Elsevier

Revue Neurologique

Volume 178, Issue 9, November 2022, Pages 878-885
Revue Neurologique

History of neurology
A history of oculogyric crises during the encephalitis lethargica pandemic

https://doi.org/10.1016/j.neurol.2022.01.013Get rights and content

Abstract

A pandemic of what came to be known as encephalitis lethargica spread starting in the winter of 1916-1917 and continued into the 1930s. Neurological after-effects, namely permanent parkinsonian syndromes and various abnormal movements, permanently disabled the survivors of the one or two million victims, often children or young adults. Among them, a small proportion developed a symptom that was little known up to that point and that is currently exceptional: oculogyric crises; that is, a lateralised, dystonic upward movement of the eyes known as a tonic eye fit. This paper proposes a history of the recognition of this symptom, its inclusion in the neurological nosography, and the pathophysiological hypotheses postulated a century ago.

Section snippets

What is an oculogyric crisis?

Without a prodrome, or only a trembling of the eyelids or blepharospasm, “the eyes suddenly deviate, most often upward, hidden under the upper eyelid which is itself retracted. The patient tries repeatedly to lower their eyes and sometimes adopts a compensatory position of the head” [13], according to Georges Guillain (1876–1961) and Pierre Mollaret (1898–1987). This oculogyric crisis is sometimes also called a “tonic eye fit” (Fig. 1, Fig. 2). Physical exertion, emotions, and peripheral

Inclusion of oculogyric crises in the nosography of encephalitis lethargica

During the session on 13 February 1920 of the Medical Society of the Hospitals in Paris, Victor Morax (1866–1935) and Jacques Bollack (1883–1951) highlighted the frequency of associated ocular movement disturbances compared to the rarity of real opthalmoplegia [27] during the acute phase of encephalitis. They did not mention oculogyric crises. Demonstrating that these crises were little known (noticed), Louis Dor (1866–1954), an ophthalmologist from Lyon, did not mention them in his general

Pathogenic theories

Von Economo's encephalitis lethargica initiated the study of behavioural consequences of subcortical disturbances induced by a supposed viral infection. The acute manifestations, highly variable, included extrapyramidal disturbances, myoclonia, ocular movement disorders, paralysis, psychosis, mood swings, sleep inversion, and catatonia. These main pathological changes implicated the substantia nigra, globus pallidus, and hypothalamus (blocked striatum dopamine receptors). For those that

Conclusion

A century after the emergence of this pandemic, its aetiology remains unknown. After eliminating a toxic origin (botulism), authors writing at the time most often subscribed to the idea of viral infection, but lacked the means to prove it. The fact that this pandemic and the so-called Spanish flu were simultaneous supported this theory even though no virus was recognised as causing the parkinsonian syndrome. The current hypothesis centres on a post-infectious, auto-immune encephalitis primarily

Statement of ethics

This work required no approval from an institutional review board and was prepared in accordance with ethical guidelines of the journal.

Disclosure of interest

The author declare that he has no competing interest.

Funding sources

No funding was obtained for this work.

Acknowledgements

Many thanks to Heinz Krestel, Jacques Poirier, Hubert Déchy, Edward Shorter and to Anna Fitzgerald for her translation.

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