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==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.  
Paraneoplastic cerebellar degeneration (PCD) is a rare [[paraneoplastic syndrome]] associated with [[lung]], [[ovarian]], [[breast]], [[Hodgkin’s lymphoma]], and other types of [[Tumor|tumors]]. Paraneoplastic cerebellar degeneration occurs in less than 1 to 3% of [[cancer]] [[Patient|patients]]. The [[pathogenesis]] of paraneoplastic cerebellar degeneration is due to an [[Autoimmunity|autoimmune reaction]] targeted against components of the [[central nervous system]]. The presence of anti-[[Purkinje cell]] is triggered by [[Tumor cell|tumor cells]], that normally express a Purkinje [[Neuron|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 [[Protein|proteins]], and [[antibodies]] to the Zic4.  Paraneoplastic cerebellar degeneration is more commonly observed among [[Patient|patients]] between 40 to 60 years old. Paraneoplastic cerebellar degeneration affects [[Female|females]] more commonly than [[Male|males]]. The majority of [[Patient|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 [[Cerebellum|cerebellar]] [[atrophy]] on [[imaging]], and positive [[Medicine|medical]] history of [[cancer]]. Common [[Medicine|medical]] [[Therapy|therapies]] for paraneoplastic cerebellar degeneration include [[intravenous]] [[immunoglobulins]], [[cyclophosphamide]], and [[methylprednisolone]]. There are no [[Prevention|primary and secondary preventive]] measures available for paraneoplastic cerebellar degeneration.  


==Historical Perspective==
==Historical Perspective==
Line 13: Line 13:


==Classification==
==Classification==
 
* Paraneoplastic cerebellar degeneration may be [[Classification|classified]] according to the presence or absence of an [[antibody]].  
Paraneoplastic cerebellar degeneration may be classified according to the presence or absence of an antibody.  


==Pathophysiology==
==Pathophysiology==
*The pathogenesis of paraneoplastic cerebellar degeneration is characterized by the presence of anti-Purkinje cell antibodies.  
*The [[pathogenesis]] of paraneoplastic cerebellar degeneration is characterized by the presence of anti-[[Purkinje cell]] [[antibodies]].  
*The pathogenesis of paraneoplastic cerebellar degeneration is due to an autoimmune reaction targeted against components of the central nervous system.  
*The [[pathogenesis]] of paraneoplastic cerebellar degeneration is due to an [[Autoimmunity|autoimmune reaction]] targeted against components of the [[central nervous system]].  
*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.  
*The [[pathogenesis]] of paraneoplastic cerebellar degeneration is triggered by [[Tumor cell|tumor cells]], that normally express a [[protein]] (Purkinje [[Neuron|neuronal]] [[protein]] termed cdr2). This [[protein]] is believed to trigger an anti-[[tumor]] [[Immunity (medical)|immune]] and anti-[[Neuron|neuronal]] [[Immunity (medical)|immune]] response.  
*The antibodies that have been associated with the development of paraneoplastic cerebellar degeneration, include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
*The [[antibodies]] that have been associated with the development of paraneoplastic cerebellar degeneration include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:*Anti-P/Q type calcium channel antibodies  
:*Anti - P/Q type [[calcium channel]] [[antibodies]]
:*Anti-Tr antibodies
:*Anti -Tr [[antibodies]]
:*Anti-Ri (ANNA-2)
:*Anti - Ri (ANNA-2)
:*Anti-CV2
:*Anti - CV2
:*Antibodies to Ma proteins
:*[[Antibodies]] to Ma [[Protein|proteins]]
:*Antibodies to the Zic4  
:*[[Antibodies]] to the Zic4  
==Causes==
==Causes==
* Causes of paraneoplastic cerebellar degeneration, include:<ref name="wiki> Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  
* Causes of paraneoplastic cerebellar degeneration include:<ref name="wiki">Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  
:*Lung cancer
:*[[Lung cancer]]
:*Ovarian cancer
:*[[Ovarian cancer]]
:*Breast cancer
:*[[Breast cancer]]
:*Hodgkin's lymphoma
:*[[Hodgkin's lymphoma]]


