MCID: VNC001
MIFTS: 42

Von Economo's Disease

Categories: Eye diseases, Mental diseases, 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 55 15
Encephalitis Lethargica 12 77 54 55 60 74
Von Economo Encephalitis 60
Von Economo Disease 12

Characteristics:

Orphanet epidemiological data:

60
encephalitis lethargica
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 60  
Rare neurological diseases


Summaries for Von Economo's Disease

NIH Rare Diseases : 54 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 amyotrophic lateral sclerosis 1 and sleeping sickness. An important gene associated with Von Economo's Disease is GSR (Glutathione-Disulfide Reductase), and among its related pathways/superpathways is Neuroscience. Affiliated tissues include eye, brain and t cells, and related phenotypes are sleep disturbance and encephalopathy

NINDS : 55 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 : 77 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 all 38)
# Related Disease Score Top Affiliating Genes
1 amyotrophic lateral sclerosis 1 29.3 GFAP GSR SNCA
2 sleeping sickness 11.6
3 influenza 10.7
4 viral encephalitis 10.5
5 poliomyelitis 10.2
6 limbic encephalitis 10.2 GRIN2B GSR
7 valproate embryopathy 10.2 GRIN2B GSR
8 postencephalitic parkinson disease 10.2
9 encephalitis 10.2
10 fetal alcohol syndrome 10.1 GFAP GRIN2B
11 autoimmune disease of peripheral nervous system 10.1 DDX41 HCRT
12 autoimmune disease 10.1
13 multiple sclerosis 10.1
14 schizophrenia 10.1
15 autoimmune disease 1 10.1
16 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
17 lateral sclerosis 10.1
18 relapsing polychondritis 10.1
19 movement disease 10.1
20 dystonia 10.1
21 herpes simplex 10.1
22 meningitis 10.1
23 tuberculous meningitis 10.1
24 myoclonus 10.1
25 eumycotic mycetoma 10.1 ACTB DDX41
26 neuromyelitis optica 10.1 GFAP HCRT
27 rem sleep behavior disorder 10.0 HCRT SNCA
28 childhood oligodendroglioma 10.0 GFAP SNCA
29 retinitis pigmentosa 42 10.0 DDX41 GFAP
30 perry syndrome 9.9 HCRT SNCA
31 status epilepticus 9.9 GFAP GRIN2B
32 multiple system atrophy 1 9.9 GFAP SNCA
33 disease of mental health 9.9 GRIN2B HCRT SNCA
34 leukoencephalopathy, hereditary diffuse, with spheroids 9.8 GFAP SNCA
35 toxic encephalopathy 9.8 GFAP GRIN2B SNCA
36 nervous system disease 9.8 GFAP GRIN2B SNCA
37 central nervous system disease 9.8 GFAP HCRT SNCA
38 syphilis 9.8 B2M DDX41

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:

60 33 (show all 31)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sleep disturbance 60 33 hallmark (90%) Very frequent (99-80%) HP:0002360
2 encephalopathy 60 33 hallmark (90%) Very frequent (99-80%) HP:0001298
3 diplopia 60 33 frequent (33%) Frequent (79-30%) HP:0000651
4 tremor 60 33 frequent (33%) Frequent (79-30%) HP:0001337
5 dyskinesia 60 33 frequent (33%) Frequent (79-30%) HP:0100660
6 fever 60 33 frequent (33%) Frequent (79-30%) HP:0001945
7 autoimmunity 60 33 frequent (33%) Frequent (79-30%) HP:0002960
8 myalgia 60 33 frequent (33%) Frequent (79-30%) HP:0003326
9 mental deterioration 60 33 frequent (33%) Frequent (79-30%) HP:0001268
10 headache 60 33 frequent (33%) Frequent (79-30%) HP:0002315
11 lethargy 60 33 frequent (33%) Frequent (79-30%) HP:0001254
12 upper limb muscle weakness 60 33 frequent (33%) Frequent (79-30%) HP:0003484
13 increased antibody level in blood 60 33 frequent (33%) Frequent (79-30%) HP:0010702
14 parkinsonism 60 33 frequent (33%) Frequent (79-30%) HP:0001300
15 increased csf protein 60 33 frequent (33%) Frequent (79-30%) HP:0002922
16 pharyngitis 60 33 frequent (33%) Frequent (79-30%) HP:0025439
17 abnormal involuntary eye movements 60 33 frequent (33%) Frequent (79-30%) HP:0012547
18 bilateral basal ganglia lesions 60 33 frequent (33%) Frequent (79-30%) HP:0007146
19 recurrent viral infections 33 frequent (33%) HP:0004429
20 seizures 60 33 occasional (7.5%) Occasional (29-5%) HP:0001250
21 bowel incontinence 60 33 occasional (7.5%) Occasional (29-5%) HP:0002607
22 photophobia 60 33 occasional (7.5%) Occasional (29-5%) HP:0000613
23 psychosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000709
24 coma 60 33 occasional (7.5%) Occasional (29-5%) HP:0001259
25 urinary incontinence 60 33 occasional (7.5%) Occasional (29-5%) HP:0000020
26 stiff neck 60 33 occasional (7.5%) Occasional (29-5%) HP:0025258
27 hyperventilation 60 33 occasional (7.5%) Occasional (29-5%) HP:0002883
28 bradycardia 60 33 occasional (7.5%) Occasional (29-5%) HP:0001662
29 limb pain 60 33 occasional (7.5%) Occasional (29-5%) HP:0009763
30 severe viral infections 60 Frequent (79-30%)
31 limitation of neck motion 60 Frequent (79-30%)

