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 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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