Cognitive and psychiatric symptoms in genetically determined Parkinson's disease: a systematic review.

The aim was to review the existing reports on cognitive and behavioural symptoms in monogenic forms of Parkinson's disease (PD) and to identify recurring patterns of clinical manifestations in those with specific mutations. A systematic literature search was conducted to retrieve observational studies of monogenic PD. Data pertaining to cognitive and psychiatric manifestations were extracted using standardized templates. The PRISMA guidelines were followed. Of the 1889 citations retrieved, 95 studies on PD-related gene mutations were included: 35 in SNCA, 35 in LRRK2, four in VPS35, 10 in Parkin, three in DJ1 and eight in PINK1. Nineteen studies (20%) provided adequate data from comprehensive cognitive assessment and 31 studies (32.6%) outlined psychiatric manifestations through the use of neuropsychiatric scales. Cognitive impairment was reported in all monogenic PD forms with variable rates (58.8% PINK1, 53.9% SNCA, 50% DJ1, 29.2% VPS35, 15.7% LRRK2 and 7.4% Parkin). In this regard, executive functions and attention were the domains most affected. With respect to psychiatric symptoms, depression was the most frequent symptom, occurring in 37.5% of PINK1 cases and 41.7% of VPS35 and LRRK2 cases. Co-occurrence of cognitive decline with visual hallucinations was evidenced. Widespread accumulation of Lewy bodies, distinctive of SNCA, PINK1 and DJ1 mutations, results in higher rates of cognitive impairment. Similarly, a higher degree of visual hallucinations is observed in SNCA mutations, probably owing to the more widespread accumulation. The lower rates of α-synuclein pathology in LRRK2 and Parkin may underpin the more benign disease course in these patients.

[Penaly ordered care in France: principles of procedures and caregiver's role]. / Les soins ordonnés par la Justice en France : principes des procédures et place du médecin.

BACKGROUND: Penaly ordered care constitutes a type of legal penalty and a form of special care, linking health and legal environments, and as such is a difficult exercise for the various parties involved. METHODS: This article is based on a comprehensive study of medical and legal literature on the subject. RESULTS: Each of the measures presented has a legal basis, procedures for implementation and different application fields. According to the measure, the caregiver has a defined role in the organization of care and flexibility in dealing with specific legal authorities. Doctors are often uncertain of their rights and duties in this type of care. CONCLUSION: Penaly ordered care requires cooperation between two professional bodies with different ethical and professional requirements. Beyond this first difficulty, it appears that these measures are also complicated by the many pieces of legislation published recently, stressing the political will of the ever-expanding scope of ordered care, despite the lack of means downstream, sometimes to the detriment of their effectiveness.

Severe obsessive-compulsive syndrome in a 10 year old: a 3 year follow-up.

Obsessive-Compulsive Disorder is rare in children but when present, its very nature is so pervasive and so complex that the therapy must be specially tailored to fit the distinct character of the young patient. We report the course and treatment of one case; then we discuss the interplay of implicit as well as explicit factors in the active process of alleviating dysfunctional behavior.

[Comparison of masked and endogenous depression using psychometric scales, endocrinological markers and pharmacological responses. Masked depression versus endogenous depression]. / Comparaison de la dépression masquée et de la dépression endogène à l'aide d'échelles psychométriques, de marqueurs endocrinologiques et de réponses pharmacologiques. Dépression masquée versus dépression endogène.

Masked depression refers to a concept of a phenomenological state, either endogenous or psychogenic where somatic symptoms replace sadness: Thirty patients were evaluated by RDC (22 endogenous and 8 masked depressions) wherein in the latter dysphoria was replaced by a nonreactive persistent somatic complaint. They were rated on Beck and Hamilton Depression Scales, on Hamilton and Trait-State Anxiety Scales and the NOSIE. All patients presented with insomnia, anorexia, loss of weight, diminished libido and anhedonia. Initial ratings were similar for both diagnostic groups except for a significantly higher agitation factor and lower retardation in masked depression. Although 59.9 percent of the subjects are positive on the dexamethasone test, only 1 masked depression did not suppress secretion of cortisol. After a randomized 30-day drug trial where patients were assigned to Clomipramine or Desipramine, patients in both groups show significant improvement on rating scales but diagnostic group drug treatment interaction exists on anxiety and agitation criteria.