PCD
MCID: PRN033
MIFTS: 31

Paraneoplastic Neurologic Disorders (PCD)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Paraneoplastic Neurologic Disorders

MalaCards integrated aliases for Paraneoplastic Neurologic Disorders:

Name: Paraneoplastic Neurologic Disorders 20
Paraneoplastic Cerebellar Degeneration 58
Paraneoplastic Neurologic Syndrome 58
Pcd 58
Pnd 20
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 71 C0393534 C0751911 C3267031
Orphanet 58 ORPHA36388

Summaries for Paraneoplastic Neurologic Disorders

GARD : 20 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 primary ciliary dyskinesia and paraneoplastic cerebellar degeneration. An important gene associated with Paraneoplastic Neurologic Disorders is AP3B2 (Adaptor Related Protein Complex 3 Subunit Beta 2). The drugs Immunoglobulins, Intravenous and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include eye, t cells and spinal cord, 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 347)
# Related Disease Score Top Affiliating Genes
1 primary ciliary dyskinesia 11.7
2 paraneoplastic cerebellar degeneration 11.4
3 subacute cerebellar degeneration 11.2
4 premature centromere division 11.2
5 ciliary dyskinesia, primary, 1 11.1
6 guillain-barre syndrome 11.1
7 hyperphenylalaninemia, bh4-deficient, d 11.1
8 kartagener syndrome 11.0
9 peripheral nervous system neoplasm 11.0
10 peripheral nervous system ganglioneuroblastoma 11.0
11 oculopharyngeal muscular dystrophy 10.9
12 mcleod syndrome 10.9
13 pathologic nystagmus 10.7
14 tremor 10.5
15 ovary adenocarcinoma 10.4
16 lymphoma 10.4
17 breast cancer 10.4
18 dysphagia 10.4
19 fallopian tube adenocarcinoma 10.3
20 small cell cancer of the lung 10.3
21 diffuse large b-cell lymphoma 10.3
22 gastric adenocarcinoma 10.3
23 wallerian degeneration 10.3
24 bladder cancer 10.3
25 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.3
26 limbic encephalitis 10.2
27 lung disease 10.2
28 bronchiectasis 10.2
29 autoimmune disease 10.2
30 endometrial cancer 10.2
31 neuroendocrine carcinoma 10.2
32 melanoma 10.2
33 fallopian tube carcinoma 10.2
34 otitis media 10.2
35 retinal degeneration 10.2
36 krabbe disease 10.2
37 postpartum depression 10.1
38 leukodystrophy 10.1
39 demyelinating disease 10.1
40 spinal cord injury 10.1
41 papillomatosis, confluent and reticulated 10.1
42 lung cancer 10.1
43 follicular lymphoma 10.1
44 ptosis 10.1
45 combined immunodeficiency 10.1
46 nephrotic syndrome 10.1
47 sensory peripheral neuropathy 10.1
48 cerebellar disease 10.1
49 papillary carcinoma 10.1
50 serous cystadenocarcinoma 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:

46
# 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 Immunoglobulins, Intravenous Phase 2
2 Immunologic Factors Phase 2
3 gamma-Globulins Phase 2
4 Rho(D) Immune Globulin Phase 2
5
gamma-Aminobutyric acid Approved, Investigational 56-12-2 119
6
Glutamic acid Approved, Nutraceutical 56-86-0 33032
7
Aspartic acid Approved, Nutraceutical 56-84-8 5960
8 Antibodies
9 Immunoglobulins
10 N-Methylaspartate

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 A Research of Value of Free Methylated DNA Detect in Lung Cancer on the Diagnosis and Treatment of Paraneoplastic Syndrome of Nervous System Completed NCT04454853
3 Killer T Cell Activity in the Paraneoplastic Neurologic Syndromes Completed NCT00327236
4 Prospective Collection of Biological Samples From Patients With Rare Neurological Diseases Recruiting NCT04698421
5 Cumulative Incidence of Autoimmune Encephalitides and Paraneoplastic Neurological Syndromes in Sweden Recruiting NCT04708626
6 Genomic Profile and Tumor Immune Attack in Paraneoplastic Neurological Syndromes (PNS) Active, not recruiting NCT03963700
7 Incidence of Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis in France Between 2016 and 2018 Not yet recruiting NCT03957616

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
Eye, T Cells, Spinal Cord, Colon, Prostate, Tonsil, Pineal

Publications for Paraneoplastic Neurologic Disorders

Articles related to Paraneoplastic Neurologic Disorders:

