Encephalitis lethargica and the influenza virus. II. The influenza pandemic of 1918/19 and encephalitis lethargica: epidemiology and symptoms

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Abstract

This is the first of two papers which critically examine the relationship between the 1918/19 influenza pandemic and encephalitis lethargica (EL). The role of influenza in the etiology of EL was vigorously debated until 1924. It is notable, however, that the unitarian camp were largely reactive in their argumentation; while the influenza skeptics provided detail descriptions of EL and the features they argued to be unique or at least unusual, influenza supporters focused on sequentially refuting the evidence of their opponents. The impression which emerges from this debate is that the individual features identified by the skeptics were not absolutely pathognomic for EL, but, on the other hand, their combination in one disorder had not previously been described for any other disease.

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Updating the Accounts: Global Mortality of the 1918-1920 "Spanish" Influenza Pandemic

Niall Johnson, Juergen Mueller (2002)

The influenza pandemic of 1918-20 is recognized as having generally taken place in three waves, starting in the northern spring and summer of 1918. This pattern of three waves, however, was not universal: in some locations influenza seems to have persisted into or returned in 1920. The recorded statistics of influenza morbidity and mortality are likely to be a significant understatement. Limitations of these data can include nonregistration, missing records, misdiagnosis, and nonmedical certification, and may also vary greatly between locations. Further research has seen the consistent upward revision of the estimated global mortality of the pandemic, which a 1920s calculation put in the vicinity of 21.5 million. A 1991 paper revised the mortality as being in the range 24.7-39.3 million. This paper suggests that it was of the order of 50 million. However, it must be acknowledged that even this vast figure may be substantially lower than the real toll, perhaps as much as 100 percent understated.

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Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity.

Russell Dale, Andrew J. Church, Robert Surtees … (2004)

In 1916, von Economo first described encephalitis lethargica (EL), a CNS disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism) and neuropsychiatric sequelae. Since the 1916-1927 epidemic, only sporadic cases have been described. Pathological studies revealed an encephalitis of the midbrain and basal ganglia, with lymphocyte (predominantly plasma cell) infiltration. The EL epidemic occurred during the same time period as the 1918 influenza pandemic, and the two outbreaks have been linked in the medical literature. However, von Economo and other contemporary scientists thought that the 1918 influenza virus was not the cause of EL. Recent examination of archived EL brain material has failed to demonstrate influenza RNA, adding to the evidence that EL was not an invasive influenza encephalitis. By contrast, the findings of intrathecal oligoclonal bands (OCB) and beneficial effects of steroid treatments have provoked the hypothesis that EL may be immune-mediated. We have recently seen 20 patients with a similar EL phenotype, 55% of whom had a preceding pharyngitis. The patients had remarkable similarity to the historical descriptions of EL: sleep disorder (somnolence, sleep inversion or insomnia), lethargy, parkinsonism, dyskinesias and neuropsychiatric symptoms. CSF examination commonly showed elevated protein and OCB (75 and 69% respectively). Investigation found no evidence of viral encephalitis or other recognized causes of rapid-onset parkinsonism. MRI of the brain was normal in 60% but showed inflammatory changes localized to the deep grey matter in 40% of patients. We investigated the possibility that this phenotype could be a postinfectious autoimmune CNS disorder, and therefore similar to Sydenham's chorea. Anti-streptolysin-O titres were elevated in 65% of patients. Furthermore, western immunoblotting showed that 95% of EL patients had autoantibodies reactive against human basal ganglia antigens. These antibodies were also present in the CSF in four patients tested. By contrast, antibodies reactive against the basal ganglia were found in only 2-4% of child and adult controls (n = 173, P < 0.0001). Rather than showing polyspecific binding, these antibodies bound to common neural autoantigens of molecular weight 40, 45, 60 and 98 kDa. Regional tissue comparisons showed that the majority of these autoantigens were specific to or enriched in CNS tissue. Immunohistochemistry with secondary staining localized antibody binding to neurons rather than glial populations. Further investigation is required to determine whether these antibodies affect neuronal function (i.e. whether they are pathogenic anti-neuronal antibodies). Histopathology in one case demonstrated striatal encephalitis with perivenous B- and T-lymphocytic infiltration. We believe an EL-like syndrome is still prevalent, and propose that this syndrome may be secondary to autoimmunity against deep grey matter neurons.

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Influenza-associated central nervous system dysfunction: a literature review.

Stephen Toovey (2008)

Influenza is a viral pathogen that imposes an under-recognized burden of central nervous system (CNS) disease. To describe the epidemiology, clinical features and etiology of the CNS disease entities associated with influenza. English-language publications from MEDLINE. Articles were identified using "influenza, human"[Mesh] AND "nervous system diseases"[Mesh] and screened for inclusion based on relevance and scientific rigor. Febrile seizure is the most frequently encountered influenza-associated CNS complication, with one in five children hospitalized with influenza experiencing one or more events. In most instances, symptoms resolve without neurological sequelae, although the risk for subsequent afebrile seizure may be increased. Influenza-associated encephalitis/encephalopathy is a less common but potentially more serious complication that is widely reported in Japanese populations, although cases from other East Asian countries, North America, and Europe have been described. Clinical manifestations are diverse, and typically involve febrile seizures and abnormal behaviors in mild cases, with rapid evolution through decreased consciousness to coma in severe forms. In cases of serious disease, the prognosis is often poor, with outcomes including death or severe neurological sequelae. Influenza is also a known trigger for a number of rarely encountered, yet often serious, CNS diseases, including the encephalopathic condition of Reye's syndrome, the peripheral neuropathy known as Guillain-Barré syndrome, and the lesser known complaints of Kleine-Levin syndrome and post-encephalitic Parkinson's disease. Influenza imposes a sizeable burden of CNS disease. Increased awareness and monitoring of CNS function is indicated, especially in infants and young children.

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Journal

Title: Journal of Neural Transmission

Abbreviated Title: J Neural Transm

Publisher: Springer Science and Business Media LLC

ISSN (Print): 0300-9564

ISSN (Electronic): 1435-1463

Publication date Created: October 2009

Publication date (Electronic): August 26 2009

Publication date (Print): October 2009

Volume: 116

Issue: 10

Pages: 1295-1308

Article

DOI: 10.1007/s00702-009-0295-9

PMC ID: 2758910

PubMed ID: 19707848

SO-VID: 37a1f42c-859c-4442-8454-08d1012c344c

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http://www.springer.com/tdm

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Encephalitis lethargica and the influenza virus. II. The influenza pandemic of 1918/19 and encephalitis lethargica: epidemiology and symptoms

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Most cited references 52

Updating the Accounts: Global Mortality of the 1918-1920 "Spanish" Influenza Pandemic

Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity.

Influenza-associated central nervous system dysfunction: a literature review.

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