==Differentiating Paraneoplastic Cerebellar Degeneration from other Diseases==
==Differentiating Paraneoplastic Cerebellar Degeneration 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 [[ataxia]], [[dizziness]], and [[nausea]] such as:
**[[Cerebellum|Cerebellar]] [[metastasis]]
**[[Brain stem|Brainstem]] [[metastasis]]
**[[Metabolic]] derangement
**[[Intoxication]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
* Paraneoplastic cerebellar degeneration affects is approximately 1-3% of all cancer patients.<ref name="wiki> Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  
* Paraneoplastic cerebellar degeneration affects approximately 1 - 3% of all [[cancer]] [[Patient|patients]].<ref name="wiki">Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  


===Age===
===Age===
*Paraneoplastic cerebellar degeneration is more commonly observed among patients between 40 to 60 years old.
*Paraneoplastic cerebellar degeneration is more commonly observed among [[Patient|patients]] between 40 to 60 years old.
*Paraneoplastic cerebellar degeneration is more commonly observed among middle aged adults  
*Paraneoplastic cerebellar degeneration is more commonly observed among middle aged [[Adult|adults]].
   
   
===Gender===
===Gender===
*Paraneoplastic cerebellar degeneration affects females more commonly than males.  
*Paraneoplastic cerebellar degeneration affects [[Female|females]] more commonly than [[Male|males]].  
   
   
===Race===
===Race===
*There is no racial predilection for paraneoplastic cerebellar degeneration.
*There is no racial predilection to paraneoplastic cerebellar degeneration.
   
   
==Risk Factors==
==Risk Factors==
*There are no known risk factors for paraneoplastic cerebellar degeneration.<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>
*There are no known [[Risk factor|risk factors]] for paraneoplastic cerebellar degeneration.<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>
 
== Natural History, Complications and Prognosis==
== Screening ==
*The majority of patients with paraneoplastic cerebellar degeneration are typically symptomatic.  
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for paraneoplastic cerebellar degeneration.
*Early clinical features include dizziness, nausea, and vomiting.<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>
 
*If left untreated,  the majority of patients with paraneoplastic cerebellar degeneration may progress to develop severe disability with inability to walk
== Natural History, Complications, and Prognosis==
*The most common complication of paraneoplastic cerebellar degeneration is cerebellar dysfunction.  
*The majority of [[Patient|patients]] with paraneoplastic cerebellar degeneration are typically [[symptomatic]].  
*Prognosis is generally poor, and the median survival rate of patients with paraneoplastic cerebellar degeneration is approximately 13 months.<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
*Early clinical features include [[dizziness]], [[nausea]], and [[vomiting]].<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>
*If left untreated,  the majority of [[Patient|patients]] with paraneoplastic cerebellar degeneration may progress to develop severe [[disability]] with inability to walk.
*The most common [[Complication (medicine)|complication]] of paraneoplastic cerebellar degeneration is [[Cerebellum|cerebellar]] [[dysfunction]].  
*[[Prognosis]] is generally poor, and the [[median]] [[survival rate]] of [[Patient|patients]] with paraneoplastic cerebellar degeneration is approximately 13 months.<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
   
   
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Study of Choice===
*The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
*The [[diagnosis]] of paraneoplastic cerebellar degeneration is made with the following criteria:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:*Positive antibody-mediated immune response
:*Positive [[antibody]]-mediated [[immune response]]
:*Diffuse cerebellar atrophy in imaging findings
:*Diffuse [[Cerebellum|cerebellar]] [[atrophy]] on [[imaging]]
:*Positive medical history for cancer.
:*Positive [[Medicine|medical]] history of [[cancer]]


=== Symptoms ===
=== History and Symptoms ===
*Symptoms of paraneoplastic cerebellar degeneration may include the following:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
*[[Symptom|Symptoms]] of paraneoplastic cerebellar degeneration may include the following:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:*Dysarthria
:*[[Dysarthria]]
:*Truncal, limb and gait ataxia
:*[[Trunk|Truncal]], [[Limb (anatomy)|limb]] and [[Gait (human)|gait]] [[ataxia]]
:*Vertigo
:*[[Vertigo]]
:*Nausea
:*[[Nausea]]
:*Vomiting
:*[[Vomiting]]
:*Diplopia  
:*[[Diplopia|Double vision]]
:*Slurred speech  
:*[[Slurred speech]]
:*Dysphagia  
:*[[Dysphagia|Difficulty swallowing]]
:*Nystagmus
:*[[Nystagmus]]


