Long-term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike-waves during slow-wave sleep.

PURPOSE: To present the long-term follow-up of 10 adolescents and young adults with documented cognitive and behavioral regression as children due to nonlesional focal, mainly frontal, epilepsy with continuous spike-waves during slow wave sleep (CSWS). METHODS: Past medical and electroencephalography (EEG) data were reviewed and neuropsychological tests exploring main cognitive functions were administered. KEY FINDINGS: After a mean duration of follow-up of 15.6 years (range, 8-23 years), none of the 10 patients had recovered fully, but four regained borderline to normal intelligence and were almost independent. Patients with prolonged global intellectual regression had the worst outcome, whereas those with more specific and short-lived deficits recovered best. The marked behavioral disorders resolved in all but one patient. Executive functions were neither severely nor homogenously affected. Three patients with a frontal syndrome during the active phase (AP) disclosed only mild residual executive and social cognition deficits. The main cognitive gains occurred shortly after the AP, but qualitative improvements continued to occur. Long-term outcome correlated best with duration of CSWS. SIGNIFICANCE: Our findings emphasize that cognitive recovery after cessation of CSWS depends on the severity and duration of the initial regression. None of our patients had major executive and social cognition deficits with preserved intelligence, as reported in adults with early destructive lesions of the frontal lobes. Early recognition of epilepsy with CSWS and rapid introduction of effective therapy are crucial for a best possible outcome.

Suivi neurodéveloppemental de l'enfant né prématuré dans l'Arc lémanique [Neurodevelopmental follow-up of premature children in Lausanne and Geneva].

Preterm children born before 32 weeks of gestation represent 1% of the annual births in Switzerland, and are the most at risk of neurodevelopmental disabilities. A neurological surveillance is thus implemented in the neonatal units, and multidisciplinary neurodevelopmental follow-up is offered to all our preterm patients. The follow-up clinics of the University hospitals in Lausanne and Geneva follow the Swiss guidelines for follow-up. An extended history and neurological examination is taken at each appointment, and a standardized test of development is performed. These examinations, which take place between the ages of 3 months and 9 years old, allow the early identification and treatment of developmental disorders frequent in this population, such as motor, cognitive or behavioral disorders, as well as the monitoring of the quality of neonatal care.

Perceived behavioral changes in early multiple sclerosis.

Acquired behavioral changes have essentially been described in advanced multiple sclerosis (MS). The present study was designed to determine whether behavioral modifications specifically related to the MS pathological process could be identified in the initial phase of the disease, as compared to control patients with chronic, relapsing and progressive inflammatory disorders not involving the central nervous system (CNS). Eighty-eight early MS patients (Expanded Disability Status Scale score <or= 2.5) and 48 controls were tested. Perceived changes by informants in behavioral control, goal-directed behavior, decision making, emotional expression, insight and interpersonal relationships were assessed using the Iowa Scale of Personality Change (ISPC). Executive behavioral disturbances were screened using the Dysexecutive Questionnaire (DEX). The mean change between the premorbid and postmorbid ISPC ratings was similar in the MS [12.2 (SD 15.6)] and in the control [11.5 (SD 15.1)] group. The perceived behavioral changes (PBCs) most frequently reported in both groups were lack of stamina, lability/moodiness, anxiety, vulnerability to stress and irritability. Pathological scores in the DEX were also similar in both groups. Correlations between PBCs and DEX scores were different in MS and control groups. MS patients with cognitive impairment had a marginally higher number of PBCs than control patients (p=0.056) and a significantly higher DEXp score (p=0.04). These results suggest that (1) PBCs occurring in early MS patients were not different from those induced by comparable chronic non-CNS disorders, (2) qualitative differences in the relationship between behavioral symptoms and executive-behavioral changes may exist between MS and control groups, and (3) behavioral symptoms seem associated with cognitive deficits in MS. We further plan to assess these observations longitudinally.

Behavioral and psychological symptoms and cognitive decline in patients with amnestic MCI and mild AD: a two-year follow-up study.

