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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 paraneoplastic syndrome associated with lung, ovarian, breast, Hodgkin’s lymphoma and other cancers. PCD is a rare condition that occurs in less than 1% of cancer patients and usually occurs in middle-aged women.

Historical Perspective

• Paraneoplastic cerebellar degeneration was first described in early 1980.

Classification

Paraneoplastic cerebellar degeneration according to the presence or absence of an antibody, into several categories.

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 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
• Antibodies to the Zic4

• On microscopic histopathological analysis,findings of paraneoplastic cerebellar degeneration, include:

Causes

• Causes of paraneoplastic cerebellar degeneration, include:

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

• The prevalence of paraneoplastic cerebellar degeneration is approximately [number or range] per 100,000 individuals worldwide.

Age

• Patients of all age groups may develop [disease name].

• Paraneoplastic cerebellar degeneration is more commonly observed among patients aged [age range] years old.
• Paraneoplastic cerebellar degeneration is more commonly observed among [elderly patients/young patients/children].

Gender

• Paraneoplastic cerebellar degeneration affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• Paraneoplastic cerebellar degeneration usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Paraneoplastic cerebellar degeneration is usually asymptomatic.
• Symptoms of may include the following:

• Dysarthria
• Truncal, limb and gait ataxia
• Vertigo
• Nausea
• Vomiting
• Diplopia
• Nystagmus

Physical Examination

• Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.
• Physical examination may be remarkable for:

• Hyperactive reflexes
• Gait disturbance
• Babinski sign
• Speech disturbance
• Lack of coordination

Laboratory Findings

• There are no specific laboratory findings associated with paraneoplastic cerebellar degeneration.

Imaging Findings

• Magnetic resonance imaging is the imaging modality of choice for paraneoplastic cerebellar degeneration.
• On MRI, findings of paraneoplastic cerebellar degeneration, include:

• Diffuse cerebellar atrophy
• No atrophy of the cerebral cortex, midbrain, pons, or medulla

Other Diagnostic Studies

• Paraneoplastic cerebellar degeneration may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].