Clinical significance of self-descriptive apathy assessment in patients with neurological form of Wilson's disease.

BACKGROUND AND AIM: Apathy is one of the neuropsychiatric symptoms of Wilson's disease (WD) which typically affects the brain's fronto-basal circuits. Lack of agreed diagnostic criteria and common use of self-description assessment tools lead to underestimation of this clinical phenomenon. The aim of this study was to investigate whether subjective and informant-based clinical features of apathy in patients with WD enable clinicians to make a valid diagnosis. METHODS: Multiple aspects of goal-oriented behavior were assessed in 30 patients with the neurological form of WD and 30 age-matched healthy participants using two questionnaires, the Lille Apathy Rating Scale (LARS) and the Dysexecutive Questionnaire (DEX). Both included a self-descriptive and a caregiver/proxy version. Cognitive functioning was estimated with the use of Addenbrooke's Cognitive Examination-Revised. RESULTS: Patients obtained significantly worse scores on all clinical scales when more objective measures were considered. Features of apathy and executive dysfunction were revealed in patients' caregiver versions of LARS and DEX, which may indicate poor self-awareness of patients with WD. Roughly 30% of participants were likely to present with clinically meaningful symptoms, independent of cognitive dysfunction. CONCLUSIONS: Methods relying on self-description appear inferior to informant-based scales when diagnosing apathy. More objective criteria and measurement tools are needed to better understand this clinical syndrome.

Neuropsychological characteristics of encephalopathy in Susac's Syndrome - Case report.

Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning. The initial assessment revealed attention, memory and executive dysfunctions, as well as behavioral changes including impulsivity, affective dysregulation and reduced self-awareness of disease deficits. After five weeks recovery process supported by rehabilitation program, improvement was observed, although some cognitive-behavioral deficits were still present in the follow-up assessment.

[The value of self-report methods in neuropsychological diagnostics of patients after brain injury]. / Wartosc pomiaru samoopisowego w diagnostyce neuropsychologicznej pacjentów z uszkodzeniem mózgu.

Self-report methods are commonly applied in medicine and psychology. However, their diagnostic value in assessment of patients with acquired brain damage can be limited due to a number of various difficulties encountered by these patients, including cognitive disorders, fatigue, similarity of psychopathological and somatic symptoms, psychological reaction to the disease and limited or lack of insight (anosognosia). In our article we highlight the most important limitations of application of popular frequently used questionnaires in evaluation of brain-injured patients. We also discuss the possible ways of adjusting self-report techniques to requirements of diagnostic process in this clinical population. Proposed modifications refer to, among others, specific construction of diagnostic tools (appropriate content and number of questions, methods of measuring responses), collecting information from relatives, using questionnaires along with more objective measures, and controlling conditions in which the assessment is carried out. Although relying only on self-report methods can be misleading, for an aware clinician it is still an important source of data on patient's subjective opinion and on the way they experience their symptoms.