MGI Mouse Phenotypes related to Von Economo's Disease:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.1 ACTB B2M GFAP GSR HCRT SNCA

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:

42
Eye, Brain, T Cells, Cortex

Publications for Von Economo's Disease

Articles related to Von Economo's Disease:

(show top 50) (show all 190)
# Title Authors Year
1
The centennial lesson of encephalitis lethargica. ( 29555881 )
2018
2
Von Economo's disease and postencephalitic parkinsonism responsive to carbidopa and levodopa. ( 29670352 )
2018
3
Encephalitis lethargica: 100 years after the epidemic. ( 28899018 )
2017
4
Olfactory vector hypothesis for encephalitis lethargica. ( 28571798 )
2017
5
Encephalitis Lethargica With Isolated Substantia Nigra Lesions Followed by a Second Encephalitis in a Child With Humoral Immunodeficiency. ( 26431898 )
2015
6
'A disease that makes criminals': encephalitis lethargica (EL) in children, mental deficiency, and the 1927 Mental Deficiency Act. ( 25683195 )
2015
7
Encephalitis lethargica (von Economo's encephalitis). ( 25015515 )
2014
8
Medical and molecular perspectives into a forgotten epidemic: encephalitis lethargica, viruses, and high-throughput sequencing. ( 25129855 )
2014
9
Evidence for an enterovirus as the cause of encephalitis lethargica. ( 22715890 )
2012
10
From encephalitis lethargica to cerebral cytoarchitectonics: the polymath talent of Constantin von Economo (1876-1931), pioneer neuroanatomist, neurophysiologist and military aviator. ( 23138582 )
2012
11
Substantia nigra depigmentation and exposure to encephalitis lethargica. ( 23280841 )
2012
12
On doing nothing: descriptions of sleep, fatigue, and motivation in encephalitis lethargica. ( 21312282 )
2011
13
Encephalitis lethargica due to Epstein-Barr virus infection. ( 21611982 )
2011
14
A historical analysis of the relationship between encephalitis lethargica and postencephalitic parkinsonism: a complex rather than a direct relationship. ( 20629120 )
2010
15
Does the historical literature on encephalitis lethargica support a simple (direct) relationship with postencephalitic Parkinsonism? ( 20629127 )
2010
16
Influenza caused epidemic encephalitis (encephalitis lethargica): the circumstantial evidence and a challenge to the nonbelievers. ( 20060230 )
2010
17
A modern perspective on the differential diagnosis between encephalitis lethargica or anti-NMDA-receptor encephalitis. ( 20605463 )
2010
18
Encephalitis lethargica with quick response to immunoglobulin. ( 21079460 )
2010
19
Encephalitis lethargica in 5 South African children. ( 18424201 )
2009
20
Encephalitis lethargica and influenza. I. The role of the influenza virus in the influenza pandemic of 1918/1919. ( 19082525 )
2009
21
Was encephalitis lethargica a post-influenzal or some other phenomenon? Time to re-examine the problem. ( 19144247 )
2009
22
Contemporary Encephalitis Lethargica: phenotype, laboratory findings and treatment outcomes. ( 19412724 )
2009
23
Review: neuropathology of acute phase encephalitis lethargica: a review of cases from the epidemic period. ( 19490429 )
2009
24
Encephalitis lethargica and the influenza virus. III. The influenza pandemic of 1918/19 and encephalitis lethargica: neuropathology and discussion. ( 19707847 )
2009
25
Encephalitis lethargica and the influenza virus. II. The influenza pandemic of 1918/19 and encephalitis lethargica: epidemiology and symptoms. ( 19707848 )
2009
26
N-methyl-D-aspartate receptor antibodies in pediatric dyskinetic encephalitis lethargica. ( 19938173 )
2009
27
A case of encephalitis lethargica associated with relapsing polychondritis. ( 18855930 )
2008
28
From juvenile parkinsonism to encephalitis lethargica, a new phenotype of post-streptococcal disorders: case report. ( 18221898 )
