Highly purified cannabidiol in the treatment of drug-resistant epilepsies: A real-life impact on seizure frequency, quality of life, behavior, and sleep patterns from a single Italian center.

Seizure frequency in treatment-resistant epilepsies seems to be decreased by cannabidiol (CBD), but contrasting data are available on its effect on sleep, behavior, and quality of life (QoL), and no data is reported on its effect on parental stress in patients with epilepsy (PWE). Thus, we conducted a retrospective study on a cohort of children and adults with drug-resistant epilepsy (DRE) who had been treated with highly purified, pharmaceutical-grade CBD to evaluate its effects on seizure frequency, QoL, behavior, parental stress, and sleep. Eighteen patients (12 adults and 6 children) were included in the cohort and followed for a median of 9 months. At the last follow-up (Tn), nine patients (50%) were considered CBD responders with at least a 50% decrease in seizure frequency. No serious adverse effects were found. No statistically significant differences were found concerning sleep, including daytime sleepiness, and no statistically significant effect was found on parental stress at Tn. An improvement was found for social interaction in quality of life (p < 0.05) for all patients. Our results demonstrate that CBD is a safe and effective antiseizure medication (ASM). CBD doesn't seem to affect sleep measures in adults and children or worsen daytime sleepiness. However, CBD improves specific QoL measures, which could indicate a possible use of CBD for other childhood disabilities. No impact of CBD was seen on parental stress, which could possibly be due to the limited follow-up or could mean that parental stress is not dependent on seizure frequency.

Sleep and delirium in the intensive care unit.

Intensive Care Unit (ICU) patients almost uniformly suffer from sleep disruption. Even though the role of sleep disturbances is not still adequately understood, they may be related to metabolic, immune, neurological and respiratory dysfunction and could worsen the quality of life after discharge. A harsh ICU environment, underlying disease, mechanical ventilation, pain and drugs are the main reasons that underlie sleep disruption in the critically ill. Polysomnography is the gold standard in evaluating sleep, but it is not feasible in clinical practice; therefore, other objective (bispectral index score [BIS] and actigraphy) and subjective (nurse and patient assessment) methods have been proposed, but their adequacy in ICU patients is not clear. Frequent evaluation of neurological status with validated tools is necessary to avoid excessive or prolonged sedation in order to better titrate patient-focused therapy. Hypnotic agents like benzodiazepines can increase total sleep time, but they alter the physiological progression of sleep phases, and decrease the time spent in the most restorative phases compared to the phases normally mediated by melatonin; melatonin production is decreased in critically ill patients, and as such, exogenous melatonin supplementation may improve sleep quality. Sleep disruption and the development of delirium are frequently related, both because of sleep scarcity and inappropriate dosing with sedatives. Delirium is strongly related to increased ICU morbidity and mortality, thus the resolution of sleep disruption could significantly contribute to improved ICU outcomes. An early evaluation of delirium is strongly recommended because of the potential to resolve the underlying causes or to begin an appropriate therapy. Further studies are needed on the effects of strategies to promote sleep and on the evaluation of better sleep in clinical outcomes, particularly on the development of delirium.

Hallucinations and sleep-wake cycle in Alzheimer's disease: a questionnaire-based study in 218 patients.

The aim was to evaluate the relationship between hallucinations and the sleep-wake cycle in a sample of Alzheimer's disease (AD) patients in the early-moderate stage. Two hundred and eighteen AD patients (66 males, 152 females, mean age 74.3+/-6.85) were administered a sleep questionnaire in the presence of a care-giver. Twenty-six out of 218 (12%) reported the occurrence of hallucinations, mainly visual. In 18/28 (69%) hallucinations occurred when the patient was awake and in 8 (31%) hallucinations were reported to occur close to a specific phase of the sleep-wake cycle. Vivid dreams were reported in 25/218 (11%) and violent sleep-related and dream-related behaviours (probable REM behaviour episodes) in 22/218 (10%). Both REM phenomena were more frequent in AD hallucinators than in AD non-hallucinators (26.9% vs. 9.3%, and 26.9% vs. 7.8%, p<0.007). Our data indicate a lower incidence of hallucinations and presumable REM behaviour disorder (RBD) in AD, at least in the early-moderate phase, than that observed in synucleinopathies. However, the higher occurrence of vivid dreams and RBD in AD patients with hallucinations compared to those without hallucinations indicates a potential role of disordered REM sleep in influencing the occurrence of hallucinations in AD, similar to what has been observed in synucleinopathies.

Non-convulsive status epilepticus and generalised tonic-clonic seizures persisting in old age in a patient with idiopathic generalised epilepsy: a long-term observation.