BACKGROUND: Mild cognitive impairment (MCI) has been defined as a transitional state between normal aging and dementia. In many cases, MCI represents an early stage of developing cognitive impairment. Patients diagnosed with MCI do not meet the criteria for dementia as their general intellect and everyday activities are preserved, although minor changes in instrumental activities of daily living (ADL) may occur. However, they may exhibit significant behavioral and psychological signs and symptoms (BPS), also frequently observed in patients with Alzheimer's disease (AD). Hence, we wondered to what extent specific BPS are associated with cognitive decline in participants with MCI or AD. METHODS: Our sample consisted of 164 participants, including 46 patients with amnestic (single or multi-domain) MCI and 54 patients with AD, as well as 64 control participants without cognitive disorders. Global cognitive performance, BPS, and ADL were assessed using validated clinical methods at baseline and at two-year follow-up. RESULTS: The BPS variability over the follow-up period was more pronounced in the MCI group than in patients with AD: some BPS improve, others occur newly or worsen, while others still remain unchanged. Moreover, specific changes in BPS were associated with a rapid deterioration of the global cognitive level in MCI patients. In particular, an increase of euphoria, eating disorders, and aberrant motor behavior, as well as worsened sleep quality, predicted a decline in cognitive functioning. CONCLUSIONS: Our findings confirm a higher variability of BPS over time in the MCI group than in AD patients. Moreover, our results provide evidence of associations between specific BPS and cognitive decline in the MCI group that might suggest a risk of conversion of individuals with amnestic MCI to AD.

The impact of personality characteristics on the clinical expression in neurodegenerative disorders--a review.

Clinical experience suggests that longstanding personality characteristics as a person's most distinctive features of all are likely to play a role in how someone with dementia copes with his increasing deficiencies. Personality characteristics may have a pathoplastic effect on both behavioral and psychological symptoms (BPS) or on cognition as well as cognitive decline. Cognitive disorders accompanied by BPS are a tremendous burden for both the patient and their proxies. This review suggests that premorbid personality characteristics are co-determinants of BPS in cognitive disorders, but much effort is needed to clarify whether or not specific premorbid personality traits are associated with specific BPS as no strong links have so far emerged. This review further shows that a growing field of research is interested in the links not only between quite short-lived emotional states and cognitive processes, but also between longstanding personality traits and cognition in both healthy individuals and patients with neurodegenerative disorders. Furthermore, a few studies found that specific premorbid personality traits may be risk factors for neurodegenerative diseases. However, research findings in this area remain scarce despite a huge literature on personality and cognitive disorders in general. An important shortcoming that hampers so far the progress of our understanding in these domains is the confusion in the literature between longstanding premorbid personality traits and transient personality changes observed in neurodegenerative diseases. Few studies have based their assessments on accepted personality theories and carefully investigated premorbid personality traits in patients with cognitive disorders, although assessing personality may be complicated in these patients. Studying the impact of personality characteristics in cognitive disorders is an especially promising field of research in particular when concomitantly using neurobiological approaches, in particular structural brain imaging and genetic studies as suggested by as yet rare studies. Improved understanding of premorbid personality characteristics as determinants of both BPS or cognitive capacities or decline is likely to influence our attitudes towards the treatment of demented patients and ultimately to help in alleviating a patient's and their proxies' burden.

Intervention précoce dans la phase initiale des troubles psychotiques à Lausanne: quels problèmes et quelles solutions [Early intervention for the initial phase of psychotic disorders in Lausanne: what problems and what solutions?]

Schizophrenia has long been considered with pessimism, but the recent interest in the early phase of psychotic disorders has modified this often unjustified perception. Literature has demonstrated the benefit of the development of programs specialised in the treatment of early psychosis, which tend to be developed in many countries. It is however important to match them to local needs as well as to the structure of local health services. This paper reviews elements that justify such a development in Lausanne, Switzerland, and describe its various elements.

Sleep disorders in boys with Duchenne muscular dystrophy.

