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paraneoplastic cerebellar degeneration
Etiology:
1) breast cancer
2) ovarian cancer
3) female genital tract cancer
4) small cell carcinoma of the lung
5) Hodgkin's disease
6) testicular cancer
7) thymoma
Pathology:
1) Purkinje cell auto-antibodies (anti-Yo antibodies)
a) in patients with breast & ovarian cancer
b) but not lung cancer or Hodgkin's disease
2) loss of cerebellar Purkinje cells
Clinical manifestations:
1) symptoms may precede diagnosis of cancer by years
2) onset of symptoms may be abrupt of progress over a period of weeks to months
3) signs of cerebellar degeneration
a) dysarthria
b) ataxia
c) nystagmus
d) dizziness, vertigo
4) cognitive decline may occur
5) lethargy
Laboratory:
- serology:
- autoantibodies against CDR1
- ANNA-1 Ab (anti-Hu)
- ANNA-3 Ab
- anti-MA
- anti-Yo
- anti-CRMP-5
- anti-PCA-2
- anti-VGCC
Management:
1) symptomatic treatment is generally unsuccessful
2) treatment of underlying malignancy may result in clinical improvement
Interactions
disease interactions
Related
anti-Purkinje cell antibody (anti-Yo)
breast cancer
cerebellar degeneration-related antigen 1; CDR34 (CDR1)
Hodgkin's disease (Hodgkin's lymphoma)
ovarian cancer
small cell carcinoma
General
cerebellar degeneration
paraneoplastic syndrome
Properties
AUTOANTIBODY: anti-Purkinje cell antibody
Database Correlations
OMIM correlations
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17.
American College of Physicians, Philadelphia 1998, 2012, 2015
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624