Persisting non-convulsive status epilepticus in a man with idiopathic generalised epilepsy is reported. After a first generalised tonic/clonic seizure on awakening one day at the age of 20, the patient experienced rare non-convulsive status epilepticus until the age of 73, when the frequency of the episodes increased, in spite of the initiation of treatment with antiepileptic drugs. No significant cognitive decline was documented when the patient was 83. The existence of such conditions in the context of idiopathic generalised epilepsy shows the problems of syndromic diagnosis and of age dependency of some epileptic phenomena over the course of life with potential bidirectional influences between epileptic manifestations and senile processes.

Apo(a) size in ischemic stroke: relation with subtype and severity on hospital admission.

OBJECTIVE: To determine the distribution of apolipoprotein (a) (apo[a]) isoforms and their relation to the clinical severity of different ischemic stroke subtypes. METHODS: Ninety-four hospital cases with a first-ever ischemic stroke and 188 randomly selected control subjects matched for age, gender, and ethnicity were enrolled. Stroke etiology was defined according to Trial of Org 10172 in Acute Stroke Treatment criteria. NIH Stroke Scale (NIHSS) was used to assess the severity of stroke on admission. RESULTS: In univariate analysis, the presence of at least one small apo(a) isoform was associated with ischemic stroke in men (p = 0.02) but not in women (p = 0.33). After allowance for age, gender and traditional vascular risk factors, subjects carrying at least one small apo(a) isoform were at increased risk of atherothrombotic stroke (odds ratio [OR] 7.1, 95% CI 2.8 to 17.5, p = 0.00001) but not of lacunar infarction (OR 1.1, 95% CI 0.5 to 2.7, p = 0.78). Multivariate logistic regression analysis revealed that in the atherothrombotic stroke group, the presence of at least one small-sized apo(a) phenotype was associated with an NIHSS score > or =6 (OR 13.6, 95% CI 1.6 to 111.9, p = 0.015). CONCLUSION: Small apolipoprotein (a) isoforms distinguish atherothrombotic stroke from lacunar infarction and are associated with the severity of atherothrombotic stroke.

A possible case of unruptured middle cerebral artery aneurysm presenting as epileptic seizures.

We report the case of a 73-year-old man with an unruptured aneurysm of the left middle cerebral artery. The initial sign was complex partial seizures. A standard scalp electroencephalogram was normal while neuropsychological tests revealed a slight deficit of episodic memory. Brain MRI showed an aneurysm at the left middle cerebral artery bifurcation. Cerebral angiography confirmed the presence of a saccular aneurysm at the left middle cerebral artery bifurcation, with a maximum diameter of 12 mm. This case had two main characteristic features: seizures had a quite late onset and were the only symptom the patient experienced.

Symptomatic epilepsy with facial myoclonus triggered by language.

We report on a patient with a left frontal lesion who, many years after an injury, developed non-fluent aphasia and facial myoclonic jerks triggered by speaking and listening to spoken language. At age 57, the patient first noted that he would begin to stutter when delivering lectures at conferences. The stuttering would worsen if he continued talking. The video-polygraphic EEG recording shows brief paroxysms of spikes and polyspikes, followed by a slow wave, more evident in the left fronto-temporal region. The myoclonic jerks originating from the submental area correlate with EEG abnormalities. Clinically, these jerks determined a form of stuttering. The triggering factors were reading, speaking and listening to spoken language. This case had several characteristic features: facial myoclonus was the only seizure type experienced by the patient; the seizures and language impairment had a very late onset--about 50 years after the traumatic event that produced a dramatic lesion in the left fronto-polar region. (Published with videosequences.)

Experiences from the accident of Seveso.

Provisional data on selected sanitary events which took place at Seveso after July 10 1976 are reported. 187 cases of chloracne, mostly in children, were detected, 50 just after the accident, the others within a year. Most polluted area (zone A) provided almost all "early" and most severe cases, but the territorial distribution of chloracne prevalence rates showed some inconsistencies with the soil TCDD pollution map; interpretations for such findings are discussed. Thirty-eight birth defects were detected in 1977 (none in zones A and B), more than in previous years, but still less than expected in a well controlled "normal" population: no clustering around a given type was observed. Spontaneous abortions, evaluated both as abortion rates and as pregnancy loss rates, showed scattered and statistically non-significant variations, inconsistent with the pollution map. No differences in birth and death rates compared to surrounding areas were observed. Data on ad hoc cytogenetic, neurological and immunological surveys are commented. Limitations of the presently available data are discussed and further research lines are anticipated.