=== Physical Examination ===
=== Physical Examination ===
*Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.<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>
*[[Patient|Patients]] with paraneoplastic cerebellar degeneration usually appear [[Confusion|confused]], or [[Fatigue|lethargic]].<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>
*Neurological examination may be remarkable for:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
*[[Neurological examination]] may be remarkable for:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:*Hyperactive reflexes
:*[[Hyperactive reflexes]]
:*Gait disturbance
:*[[Gait disturbance]]
:*Babinski sign
:*[[Babinski sign]]
:*Speech disturbance
:*[[Speech disturbances|Speech disturbance]]
:*Lack of coordination
:*[[Lack of coordination of muscle movement|Lack of coordination]]
:*Nystagmus
:*[[Nystagmus]]


=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with paraneoplastic cerebellar degeneration.
*There are no specific [[Medical laboratory|laboratory]] findings associated with paraneoplastic cerebellar degeneration.
*Laboratory testing may include thyroid function tests, vitamin levels, and antibody titers (anti-gliadin, or anti-GAD antibodies)  
*[[Medical laboratory|Laboratory]] [[Test|testing]] may include [[thyroid function tests]], [[vitamin]] levels, and [[antibody]] [[Titer|titers]] ([[Anti-gliadin antibodies|anti-gliadin]], or anti-GAD [[antibodies]]).
 
=== Electrocardiogram ===
* There are no [[The electrocardiogram|ECG]] findings associated with paraneoplastic cerebellar degeneration.
 
=== X-ray ===
* There are no [[X-rays|x-ray]] findings associated with paraneoplastic cerebellar degeneration.
 
=== Echocardiography or Ultrasound ===
* There are no [[echocardiography]]/[[ultrasound]] findings associated with paraneoplastic cerebellar degeneration.
 
=== CT scan ===
* There are no [[Computed tomography|CT scan]] findings associated with paraneoplastic cerebellar degeneration.
 
===MRI===
*[[Magnetic resonance imaging|MRI]] is the [[imaging]] modality of choice for paraneoplastic cerebellar degeneration.
*On [[Magnetic resonance imaging|MRI]], findings of paraneoplastic cerebellar degeneration include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:* Diffuse [[Cerebellum|cerebellar]] [[atrophy]]
:* No [[atrophy]] of the [[cerebral cortex]], [[Mesencephalon|midbrain]], [[pons]], or [[Medulla oblongata|medulla]]


===Imaging Findings===
=== Other Imaging Findings ===
*Magnetic resonance imaging is the imaging modality of choice for paraneoplastic cerebellar degeneration.
* There are no other [[imaging]] findings associated with paraneoplastic cerebellar degeneration.
*On MRI, findings of paraneoplastic cerebellar degeneration, include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>
:* Diffuse cerebellar atrophy
:* No atrophy of the cerebral cortex, midbrain, pons, or medulla


=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*Paraneoplastic cerebellar degeneration may also be diagnosed using PET scan.
*Paraneoplastic cerebellar degeneration may also be [[Diagnosis|diagnosed]] using [[Positron emission tomography|PET scan]].
*Findings on PET scan are often unspecific, but may include hypermetabolism.<ref name="wiki> Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  
*Findings on [[Positron emission tomography|PET scan]] are often unspecific, but may include [[hypermetabolism]].<ref name="wiki">Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration  Accessed on April 13, 2016</ref>  
   
   
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*The mainstay of therapy for paraneoplastic cerebellar degeneration is supportive care.
*The mainstay of [[therapy]] for paraneoplastic cerebellar degeneration is supportive care.
*Common medical therapies for paraneoplastic cerebellar degeneration, include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>  
*Common [[Medicine|medical]] [[Therapy|therapies]] for paraneoplastic cerebellar degeneration include:<ref name="pmid18339348">{{cite journal |vauthors=Dalmau J, Rosenfeld MR |title=Paraneoplastic syndromes of the CNS |journal=Lancet Neurol |volume=7 |issue=4 |pages=327–40 |year=2008 |pmid=18339348 |pmc=2367117 |doi=10.1016/S1474-4422(08)70060-7 |url=}}</ref>  
:*Intravenous immunoglobulins  
:*[[Intravenous]] [[Antibody|immunoglobulins]]
:*Cyclophosphamide
:*[[Cyclophosphamide]]
:*Methylprednisolone
:*[[Methylprednisolone]]


=== Surgery ===
=== Surgery ===
*Surgery is not recommended for patients with paraneoplastic cerebellar degeneration.
*[[Surgery]] is not recommended for [[Patient|patients]] with paraneoplastic cerebellar degeneration.
 