2008
29
The relationship between encephalitis lethargica and influenza: a critical analysis. ( 18569452 )
2008
30
Encephalitis lethargica in the Soviet Union. ( 18628628 )
2008
31
Sporadic encephalitis lethargica. ( 16647855 )
2007
32
Encephalitis lethargica following Bartonella henselae infection. ( 17351727 )
2007
33
Steroid-responsive encephalitis lethargica syndrome with malignant catatonia. ( 17379999 )
2007
34
Children and encephalitis lethargica: a historical review. ( 17675021 )
2007
35
Contemporary encephalitis lethargica presenting with agitated catatonia, stereotypy, and dystonia-parkinsonism. ( 17914719 )
2007
36
Movement disorders associated with encephalitis lethargica: a video compilation. ( 16200538 )
2006
37
Acute reversible parkinsonism in Epstein-Barr virus-related encephalitis lethargica-like illness. ( 16250020 )
2006
38
The percipient observations of Constantin von Economo on encephalitis lethargica and sleep disruption and their lasting impact on contemporary sleep research. ( 16564419 )
2006
39
Encephalitis lethargica like illness: case report and literature review. ( 17100395 )
2006
40
Cognitive recovery from Encephalitis Lethargica. ( 16286345 )
2005
41
Encephalitis lethargica: part of a spectrum of post-streptococcal autoimmune diseases? ( 14679031 )
2004
42
Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. ( 14570817 )
2004
43
The two faces of L-DOPA: benefits and adverse side effects in the treatment of Encephalitis lethargica, Parkinson's disease, multiple sclerosis and amyotrophic lateral sclerosis. ( 14962622 )
2004
44
Lack of detection of influenza genes in archived formalin-fixed, paraffin wax-embedded brain samples of encephalitis lethargica patients from 1916 to 1920. ( 12695912 )
2003
45
Vaccines to treat encephalitis lethargica: human experiments at the Neurological Institute of New York, 1929-1940. ( 12223039 )
2002
46
A contemporary case of encephalitis lethargica. ( 11220691 )
2001
47
Experimenting on the past: the enigma of von Economo's encephalitis lethargica. ( 11444794 )
2001
48
Influenza RNA not detected in archival brain tissues from acute encephalitis lethargica cases or in postencephalitic Parkinson cases. ( 11444798 )
2001
49
Bilateral substantia nigra lesions on magnetic resonance imaging in a patient with encephalitis lethargica. ( 11459912 )
2001
50
Influenza RNA not detected in archival brain tissues from acute encephalitis lethargica cases or in postencephalitic Parkinson cases. ( 11706941 )
2001

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 GFAP GRIN2B HCRT SNCA

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 cytosol GO:0005829 9.5 ACOT8 ACTB B2M DDX41 GFAP GSR
2 rough endoplasmic reticulum GO:0005791 8.62 HCRT SNCA

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

# Name GO ID Score Top Affiliating Genes
1 chemical synaptic transmission GO:0007268 9.58 GRIN2B HCRT SNCA
2 ephrin receptor signaling pathway GO:0048013 9.48 ACTB GRIN2B
3 negative regulation of neuron projection development GO:0010977 9.46 B2M GFAP
4 retina homeostasis GO:0001895 9.43 ACTB B2M
5 excitatory postsynaptic potential GO:0060079 9.43 GRIN2B HCRT SNCA
6 positive regulation of neuron death GO:1901216 9.37 GRIN2B SNCA
7 regulation of neurotransmitter secretion GO:0046928 9.32 HCRT SNCA
8 regulation of transmembrane transporter activity GO:0022898 9.16 ACTB SNCA
9 long-term synaptic potentiation GO:0060291 9.13 GFAP GRIN2B SNCA
10 regulation of norepinephrine uptake GO:0051621 8.62 ACTB SNCA

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 ACTB B2M GFAP SNCA
2 kinesin binding GO:0019894 8.62 ACTB SNCA

Sources for Von Economo's Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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