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VisualWikitext

Revision as of 18:23, 13 April 2016

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.

[terrance-1] 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.

[Abdul-2] 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-3] 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.

[1]

[2]

[3]

@@ Line 17: / Line 17: @@
 *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
@@ Line 24: / Line 25: @@
 :*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:
+:*Lung cancer
+:*Ovarian cancer
+:*Breast cancer
+:*Hodgkin's lymphoma
 ==Differentiating Paraneoplastic Cerebellar Degeneration from other Diseases==
@@ Line 37: / Line 38: @@
 ==Epidemiology and Demographics==
-* The prevalence of paraneoplastic cerebellar degeneration is approximately [number or range] per 100,000 individuals worldwide.
+* Paraneoplastic cerebellar degeneration affects is approximately 1-3% of all cancer patients.
 ===Age===
-*Patients of all age groups may develop [disease name].
+*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
-*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].
+*There is no racial predilection for paraneoplastic cerebellar degeneration.
-*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].
+*There are no known risk factors for paraneoplastic cerebellar degeneration.
 == Natural History, Complications and Prognosis==
-*The majority of patients with [disease name] remain asymptomatic for [duration/years].
+*The majority of patients with paraneoplastic cerebellar degeneration are typically symptomatic.
-*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
+*Early clinical features include dizziness, nausea, and vomiting.
-*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
+*If left untreated,  the majority of patients with paraneoplastic cerebellar degeneration may progress to develop severe disability with inability to walk
-*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
+*Common complications of paraneoplastic cerebellar degeneration, include:
-*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
+*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:
-:*[criterion 1]
+:*Positive antibody-mediated immune response
-:*[criterion 2]
+:*Diffuse cerebellar atrophy in imaging findings
-:*[criterion 3]
+:*Positive medical history for cancer.
-:*[criterion 4]
 === Symptoms ===
-*Paraneoplastic cerebellar degeneration is usually asymptomatic.
+*Symptoms of paraneoplastic cerebellar degeneration may include the following:
-*Symptoms of  may include the following:
 :*Dysarthria
 :*Truncal, limb and gait ataxia
@@ Line 84: / Line 74: @@
 :*Vomiting
 :*Diplopia
+:*Slurred speech
+:*Dysphagia
 :*Nystagmus
 === Physical Examination ===
 *Patients with paraneoplastic cerebellar degeneration usually appear confused, or lethargic.
-*Physical examination may be remarkable for:
+*Neurological examination may be remarkable for:
 :*Hyperactive reflexes
 :*Gait disturbance
@@ Line 94: / Line 86: @@
 :*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:
@@ Line 105: / Line 98: @@
 :* No atrophy of the cerebral cortex, midbrain, pons, or medulla
 === Other Diagnostic Studies ===
-*Paraneoplastic cerebellar degeneration may also be diagnosed using [diagnostic study name].
+*Paraneoplastic cerebellar degeneration may also be diagnosed using PET scan.
-*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
+*Findings on PET scan are often unspecific, but may include hypermetabolism.
 == Treatment ==
 === Medical Therapy ===
-*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
+*The mainstay of therapy for paraneoplastic cerebellar degeneration is supportive care.
+*Common medical therapies, include:
-*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
+:*Intravenous immunoglobulins
-*[Medical therapy 1] acts by [mechanism of action1].
+:*Cyclophosphamide
-*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
+:*Methylprednisolone
 === Surgery ===
-*Surgery is the mainstay of therapy for [disease name].
+*Surgery is not recommended for patients with paraneoplastic cerebellar degeneration.
-*[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].
+*There are no primary preventive measures available for paraneoplastic cerebellar degeneration.
-*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].
 ==References==
 {{Reflist|2}}
 [[Category: Oncology]]