PCD
MCID: PRN033
MIFTS: 32

Paraneoplastic Neurologic Disorders (PCD)

Categories: Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Paraneoplastic Neurologic Disorders

MalaCards integrated aliases for Paraneoplastic Neurologic Disorders:

Name: Paraneoplastic Neurologic Disorders 52
Paraneoplastic Cerebellar Degeneration 58
Paraneoplastic Neurologic Syndrome 58
Pcd 58
Pnd 52
Pns 58

Characteristics:

Orphanet epidemiological data:

58
paraneoplastic neurologic syndrome
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

UMLS via Orphanet 72 C0393534 C0751911 C3267031
Orphanet 58 ORPHA36388

Summaries for Paraneoplastic Neurologic Disorders

NIH Rare Diseases : 52 Paraneoplastic neurologic disorders are a group of rare degenerative conditions that are triggered by a person's immune system response to a cancerous tumor . Researchers believe these disorders occur when cancer -fighting antibodies or white blood cells known as T cells mistakenly attack normal cells in the nervous system. Paraneoplastic neurologic disorders typically develop after mid-adulthood and are most common in people with lung, ovarian, lymphatic, or breast cancer. Symptoms generally develop over a period of days to weeks and usually occur prior to tumor detection. These symptoms may include difficulty in walking and/or swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia , seizures , sensory loss in the limbs, and vertigo . Paraneoplastic neurologic disorders include Lambert-Eaton myasthenic syndrome , stiff-person syndrome , encephalomyelitis (inflammation of the brain and spinal cord), myasthenia gravis , cerebellar degeneration , limbic and/or brainstem encephalitis , neuromyotonia , and opsoclonus (involving eye movement) and sensory neuropathy . Treatment involves treatment of the underlying tumor, immunotherapy , and supportive therapy.

MalaCards based summary : Paraneoplastic Neurologic Disorders, also known as paraneoplastic cerebellar degeneration, is related to dandy-walker syndrome and primary ciliary dyskinesia. An important gene associated with Paraneoplastic Neurologic Disorders is AP3B2 (Adaptor Related Protein Complex 3 Subunit Beta 2). The drugs Immunologic Factors and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include lung, breast and t cells, and related phenotypes are behavior/neurological and nervous system

Related Diseases for Paraneoplastic Neurologic Disorders

Diseases related to Paraneoplastic Neurologic Disorders via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 454)
# Related Disease Score Top Affiliating Genes
1 dandy-walker syndrome 29.1 ZIC4 ZIC1
2 primary ciliary dyskinesia 12.5
3 poikiloderma with neutropenia 12.4
4 premature centromere division 11.7
5 guillain-barre syndrome 11.7
6 prurigo nodularis 11.6
7 kartagener syndrome 11.6
8 stiff-person syndrome 11.5
9 ciliary dyskinesia, primary, 1 11.4
10 hyperphenylalaninemia, bh4-deficient, d 11.4
11 peripheral nervous system neoplasm 11.2
12 peripheral nervous system ganglioneuroblastoma 11.2
13 oculopharyngeal muscular dystrophy 11.2
14 mcleod syndrome 11.2
15 pathologic nystagmus 10.6
16 ovary adenocarcinoma 10.4
17 lung disease 10.4
18 tremor 10.4
19 wallerian degeneration 10.3
20 bronchiectasis 10.3
21 lymphoma 10.3
22 retinal degeneration 10.3
23 postpartum depression 10.3
24 diffuse large b-cell lymphoma 10.3
25 fallopian tube adenocarcinoma 10.3
26 gastric adenocarcinoma 10.3
27 otitis media 10.3
28 paraneoplastic limbic encephalitis 10.3
29 anxiety 10.3
30 bladder cancer 10.2
31 lung oat cell carcinoma 10.2
32 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.2
33 esophageal cancer 10.2
34 myoclonus 10.2
35 hypertension, essential 10.2
36 krabbe disease 10.2
37 short bowel syndrome 10.2
38 paraneoplastic syndromes 10.2
39 premature chromatid separation trait 10.2
40 mosaic variegated aneuploidy syndrome 1 10.2
41 male infertility 10.2
42 rhinitis 10.2
43 endometrial cancer 10.2
44 encephalopathy 10.2
45 panic disorder 10.2
46 leukodystrophy 10.2
47 cerebellar degeneration 10.2
48 limbic encephalitis 10.2
49 opsoclonus-myoclonus syndrome 10.2
50 wilms tumor 5 10.1

