MCID: VNC001
MIFTS: 41

Von Economo's Disease

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Von Economo's Disease

MalaCards integrated aliases for Von Economo's Disease:

Name: Von Economo's Disease 12 20 53 15
Encephalitis Lethargica 12 73 20 53 58 70
Von Economo Encephalitis 20 58
Von Economo Disease 12

Characteristics:

Orphanet epidemiological data:

58
encephalitis lethargica
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


Summaries for Von Economo's Disease

GARD : 20 Encephalitis lethargica (EL) is a disease characterized by high fever, headache, double vision, delayed physical and mental response, extreme tiredness ( lethargy ), and sometimes coma. Patients may also experience abnormal eye movements, upper body weakness, muscle pain, tremors, neck rigidity, and behavioral changes including psychosis. A worldwide epidemic of EL occurred from 1917 to 1928 with more than one million reported cases. Although occasional cases are reported with similar symptoms, EL epidemics have not recurred. The cause of this condition is unknown, but a viral origin is suspected. Treatment depends on a person's symptoms. Levodopa and other antiparkinson drugs may be effective in alleviating some symptoms.

MalaCards based summary : Von Economo's Disease, also known as encephalitis lethargica, is related to akinetic mutism and mutism. An important gene associated with Von Economo's Disease is HCRT (Hypocretin Neuropeptide Precursor), and among its related pathways/superpathways is Neuroscience. Affiliated tissues include eye, brain and cortex, and related phenotypes are sleep disturbance and encephalopathy

NINDS : 53 Encephalitis lethargica is a disease characterized by high fever, headache, double vision, delayed physical and mental response, and lethargy. In acute cases, patients may enter coma. Patients may also experience abnormal eye movements, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis. The cause of encephalitis lethargica is unknown. Between 1917 to 1928, an epidemic of encephalitis lethargica spread throughout the world, but no recurrence of the epidemic has since been reported. Postencephalitic Parkinson's disease may develop after a bout of encephalitis-sometimes as long as a year after the illness.

Wikipedia : 73 Encephalitis lethargica is an atypical form of encephalitis. Also known as "sleeping sickness" or... more...

Related Diseases for Von Economo's Disease

Diseases related to Von Economo's Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 113)
# Related Disease Score Top Affiliating Genes
1 akinetic mutism 30.5 SNCA PNMA2
2 mutism 30.4 PNMA2 LGI1 CNTNAP2
3 encephalitis 30.3 PNMA2 LGI1 CNTNAP2
4 postencephalitic parkinson disease 30.1 SNCA LRRK2 IGLON5
5 movement disease 29.7 SNCA LRRK2 LGI1 IGLON5 DPYSL5 CNTNAP2
6 acute disseminated encephalomyelitis 29.7 PNMA2 LGI1 HCRT CNTNAP2
7 choreatic disease 29.2 SNCA PNMA2 LGI1 IGLON5 DPYSL5 CNTNAP2
8 sleep disorder 28.7 SNCA LRRK2 IGLON5 HCRTR2 HCRTR1 HCRT
9 sleeping sickness 11.4
10 viral encephalitis 10.5
11 kearns-sayre syndrome 10.3
12 influenza 10.3
13 parkinsonism 10.2
14 dissociative amnesia 10.2 LGI1 CNTNAP2
15 postinfectious encephalitis 10.2 LGI1 CNTNAP2
16 adjustment disorder 10.2 HCRT GAL
17 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
18 central nervous system disease 10.2
19 myoclonus 10.2
20 tremor 10.2
21 periodic limb movement disorder 10.2 HCRTR2 HCRT
22 parkinson disease 10 10.2 SNCA LRRK2
23 autoimmune encephalitis 10.1 LGI1 HCRT CNTNAP2
24 retrograde amnesia 10.1 LGI1 FOS
25 ocular motor apraxia 10.1
26 chorea, childhood-onset, with psychomotor retardation 10.1
27 oculogyric crisis 10.1
28 poliomyelitis 10.1
29 subacute delirium 10.1
30 perry syndrome 10.1 SNCA HCRT
31 chorea gravidarum 10.1 DPYSL5 CNTNAP2
32 autosomal dominant epilepsy with auditory features 10.1 LGI1 GAL CNTNAP2
33 cerebellopontine angle tumor 10.1 DPYSL5 CNTNAP2
34 rohhad 10.1 HCRTR2 HCRTR1 HCRT
35 parkinson disease 3, autosomal dominant 10.1 SNCA LRRK2
36 withdrawal disorder 10.1 HCRT FOS
37 autoimmune neuropathy 10.1 DPYSL5 CNTNAP2
38 parkinson disease 15, autosomal recessive early-onset 10.1 SNCA LRRK2
39 speech and communication disorders 10.1 SNCA LGI1 CNTNAP2
40 parkinson disease 1, autosomal dominant 10.1 SNCA LRRK2
41 morphine dependence 10.0 HCRTR1 HCRT FOS
42 fatal familial insomnia 10.0 SNCA HCRT
43 central hypoventilation syndrome, congenital 10.0 HCRT GAL FOS
44 status epilepticus 10.0 LGI1 GAL FOS
45 miller fisher syndrome 10.0 PNMA2 CNTNAP2
46 amnestic disorder 10.0 LGI1 FOS CNTNAP2
47 kufor-rakeb syndrome 10.0 SNCA LRRK2
48 cerebellopontine angle meningioma 10.0 LGI1 DPYSL5 CNTNAP2
49 color agnosia 10.0 SNCA LRRK2 CNTNAP2
50 dementia, lewy body 10.0 SNCA LRRK2 HCRT

