On doing nothing: Descriptions of sleep, fatigue, and motivation in encephalitis lethargica

Abstract

Epidemics of encephalitis lethargica (EL), from 1917 to the 1930s, are an important milestone in the history of movement disorders. Today, the two best-known features of EL are somnolence and parkinsonism but the full clinical picture was variable and complex. States of wakeful inactivity--as opposed to drowsiness--were often described both in the acute and postacute stages and were referred to in the EL literature as "lethargy" or "torpor." The study described here is based on a survey of clinical descriptions published in English, French, and German from 1917 to 1942. Its focus is on the history of clinical ideas, rather than applying modern pathophysiological concepts retrospectively. Descriptions of lethargy are explored as a way of elucidating concepts of sleep, fatigue, and motivation during the study period. The literature described many patients who had (1) lethargy without interruption in consciousness; (2) slowness of movement and catalepsy without other prominent parkinsonian features; and (3) apathy and lack of initiative without severe disorders of mood or thought content. Hence observers distinguished a state of wakeful inactivity from primary disorders of sleep, movement and behavior. Contemporaneous accounts suggest that writers had difficulty in reconciling their observations with preexisting concepts; there still may be limitations in our ability to describe and classify the clinical states connected during the epidemic era with the term "lethargy."

Post-Encephalitic Parkinsonism and Sleep Disorder Responsive to Immunological Treatment

“…Three variants have been classified: the "somnolent-ophthalmoplegic" pattern, the "hyperkinetic" form, more frequent in children, and the "amyostatic-akinetic" form, which had a parkinsonian character. 9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus.…”

“…9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus. 10 In our case, the delay (1-week time lag in our case) between the resolution of fever and the onset of the movement and the sleep disorder suggests that this syndrome may be considered a post-encephalitic (or para-encephalitic) condition; the results of the immunohistochemical analysis and the prompt and stable response of the neurological symptoms to Igiv support an immune-mediated pathogenesis.…”

“…In the encephalitis lethargica literature, the term "lethargy" or "torpor" is used to indicate states of wakeful inactivity, as opposed to drowsiness. 9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B).…”

“…9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B). As concerns sleep disorders, Dale et al, 3 in a series of 20 patients, reported that 19 (95%) had sleep disturbances: 12 had hypersomnolence, 2 had insomnia and 5 sleep-wake inversion; other sleep abnormalities included vivid nightmares in 2 patients and sleep-walking in 1 patient.…”

Post-Encephalitic Parkinsonism and Sleep Disorder Responsive to Immunological Treatment

“…Three variants have been classified: the "somnolent-ophthalmoplegic" pattern, the "hyperkinetic" form, more frequent in children, and the "amyostatic-akinetic" form, which had a parkinsonian character. 9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus.…”

“…9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus. 10 In our case, the delay (1-week time lag in our case) between the resolution of fever and the onset of the movement and the sleep disorder suggests that this syndrome may be considered a post-encephalitic (or para-encephalitic) condition; the results of the immunohistochemical analysis and the prompt and stable response of the neurological symptoms to Igiv support an immune-mediated pathogenesis.…”

“…In the encephalitis lethargica literature, the term "lethargy" or "torpor" is used to indicate states of wakeful inactivity, as opposed to drowsiness. 9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B).…”

“…9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B). As concerns sleep disorders, Dale et al, 3 in a series of 20 patients, reported that 19 (95%) had sleep disturbances: 12 had hypersomnolence, 2 had insomnia and 5 sleep-wake inversion; other sleep abnormalities included vivid nightmares in 2 patients and sleep-walking in 1 patient.…”

The sleep relay—the role of the thalamus in central and decentral sleep regulation

“…Reducing sleep time is a common route in a notoriously busy society, but prolonged sleep loss will cause (among other defects) impaired metabolism, impaired immune function, impaired cognitive function, and will, ultimately, lead to death [149]. Many pathophysiological states, such as lethargic encephalitis [190], insomnia [47], narcolepsy [114], apnoea [186], cataplexy [126], or epilepsy [104], are related to disturbances in the mechanisms of sleep induction or maintenance.…”

