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{{Infobox disease |
  Name          = Paraneoplastic cerebellar degeneration |
  Image          = |
  Caption        = |
  DiseasesDB    = 33977 |
  ICD10          = {{ICD10|G|13|0|g|10}} |
  ICD9          = {{ICD9|334.9}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = neuro |
  eMedicineTopic = 299 |
  MeshID          = D020362 |
}}
__NOTOC__
__NOTOC__
{{CMG}}
{{SI}}                                                                 
{{CMG}} {{AE}} {{MV}}
{{SK}} 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 [[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==
*Paraneoplastic cerebellar degeneration was first described in early 1980.
 
==Classification==
* Paraneoplastic cerebellar degeneration may be [[Classification|classified]] according to the presence or absence of an [[antibody]].
 
==Pathophysiology==
*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 [[Autoimmunity|autoimmune reaction]] targeted against components of the [[central nervous system]].
*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>
:*Anti - P/Q type [[calcium channel]] [[antibodies]]
:*Anti -Tr [[antibodies]]
:*Anti - Ri (ANNA-2)
:*Anti - CV2
:*[[Antibodies]] to Ma [[Protein|proteins]]
:*[[Antibodies]] to the Zic4
==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>
:*[[Lung cancer]]
:*[[Ovarian cancer]]
:*[[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:
**[[Cerebellum|Cerebellar]] [[metastasis]]
**[[Brain stem|Brainstem]] [[metastasis]]
**[[Metabolic]] derangement
**[[Intoxication]]
 
==Epidemiology and Demographics==
* 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===
*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 [[Adult|adults]].
===Gender===
*Paraneoplastic cerebellar degeneration affects [[Female|females]] more commonly than [[Male|males]].
===Race===
*There is no racial predilection to paraneoplastic cerebellar degeneration.
==Risk Factors==
*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>
 
== Screening ==
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for paraneoplastic cerebellar degeneration.
 
== Natural History, Complications, and Prognosis==
*The majority of [[Patient|patients]] with paraneoplastic cerebellar degeneration are typically [[symptomatic]].
*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 ==
===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>
:*Positive [[antibody]]-mediated [[immune response]]
:*Diffuse [[Cerebellum|cerebellar]] [[atrophy]] on [[imaging]]
:*Positive [[Medicine|medical]] history of [[cancer]]
 
=== History and Symptoms ===
*[[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]]
:*[[Trunk|Truncal]], [[Limb (anatomy)|limb]] and [[Gait (human)|gait]] [[ataxia]]
:*[[Vertigo]]
:*[[Nausea]]
:*[[Vomiting]]
:*[[Diplopia|Double vision]]
:*[[Slurred speech]]
:*[[Dysphagia|Difficulty swallowing]]
:*[[Nystagmus]]
 
=== Physical Examination ===
*[[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>
:*[[Hyperactive reflexes]]
:*[[Gait disturbance]]
:*[[Babinski sign]]
:*[[Speech disturbances|Speech disturbance]]
:*[[Lack of coordination of muscle movement|Lack of coordination]]
:*[[Nystagmus]]
 
=== Laboratory Findings ===
*There are no specific [[Medical laboratory|laboratory]] findings associated with paraneoplastic cerebellar degeneration.
*[[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.


{{SK}} Cerebellar ataxia due to neoplasia
=== X-ray ===
* There are no [[X-rays|x-ray]] findings associated with paraneoplastic cerebellar degeneration.


==Overview==
=== Echocardiography or Ultrasound ===
'''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.  
* 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.


As is the case with other [[paraneoplastic syndromes]],<ref>[http://books.google.com/books?id=TGFmbuOFot0C&dq=darnell+posner&source=gbs_navlinks_s Paraneoplastic Syndromes, 2011, Darnell & Posner]</ref> 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]]<ref>{{citation |journal=Annals of Neurology |author=Jaeckle,K.A., Graus,F., Houghton,A., Cardon-Cardo,C., Nielsen,S.L., Posner,J.B. |title=Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen |volume=18|issue=5 |pages=592–600 |year=1985 |pmid=2416270 |doi=10.1002/ana.410180513}}</ref> in the cerebellum,<ref name=Brock>{{cite journal|last=Phuphanich|first=Surasak|author2=Brock |author3=Charles |title=Neurologic improvement after high-dose intravenous immunoglobulin therapy in patients with paraneoplastic cerebellar degeneration associated with anti-Purkinje cell antibody|journal=Journal of Neuro-oncology|year=2007|volume=81|pages=67–69|doi=10.1007/s11060-006-9198-x}}</ref> sometimes  accompanied  by  a  proliferation  of  Berg- mann [[astrocytes]] and [[microglia]] in the molecular layer of the cerebellum and a loss of granule cells<ref name=Brock /><ref name=anderson>{{cite journal|last=Anderson|first=NE|author2=Rosenblum, MK |author3=Posner, JB |title=Paraneoplastic cerebellar degeneration: clinical-immunological correlations|journal=Annals of Neurology|year=1988|volume=24|issue=5|pages=559–567|doi=10.1002/ana.410240413}}</ref> ).  It is thought to be triggered when tumor cells (in PCD, most commonly ovarian or breast cancer<ref>{{citation |journal=Neurology |author=Peterson,K., Rosenblum, J.K., Kotanides,H., Posner,J.B. |title=Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients |volume=42|issue=10 |pages=1931–1937 |year=1992 |pmid= 1407575 |doi=10.1212/wnl.42.10.1931}}</ref><ref>Sillevis Smith et al, Chapter 97</ref>) 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.<ref>{{citation |journal=Current Opinion in Immunology |author=Roberts,W.K., Darnell,R.B. |title=Neuroimmunology of the paraneoplastic neurological degenerations |volume=16|issue=5 |pages=616–622 |year=2004 |pmid= 15342008 |doi=10.1016/j.coi.2004.07.009}}</ref>  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<ref name=Finsterer />  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.<ref name=Abdul /> 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.<ref name=Brock />
===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]]


