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Background: Sleep disorders are increasingly recognized in the symptomatology of many neurodegenerative diseases. Gerstmann-Sträussler-Scheinker (GSS) disease is a hereditary prion disease featuring cerebellar ataxia, akinetic parkinsonism, pyramidal signs and cognitive decline. Methods: We performed a polysomnographic study (PSG) of sleep and body core temperature (BcT degrees ) in two sisters with GSS. Results: Our study showed protracted nocturnal awakenings, reduced sleep efficiency and brief daytime naps but also qualitatively preserved slow-wave and REM sleep and substantially normal arousal and periodic limb movements in sleep indices and BcT degrees rhythm. Conclusions: These findings conflict with those in multiple system atrophy and other prion diseases such as fatal familial insomnia, which enter the differential diagnosis of GSS and are characterized by prominently disrupted sleep-wake and BcT degrees cycles. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To study sleep–wake and body core temperature (BCT) circadian rhythms in patients with multiple sclerosis (MS)-associated with chronic fatigue. Methods: Six relapsing–remitting MS patients with chronic fatigue underwent 48 consecutive hours polysomnography (PSG) with BCT measurement, followed by a Multiple Sleep Latency Test (MSLT). All patients were relapse- and drug-free. Mood depression, brain and cervical cord enhanced MRI, dynamic spirometry and Fatigue Severity Scale (FSS) were assessed just before PSG. Results: In all patients mood depression was absent and dynamic spirometry normal, but FSS confirmed fatigue. MRI showed non-enhancing lesions. Nocturnal sleep was characterized by normal architecture and mean sleep efficiency was only slightly reduced. Arousal index was normal and periodic limb movements during sleep (PLMS) were present in four patients, with an increased index (PLMS-I) in only two of them. Upon MSLT, mean sleep latency was normal in all patients with one sleep onset REM period in one patient. All patients displayed a normal BCT 24-h rhythm. Mesor, amplitude and acrophase of BCT rhythm did not show significant differences between MS and controls. Conclusions: We found substantially normal sleep–wake and BCT rhythmicity in six patients with MS and fatigue. Non-restorative sleep and abnormal BCT regulation were unlikely mechanisms of chronic fatigue in our MS patients. Significance: Subjective fatigue and abnormal sleep and BCT can be independent manifestation in MS patients. The findings support the notion that objective measures of fatigue comparable to the MSLT for sleepiness do not exist. [Copyright &y& Elsevier]

Abstract: Aims: To describe the clinical and polygraphic features of propriospinal myoclonus (PSM) at sleep onset. Material and methods: PSM was first described in 1997 in patients with jerks occurring in the relaxation period preceding sleep. EMG showed jerks to arise in spinally innervated muscles, propagating thereafter to rostral and caudal muscles at a low speed, typical of propriospinal pathways. Results: PSM arose when EEG alpha activity spread over the scalp and disappeared during either active wakefulness or actual sleep. In some patients EMG activity could sometimes remain localized to the abdominal muscles, propagating to other segments only in fully developed jerks. Neurological examination, brain and spinal MRI were usually normal and clonazepam afforded partial improvement. PSM has been recently observed also in restless legs syndrome, during relaxed wakefulness preceding falling asleep, coexisting with motor restlessness and sensory discomfort. PSM disappeared when spindles and K-complexes and typical Periodic Limb Movements appeared with EMG activity limited to leg muscles, without propriospinal propagation. Conclusions: Conceivably, PSM arises in axial muscles due to some spinal generator set into motion by facilitating influences characteristic of the wake–sleep transition and then undergoes multimeric propriospinal propagation. In the International Classification of Sleep Disorders (ICSD-2), PSM is listed in chapter VII, among the “Isolated symptoms, apparently normal variants and unresolved issues”. [Copyright &y& Elsevier]