Graphical network of the top 20 diseases related to Von Economo's Disease:



Diseases related to Von Economo's Disease

Symptoms & Phenotypes for Von Economo's Disease

Human phenotypes related to Von Economo's Disease:

58 31 (show all 33)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sleep disturbance 58 31 hallmark (90%) Very frequent (99-80%) HP:0002360
2 encephalopathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0001298
3 diplopia 58 31 frequent (33%) Frequent (79-30%) HP:0000651
4 tremor 58 31 frequent (33%) Frequent (79-30%) HP:0001337
5 dyskinesia 58 31 frequent (33%) Frequent (79-30%) HP:0100660
6 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
7 autoimmunity 58 31 frequent (33%) Frequent (79-30%) HP:0002960
8 myalgia 58 31 frequent (33%) Frequent (79-30%) HP:0003326
9 mental deterioration 58 31 frequent (33%) Frequent (79-30%) HP:0001268
10 headache 58 31 frequent (33%) Frequent (79-30%) HP:0002315
11 lethargy 58 31 frequent (33%) Frequent (79-30%) HP:0001254
12 upper limb muscle weakness 58 31 frequent (33%) Frequent (79-30%) HP:0003484
13 parkinsonism 58 31 frequent (33%) Frequent (79-30%) HP:0001300
14 increased csf protein 58 31 frequent (33%) Frequent (79-30%) HP:0002922
15 abnormal involuntary eye movements 58 31 frequent (33%) Frequent (79-30%) HP:0012547
16 bilateral basal ganglia lesions 58 31 frequent (33%) Frequent (79-30%) HP:0007146
17 pharyngitis 58 31 frequent (33%) Frequent (79-30%) HP:0025439
18 recurrent viral infections 31 frequent (33%) HP:0004429
19 increased circulating antibody level 31 frequent (33%) HP:0010702
20 bowel incontinence 58 31 occasional (7.5%) Occasional (29-5%) HP:0002607
21 photophobia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000613
22 psychosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000709
23 coma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001259
24 urinary incontinence 58 31 occasional (7.5%) Occasional (29-5%) HP:0000020
25 limb pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0009763
26 hyperventilation 58 31 occasional (7.5%) Occasional (29-5%) HP:0002883
27 bradycardia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001662
28 stiff neck 58 31 occasional (7.5%) Occasional (29-5%) HP:0025258
29 seizure 31 occasional (7.5%) HP:0001250
30 seizures 58 Occasional (29-5%)
31 increased antibody level in blood 58 Frequent (79-30%)
32 severe viral infections 58 Frequent (79-30%)
33 limitation of neck motion 58 Frequent (79-30%)

MGI Mouse Phenotypes related to Von Economo's Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.7 CNTNAP2 DPYSL5 FOS GAL HAPLN1 HCRT
2 nervous system MP:0003631 9.4 CNTNAP2 DPP6 DPYSL5 FOS GAL HAPLN1

