Sandbox: Maria12
|
WikiDoc Resources for Sandbox: Maria12 |
|
Articles |
|---|
|
Most recent articles on Sandbox: Maria12 Most cited articles on Sandbox: Maria12 |
|
Media |
|
Powerpoint slides on Sandbox: Maria12 |
|
Evidence Based Medicine |
|
Clinical Trials |
|
Ongoing Trials on Sandbox: Maria12 at Clinical Trials.gov Trial results on Sandbox: Maria12 Clinical Trials on Sandbox: Maria12 at Google
|
|
Guidelines / Policies / Govt |
|
US National Guidelines Clearinghouse on Sandbox: Maria12 NICE Guidance on Sandbox: Maria12
|
|
Books |
|
News |
|
Commentary |
|
Definitions |
|
Patient Resources / Community |
|
Patient resources on Sandbox: Maria12 Discussion groups on Sandbox: Maria12 Patient Handouts on Sandbox: Maria12 Directions to Hospitals Treating Sandbox: Maria12 Risk calculators and risk factors for Sandbox: Maria12
|
|
Healthcare Provider Resources |
|
Causes & Risk Factors for Sandbox: Maria12 |
|
Continuing Medical Education (CME) |
|
International |
|
|
|
Business |
|
Experimental / Informatics |
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[1] and other cancers. PCD is a rare condition that occurs in less than 1% of cancer patients[1][2][3] 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 pathophysiology mechanism of paraneoplastic cerebellar degeneration is triggered by tumor cells, that normally express a protein (Purkinje neuronal protein termed cdr2). This protein is believed to trigger an anti-tumor immune and anti-neuronal immune response.
- 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
Causes
- Causes of paraneoplastic cerebellar degeneration, include:
- Lung cancer
- Ovarian cancer
- Breast cancer
- Hodgkin's lymphoma
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
- Paraneoplastic cerebellar degeneration affects is approximately 1-3% of all cancer patients.
Age
- Paraneoplastic cerebellar degeneration is more commonly observed among patients aged 45 years old.
- Paraneoplastic cerebellar degeneration is more commonly observed among elderly patients and adults
Gender
- Paraneoplastic cerebellar degeneration affects men and women equally.
Race
- There is no racial predilection for paraneoplastic cerebellar degeneration.
Risk Factors
- There are no known risk factors for paraneoplastic cerebellar degeneration.
Natural History, Complications and Prognosis
- The majority of patients with paraneoplastic cerebellar degeneration are typically symptomatic.
- Early clinical features include dizziness, nausea, and vomiting.
- If left untreated, the majority of patients with paraneoplastic cerebellar degeneration may progress to develop severe disability with inability to walk
- Common complications of paraneoplastic cerebellar degeneration, include:
- Prognosis is generally poor, and the median survival rate of patients with paraneoplastic cerebellar degeneration is approximately 13 months.
Diagnosis
Diagnostic Criteria
- The diagnosis of paraneoplastic cerebellar degeneration is made with the following criteria:
- Positive antibody-mediated immune response
- Diffuse cerebellar atrophy in imaging findings
- Positive medical history for cancer.
Symptoms
- Symptoms of paraneoplastic cerebellar degeneration may include the following:
- Dysarthria
- Truncal, limb and gait ataxia
- Vertigo
- Nausea
- Vomiting
- Diplopia
- Slurred speech
- Dysphagia
- Nystagmus
Physical Examination
- Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.
- Neurological examination may be remarkable for:
- Hyperactive reflexes
- Gait disturbance
- Babinski sign
- Speech disturbance
- Lack of coordination
- Nystagmus
Laboratory Findings
- There are no specific laboratory findings associated with paraneoplastic cerebellar degeneration.
- Laboratory testing may include thyroid function tests, vitamin levels, and antibody titers (anti-gliadin, or anti-GAD antibodies)
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 PET scan.
- Findings on PET scan are often unspecific, but may include hypermetabolism.
Treatment
Medical Therapy
- The mainstay of therapy for paraneoplastic cerebellar degeneration is supportive care.
- Common medical therapies, include:
- Intravenous immunoglobulins
- Cyclophosphamide
- Methylprednisolone
Surgery
- Surgery is not recommended for patients with paraneoplastic cerebellar degeneration.
Prevention
- There are no primary preventive measures available for paraneoplastic cerebellar degeneration.
References
- ↑ 1.0 1.1 O'Brien, Terrence J.; Pasaliaris, Bill; D'Apince, Anthony; Byrne, Edward (1995). "Anti-Yo positive paraneoplastic cerebellar degeneration: a report of three cases and review of the literature". Journal of Clinical Neuroscience. 2 (4): 316–320. doi:10.1016/0967-5868(95)90052-7.
- ↑ Rana, Abdul, Oayyu; Ranna, A.N.; Adul, Ashfique (2012). "Acute ataxia due to anti-Yo antibody paraneoplastic cerebellar degeneration 4 months prior to diagnosis of uterine carcinoma". Acta Neurologica Belgica.
- ↑ Finsterer, Josef; Voigtlander, Till; Grisold, Wolfgang (2011). "Deterioration of anti-Yo-associated paraneoplastic cerebellar degeneration". Journal of the Neurological Sciences. 308: 139–141. doi:10.1016/j.jns.2011.06.051.