Graphical network of the top 20 diseases related to Paraneoplastic Neurologic Disorders:



Diseases related to Paraneoplastic Neurologic Disorders

Symptoms & Phenotypes for Paraneoplastic Neurologic Disorders

MGI Mouse Phenotypes related to Paraneoplastic Neurologic Disorders:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.26 AP3B2 ELAVL4 ZIC1 ZIC4
2 nervous system MP:0003631 8.92 AP3B2 ELAVL4 ZIC1 ZIC4

Drugs & Therapeutics for Paraneoplastic Neurologic Disorders

Drugs for Paraneoplastic Neurologic Disorders (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunologic Factors Phase 2
2 Antibodies Phase 2
3 Immunoglobulins, Intravenous Phase 2
4 Rho(D) Immune Globulin Phase 2
5 gamma-Globulins Phase 2
6 Immunoglobulins Phase 2
7
Prednisone Approved, Vet_approved 53-03-2 5865
8
Tacrolimus Approved, Investigational 104987-11-3 445643 439492 6473866
9 Immunosuppressive Agents
10 Calcineurin Inhibitors

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Early Onset Immunotherapy by Intravenous Immunoglobulin in Well-characterized Onconeural-antibody-positive Paraneoplastic Neurological Syndromes Completed NCT02343211 Phase 2 Immunoglobulin
2 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Tacrolimus
3 Tumor Immunity in Neurologically Normal Patients Completed NCT00481637

Search NIH Clinical Center for Paraneoplastic Neurologic Disorders

Genetic Tests for Paraneoplastic Neurologic Disorders

Anatomical Context for Paraneoplastic Neurologic Disorders

MalaCards organs/tissues related to Paraneoplastic Neurologic Disorders:

40
Lung, Breast, T Cells, Eye, Brain, Spinal Cord, Colon

Publications for Paraneoplastic Neurologic Disorders

Articles related to Paraneoplastic Neurologic Disorders:

(show top 50) (show all 102)
# Title Authors PMID Year
1
An autoimmune-based, paraneoplastic neurologic syndrome following checkpoint inhibition and concurrent radiotherapy for merkel cell carcinoma: case report. 61
32006066 2020
2
Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies. 61
32170042 2020
3
Worsening of anti-Hu paraneoplastic neurological syndrome related to anti-PD-1 treatment: Case report and review of literature. 61
32058173 2020
4
Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors. 61
31454760 2019
5
18 F-Fluorodeoxyglucose positron-emission tomography for the investigation of malignancy in patients with suspected paraneoplastic neurologic syndromes and negative or indeterminate conventional imaging: a retrospective analysis of the Ontario PET Access Program, with systematic review and meta-analysis. 61
31548813 2019
6
Paraneoplastic neurologic syndrome as a presentation of underlying breast malignancy. 61
30240068 2018
7
Anti-Ma2-antibody-associated encephalitis: An atypical paraneoplastic neurologic syndrome. 61
31754496 2018
8
Acquired Hemophilia and Anti-Hu Paraneoplastic Neurologic Syndrome in Small Cell Lung Cancer. 61
29078851 2017
9
Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report. 61
28984777 2017
10
FDG-Avid Keloid on PET/CT Imaging. 61
28481801 2017
11
Thymoma-associated panencephalitis: a newly emerging paraneoplastic neurologic syndrome. 61
29479559 2017
12
Limbic encephalitis - a report of four cases. 61
28860940 2017
13
CTLA4 blockade elicits paraneoplastic neurological disease in a mouse model. 61
27604307 2016
14
A destructive feedback loop mediated by CXCL10 in central nervous system inflammatory disease. 61
26224283 2015
15
Paraneoplastic neurologic disorders in small cell lung carcinoma: A prospective study. 61
26109714 2015
16
Neuronal Na+/K+ ATPase is an autoantibody target in paraneoplastic neurologic syndrome. 61
25809299 2015
17
Neoplastic myelopathies. 61
25651222 2015
18
Paraneoplastic syndrome: a masquerade of autoimmune inner ear disease. 61
25427223 2015
19
Neural autoantibody clusters aid diagnosis of cancer. 61
24833664 2014
20
Autoimmune encephalitis update. 61
24637228 2014
21
Seizures as a presentation of a pelvic neuroblastoma in a 5-month-old infant. 61
23112240 2014
22
Occam's Razor or Hickam's dictum: a paraneoplastic or coincidental occurrence of lung cancer and Guillain-Barré syndrome. 61
25030575 2014
23
"Dancing eye syndrome" secondary to opsoclonus-myoclonus syndrome in small-cell lung cancer. 61
24778658 2014
24
Trigeminal neuralgia as the first clinical manifestation of anti-hu paraneoplastic syndrome induced by a borderline ovarian mucinous tumor. 61
24575026 2014
25
Neurologic complications of lung cancer. 61
24365305 2014
26
Diagnosis and management of paraneoplastic neurologic disorders. 61
23900965 2013
27
Hypopharyngeal paraganglioma with a paraneoplastic neurologic syndrome: a case report. 61
22987597 2013
28
Case report: Paraneoplastic neurologic syndrome associated with squamous cell carcinoma of the tonsil. 61
24170469 2013
29
[Paraneoplastic limbic encephalitis: 2 pediatric cases]. 61
23474034 2013
30
Antibodies against GM1 gangliosides associated with metastatic melanoma. 61
24001415 2013
31
Prognostic effect of early treatment of paraneoplastic limbic encephalitis in a patient with small-cell lung cancer. 61
23144583 2012
32
Breast cancer presents with a paraneoplastic neurologic syndrome. 61
23275775 2012
33
Paraneoplastic Neurologic Disorders: A Brief Overview. 61
23264806 2012
34
Neuronal Elav-like (Hu) proteins regulate RNA splicing and abundance to control glutamate levels and neuronal excitability. 61
22998874 2012
35
Cellular immune suppression in paraneoplastic neurologic syndromes targeting intracellular antigens. 61
22566506 2012
36
Neurological manifestation of colonic adenocarcinoma. 61
22826789 2012
37
A 61-year-old man presented with myopathy, neuropathy, and inflammatory dermatitis responsive to chronic lymphocytic leukemia treatment. 61
22924009 2012
38
Paraneoplastic neurologic syndrome: A practical approach. 61
22412264 2012
39
Utility of F-18 FDG PET/CT in screening for paraneoplastic neurological syndromes. 61
22157026 2012
40
An unusual case of altered mental status in a young woman. 61
22361499 2011
41
Anti-NMDA-Receptor Encephalitis and Other Synaptic Autoimmune Disorders. 61
21298406 2011
42
Paraneoplastic neurologic disorders in children. 61
21161703 2011
43
Harnessing naturally occurring tumor immunity: a clinical vaccine trial in prostate cancer. 61
20824184 2010
44
Update on paraneoplastic and autoimmune disorders of the central nervous system. 61
20577938 2010
45
Treatment of paraneoplastic neurologic disorders. 61
20842583 2010
46
Update on paraneoplastic neurologic disorders. 61
21572897 2010
47
Positron emission tomography-computed tomography in paraneoplastic neurologic disorders: systematic analysis and review. 61
20065123 2010
48
Paraneoplastic neurologic syndrome in the PNS Euronetwork database: a European study from 20 centers. 61
20212230 2010
49
Update on paraneoplastic neurologic disorders. 61
20479279 2010
50
Patients with lung cancer and paraneoplastic Hu syndrome harbor HuD-specific type 2 CD8+ T cells. 61
19509467 2009

Variations for Paraneoplastic Neurologic Disorders

Expression for Paraneoplastic Neurologic Disorders

Search GEO for disease gene expression data for Paraneoplastic Neurologic Disorders.

Pathways for Paraneoplastic Neurologic Disorders

GO Terms for Paraneoplastic Neurologic Disorders

Biological processes related to Paraneoplastic Neurologic Disorders according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 central nervous system development GO:0007417 8.62 ZIC4 ZIC1

Molecular functions related to Paraneoplastic Neurologic Disorders according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II regulatory region sequence-specific DNA binding GO:0000977 8.96 ZIC4 ZIC1
2 nucleic acid binding GO:0003676 8.8 ZIC4 ZIC1 ELAVL4

Sources for Paraneoplastic Neurologic Disorders

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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