Drugs & Therapeutics for Von Economo's Disease

Search Clinical Trials , NIH Clinical Center for Von Economo's Disease

Genetic Tests for Von Economo's Disease

Anatomical Context for Von Economo's Disease

MalaCards organs/tissues related to Von Economo's Disease:

40
Eye, Brain, Cortex, Hypothalamus, Pituitary

Publications for Von Economo's Disease

Articles related to Von Economo's Disease:

(show top 50) (show all 292)
# Title Authors PMID Year
1
Medical and molecular perspectives into a forgotten epidemic: encephalitis lethargica, viruses, and high-throughput sequencing. 20 61
25129855 2014
2
Tics in patients with encephalitis. 61
33486621 2021
3
A role for pathogen risk factors and autoimmunity in encephalitis lethargica? 61
33549696 2021
4
Historical Insight into Infections and Disorders Associated with Neurological and Psychiatric Sequelae Similar to Long COVID. 61
33633106 2021
5
The first psychiatric pandemic: Encephalitis lethargica, 1917-27. 61
33268001 2021
6
Encephalitis lethargica in Peru. 61
33320774 2020
7
Encephalitis lethargica epidemic milestones in early sleep neurobiology researches. 61
32957006 2020
8
From encephalitis lethargica to COVID-19: Is there another epidemic ahead? 61
32659638 2020
9
COVID-19: can we learn from encephalitis lethargica? 61
32562680 2020
10
Centenary of Tretiakoff's thesis on the morphology of Parkinson's disease, evolved on the grounds of encephalitis lethargica pathology. 61
31226240 2019
11
Encephalitis lethargica: a dying fall. 61
32445463 2019
12
La Von Economo‚Äôs encephalitis lethargica and the Spanish flu pandemic in Bogotá and Medellín (Colombia): An historic review one hundred years after 61
31021542 2019
13
[Encephalitis lethargica. The epidemic at the dawn of neurology]. 61
30638258 2019
14
Infectious Etiologies of Parkinsonism: Pathomechanisms and Clinical Implications. 61
31275235 2019
15
Historical and conceptual aspects of motor disorders in the psychoses. 61
28941779 2018
16
The centennial lesson of encephalitis lethargica. 61
29555881 2018
17
Von Economo's disease and postencephalitic parkinsonism responsive to carbidopa and levodopa. 61
29670352 2018
18
Virus-like particles and enterovirus antigen found in the brainstem neurons of Parkinson's disease. 61
29899977 2018
19
Encephalitis lethargica: 100 years after the epidemic. 61
28899018 2017
20
Olfactory vector hypothesis for encephalitis lethargica. 61
28571798 2017
21
The terminology of akinesia, bradykinesia and hypokinesia: Past, present and future. 61
28153525 2017
22
[NMDA-GluR Subunit Antibody-Positive Encephalitis: A Clinical Analysis of Five Cases]. 61
27667494 2016
23
Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency. 61
26431898 2015
24
'A disease that makes criminals': encephalitis lethargica (EL) in children, mental deficiency, and the 1927 Mental Deficiency Act. 61
25683195 2015
25
Tropism and Induction of Cytokines in Human Embryonic-Stem Cells-Derived Neural Progenitors upon Inoculation with Highly- Pathogenic Avian H5N1 Influenza Virus. 61
26274828 2015
26
Rating Parkinson's Disease: A Contemporary Eye to Archival Patients. 61
30713854 2014
27
Postencephalitic parkinsonism: interesting clinico-imaging correlation. 61
24993469 2014
28
Encephalitis lethargica (von Economo's encephalitis). 61
25015515 2014
29
Brain and aviation: on the 80th anniversary of Constantin von Economo's (1876-1931) death. 61
22569570 2013
30
The Demise of Poskanzer and Schwab's Influenza Theory on the Pathogenesis of Parkinson's Disease. 61
23853734 2013
31
Immune-mediated extrapyramidal movement disorders, including Sydenham chorea. 61
23622334 2013
32
Substantia nigra depigmentation and exposure to encephalitis lethargica. 61
23280841 2012
33
Autoimmune basal ganglia disorders. 61
22832771 2012
34
From encephalitis lethargica to cerebral cytoarchitectonics: the polymath talent of Constantin von Economo (1876-1931), pioneer neuroanatomist, neurophysiologist and military aviator. 61
23138582 2012
35
Evidence for an enterovirus as the cause of encephalitis lethargica. 61
22715890 2012
36
Encephalitis lethargica due to Epstein-Barr virus infection. 61
21611982 2011
37
Parkinson's disease or Parkinson symptoms following seasonal influenza. 61
21668692 2011
38
The diagnosis of postencephalitic parkinsonism at the neurological unit of Boston City Hospital, 1930-1981. 61
21327402 2011
39
On doing nothing: descriptions of sleep, fatigue, and motivation in encephalitis lethargica. 61
21312282 2011
40
Encephalitis lethargica with quick response to immunoglobulin. 61
21079460 2010
41
Anti-NMDA receptor encephalitis with atypical brain changes on MRI. 61
20837307 2010
42
A modern perspective on the differential diagnosis between encephalitis lethargica or anti-NMDA-receptor encephalitis. 61
20605463 2010
43
Does the historical literature on encephalitis lethargica support a simple (direct) relationship with postencephalitic Parkinsonism? 61
20629127 2010
44
A historical analysis of the relationship between encephalitis lethargica and postencephalitic parkinsonism: a complex rather than a direct relationship. 61
20629120 2010
45
Severe childhood encephalopathy with dyskinesia and prolonged cognitive disturbances: evidence for anti-N-methyl-D-aspartate receptor encephalitis. 61
20041934 2010
46
Influenza caused epidemic encephalitis (encephalitis lethargica): the circumstantial evidence and a challenge to the nonbelievers. 61
20060230 2010
47
[From Bulgaria to Italy: the "Bulgarian cure" of encephalitis lethargica]. 61
21563487 2010
48
N-methyl-D-aspartate receptor antibodies in pediatric dyskinetic encephalitis lethargica. 61
19938173 2009
49
Encephalitis lethargica and the influenza virus. III. The influenza pandemic of 1918/19 and encephalitis lethargica: neuropathology and discussion. 61
19707847 2009
50
Review: neuropathology of acute phase encephalitis lethargica: a review of cases from the epidemic period. 61
19490429 2009

