ALS is a devastating neurodegenerative disorder with no effective treatment. In the present study, we found that daily doses of lithium, leading to plasma levels ranging from 0.4 to 0.8 mEq/liter, delay disease progression in human patients affected by ALS. None of the patients treated with lithium died during the 15 months of the follow-up, and disease progression was markedly attenuated when compared with age-, disease duration-, and sex-matched control patients treated with riluzole for the same amount of time. In a parallel study on a genetic ALS animal model, the G93A mouse, we found a marked neuroprotection by lithium, which delayed disease onset and duration and augmented the life span. These effects were concomitant with activation of autophagy and an increase in the number of the mitochondria in motor neurons and suppressed reactive astrogliosis. Again, lithium reduced the slow necrosis characterized by mitochondrial vacuolization and increased the number of neurons counted in lamina VII that were severely affected in saline-treated G93A mice. After lithium administration in G93A mice, the number of these neurons was higher even when compared with saline-treated WT. All these mechanisms may contribute to the effects of lithium, and these results offer a promising perspective for the treatment of human patients affected by ALS

Background/Aims: Sleep disturbances are common in the elderly and in persons with cognitive decline. The aim of this study was to describe frequency and characteristics of insomnia, excessive daytime sleepiness, sleep-disordered breathing, REM behavior disorder and restless legs syndrome in a large cohort of persons with mild cognitive impairment or dementia. Methods: 431 consecutive patients were enrolled in 10 Italian neurological centers: 204 had Alzheimer’s disease, 138 mild cognitive impairment, 43 vascular dementia, 25 frontotemporal dementia and 21 Lewy body dementia or Parkinson’s disease dementia. Sleep disorders were investigated with a battery of standardized questions and questionnaires. Results: Over 60% of persons had one or more sleep disturbances almost invariably associated one to another without any evident and specific pattern of co-occurrence. Persons with Alzheimer’s disease and those with mild cognitive impairment had the same frequency of any sleep disorder. Sleep-disordered breathing was more frequent in vascular dementia. REM behavior disorder was more represented in Lewy body or Parkinson’s disease dementia. Conclusion: A careful clinical evaluation of sleep disorders should be performed routinely in the clinical setting of persons with cognitive decline. Instrumental supports should be used only in selected patients.

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Summary: Purpose: Because several reports have described the relation between epilepsy and cardiac arrhythmias and suggest that changes in autonomic neural control of the heart could be involved in the pathogenesis of sudden unexplained death in patients with epilepsy, the aim of this study was to evaluate cardiac function in patients with temporal lobe epilepsy.Methods: Sixty-five patients with epilepsy were evaluated by simultaneous ambulatory 24-h EEG-ECG monitoring, and in 30 of these, power spectral analysis of relative-risk (RR) variability also was carried out, both in the supine position and in a passive tilt position at 60". The power spectrum of RR variability, the two major spectral components detectable at low frequency (LF) and at high frequency (HF), respectively, and the LF/HF ratio were calculated.Results: By EEG-ECG monitoring, we recorded six partial seizures, and in four cases, discharges were associated with sinus tachycardia. However, interictally the occurrence of ventricular and supraventricular arrhythmias was not different from that in normal subjects. The spectral analysis of RR variability, on the other hand, demonstrated in patients with epilepsy a significant decrease in the total RR variability and in both of its components (LF and HF) in the supine position, and of the LF/HF ratio in orthostatic position.Conclusions: These findings suggest that the spectral analysis of RR variability may detect disorders of autonomic cardiac control in patients with epilepsy, even in the absence of abnormal findings during ECG monitoring. This alteration, which is more severe in cases with right EEG focus, could play a role in the pathogenesis of cardiac arrhythmias. Key Words: Temporal lobe epilepsy-Sudden unexplained death in patients with epilepsy-ECG monitoring-Heart rate variabilityAutonomic disorders.Sudden unexplained death in patients with epilepsy (SUDEP) is a well-recognized phenomenon (l), although its pathogenesis is not yet completely understood. In a recent cohort study (2), the maximal overall incidence of SUDEP observed was 1.35 cases/l,OOO person-years, which was similar to values described in previous studies (3,4). In particular, the incidence of SUDEP is increased in males and is correlated with the number of antiepileptic drugs (AEDs) prescribed and with treatment. with psychotropic drugs. It is also associated with alcohol abuse and slightly with mental retardation.With regard to the pathogenesis of SUDEP, the hypothesis of an acute disturbance of the cardiac rhythm as a possible mechanism is supported by various clinical case reports of patients with temporal lobe epilepsy (5,6) Accepted September 13, 1996. Address correspondence and reprint requests to Dr. L. Mum at Department of Neurosciences, Institute of Neurology, Via Roma, 67, 56126 Pisa, Italy and by the finding that both simple and complex partial seizures (CPSs) are consistently associated with tachycardia (7,s) and rarely with bradycardia (9,lO). On the other hand, certain data obtained interictally provide only par...