority of patients with paraneoplastic cerebellar degeneration are typically symptomatic.  Early clinical features include dizziness, nausea, and vomiting. The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria: positive antibody-mediated immune response, diffuse cerebellar atrophy in imaging findings, and positive medical history for cancer. Common medical therapies for paraneoplastic cerebellar degeneration, include:  intravenous immunoglobulins, cyclophosphamide, and methylprednisolone.
==Historical Perspective==
*Paraneoplastic cerebellar degeneration was first described in early 1980.


==Classification==
==Classification==
Paraneoplastic cerebellar degeneration according to the presence or absence of an antibody, into several categories.  
*[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
:*[group1]
:*[group2]
:*[group3]
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of paraneoplastic cerebellar degeneration is characterized by the presence of anti-Purkinje cell antibodies.
*The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
*The pathogenesis of paraneoplastic cerebellar degeneration is due to an autoimmune reaction targeted against components of the central nervous system.
*The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
*The pathophysiology mechanism of paraneoplastic cerebellar degeneration is triggered by tumor cells, that normally express a protein (Purkinje neuronal protein termed cdr2). This protein is believed to trigger an anti-tumor immune and anti-neuronal immune response.  
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*The antibodies that have been associated with the development of paraneoplastic cerebellar degeneration, include:
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
:*Anti-P/Q type calcium channel antibodies
:*Anti-Tr antibodies
:*Anti-Ri (ANNA-2)
:*Anti-CV2
:*Antibodies to Ma proteins
:*Antibodies to the Zic4
==Causes==
==Causes==
* Causes of paraneoplastic cerebellar degeneration, include:
* [Disease name] may be caused by either [cause1], [cause2], or [cause3].
:*Lung cancer
* [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
:*Ovarian cancer
* There are no established causes for [disease name].
:*Breast cancer
:*Hodgkin's lymphoma
 
==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==
* Paraneoplastic cerebellar degeneration affects is approximately 1-3% of all cancer patients.  
 
===Age===
*Paraneoplastic cerebellar degeneration is more commonly observed among patients between 40 to 60 years old.
*Paraneoplastic cerebellar degeneration is more commonly observed among middle aged adults
===Gender===
*Paraneoplastic cerebellar degeneration affects females more commonly than males.
===Race===
*There is no racial predilection for paraneoplastic cerebellar degeneration.
   
   
==Differentiating [disease name] from other Diseases==
*[Disease name] 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]
 
==Risk Factors==
==Risk Factors==
*There are no known risk factors for paraneoplastic cerebellar degeneration.
*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 paraneoplastic cerebellar degeneration are typically symptomatic.
*Early clinical features include dizziness, nausea, and vomiting.  
*If left untreated,  the majority of patients with paraneoplastic cerebellar degeneration may progress to develop severe disability with inability to walk
*Common complications of paraneoplastic cerebellar degeneration, include:
*Prognosis is generally poor, and the median survival rate of patients with paraneoplastic cerebellar degeneration is approximately 13 months.
   
   
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*Positive antibody-mediated immune response
:*[criterion 1]
:*Diffuse cerebellar atrophy in imaging findings
:*[criterion 2]
:*Positive medical history for cancer.
:*[criterion 3]
 
:*[criterion 4]
=== Symptoms ===
=== Symptoms ===
*Symptoms of paraneoplastic cerebellar degeneration may include the following:
*[Disease name] is usually asymptomatic.
:*Dysarthria
*Symptoms of [disease name] may include the following:
:*Truncal, limb and gait ataxia
:*[symptom 1]
:*Vertigo
:*[symptom 2]
:*Nausea
:*[symptom 3]
:*Vomiting
:*[symptom 4]
:*Diplopia
:*[symptom 5]
:*Slurred speech
:*[symptom 6]
:*Dysphagia
:*Nystagmus
 
=== Physical Examination ===
=== Physical Examination ===
*Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.
*Patients with [disease name] usually appear [general appearance].
*Neurological examination may be remarkable for:<ref name="pmid16543537">{{cite journal |vauthors=Scheid R, Voltz R, Briest S, Kluge R, von Cramon DY |title=Clinical insights into paraneoplastic cerebellar degeneration |journal=J. Neurol. Neurosurg. Psychiatr. |volume=77 |issue=4 |pages=529–30 |year=2006 |pmid=16543537 |pmc=2077487 |doi=10.1136/jnnp.2005.082206 |url=}}</ref>
*Physical examination may be remarkable for:
:*Hyperactive reflexes
:*[finding 1]
:*Gait disturbance
:*[finding 2]
:*Babinski sign
:*[finding 3]
:*Speech disturbance
:*[finding 4]
:*Lack of coordination
:*[finding 5]
:*Nystagmus
:*[finding 6]
 
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with paraneoplastic cerebellar degeneration.
*There are no specific laboratory findings associated with [disease name].
*Laboratory testing may include thyroid function tests, vitamin levels,  and antibody titers (anti-gliadin, or anti-GAD antibodies)


*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===
===Imaging Findings===
*Magnetic resonance imaging is the imaging modality of choice for paraneoplastic cerebellar degeneration.  
*There are no [imaging study] findings associated with [disease name].
*On MRI, findings of paraneoplastic cerebellar degeneration, include:
:* Diffuse cerebellar atrophy
*[Imaging study 1] is the imaging modality of choice for [disease name].
:* No atrophy of the cerebral cortex, midbrain, pons, or medulla
*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 ===
=== Other Diagnostic Studies ===
*Paraneoplastic cerebellar degeneration may also be diagnosed using PET scan.
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on PET scan are often unspecific, but may include hypermetabolism.  
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
   
   
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*The mainstay of therapy for paraneoplastic cerebellar degeneration is supportive care.
*Common medical therapies for paraneoplastic cerebellar degeneration, include:
:*Intravenous immunoglobulins
:*Cyclophosphamide
:*Methylprednisolone


=== Surgery ===
=== Surgery ===
*Surgery is not recommended for patients with paraneoplastic cerebellar degeneration.


=== Prevention ===
*There are no primary preventive measures available for paraneoplastic cerebellar degeneration.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
   
   
[[Category: Oncology]]
[[Category:Pick One of 28 Approved]]

Revision as of 18:58, 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: Synonym 1; Synonym 2; Synonym 3

Overview

Classification

  • [Disease name] may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

  • [Disease name] may be caused by either [cause1], [cause2], or [cause3].
  • [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

  • [Disease name] 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]

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].


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

  • [Disease name] 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

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

Treatment

Medical Therapy

Surgery

References

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