Variations for Von Economo's Disease

Expression for Von Economo's Disease

Search GEO for disease gene expression data for Von Economo's Disease.

Pathways for Von Economo's Disease

Pathways related to Von Economo's Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.46 SNCA LRRK2 HCRT CNTNAP2

GO Terms for Von Economo's Disease

Cellular components related to Von Economo's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.72 SNCA LRRK2 LGI1 HCRT CNTNAP2
2 neuronal cell body GO:0043025 9.35 SNCA LRRK2 GAL DPYSL5 CNTNAP2
3 synaptic vesicle GO:0008021 9.33 SNCA LRRK2 HCRT
4 inclusion body GO:0016234 9.26 SNCA LRRK2
5 synapse GO:0045202 9.17 SNCA LRRK2 LGI1 HCRTR2 HCRTR1 HCRT

Biological processes related to Von Economo's Disease according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 chemical synaptic transmission GO:0007268 9.56 SNCA HCRTR2 HCRTR1 HCRT
2 response to immobilization stress GO:0035902 9.49 GAL FOS
3 regulation of neuron death GO:1901214 9.48 SNCA LRRK2
4 regulation of neurotransmitter secretion GO:0046928 9.46 SNCA HCRT
5 positive regulation of synaptic transmission GO:0050806 9.43 SNCA LGI1
6 striatum development GO:0021756 9.4 LRRK2 CNTNAP2
7 sleep GO:0030431 9.37 HCRT FOS
8 excitatory postsynaptic potential GO:0060079 9.33 SNCA LRRK2 HCRT
9 regulation of locomotion GO:0040012 9.32 SNCA LRRK2
10 neuropeptide signaling pathway GO:0007218 9.26 HCRTR2 HCRTR1 HCRT GAL
11 feeding behavior GO:0007631 8.92 HCRTR2 HCRTR1 HCRT GAL

Molecular functions related to Von Economo's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuropeptide hormone activity GO:0005184 8.96 HCRT GAL
2 orexin receptor activity GO:0016499 8.62 HCRTR2 HCRTR1

Sources for Von Economo's Disease

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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