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Disease Profile
Encephalitis lethargica
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
Adult
ICD-10
A85.8
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Von Economo’s disease; Von Economo encephalitis
Categories
Nervous System Diseases
Summary
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.[1][2] Although occasional cases are reported with similar symptoms, EL epidemics have not recurred.[1] The cause of this condition is unknown, but a viral origin is suspected.[2] Treatment depends on a person's symptoms. Levodopa and other antiparkinson drugs may be effective in alleviating some symptoms.[1]
Symptoms
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
0001298 | ||
Sleep disturbance |
Difficulty sleeping
Trouble sleeping
[ more ] |
0002360 |
30%-79% of people have these symptoms | ||
Abnormal involuntary eye movements | 0012547 | |
Autoimmunity |
Autoimmune disease
Autoimmune disorder
[ more ] |
0002960 |
Bilateral basal ganglia lesions | 0007146 | |
Diplopia |
Double vision
|
0000651 |
Dyskinesia |
Disorder of involuntary muscle movements
|
0100660 |
Fever | 0001945 | |
Headache |
Headaches
|
0002315 |
Increased circulating |
0010702 | |
Increased CSF |
0002922 | |
Lethargy | 0001254 | |
Mental deterioration |
Cognitive decline
Cognitive decline, progressive
Intellectual deterioration
Progressive cognitive decline
[ more ] |
0001268 |
Myalgia |
Muscle ache
Muscle pain
[ more ] |
0003326 |
Parkinsonism | 0001300 | |
Pharyngitis | 0025439 | |
Recurrent viral infections | 0004429 | |
Tremor | 0001337 | |
Upper limb muscle weakness |
Decreased arm strength
Weak arm
[ more ] |
0003484 |
5%-29% of people have these symptoms | ||
Bowel incontinence |
Loss of bowel control
|
0002607 |
Bradycardia |
Slow heartbeats
|
0001662 |
Coma | 0001259 | |
Hyperventilation |
Rapid breathing
|
0002883 |
Limb pain | 0009763 | |
Photophobia |
Extreme sensitivity of the eyes to light
Light hypersensitivity
[ more ] |
0000613 |
Psychosis | 0000709 | |
0001250 | ||
Stiff neck |
Neck stiffness
|
0025258 |
Urinary incontinence |
Loss of bladder control
|
0000020 |
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
In-Depth Information
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Encephalitis lethargica. Click on the link to view a sample search on this topic.
References
- NINDS Encephalitis Lethargica Information Page. National Institute of Neurological Disorders and Stroke Website. February 12, 2007; https://www.ninds.nih.gov/disorders/encephalitis_lethargica/encephalitis_lethargica.htm. Accessed 6/7/2016.
- Dennis Tappea, David E. Alquezar-Planas. Medical and molecular perspectives into a forgotten epidemic: Encephalitis lethargica, viruses, and high-throughput sequencing. Journal of Clinical Virology. October 2014; 61(2):189-195. https://www.ncbi.nlm.nih.gov/pubmed/25129855.