Clinical Features of Mental Impairments in Amyotrophic Lateral Sclerosis

摘要

Objectives. To study the clinical features of mental impairments in amyotrophic lateral sclerosis (ALS). Materials and methods. A total of 27 patients with lesions to central and peripheral motoneurons (nine men and 18 women) were investigated. Patients’ mean age was 59.8 ± 11.9 years. Mean duration of illness at the moment of investigation was 3.2 ± 2.4 years. Clinical-psychopathological and clinical follow-up methods were used. Quantitative assessment of the severity of cognitive impairments were obtained using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) and depressive impairments on the Montgomery– Asberg Depression Rating Scale (MADRS). Results. Cognitive impairments were found in 88.9 of subjects. Mnestic impairments were predominantly hypo- and dysmnesia. Thinking speed in these patients was slow, and torpidity was combined with circumstantiality and a tendency to excess detail. Attention on the background of general instability was characterized by a decrease in volume, an increase in distractibility, and difficulty concentrating. At the late stages of illness, there was an overall decline in the level of thinking and intellect, with marked perseveration and an inability to make appropriate self-assessment. More than half the patients showed symptoms of dynamic aphasia, as well as impairments to gnosis and praxis. Most (92.3) patients showed affective impairments. Increases in the severity of symptomatology of the main neurological disease were linked with associated depressive symptomatology coming to the fore (62.9). Particular attention should be paid to clinical cases of ALS associated with psychotic disorders in the form of acute schizophrenia-like psychotic states. Conclusions. The data obtained here provide evidence of affective, cognitive, and behavioral impairments, along with a specific neuroimaging picture, the totality of which constituted a manifestation of the behavioral variant of frontotemporal dementia. This supports the current view that ALS and frontotemporal dementia have much in common, which allows these diseases to be regarded as different phenotypic manifestations of single pathological process.