No abstract

Forty-four elderly patients of both sexes (mean age 76.5 years) suffering from depressive pseudodementia were intensively treated for the depression. When that cleared up, cognitive function reverted to premorbid level. Patients were regularly interviewed and retested at six months intervals for four to 18 years (average 8). Some patients experienced, during the follow-up period, a recurrence of the depression for which they were again successfully treated. At the end of the observation period, 39 of the 44 patients (89%) had developed a dementia syndrome of the Alzheimer type.

The symptomatology and treatment of the various types of depressions found in Alzheimer patients are briefly described as well as a discussion of some of the interesting theoretical problems that are posed.

Fabry's disease was diagnosed in an adult patient as a lipid storage-induced non-obstructive hypertrophic cardiomyopathy. Stable angina pectoris started 15 years before death, was followed by slowly progressive heart failure and repeated pulmonary thromboembolism with death at 63 years. Autopsy disclosed enormous cardiomegaly (1100 g), cardiac storage of ceramide trihexoside (CTH) of the same intensity as in classical cases of generalized Fabry's disease (11 mg lipid/g wet weight) restricted to cardiocytes. Other tissues (liver, kidney, brain, pancreas, pulmonary artery, coronary arteries) were free of storage. Using proton magnetic resonance analysis on formaldehyde-fixed tissue the stored CTH was identified as globotriaosylceramide. It was enzymatically degradable by control cell cultures but left uncleaved by mutant reference Fabry cells. Alpha-galactosidase activities in peripheral leucocytes of all four of the patient's daughters were in the heterozygous range. The diagnostic difficulties in this monosymptomatic novel variant of Fabry's disease are stressed.

The London Psychogeriatric Rating Scale (LPRS) was developed in answer to the demand for a reliable psychometric rating scale suited to a psychogeriatric population. All inpatients on a Psychogeriatric Unit (approximately 140 at any specific time) were rated with the LPRS every third month for an 18-month period. The 4 components of the total score include a measure of mental status originally derived by factor analysis. Predictive validity has been established in terms of various clinically relevant areas including the following: a) ward placement; b) outcome (continued hospitalization, discharge, or death); c) diagnosis (by scoring levels and progress patterns across time); and d) ability of the patient to function in, or benefit from, a particular treatment program. The scale has been used successfully to assess a given patient's progress quantitatively and globally over a long period.