MCID: VNC001
MIFTS: 35

Von Economo's Disease

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Von Economo's Disease

MalaCards integrated aliases for Von Economo's Disease:

Name: Von Economo's Disease 12 54 15
Encephalitis Lethargica 12 76 53 54 59 73
Von Economo Encephalitis 59
Von Economo’s Disease 53
Von Economo Disease 12

Characteristics:

Orphanet epidemiological data:

59
encephalitis lethargica
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


Summaries for Von Economo's Disease

NIH Rare Diseases : 53 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 sleeping sickness and limbic encephalitis. 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, and related phenotypes are encephalopathy and sleep disturbance

NINDS : 54 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 : 76 Encephalitis lethargica is an atypical form of encephalitis. Also known as \"sleeping sickness\" or... more...

Related Diseases for Von Economo's Disease

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:

59 32 (show all 30)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 encephalopathy 59 32 hallmark (90%) Very frequent (99-80%) HP:0001298
2 sleep disturbance 59 32 hallmark (90%) Very frequent (99-80%) HP:0002360
3 diplopia 59 32 frequent (33%) Frequent (79-30%) HP:0000651
4 lethargy 59 32 frequent (33%) Frequent (79-30%) HP:0001254
5 mental deterioration 59 32 frequent (33%) Frequent (79-30%) HP:0001268
6 parkinsonism 59 32 frequent (33%) Frequent (79-30%) HP:0001300
7 tremor 59 32 frequent (33%) Frequent (79-30%) HP:0001337
8 fever 59 32 frequent (33%) Frequent (79-30%) HP:0001945
9 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
10 increased csf protein 59 32 frequent (33%) Frequent (79-30%) HP:0002922
11 autoimmunity 59 32 frequent (33%) Frequent (79-30%) HP:0002960
12 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
13 upper limb muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0003484
14 severe viral infections 59 32 frequent (33%) Frequent (79-30%) HP:0005364
15 bilateral basal ganglia lesions 59 32 frequent (33%) Frequent (79-30%) HP:0007146
16 increased antibody level in blood 59 32 frequent (33%) Frequent (79-30%) HP:0010702
17 abnormal involuntary eye movements 59 32 frequent (33%) Frequent (79-30%) HP:0012547
18 pharyngitis 59 32 frequent (33%) Frequent (79-30%) HP:0025439
19 dyskinesia 59 32 frequent (33%) Frequent (79-30%) HP:0100660
20 urinary incontinence 59 32 occasional (7.5%) Occasional (29-5%) HP:0000020
21 photophobia 59 32 occasional (7.5%) Occasional (29-5%) HP:0000613
22 psychosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000709
23 seizures 59 32 occasional (7.5%) Occasional (29-5%) HP:0001250
24 coma 59 32 occasional (7.5%) Occasional (29-5%) HP:0001259
25 bradycardia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001662
26 bowel incontinence 59 32 occasional (7.5%) Occasional (29-5%) HP:0002607
27 hyperventilation 59 32 occasional (7.5%) Occasional (29-5%) HP:0002883
28 limb pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0009763
29 stiff neck 59 32 occasional (7.5%) Occasional (29-5%) HP:0025258
30 limitation of neck motion 59 Frequent (79-30%)

MGI Mouse Phenotypes related to Von Economo's Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.17 GFAP GRIN2B GSR HCRT SNCA ACTB

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:

41
Eye

Publications for Von Economo's Disease

Articles related to Von Economo's Disease:

# Title Authors Year
1
Von Economo's disease and postencephalitic parkinsonism responsive to carbidopa and levodopa. ( 29670352 )
2018
2
A case of encephalitis lethargica associated with relapsing polychondritis. ( 18855930 )
2008
3
Atypical affective disorder with episodic dyscontrol: a case of von Economo's disease (encephalitis lethargica). ( 1348657 )
1992

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 rough endoplasmic reticulum GO:0005791 8.96 HCRT SNCA
2 postsynapse GO:0098794 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 9.54 GRIN2B HCRT SNCA
2 negative regulation of neuron projection development GO:0010977 9.4 B2M GFAP
3 retina homeostasis GO:0001895 9.32 ACTB B2M
4 positive regulation of neuron death GO:1901216 9.26 GRIN2B SNCA
5 regulation of neurotransmitter secretion GO:0046928 9.16 HCRT SNCA
6 excitatory postsynaptic potential GO:0060079 9.13 GRIN2B HCRT SNCA
7 long-term synaptic potentiation GO:0060291 8.8 GFAP GRIN2B 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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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