Diagnostic criteria for apathy in clinical practice.

BACKGROUND: Apathy is an important and distressing behavioural symptom in Alzheimer's disease and in various neuropsychiatric disorders. Recently, diagnostic criteria for apathy have been proposed. OBJECTIVES: In groups of patients suffering from different neuropsychiatric diseases, (i) to estimate the prevalence of patients meeting the proposed diagnostic criteria; (ii) to estimate the concurrent validity of the criteria with the neuropsychiatric inventory (NPI) apathy item; (iii) to identify the most frequently met criteria or sub-criteria in each specific neuropsychiatric disease and (iv) to estimate the inter-observer reliability of the diagnostic criteria for apathy. METHODS: This cross-sectional, multicentric, observational study was performed on 306 patients. Each of the participating centres had to check the presence of apathy according to the diagnostic criteria for apathy in consecutive patients belonging to the following diagnoses list: Alzheimer disease (AD), mixed dementia, mild cognitive impairment (MCI), Parkinson's disease (PD), Schizophrenia (DSM-IV) and major depressive episode. In addition to the clinical interview, the assessment included the Mini Mental Score Examination (MMSE) and the NPI. At the end of the visit, clinicians were required to check the diagnostic criteria for apathy. RESULTS: Using the diagnostic criteria for apathy, the frequency of apathy was of 53% in the whole population, 55% in AD, 70% in mixed dementia, 43% in MCI, 27% in PD, 53% in schizophrenia and 94% in major depressive episode. In AD, mixed dementia, MCI and PD, the NPI apathy score was significantly higher for patient fulfilling the apathy criteria. Goal-directed cognitive activity (criteria B2-Cognition) was the most frequently observed domain followed by goal-directed behaviour (criteria B1-Behaviour) and emotion (criteria B3), respectively. Inter-rater reliability was high for the overall diagnostic (κ coefficient = 0.93; p = 0.0001) and for each criteria. CONCLUSION: This study is the first one to test the diagnostic criteria for apathy in clinical practice. Results make the diagnostic criteria useful for clinical practice and research.

[Evolution of locomotion disorders in Huntington's disease]. / Caractéristiques évolutives des troubles de locomotion dans la maladie de Huntington.

INTRODUCTION: Locomotion disorders are important in Huntington's disease (HD). Although the rates of evolution of motor, functional or cognitive aspects of HD have been studied, the evolution of locomotion disorders in early stages of the disease remains unknown. OBJECTIVES: To determine the rate of evolution of the HD-associated gait and gait initiation disorders and their correlates. PATIENTS AND METHODS: Eighteen HD patients were recorded with a minimum interevaluation interval of one year. Akinesia was studied by evaluating the anticipatory postural adjustment (APA) phase preceding the first step. We also evaluated gait speed, stride time and stride length. RESULTS: We observed an alteration in the APA phase, whose evolution was correlated with that of akinesia. We also observed a decrease in gait speed, which was due both to an increase in stride time and a decrease in stride length. Stride-to-stride variability did not worsen between both evaluations. CONCLUSIONS: A worsening in both gait initiation and gait performance was observed in HD. Initial weak functional capacity and more severe motor impairment seem to be associated with a faster progression of locomotion parameters in these mildly impaired HD patients.

[About Huntington's disease: role of families and health professionals in information transmission]. / A propos de la maladie de Huntington : rôles respectifs de la famille et des professionnels de santé dans la transmission de l'information.

INTRODUCTION: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. However, little information is available concerning the way each patient learns about the existence of Huntington's disease in his family and the way he transmits the information to his descendants. This study aims to specify the role of families and healthcare professionals in delivering information about the disease and its hereditary risk. PATIENTS AND METHODS: Data from 105 consecutive patients were analyzed. The patients were categorized in four classes according to the way they received information about HD in their family: firstly, families where the disease was known; secondly, families where the HD was "poorly known"; thirdly, families where no antecedent could be found; fourthly, families where the disease was voluntarily hidden. The majority (52%) of the patients did not know the name of HD before being diagnosed. The patient choices for disclosure of hereditary risks to their relatives were influenced by the information they received about the disease in their own family. Patients from the second category (disease "poorly known") had the most difficulty in transmitting the information. DISCUSSION: Despite the high risk of transmission, information about the disease is poorly known and transmitted in families concerned by HD. Although healthcare professionals confronted with the question of information delivery to relatives must always respect patient confidentiality, our results underline the need to more fully inform patients about the disease and transmission patterns. More help from healthcare professionals is needed to accompany HD patients concerning the question of transmitting information. The efficacy of a specific educational program should be assessed.