Post-Encephalitic Parkinsonism and Sleep Disorder Responsive to Immunological Treatment

“…Three variants have been classified: the "somnolent-ophthalmoplegic" pattern, the "hyperkinetic" form, more frequent in children, and the "amyostatic-akinetic" form, which had a parkinsonian character. 9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus.…”

“…9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus. 10 In our case, the delay (1-week time lag in our case) between the resolution of fever and the onset of the movement and the sleep disorder suggests that this syndrome may be considered a post-encephalitic (or para-encephalitic) condition; the results of the immunohistochemical analysis and the prompt and stable response of the neurological symptoms to Igiv support an immune-mediated pathogenesis.…”

“…In the encephalitis lethargica literature, the term "lethargy" or "torpor" is used to indicate states of wakeful inactivity, as opposed to drowsiness. 9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B).…”

“…9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B). As concerns sleep disorders, Dale et al, 3 in a series of 20 patients, reported that 19 (95%) had sleep disturbances: 12 had hypersomnolence, 2 had insomnia and 5 sleep-wake inversion; other sleep abnormalities included vivid nightmares in 2 patients and sleep-walking in 1 patient.…”

The sleep relay—the role of the thalamus in central and decentral sleep regulation

“…Reducing sleep time is a common route in a notoriously busy society, but prolonged sleep loss will cause (among other defects) impaired metabolism, impaired immune function, impaired cognitive function, and will, ultimately, lead to death [149]. Many pathophysiological states, such as lethargic encephalitis [190], insomnia [47], narcolepsy [114], apnoea [186], cataplexy [126], or epilepsy [104], are related to disturbances in the mechanisms of sleep induction or maintenance.…”

Post-Encephalitic Parkinsonism and Sleep Disorder Responsive to Immunological Treatment

“…Three variants have been classified: the "somnolent-ophthalmoplegic" pattern, the "hyperkinetic" form, more frequent in children, and the "amyostatic-akinetic" form, which had a parkinsonian character. 9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus.…”

“…9 Parkinsonism and other movement disorders were described as developing insidiously from the acute phase or after a latent period. 9 Review of pathological specimens of brain autopsies of original encephalitis lethargica patients has indicated evidence of predominantly enterovirus pathology, specifically poliovirus and coxsackie B virus. 10 In our case, the delay (1-week time lag in our case) between the resolution of fever and the onset of the movement and the sleep disorder suggests that this syndrome may be considered a post-encephalitic (or para-encephalitic) condition; the results of the immunohistochemical analysis and the prompt and stable response of the neurological symptoms to Igiv support an immune-mediated pathogenesis.…”

“…In the encephalitis lethargica literature, the term "lethargy" or "torpor" is used to indicate states of wakeful inactivity, as opposed to drowsiness. 9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B).…”

“…9 No EEG correlate was available, because when the EEG was developed, the pandemic was over. 9 In our patient, the EEG during such lethargic periods showed diffuse slowing, similar to sleep stage N1, without focal abnormalities or epileptiform discharges ( Figure 2B). As concerns sleep disorders, Dale et al, 3 in a series of 20 patients, reported that 19 (95%) had sleep disturbances: 12 had hypersomnolence, 2 had insomnia and 5 sleep-wake inversion; other sleep abnormalities included vivid nightmares in 2 patients and sleep-walking in 1 patient.…”

The sleep relay—the role of the thalamus in central and decentral sleep regulation

“…Reducing sleep time is a common route in a notoriously busy society, but prolonged sleep loss will cause (among other defects) impaired metabolism, impaired immune function, impaired cognitive function, and will, ultimately, lead to death [149]. Many pathophysiological states, such as lethargic encephalitis [190], insomnia [47], narcolepsy [114], apnoea [186], cataplexy [126], or epilepsy [104], are related to disturbances in the mechanisms of sleep induction or maintenance.…”