Aim:  Determine the frequency and predictors of sleep disorders in boys with Duchenne Muscular Dystrophy (DMD). Method:  Cross-sectional study by postal questionnaire. Sleep disturbances were assessed using the Sleep Disturbance Scale for Children (validated on 1157 healthy children). A total sleep score and six sleep disturbance factors representing the most common sleep disorders were computed. Potential associations between pathological scores and personal, medical and environmental factors were assessed. Results:  Sixteen of 63 boys (25.4%) had a pathological total sleep score compared with 3% in the general population. The most prevalent sleep disorders were disorders of initiating and maintaining sleep (DIMS) 29.7%, sleep-related breathing disorders 15.6% and sleep hyperhydrosis 14.3%. On multivariate analysis, pathological total sleep scores were associated with the need to be moved by a carer (OR = 9.4; 95%CI: 2.2-40.7; p = 0.003) and being the child of a single-parent family (OR = 7.2; 95%CI: 1.5-35.1; p = 0.015) and DIMS with the need to be moved by a carer (OR = 18.0; 95%CI: 2.9-110.6; p = 0.002), steroid treatment (OR = 7.7; 95%CI: 1.4-44.0; p = 0.021) and being the child of a single-parent family (OR = 7.0; 95%CI: 1.3-38.4; p = 0.025). Conclusion:  Sleep disturbances are frequent in boys with DMD and are strongly associated with immobility. Sleep should be systematically assessed in DMD to implement appropriate interventions.

Enjeux ethiques de la psychiatrie sous contrainte [Ethical issues in psychiatry under coercion].

Psychiatry is now subject to two apparently contradictory movements. On the one hand, the need to respect the autonomy and rights of patients is reinforced and coercive measures are strictly defined and limited. On the other hand, security concerns in our society leads to prosecution of psychiatric disorders, especially when accompanied by behavioral problems or criminal acts. In these situations of compulsory treatment or care provided in prisons, a number of dilemmas emerge. The place of the healthcare professional in treatments ordered by the Justice and problems related to administrative detention are discussed in more detail.

Prise en charge des troubles oculomoteurs après effraction iatrogène de l'orbite au cours d'interventions sinusiennes endoscopiques [Management of motility disorders secondary to iatrogenic orbital fracture during endoscopic sinus surgery.]

PURPOSE: Orbital wall fracture may occur during endoscopic sinus surgery, resulting in oculomotor disorders. We report the management of four cases presenting with this surgical complication. METHODS: A non-comparative observational retrospective study was carried out on four patients presenting with diplopia after endoscopic ethmoidal sinus surgery. All patients underwent full ophthalmologic and orthoptic examination as well as orbital imaging. RESULTS: All four patients presented with diplopia secondary to a medial rectus lesion confirmed by orbital imaging. A large horizontal deviation as well as limitation of adduction was present in all cases. Surgical management consisted of conventional recession-resection procedures in three cases and muscle transposition in one patient. A useful field of binocular single vision was restored in two of the four patients. CONCLUSION: Orbital injury may occur during endoscopic sinus surgery and cause diplopia, usually secondary to medial rectus involvement due to the proximity of this muscle to the lamina papyracea of the ethmoid bone. Surgical management is based on orbital imaging, duration of the lesion, evaluation of anterior segment vasculature, results of forced duction testing and intraoperative findings. In most cases, treatment is aimed at the symptoms rather than the cause, and the functional prognosis remains guarded.

Troubles de l'humeur et VIH: épidémiologie, clinique et prise en charge thérapeutique [Mood disorders in HIV patients: a challenge for liaison psychiatry consultation].

Mood disorders represent the most prevalent psychiatric condition in patients infected by HIV virus. Screening and treatment of depression as well as the evaluation of the risk suicide is of the utmost importance. When psychopharmacological treatment is required, interaction with antiretroviral treatment must be carefully considered. More generally a close collaboration between the physician and the psychiatrist is recommended.

Controversies about a common etiology for eating and mood disorders.

Obesity and depression represent a growing health concern worldwide. For many years, basic science and medicine have considered obesity as a metabolic illness, while depression was classified a psychiatric disorder. Despite accumulating evidence suggesting that obesity and depression may share commonalities, the causal link between eating and mood disorders remains to be fully understood. This etiology is highly complex, consisting of multiple environmental and genetic risk factors that interact with each other. In this review, we sought to summarize the preclinical and clinical evidence supporting a common etiology for eating and mood disorders, with a particular emphasis on signaling pathways involved in the maintenance of energy balance and mood stability, among which orexigenic and anorexigenic neuropeptides, metabolic factors, stress responsive hormones, cytokines, and neurotrophic factors.