Encephalitis lethargica
Updates to Article Attributes
Encephalitis lethargica (von Economo encephalitis (EL) is a rare disease that is also known as von Economo encephalitis. It affects the midbrain and misterious deaseas first decriebedbasal ganglia, and the exact aetiology is unknown.
Clinical presentation
Symptoms are initially that of pharyngitis followed by lethargy, extrapyramidal movements (parkinsonism and dyskinesias), neuropsychiatricdisturbance (obsessive, compulsive disorder, catatonia, mutism, apathy and conduct disorders), and ocular movement disorder (oculogyric crises).
Pathology
The exact aetiology is unknown but there is lymphocyte infiltration of the midbrain and basal ganglia.
Markers
Autoantibodies reactive against human basal ganglia antigens present in the majority of EL patients.
Radiographic features
MRI
Brain MRI demonstrates inflammatory changes localised to the deep grey matterin 40% of patients.
- T1: low signal
- T2/FLAIR: high signal in the basal ganglia (including the substantia nigra), midbrain, thalamus, cerebral peduncle and/or temporal cortex
- T1 C+ (Gd) : leptomeningeal and focal patchy enhancement
- DWI/ADC : isointense without restriction of ADC
Nuclear medicine
- PET-CT:increased glucose metabolism in the basal ganglia
Treatment and prognosis
Treatment approaches to encephalitis lethargica include immunomodulating therapies and steroids.
History and etymology
First described by von Economo in 1916 during an epydemyepidemic affecting 500500,000 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, extrapyramidal movements (parkinsonism and dyskinesias), neuropsychiatricdisturbance (obsessive, compulsive disorder, catatonia, mutism, apathy and conduct disorders) and ocular movement 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:
-<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>- +<p><strong>Encephalitis lethargica</strong> (<strong>EL</strong>) is a rare disease that is also known as <strong>von Economo encephalitis</strong>. It affects the midbrain and basal ganglia, and the exact aetiology is unknown. </p><h4>Clinical presentation</h4><p>Symptoms are initially that of pharyngitis followed by 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><h4>Pathology</h4><p>The exact aetiology is unknown but there is lymphocyte infiltration of the midbrain and basal ganglia.</p><h5>Markers</h5><p>Autoantibodies reactive against human basal ganglia antigens present in the majority of EL patients. </p><h4>Radiographic features</h4><h5>MRI</h5><p>Brain MRI demonstrates inflammatory changes localised to the deep grey matter<br>in 40% of patients.</p><ul>
- +<li>
- +<strong>T1:</strong> low signal</li>
- +<li>
- +<strong>T2/FLAIR: </strong>high signal in the basal ganglia (including the substantia nigra), midbrain, thalamus, cerebral peduncle and/or temporal cortex</li>
- +<li>
- +<strong>T1 C+ (Gd) : </strong>leptomeningeal and focal patchy enhancement </li>
- +<li>
- +<strong>DWI/ADC : </strong>isointense without restriction of ADC</li>
- +</ul><h5>Nuclear medicine</h5><ul><li>PET-CT:<strong> </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 steroids.</p><h4>History and etymology</h4><p>First described by <strong>von Economo</strong> in 1916 during an epidemic 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><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
-<li><p>Herpes simplex encephalitis</p></li>-<li><a href="/articles/leptospirosis">Leptospirosis</a></li>- +<li><a href="/articles/herpes-simplex-encephalitis">herpes simplex encephalitis</a></li>
- +<li><a href="/articles/leptospirosis">leptospirosis</a></li>
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>
- K. von Economo. Encepahlitis lethargica. Wiener klinische Wochenschrift, May 10, 1917, 30: 581-585. Die Encephalitis lethargica. Leipzig and Vienna, Franz Deuticke, 1918
- Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity Brain, 127 (2004), pp. 21–33
- Morris S. Dickman, MD von Economo Encephalitis Arch Neurol. 2001; 58; 1696-1698
- Lopez-Alberola R, Georgiou M, Sfakianakis G, Singer C, Papapetropoulos S. Contemporary Encephalitis Lethargica: Phenotype, laboratory findings and treatment outcomes. J Neurol (2009) 256:396–404
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