=== Primary Prevention ===
*There are no [[Prevention (medical)|primary preventive]] measures available for paraneoplastic cerebellar degeneration.
 
=== Secondary Prevention ===
* There are no [[Prevention (medical)|secondary preventive]] measures available for paraneoplastic cerebellar degeneration.


=== Prevention ===
*There are no primary preventive measures available for paraneoplastic cerebellar degeneration.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
   
   
[[Category: Oncology]]
[[Category: Oncology]]
[[Category:Rheumatology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Neurology]]

Latest revision as of 18:00, 20 May 2019

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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 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 on imaging, and positive medical history of cancer. Common medical therapies for paraneoplastic cerebellar degeneration include intravenous immunoglobulins, cyclophosphamide, and methylprednisolone. There are no primary and secondary preventive measures available for paraneoplastic cerebellar degeneration.

Historical Perspective

  • Paraneoplastic cerebellar degeneration was first described in early 1980.

Classification

  • Paraneoplastic cerebellar degeneration may be classified according to the presence or absence of an antibody.

Pathophysiology

Causes

  • Causes of paraneoplastic cerebellar degeneration include:[2]

Differentiating Paraneoplastic Cerebellar Degeneration from Other Diseases

Epidemiology and Demographics

  • Paraneoplastic cerebellar degeneration affects approximately 1 - 3% of all cancer patients.[2]

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 to paraneoplastic cerebellar degeneration.

Risk Factors

  • There are no known risk factors for paraneoplastic cerebellar degeneration.[3]

Screening

  • There is insufficient evidence to recommend routine screening for paraneoplastic cerebellar degeneration.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

  • The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:[1]

History and Symptoms

  • Symptoms of paraneoplastic cerebellar degeneration may include the following:[1]

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with paraneoplastic cerebellar degeneration.

X-ray

  • There are no x-ray findings associated with paraneoplastic cerebellar degeneration.

Echocardiography or Ultrasound

CT scan

  • There are no CT scan findings associated with paraneoplastic cerebellar degeneration.

MRI

  • MRI is the imaging modality of choice for paraneoplastic cerebellar degeneration.
  • On MRI, findings of paraneoplastic cerebellar degeneration include:[1]

Other Imaging Findings

  • There are no other imaging findings associated with paraneoplastic cerebellar degeneration.

Other Diagnostic Studies

Treatment

Medical Therapy

  • The mainstay of therapy for paraneoplastic cerebellar degeneration is supportive care.
  • Common medical therapies for paraneoplastic cerebellar degeneration include:[1]

Surgery

  • Surgery is not recommended for patients with paraneoplastic cerebellar degeneration.

Primary Prevention

  • There are no primary preventive measures available for paraneoplastic cerebellar degeneration.

Secondary Prevention

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Dalmau J, Rosenfeld MR (2008). "Paraneoplastic syndromes of the CNS". Lancet Neurol. 7 (4): 327–40. doi:10.1016/S1474-4422(08)70060-7. PMC 2367117. PMID 18339348.
  2. ↑ 2.0 2.1 2.2 Paraneoplastic cerebellar degeneration. Wikipedia. https://en.wikipedia.org/wiki/Paraneoplastic_cerebellar_degeneration Accessed on April 13, 2016
  3. ↑ 3.0 3.1 3.2 Scheid R, Voltz R, Briest S, Kluge R, von Cramon DY (2006). "Clinical insights into paraneoplastic cerebellar degeneration". J. Neurol. Neurosurg. Psychiatr. 77 (4): 529–30. doi:10.1136/jnnp.2005.082206. PMC 2077487. PMID 16543537.
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