Review

Neuropathology of acute phase encephalitis lethargica: A review of cases from the epidemic period

L. L. Anderson, Joel Vilensky, R. C. Duvoisin

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Introduction: Encephalitis lethargica (EL), an epidemic disease of the early 20th century, has continued to be diagnosed sporadically since that time, including a report of 20 new cases in 2004. Many of the recent case reports state that the primary neuropathology of acute EL consists of inflammatory changes and lesions within the midbrain, basal ganglia and substantia nigra. However, the neuropathology of acute EL cases from the epidemic period was actually much more widespread. Methods: In order to characterize the neuropathology of acute phase EL, we developed a database of EL pathology based on 112 cases from the years 1915 to 1940, of which most died within 2 weeks of EL onset. Results: Our analysis revealed that cortical damage was prevalent in 75% of the 112 cases; damage to the meninges and brainstem occurred in approximately half of the cases; and the substantia nigra was damaged in only 13% of these acute cases. We also found that after 1921, damage to cranial nerve nuclei was not reported. An analysis of the neuropathology and clinical symptoms revealed little correlation. Conclusions: Based on these findings, putative modern cases of acute EL with MRI/CT indicated lesions confined solely to the midbrain, brainstem, and/or basal ganglia should not be considered, consistent with that reported during epidemic period.

Original languageEnglish
Pages (from-to)462-472
Number of pages11
JournalNeuropathology and Applied Neurobiology
Volume35
Issue number5
DOIs
StatePublished - Oct 2009

Fingerprint

Encephalitis
Substantia Nigra
Mesencephalon
Basal Ganglia
Brain Stem
Arbovirus Encephalitis
Meninges
Cranial Nerves
Neuropathology
Databases
Pathology

Keywords

  • Epidemic encephalitis
  • Postencephalitic Parkinsonism
  • Von Economo's disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neurology
  • Histology
  • Physiology (medical)

Cite this

Review : Neuropathology of acute phase encephalitis lethargica: A review of cases from the epidemic period. / Anderson, L. L.; Vilensky, Joel; Duvoisin, R. C.

In: Neuropathology and Applied Neurobiology, Vol. 35, No. 5, 10.2009, p. 462-472.

Research output: Contribution to journalArticle

@article{1355d5144a9149d6a2f88fbe517d2f1c,
title = "Review: Neuropathology of acute phase encephalitis lethargica: A review of cases from the epidemic period",
abstract = "Introduction: Encephalitis lethargica (EL), an epidemic disease of the early 20th century, has continued to be diagnosed sporadically since that time, including a report of 20 new cases in 2004. Many of the recent case reports state that the primary neuropathology of acute EL consists of inflammatory changes and lesions within the midbrain, basal ganglia and substantia nigra. However, the neuropathology of acute EL cases from the epidemic period was actually much more widespread. Methods: In order to characterize the neuropathology of acute phase EL, we developed a database of EL pathology based on 112 cases from the years 1915 to 1940, of which most died within 2 weeks of EL onset. Results: Our analysis revealed that cortical damage was prevalent in 75{\%} of the 112 cases; damage to the meninges and brainstem occurred in approximately half of the cases; and the substantia nigra was damaged in only 13{\%} of these acute cases. We also found that after 1921, damage to cranial nerve nuclei was not reported. An analysis of the neuropathology and clinical symptoms revealed little correlation. Conclusions: Based on these findings, putative modern cases of acute EL with MRI/CT indicated lesions confined solely to the midbrain, brainstem, and/or basal ganglia should not be considered, consistent with that reported during epidemic period.",
keywords = "Epidemic encephalitis, Postencephalitic Parkinsonism, Von Economo's disease",
author = "Anderson, {L. L.} and Joel Vilensky and Duvoisin, {R. C.}",
year = "2009",
month = "10",
doi = "10.1111/j.1365-2990.2009.01024.x",
language = "English",
volume = "35",
pages = "462--472",
journal = "Neuropathology and Applied Neurobiology",
issn = "0305-1846",
publisher = "Wiley-Blackwell",
number = "5",

}

TY - JOUR

T1 - Review

T2 - Neuropathology of acute phase encephalitis lethargica: A review of cases from the epidemic period

AU - Anderson, L. L.

AU - Vilensky, Joel

AU - Duvoisin, R. C.

PY - 2009/10

Y1 - 2009/10

N2 - Introduction: Encephalitis lethargica (EL), an epidemic disease of the early 20th century, has continued to be diagnosed sporadically since that time, including a report of 20 new cases in 2004. Many of the recent case reports state that the primary neuropathology of acute EL consists of inflammatory changes and lesions within the midbrain, basal ganglia and substantia nigra. However, the neuropathology of acute EL cases from the epidemic period was actually much more widespread. Methods: In order to characterize the neuropathology of acute phase EL, we developed a database of EL pathology based on 112 cases from the years 1915 to 1940, of which most died within 2 weeks of EL onset. Results: Our analysis revealed that cortical damage was prevalent in 75% of the 112 cases; damage to the meninges and brainstem occurred in approximately half of the cases; and the substantia nigra was damaged in only 13% of these acute cases. We also found that after 1921, damage to cranial nerve nuclei was not reported. An analysis of the neuropathology and clinical symptoms revealed little correlation. Conclusions: Based on these findings, putative modern cases of acute EL with MRI/CT indicated lesions confined solely to the midbrain, brainstem, and/or basal ganglia should not be considered, consistent with that reported during epidemic period.

AB - Introduction: Encephalitis lethargica (EL), an epidemic disease of the early 20th century, has continued to be diagnosed sporadically since that time, including a report of 20 new cases in 2004. Many of the recent case reports state that the primary neuropathology of acute EL consists of inflammatory changes and lesions within the midbrain, basal ganglia and substantia nigra. However, the neuropathology of acute EL cases from the epidemic period was actually much more widespread. Methods: In order to characterize the neuropathology of acute phase EL, we developed a database of EL pathology based on 112 cases from the years 1915 to 1940, of which most died within 2 weeks of EL onset. Results: Our analysis revealed that cortical damage was prevalent in 75% of the 112 cases; damage to the meninges and brainstem occurred in approximately half of the cases; and the substantia nigra was damaged in only 13% of these acute cases. We also found that after 1921, damage to cranial nerve nuclei was not reported. An analysis of the neuropathology and clinical symptoms revealed little correlation. Conclusions: Based on these findings, putative modern cases of acute EL with MRI/CT indicated lesions confined solely to the midbrain, brainstem, and/or basal ganglia should not be considered, consistent with that reported during epidemic period.

KW - Epidemic encephalitis

KW - Postencephalitic Parkinsonism

KW - Von Economo's disease

UR - http://www.scopus.com/inward/record.url?scp=69949140639&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=69949140639&partnerID=8YFLogxK

U2 - 10.1111/j.1365-2990.2009.01024.x

DO - 10.1111/j.1365-2990.2009.01024.x

M3 - Article

VL - 35

SP - 462

EP - 472

JO - Neuropathology and Applied Neurobiology

JF - Neuropathology and Applied Neurobiology

SN - 0305-1846

IS - 5

ER -