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The Neurologic Disorders in Film
Published in Eelco F. M. Wijdicks, Neurocinema—The Sequel, 2022
What is currently known about the disorder is that after a flu-like illness, patients with encephalitis lethargica develop marked sleepiness, ocular movement disturbances, and fever. Abnormal posturing (dystonia) was not initially part of the manifestation, and stupor (hence, the word lethargic) was most prominent and persistent. Some patients could become immediately alert; others had more cyclic responsiveness, with sleep during the day and wakefulness during the night. In earlier descriptions, paralysis of eye muscles was very common, but very often, other cranial nerves were involved. Oropharyngeal dysfunction could lead to early demise. This combination of upper cranial nerve involvement and stupor now, in retrospect, would fit well with an upper brainstem lesion, and indeed, the mesencephalon showed necrosis and perivascular lymphocytic infiltrate. In some patients, the hypothalamus was involved. Many of these patients either developed a catatonic state or recovered with narcolepsy. The parkinsonian manifestations occurred often after a period of years after the infection, but more than 50% of the survivors developed parkinsonian symptoms within 5 years and 80% within 10 years. Oculogyric crisis was a common manifestation.
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
Infection:59Primary infection invading basal ganglia structures (i.e. toxoplasmosis, viral encephalitis).Postencephalitic parkinsonism; now rare, mainly reported as encephalitis lethargica followed a worldwide flu epidemic in 1918.HIV infection/AIDS.Prion disease (CJD, GSS).Whipple's disease.SSPE.West Nile virus.60
Von Economo’s encephalitis
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Psychiatric manifestations were common as long term consequences of encephalitis lethargica. Neuropsychiatric disturbances were reported in 50% [75] to 100% of survivors [16,76]. These manifestations included mood changes, e.g., mania and depression, feelings of euphoria, increased sexual drive, exhibitionism, and paraphilic behavior [9]. Hallucinations, metamorphopsia, and excessive puns, joviality, and silliness referred to as the Witzelsucht of Jastrowitz [9]. Psychosis is observed in 30%, although milder forms very common. Delusions, hallucinations, and ideas of reference accompanied the psychosis [9]. Catatonic symptoms were prominent [9]. The mental changes appeared to be more striking in children, whereas, adults more often had Parkinsonian sequelae [2]. McKenzie described these behavioral disturbances in children as manifesting as a profound emotional instability and a perversion of conduct with poor impulse control that occasionally led to criminal behavior [2]. Mild features of included nervousness, fatiguability, poor concentration, anxiety and depression, Parkinsonism with emotional impoverishment, and intellectual decline. The loss of cognitive function was most commonly observed when encephalitis lethargica occurred in infancy.
On the English (1931) and Spanish (1932) translations of von Economo’s classic monograph on encephalitis lethargica
Published in Journal of the History of the Neurosciences, 2022
Constantin von Economo provided the following note especially for the English edition (von Economo 1931): I hope that the attempt undertaken in this book to present a survey of the complex features of encephalitis lethargica from the clinician’s point of view, following the course of the disease up to its sources, will meet with a friendly reception from English readers. It was in England that very early and fundamental researches into the nature of this ‘new’ disease took place—by [Sir Edward Farquhar] Buzzard, [Francis Graham] Crookshank, [Joseph Godwin] Greenfield, [Sir Arthur John] Hall, [James] McIntosh, [Sir Arthur Salusbury] MacNalty, [Allan C.] Parsons, and many others—so that we owe a great deal of our knowledge of the disease to English and American work.C. v. E.
Encephalitis lethargica in Peru
Published in Journal of the History of the Neurosciences, 2021
Santiago Stucchi-Portocarrero, Miguel Humberto Tomas-Miranda
The clinical presentation of encephalitis lethargica was heterogeneous and typically included acute and chronic phases. There could also be a prodromal phase, with malaise, low-grade fever, pharyngitis, shivering, headache, vertigo, and vomiting. The acute phase was characterized by excessive sleepiness, disorders of ocular motility, fever, and movement disorders. Von Economo classified this phase into three forms: somnolent-ophthalmoplegic, hyperkinetic, and amyostatic-akinetic. The somnolent-ophthalmoplegic form was the most common of the three, with patients typically experiencing an overwhelming desire to sleep, and patients with the disease were indeed capable of sleeping for long periods. In the hyperkinetic form, there was an initial manic phase with chorea, vocalizations, myoclonic twitches, and myorhythmia of the ocular and masticatory muscles; this manic phase was followed by generalized restlessness, weakness, and fatigue. The amyostatic-akinetic form was the least common of the three, and was characterized by rigidity and lack of movement, with no noticeable weakness; this form could include transient Parkinsonism. The chronic phase of encephalitis lethargica usually developed one to five years after the acute phase and was characterized by Parkinsonism, oculomotor abnormalities, involuntary movements, speech and respiratory abnormalities, and psychiatric manifestations (Hoffman and Vilensky 2017).
Centenary of Tretiakoff’s thesis on the morphology of Parkinson’s disease, evolved on the grounds of encephalitis lethargica pathology
Published in Journal of the History of the Neurosciences, 2019
The first outbreak of encephalitis lethargica has been recorded in the winter 1916–1917 and extended worldwide to one of the most catastrophic epidemics, with millions of victims, especially young people. The first report of Constantin von Economo from the university clinic in Vienna appeared in 1917 on seven cases, including two autopsies that showed in two brains an infiltration of small cells of the vessels in the gray matter around the third ventricle, the area of the ocular nuclei, and the floor of the fourth ventricle with selective lesion of gray matter (polio-encephalitis; Economo, 1917a). In following papers Economo (1917b, 1917c) reported on more victims with detailed microscopic findings, chiefly located in the basal ganglia and the midbrain (Economo, 1917c, p. 315). But Economo did not mention expressly the predominant lesion of the Substantia nigra, only twice as part of a broader extended process in Case 1 with a course of four months (Economo, 1917b), and in Case 4 with a course of 24 days (Economo 1917c, pp. 302 and 315, and Figure 15). Clinically, it was obvious that somnolence and ophthalmoplegic symptoms were signs of the midbrain.