(show top 50) (show all 106)
# Title Authors PMID Year
1
Paraneoplastic Neurologic Syndrome in Ewing Sarcoma. 61
33811882 2021
2
Imaging Review of Paraneoplastic Neurologic Syndromes. 61
33093137 2020
3
Paraneoplastic Disorders of the Nervous System. 61
33273174 2020
4
Expanded Clinical Phenotype, Oncological Associations, and Immunopathologic Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis. 61
32744608 2020
5
An autoimmune-based, paraneoplastic neurologic syndrome following checkpoint inhibition and concurrent radiotherapy for merkel cell carcinoma: case report. 61
32006066 2020
6
Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies. 61
32170042 2020
7
Worsening of anti-Hu paraneoplastic neurological syndrome related to anti-PD-1 treatment: Case report and review of literature. 61
32058173 2020
8
Increased frequency of anti-Ma2 encephalitis associated with immune checkpoint inhibitors. 61
31454760 2019
9
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
10
Paraneoplastic neurologic syndrome as a presentation of underlying breast malignancy. 61
30240068 2018
11
Anti-Ma2-antibody-associated encephalitis: An atypical paraneoplastic neurologic syndrome. 61
31754496 2018
12
Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report. 61
28984777 2017
13
Acquired Hemophilia and Anti-Hu Paraneoplastic Neurologic Syndrome in Small Cell Lung Cancer. 61
29078851 2017
14
FDG-Avid Keloid on PET/CT Imaging. 61
28481801 2017
15
Thymoma-associated panencephalitis: a newly emerging paraneoplastic neurologic syndrome. 61
29479559 2017
16
Limbic encephalitis - a report of four cases. 61
28860940 2017
17
CTLA4 blockade elicits paraneoplastic neurological disease in a mouse model. 61
27604307 2016
18
A destructive feedback loop mediated by CXCL10 in central nervous system inflammatory disease. 61
26224283 2015
19
Paraneoplastic neurologic disorders in small cell lung carcinoma: A prospective study. 61
26109714 2015
20
Neuronal Na+/K+ ATPase is an autoantibody target in paraneoplastic neurologic syndrome. 61
25809299 2015
21
Neoplastic myelopathies. 61
25651222 2015
22
Paraneoplastic syndrome: a masquerade of autoimmune inner ear disease. 61
25427223 2015
23
Neural autoantibody clusters aid diagnosis of cancer. 61
24833664 2014
24
Autoimmune encephalitis update. 61
24637228 2014
25
"Dancing eye syndrome" secondary to opsoclonus-myoclonus syndrome in small-cell lung cancer. 61
24778658 2014
26
Trigeminal neuralgia as the first clinical manifestation of anti-hu paraneoplastic syndrome induced by a borderline ovarian mucinous tumor. 61
24575026 2014
27
Neurologic complications of lung cancer. 61
24365305 2014
28
Seizures as a presentation of a pelvic neuroblastoma in a 5-month-old infant. 61
23112240 2014
29
Occam's Razor or Hickam's dictum: a paraneoplastic or coincidental occurrence of lung cancer and Guillain-Barré syndrome. 61
25030575 2014
30
Diagnosis and management of paraneoplastic neurologic disorders. 61
23900965 2013
31
Hypopharyngeal paraganglioma with a paraneoplastic neurologic syndrome: a case report. 61
22987597 2013
32
Case report: Paraneoplastic neurologic syndrome associated with squamous cell carcinoma of the tonsil. 61
24170469 2013
33
[Paraneoplastic limbic encephalitis: 2 pediatric cases]. 61
23474034 2013
34
Antibodies against GM1 gangliosides associated with metastatic melanoma. 61
24001415 2013
35
Prognostic effect of early treatment of paraneoplastic limbic encephalitis in a patient with small-cell lung cancer. 61
23144583 2012
36
Breast cancer presents with a paraneoplastic neurologic syndrome. 61
23275775 2012
37
Paraneoplastic Neurologic Disorders: A Brief Overview. 61
23264806 2012
38
Cellular immune suppression in paraneoplastic neurologic syndromes targeting intracellular antigens. 61
22566506 2012
39
Neuronal Elav-like (Hu) proteins regulate RNA splicing and abundance to control glutamate levels and neuronal excitability. 61
22998874 2012
40
Neurological manifestation of colonic adenocarcinoma. 61
22826789 2012
41
Utility of F-18 FDG PET/CT in screening for paraneoplastic neurological syndromes. 61
22157026 2012
42
Paraneoplastic neurologic syndrome: A practical approach. 61
22412264 2012
43
A 61-year-old man presented with myopathy, neuropathy, and inflammatory dermatitis responsive to chronic lymphocytic leukemia treatment. 61
22924009 2012
44
An unusual case of altered mental status in a young woman. 61
22361499 2011
45
Anti-NMDA-Receptor Encephalitis and Other Synaptic Autoimmune Disorders. 61
21298406 2011
46
Paraneoplastic neurologic disorders in children. 61
21161703 2011
47
Harnessing naturally occurring tumor immunity: a clinical vaccine trial in prostate cancer. 61
20824184 2010
48
Update on paraneoplastic and autoimmune disorders of the central nervous system. 61
20577938 2010
49
Update on paraneoplastic neurologic disorders. 61
21572897 2010
50
Treatment of paraneoplastic neurologic disorders. 61
20842583 2010

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

Sources for Paraneoplastic Neurologic Disorders

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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