Abstract

Fifty patients from a longitudinal study on 178 cases of Alzheimer's disease were examined at postmortem. The clinical features, CT-scans and neuropathological findings of five patients, with verified Alzheimer's disease, who had bilateral basal ganglia mineralization (BGM; 2 male, 3 female; age 78–91 years) were compared with the data of five age- and sex-matched Alzheimer patients without BGM and of five control subjects. Persecutory and other delusions (4 patients), persistent depression (2), parkinsonism (4), myoclonus (1) and epileptic seizures (1) were observed more frequently in the patients with BGM than was expected. The BGM-group had significantly lower counts of large neurons in the pallidum internum than the demented patients without BGM or the control group. We did not find other differences between the dementia groups regarding the CT-scans, or plaque, tangle and neuron counts in neocortex and brainstem. We suggest that the combined effects of Alzheimer pathology and BGM might lead to an increased manifestation of psychotic and motor disturbances.

Highlights

  • Pick (1905) and Taft (1916) were among the first to comment on a possible relationship between extensive cerebral mineralization and neuropsychiatric disease, e.g. tetany, epilepsy, depression, schizophrenia and dementia

  • Later the name ofFahr (1930), who had described only one patient with predominant white matter mineralization, became associated with basal ganglia mineralization (BGM) even though BGM had already been reported in mid 19th century (Virchow, 1854)

  • The main findings of our study were: -Four of five patients with Alzheimer's disease and BGM suffered from delusions, two were depressed over a long period of time, four developed signs of parkinsonism, one myoclonus and one cerebral seizures. -Apart from the BGM itself, the CT-scans did not shown any other differences between the demented patients with and without BGM. -Patients with BGM showed a significantly lower number of large neurons in the pallidum internum than the patients without BGM and the controls

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Summary

INTRODUCTION

Pick (1905) and Taft (1916) were among the first to comment on a possible relationship between extensive cerebral mineralization and neuropsychiatric disease, e.g. tetany, epilepsy, depression, schizophrenia and dementia. An increased rate of BGM has been suspected in patients with seizures, in patients receiving antiepileptic drugs and in patients suffering from movement disorders (Puvanendran et at., 1982; Kazis et at., 1985). The specificity of such associations has been questioned and it has been suggested that various underlying metabolic, familial or infectious diseases, which cause the neurological and psychiatric symptoms, may be responsible for BGM (Forstl et at., 1991a, 1992; Philpot and Lewis, 1989). These results are compared to an age- and sex-matched sample ofpatients with Alzheimer's disease with and without BGM and to a control group without neuropsychiatric disease

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