Paraneoplastic Cerebellar Degeneration Workup

Updated: Nov 04, 2014
  • Author: Abbas Mehdi, MD; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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Workup

Laboratory Studies

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  • Consider a diagnosis of paraneoplastic cerebellar degeneration in patients who present with acute or subacute cerebellar degeneration and no risk factors for cerebellar disorders (eg, stroke, alcoholism, primary or metastatic neoplasms in the cerebellum, treatment with chemotherapeutic agents).

  • Once a diagnosis of paraneoplastic cerebellar degeneration is made, a thorough search for an underlying malignancy is warranted. Analysis of samples of serum and CSF for autoantibodies helps to determine the underlying primary malignancy.

  • Diagnosis and treatment of paraneoplastic cerebellar degeneration is important because the disability caused by the paraneoplastic cerebellar degeneration is severe; correct diagnosis can lead to early discovery of an occult tumor with chances of being cured.

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Imaging Studies

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  • Magnetic resonance imaging (MRI) findings are normal early in the course of paraneoplastic cerebellar degeneration but can show cerebellar atrophy in advanced cases. [25]

  • MRI of the brain with contrast is recommended to exclude any structural, demyelinating, vascular, or infectious causes. See the image below.

    MRI of a 29-year-old female with ARCA1. Sagittal T MRI of a 29-year-old female with ARCA1. Sagittal T1 shows marked diffuse cerebellar atrophy with no atrophy of the cerebral cortex, midbrain, pons, or medulla. Image from National Institutes of Health.
  • In paraneoplastic cerebellar degeneration with anti-Yo antibodies, perform radiography of the chest, mammography, and CT of the abdomen or chest to identify the primary malignancy.

  • In paraneoplastic cerebellar degeneration with anti-Hu antibodies, perform radiography and CT of the chest to identify a likely small-cell lung cancer. Also investigate other organs where small-cell cancers present, such as the cervix, esophagus, and prostate.

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Other Tests

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  • In addition to the imaging studies listed above, a thorough gynecologic examination should be performed in patients with paraneoplastic cerebellar degeneration with anti-Yo antibodies to identify the primary malignancy.

  • CSF samples demonstrate mononuclear pleocytosis, elevated protein levels (immunoglobulin G), and oligoclonal bands. These findings may normalize late in the course of the illness. Evaluate CSF for antineuronal autoantibodies. Anti-Yo and Anti-Tr antibodies have the highest specificity for cerebellar dysfunction.

  • Whole body fluorodeoxyglucose positron emission tomography (FDG-PET) is useful in demonstrating occult neoplasms or small metastatic lesions.

See the image below for an illustration of the workup of paraneoplastic cerebellar degeneration.

The workup of paraneoplastic cerebellar degenerati The workup of paraneoplastic cerebellar degeneration.
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Procedures

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  • If the initial workup of a patient who has paraneoplastic cerebellar degeneration with anti-Yo antibodies is nonrevealing, the usual next step is a total abdominal hysterectomy and a bilateral salpingo-oophorectomy in postmenopausal women. [26] If histologic examination reveals no malignancy and/or the patients are men or premenopausal women, periodic surveillance is necessary. At times, the primary malignancy is discovered up to 2 years after the initial onset of paraneoplastic cerebellar degeneration.

  • Perform tumor resection.

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Histologic Findings

The hallmark of paraneoplastic cerebellar degeneration is severe loss of Purkinje cells diffusely throughout the cerebellar cortex. These cells are completely absent on specimens. Other cell loss is observed but is rare. Occasionally, Purkinje cell loss is patchy. Inflammatory changes are also observed with lymphocytic infiltration. Atrophy of the granular and molecular layers is demonstrated, with microglial proliferation and astrocytosis but relative sparing of basket cells. The deep cerebellar nuclei and the cerebellar connections to the brain stem are normal. Patients with APCA-1/anti-Yo antibody tend to demonstrate more inflammatory changes and characteristic immunofluorescence patterns with coarse granular staining of Purkinje cell cytoplasm as well as proximal axons and dendrites; nuclei and systemic tissues are not stained. In paraneoplastic cerebellar degeneration associated with anti-Hu, the cortical and cerebellar neuronal nuclei are stained.

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