Paraneoplastic cerebellar degeneration

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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 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

Paraneoplastic cerebellar degeneration may be 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 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 antibodies that have been associated with the development of paraneoplastic cerebellar degeneration, include:[1]
  • 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 of paraneoplastic cerebellar degeneration, include:[2]

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.[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 for paraneoplastic cerebellar degeneration.

Risk Factors

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

Natural History, Complications and Prognosis

  • The majority of patients with paraneoplastic cerebellar degeneration are typically symptomatic.
  • Early clinical features include dizziness, nausea, and vomiting.[3]
  • If left untreated, the majority of patients with paraneoplastic cerebellar degeneration may progress to develop severe disability with inability to walk
  • The most common complication of paraneoplastic cerebellar degeneration is cerebellar dysfunction.
  • Prognosis is generally poor, and the median survival rate of patients with paraneoplastic cerebellar degeneration is approximately 13 months.[1]

Diagnosis

Diagnostic Criteria

  • The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:[1]
  • Positive antibody-mediated immune response
  • Diffuse cerebellar atrophy in imaging findings
  • Positive medical history for cancer.

Symptoms

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

Physical Examination

  • Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.[3]
  • Neurological examination may be remarkable for:[1]

Laboratory Findings

  • There are no specific 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)

Imaging Findings

  • Magnetic resonance imaging is the imaging modality of choice for paraneoplastic cerebellar degeneration.
  • On MRI, findings of paraneoplastic cerebellar degeneration, include:[1]
  • Diffuse cerebellar atrophy
  • No atrophy of the cerebral cortex, midbrain, pons, or medulla

Other Diagnostic Studies

  • Paraneoplastic cerebellar degeneration may also be diagnosed using PET scan.
  • Findings on PET scan are often unspecific, but may include hypermetabolism.[2]

Treatment

Medical Therapy

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

Surgery

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

Prevention

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

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. PMC 2367117Freely accessible. PMID 18339348. doi:10.1016/S1474-4422(08)70060-7. 
  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. PMC 2077487Freely accessible. PMID 16543537. doi:10.1136/jnnp.2005.082206. 

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