Encephalitis lethargica
Updates to Synonym Attributes
Updates to Article Attributes
Encephalitis lethargica (von Economo encephalitis) is was a mysteriousrare and devastating early 20th century epidemicmisterious deaseas first decriebed by von Economo in 1916 during an epydemy affecting 500,000 people worldwide in 1916-1927. There have been no further epidemics of EL since the 1920s, although sporadic cases have continued to be reported.
Exact etiology is unknown.
This encephalitis affect the midbrain and basal ganglia, with lymphocyte iniltration.
Clinical presentation
Clinic is dominated by pharyngitis followed by sleep disorder, lethargy, sleep cycle disturbances, extrapyramidal symptomatologymovements (parkinsonism and dyskinesias), neuropsychiatric manifestationsdisturbance (obsessive, compulsive disorder, catatonia, mutism, apathy and conduct disorders) and ocular featuresmovement disorder (oculogyric crises).
Pathology
Exact etiology is unknown.
Lymphocyte iniltration of the midbrain and basal ganglia.
Auto-antibodies reactive against human basal ganglia antigens present in the majority of EL patients
Radiographic features
MRI
MRI of the brain show inflammatory changes localized to the deep grey matterin 40% of patients.
- T2
The main imaging feature is increase in T2 signal (best seen on FLAIR) in variable location such as basal ganglia (including the substantia nigra), midbrain, thalamus, cerebral peduncle and temporal cortex.
- T1: low signal
- T1 C+ (Gd) : leptomeningeal and focal patchy enhancement
- DWI/ADC : iso intense without restriction of ADC.
- PET :Increased glucose metabolism in the basal ganglia
Treatment and prognosis
Treatment approaches to encephalitis lethargica include immunomodulating therapies and steroid.
Differential diagnosis
General imaging differential considerations include
- limbic encephalitis
Herpes simplex encephalitis
- Leptospirosis
-<p>Encephalitis lethargica is was a mysterious and devastating early 20th century epidemic.</p><p> </p><p>Clinic is dominated by pharyngitis followed by lethargy, sleep cycle disturbances, extrapyramidal symptomatology, neuropsychiatric manifestations and ocular features.</p><p>MRI of the brain show inflammatory changes localized to the deep grey matter<br>in 40% of patients.</p><p> </p>- +<p>Encephalitis lethargica (von Economo encephalitis) is a rare and misterious deaseas first decriebed by von Economo in 1916 during an epydemy affecting 500,000 people worldwide in 1916-1927. There have been no further epidemics of EL since the 1920s, although sporadic cases have continued to be reported.</p><p>Exact etiology is unknown.</p><p>This encephalitis affect the midbrain and basal ganglia, with lymphocyte iniltration.</p><p> </p><h4>Clinical presentation</h4><p>Clinic is dominated by pharyngitis followed by sleep disorder, lethargy, extrapyramidal movements (parkinsonism and dyskinesias), neuropsychiatric<br>disturbance (obsessive, compulsive disorder, catatonia, mutism, apathy and conduct disorders) and ocular movement disorder (oculogyric crises).</p><p> </p><h4>Pathology</h4><p>Exact etiology is unknown.</p><p>Lymphocyte iniltration of the midbrain and basal ganglia.</p><p>Auto-antibodies reactive against human basal ganglia antigens present in the majority of EL patients</p><h4>Radiographic features</h4><h5>MRI</h5><p>MRI of the brain show inflammatory changes localized to the deep grey matter<br>in 40% of patients.</p><ul><li><strong>T2</strong></li></ul><p>The main imaging feature is increase in T2 signal (best seen on FLAIR) in variable location such as basal ganglia (including the substantia nigra), midbrain, thalamus, cerebral peduncle and temporal cortex.</p><p> </p><ul><li>
- +<strong>T1:</strong> low signal</li></ul><p> </p><ul><li>
- +<strong>T1 C+ (Gd) : </strong>leptomeningeal and focal patchy enhancement </li></ul><p> </p><ul><li>
- +<strong>DWI/ADC : </strong>iso intense without restriction of ADC.</li></ul><p> </p><ul><li>
- +<strong>PET</strong> :Increased glucose metabolism in the basal ganglia</li></ul><h4>Treatment and prognosis</h4><p>Treatment approaches to encephalitis lethargica include immunomodulating therapies and steroid.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include</p><ul>
- +<li><a href="/articles/limbic-encephalitis">limbic encephalitis </a></li>
- +<li><p>Herpes simplex encephalitis</p></li>
- +<li><a href="/articles/leptospirosis">Leptospirosis</a></li>
- +</ul>
References changed:
- 1. Dale R, Church A, Surtees R et al. Encephalitis Lethargica Syndrome: 20 New Cases and Evidence of Basal Ganglia Autoimmunity. Brain. 2004;127(Pt 1):21-33. <a href="https://doi.org/10.1093/brain/awh008">doi:10.1093/brain/awh008</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/14570817">Pubmed</a>
- 2. Morris S. Dickman, MD von Economo Encephalitis Arch Neurol. 2001; 58; 1696-1698
- 3. Lopez-Alberola R, Georgiou M, Sfakianakis G, Singer C, Papapetropoulos S. Contemporary Encephalitis Lethargica: Phenotype, Laboratory Findings and Treatment Outcomes. J Neurol. 2009;256(3):396-404. <a href="https://doi.org/10.1007/s00415-009-0074-4">doi:10.1007/s00415-009-0074-4</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19412724">Pubmed</a>
Systems changed:
- Central Nervous System
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