Encephalitis lethargica

Title

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Other Names:

Von Economo’s disease; Von Economo encephalitis

Categories:

Nervous System Diseases

Summary 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]

Last updated: 6/7/2016

Symptoms 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.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Encephalopathy		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 antibody level		0010702
Increased CSF protein		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
Seizure		0001250
Stiff neck	Neck stiffness	0025258
Urinary incontinence	Loss of bladder control	0000020

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Last updated: 2/1/2021

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Learn More 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.

News & Events News & Events

News

Rare Disease Day at NIH 2021
March 1, 2021

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

When I was about 8-years-old, I was hospitalized due to encephalitis lethargica. I was extremely ill and it took me a long time to recover. Last May, I was diagnosed with a benign meningioma. Could my childhood illness be related to the development of my tumor? Are there any documented cases of meningioma in other survivors of encephalitis lethargica? See answer

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References References

NINDS Encephalitis Lethargica Information Page. National Institute of Neurological Disorders and Stroke Website. February 12, 2007; http://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.

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