ALTERNATE NAMES

Paraneoplastic Cerebellar Ataxia; PCD; Rapidly Progressive Cerebellar Syndrome; Subacute Cerebellar Degeneration

DESCRIPTION

Paraneoplastic Cerebellar Degeneration (PCD) is paraneoplastic syndrome caused by “killer T-cells,” or cytotoxic CD8+ T lymphocytes attacking normal cells in the cerebellum. Paraneoplastic syndromes are conditions believed to be the result of abnormal responses to undetected underlying malignant tumors.

PCD is a rare, non-metastatic complication that occurs in less than 1% of cancer patients (children and adults). Individuals with ovarian cancer, uterine cancer, breast cancer, small-cell lung cancer, and Hodgkin lymphoma are most often affected by PCD.

The symptoms of PCD can be rapidly progressive and very debilitating and they typically precede the diagnosis of the underlying malignancy. Prompt recognition and treatment of this disease are crucial to the prevention of significant disability.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of PCD is typically made by ruling out more common conditions that affect the cerebellum and other neurological disorders.

Testing used to diagnosis PCD may include:

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  Computed tomography scans (CT);

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  Cerebellar eye signs;

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  Various blood test panels;

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  Magnetic resonance imaging (MRI)(MRI of the brain in PCD is usually normal but can show cerebellar atrophy as the disease progresses);

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  Fluorodeoxyglucose-positron emission tomography scan; and

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  Positron emission tomography-computed tomography (PET/CT).

Physical findings: Physical findings of individuals with PCD may include:

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  Ataxia (loss of coordination);

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  Dizziness;

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  Dysarthria (slurred speech);

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  Dysphagia (difficulty swallowing);

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  Blurred vision;

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  Nystagmus (dancing eyes); and

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  Speech difficulties.

ICD-9: 331.89

ICD-10: G31.9, G32.81

PROGRESSION

Symptoms of PCD usually precede the diagnosis of the underlying cancer diagnosis. They often present very mildly and progress rapidly before ultimately reaching a severely disabled state that is followed by a variable plateau period that can last for months to years.

TREATMENT

Treatment of PCD varies depending on the source and severity of the underlying tumor. Common treatment for the condition may include tumor removal, high dose gammaglobulin therapy, or immunotherapy.

Suggested MER for Evaluation:

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  Clinical history and physical examination that describes the diagnostic features of the impairment including a description of gait; and

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  Imaging Reports Results of imaging (CT scan, MRI, PET).

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

11.17

111.17

PCD in adults must meet the conditions of 11.17 A or 11.17 B.

PCD in children must meet the conditions of 111.17.

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