To improve the specificity and sensitivity of the clinical diagnosis of progressive supranuclear palsy (PSP, Steele-Richardson-Olszewski syndrome), the National Institute of Neurological Disorders and Stroke (NINDS) and the Society for PSP, Inc. (SPSP) sponsored an international workshop to develop an accurate and universally accepted set of criteria for this disorder. The NINDS-SPSP criteria, which were formulated from an extensive review of the literature, comparison with other previously published sets of criteria, and the consensus of experts, were validated on a clinical data set from autopsy-confirmed cases of PSP. The criteria specify three degrees of diagnostic certainty: possible PSP, probable PSP, and definite PSP. Possible PSP requires the presence of a gradually progressive disorder with onset at age 40 or later, either vertical supranuclear gaze palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of onset, as well as no evidence of other diseases that could explain these features. Probable PSP requires vertical supranuclear gaze palsy, prominent postural instability, and falls in the first year of onset, as well as the other features of possible PSP. Definite PSP requires a history of probable or possible PSP and histopathologic evidence of typical PSP. Criteria that support the diagnosis of PSP, and that exclude diseases often confused with PSP, are presented. The criteria for probable PSP are highly specific, making them suitable for therapeutic, analytic epidemiologic, and biologic studies, but not very sensitive. The criteria for possible PSP are substantially sensitive, making them suitable for descriptive epidemiologic studies, but less specific. An appendix provides guidelines for diagnosing and monitoring clinical disability in PSP.

Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) Report of the NINDS-SPSP International Workshop*

Thirty asymptomatic men and 19 asymptomatic women were monitored during one night's sleep to determine the incidence of breathing abnormalities and oxygen desaturation in normal subjects. Twenty men accounted for 264 episodes of nocturnal oxygen desaturation or abnormal breathing. Women never experienced oxygen desaturation, and only three had a total of nine episodes of apnea. These sex differences were highly significant (P less than 0.003). In men, increasing age and obesity correlated positively with the incidence of nocturnal oxygen desaturation and abnormal breathing. Four asymptomatic men weighing more than 90 kg dropped their saturation to very low levels (68 to 72 per cent). Abnormal breathing and oxygen desaturation during sleep in subjects with chronic obstructive lung disease of the syndrome of hypersomnolence with periodic breathing may represent the superimposition of smoking or obesity on a normal tendency to snoring and oxygen desaturation in men.

Sleep apnea, hypopnea and oxygen desaturation in normal subjects. A strong male predominance.

Sleep in depressed patients resembles sleep in normal subjects whose circadian rhythms of temperature and rapid-eye-movement sleep are phase-advanced (shifted earlier) relative to their sleep schedules. If this analogy is relevant to the pathophysiology of depressive illness, advancing the time of sleep and awakening should temporarily compensate for the abnormal timing of depressed patients' circadian rhythms. Four of seven manic-depressive patients studied longitudinally spontaneously advanced their times of awakening (activity onset) as they emerged from the depressive phase of their illness. In a phase-shift experiment, a depressed manic-depressive woman was twice brought out of depression for 2 weeks by advancing her sleep period so that she went to sleep and arose 6 hours earlier than usual. The antidepressant effect of the procedure was temporary and similar in duration to circadian desynchronization induced by jet lag in healthy subjects. This result supports the hypothesis that abnormalities of sleep patterns in some types of depression are due to abnormal internal phase relationships of circadian rhythms.

https://science.sciencemag.org/content/sci/206/4419/710.full.pdf

Phase advance of the circadian sleep-wake cycle as an antidepressant

Summary Rapid-eye-movement (REM) sleep behaviour disorder (RBD) is characterised by loss of muscular atonia and prominent motor behaviours during REM sleep RBD can cause sleep disruption and severe injuries for the patient or bed partner The disorder is strongly associated with neurodegenerative diseases, such as multiple-system atrophy, Parkinson's disease, dementia with Lewy bodies, and progressive supranuclear palsy In many cases, the symptoms of RBD precede other symptoms of these neurodegenerative disorders by several years Furthermore, several recent studies have shown that RBD is associated with abnormalities of electroencephalographic activity, cerebral blood flow, and cognitive, perceptual, and autonomic functions RBD might be a stage in the development of neurodegenerative disorders and increased awareness of this could lead to substantial advances in knowledge of mechanisms, diagnosis, and treatment of neurodegenerative disorders

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(06)70441-0.pdf

Rapid-eye-movement sleep behaviour disorder and neurodegenerative diseases

https://www.neurologic.theclinics.com/article/S0733-8619(05)70281-4/pdf

REM sleep parasomnias.

A polygraphic study of sleep has been performed on 12 patients with ALS and 12 normal controls. Neither the total duration of sleep, nor the proportion of different stages of sleep differed significantly between the patient and control groups. A variety of morphologic peculiarities were observed in certain patients: frequent and repetitive blinking movements during the waking state (under quiet, dark conditions); persistence of EMG activity during both PS and SWS; and an early appearance of marked slow delta wave activity at the beginning of sleep. We observed in patients increased PS latency, and longer and more frequent awakenings. Certain patients also showed both qualitative and quantitative abnormalities of respiratory rhythm. A longitudinal study was performed on one patient. Results are discussed in relation to the existing literature.

A study on sleep in amyotrophic lateral sclerosis.

Sleep respiratory arrhythmias in control subjects, narcoleptics and non-cataplectic hypersomniacs.

[Polygraphic sleep recordings in 9 cases of Steele-Richardson's disease (author's transl)].

A polygraphic study of nocturnal sleep was carried out on 12 patients suffering from amyotrophic lateral sclerosis (ALS), 6 patients suffering from olivio-ponto-cerebellare-atrophia (OPCA) and 9 patients suffering from the Steele-Richardson disease (SR). A disturbance of sleep--if it exists--always runs parallel with the course of the disease. No disturbances were registered in the group of patients suffering from ALS, in the OPCA group a specific disease of paradoxal sleep is observed. The sleep disturbances are more global in the group of patients suffering from the SR disease. A particular electrooculogram was taken from the patients from the awakening and from the paradoxal sleep. Respiratory difficulties were registered on certain patients of each of the three groups. These sleep disturbances, registered by the polygraph, were compared with those observed on patients presenting a cortical disease.

[Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy].

The administration of clonidine (300 mug) suppresses REM sleep or strikingly diminishes its duration in Man. The statistical difference with placebo given in a double blind trial is highly significant. This suppression of REM sleep may depend on the stimulation of alpha-adrenergic receptors.

Beillevaire T

Papers

A study on sleep in amyotrophic lateral sclerosis.

Sleep respiratory arrhythmias in control subjects, narcoleptics and non-cataplectic hypersomniacs.

[Polygraphic sleep recordings in 9 cases of Steele-Richardson's disease (author's transl)].

[Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy].

Suppression of paradoxal sleep by clonidine in man