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.<ref name=terrance /><ref name=Abdul /><ref name=Brock /> 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]]<ref name=terrance /><ref name=Brock /> and [[nystagmus]].  Neurological symptoms precede the diagnosis of the underlying cancer in about 60% of cases.<ref name=Finsterer /><ref name=Frings>{{cite journal|last=Frings|first=Markus|coauthors=Antoch, Gerald; Knorn, Phillipp; Freudenberg, Lutz; Bier, Ulrich; Timmann, Dagar, Timmann; Maschke, Matthias|title=Strategies in detection of the  primary tumour in anti-Yo associated paraneoplastic cerebellar degeneration|journal=Journal of Neurology|year=2005|volume=252|pages=197–201|doi=10.1007/s00415-005-0635-0}}</ref> 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.<ref name=Takeda>{{cite journal|last=Takeda|first=Akihrio|author2=Manabe, Shuichi |author3=Mitsui, Takashi |author4= Nakamura, Hiromi |title=Laparoscopic management of fallopian tube carcinoma with paraneoplastic cerebellar degeneration|journal=Gyneological Surgery|year=2007|volume=4|pages=131–134|doi=10.1007/s10397-006-0228-7}}</ref> 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.<ref name=Brock />
=== Other Imaging Findings ===
* There are no other [[imaging]] findings associated with paraneoplastic cerebellar degeneration.


==Pathophysiology==
=== Other Diagnostic Studies ===
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<ref>{{citation |journal=Proc Natl Acad Sci U S A |author=Darnell,R.B. |title=Onconeural antigens and the paraneoplastic neurologic disorders: at the intersection of cancer, immunity, and the brain |volume=93|issue=10 |pages=4529–4536 |year=1996 |pmid= 8643438 |pmc=39311 |doi=10.1073/pnas.93.10.4529}}</ref> that there might be a cell-mediated component (T cell) in disease pathogenesis. cdr2 antigen-specific CD8+ T cells were subsequently described<ref>{{citation |journal=Ann Neurol |author=Albert,M.L., Austin,L.M., Darnell,R.B. |title=Detection and treatment of activated T cells in the cerebrospinal fluid of patients with paraneoplastic cerebellar degeneration |volume=47|issue=1 |pages=9–17 |year=2000 |pmid= 10632096 |doi=10.1002/1531-8249(200001)47:1<9::aid-ana5>3.3.co;2-9}}</ref> in all PCD patients,<ref>{{citation |journal=Ann Neurol |author=Darnell,R.B., Albert,M.L. |title=cdr2-specific CTLs are detected in the blood of all patients with paraneoplastic cerebellar degeneration analyzed |volume=48|issue=2 |pages=270–271 |year=2000 |pmid= 10939585 |doi=10.1002/1531-8249(200008)48:2<270::aid-ana25>3.3.co;2-p}}</ref> making them a hallmark of the disease, and likely components in both the anti-tumor immune response and in the neuronal degeneration.
*Paraneoplastic cerebellar degeneration may also be [[Diagnosis|diagnosed]] using [[Positron emission tomography|PET scan]].
*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 ==
=== Medical Therapy ===
*The mainstay of [[therapy]] for paraneoplastic cerebellar degeneration is supportive care.
*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]] [[Antibody|immunoglobulins]]
:*[[Cyclophosphamide]]
:*[[Methylprednisolone]]


==References==
=== Surgery ===
{{Reflist|2}}
*[[Surgery]] is not recommended for [[Patient|patients]] with paraneoplastic cerebellar degeneration.


==External links==
=== Primary Prevention ===
*[http://www.antibodypatterns.com/yo.php Antibody associated with cerebellar degeneration]
*There are no [[Prevention (medical)|primary preventive]] measures available for paraneoplastic cerebellar degeneration.


{{Paraneoplastic syndromes}}
=== Secondary Prevention ===
* There are no [[Prevention (medical)|secondary preventive]] measures available for paraneoplastic cerebellar degeneration.


[[Category:Paraneoplastic syndromes]]
==References==
{{Reflist|2}}
[[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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