Platelet monoamine oxidase B activity in dementia. A 4-year follow-up.

Platelet monoamine oxidase B (MAO-B) activity has been found to increase significantly in demented patients. For the first time, a 4-year follow-up of platelet MAO-B activity and Mini-Mental State (MMS) was performed in patients with probable dementia of the Alzheimer type (DAT) and age-matched controls. MAO-B activity of DAT patients increased significantly 2 years after the beginning of the study as compared with controls and remained significantly higher for the entire period of the examinations (p < 0.0001). The decrease of the MMS scores did not correlate with the time course of the increase of platelet MAO-B activity (Spearman rank correlation test). The decline of the MMS scores of DAT patients preceded the elevation of MAO-B activity. Since degenerative processes in brain areas which are responsible for cognitive function and are reflected by the MMS scores rather affect cerebral cholinergic than monoaminergic neurotransmitter systems, degeneration of the latter at late stages of DAT might be reflected by increased platelet MAO-B activity.

Platelet monoamine oxidase B activity and vitamin B12 in dementia.

The activity of platelet monoamine oxidase B (MAO-B) was highly correlated with the severity of dementia in 39 patients suffering from probable dementia of the Alzheimer type and in 18 age-matched controls. There was no association between a low vitamin B12 level and high MAO-B activity in our sample of patients, who are living in a geriatric hospital where the balanced nutrition of inpatients is controlled by diet assistants.

Platelet MAO-B activity and the psychopathology of Parkinson's disease, senile dementia and multi-infarct dementia.

Monoamine oxidase-B (MAO-B) activity of platelets of an age- and sex-matched group of controls was compared with several groups of inpatients having non-familial dementia of Alzheimer type (DAT), Parkinson's disease (PD), multi-infarct dementia (MID), mixed types of these 3 diseases and a group of other central nervous system (CNS) organic disorders. All patients were subjected to several psychometric tests, including the Sandoz Clinical Assessment--Geriatric Scale, Hamilton Rating Scale for Depression, Mini-Mental State Examination and the Organic mental Disorder Scale (OMDS). A statistically significant enhancement of MAO-B activity could be observed in DAT patients and in PD patients, whereas the MID group showed a mean activity similar to that of the control group and the group with other organic CNS disorders. In DAT patients the degree of dementia in the OMDS test and the enhancement of MAO activity were positively correlated, but PD did not show such a correlation. It is concluded that the increase of MAO activity in PD and in DAT might be due to a disease-related enhanced affinity to oxygen and to such oxygen-derived radicals as superoxide or hydroxyl radicals. However, a possible drug-induced enhancement of MAO activity in PD cannot be excluded. Furthermore, the MAO-B activity values in platelets of individual patients or controls are not indicative of diagnosis or prognosis of any of these diseases and are of no disease-related specificity.

Disorders of verbal expression in parkinsonism.

The present study shows that rigor is one of the main causative factors in the parkinsonian speech disorder, whereas tremor ordinarily can only play a minor role. The Fast Fourier Analysis of the frequency spectra demonstrated in parkinsonian speech frequency regions that are energy-deficient and also that the entire frequency spectrum is restricted to a range of 100 to 400 Hz, including the deficient regions, as against the 100- to 700-Hz range in the controls. These analytical findings seem to underline the important role played by the laryngeal and breathing dysfunctions in the parkinsonian voice and speech production.

Long-term effects of L-deprenyl in chronic levodopa treated parkinsonian patients.

In a previous communication the results of a three months clinical trial with the co-administration of the Beta-type Monoamineoxydase (MAO)-inhibitor L-deprenyl in long-term levodopa treated Parkinsonian patients were reported. In view of the favourable effects observed in this study, as well as in others, L-deprenyl was continued in this patient group and given to other patients, found suitable, for periods of four years and more. In the 29 patients reported here, special attention was addressed to fluctuating manifestations of chronic levodopa therapy. Apart from a considerable subjective improvement, L-deprenyl effected an objective improvement in the overall disability score as well as an appreciable reduction of "on-off" phenomena in the great majority of the patients. Dyskinesias appeared in 4 of the patients and increased mildly in another ten. Untoward effects of L-deprenyl were not serious, mostly transitory, and none was prohibitive. In 13 of the 29 patients (44.83%) the levodopa dose could be reduced by 26.5% +/- 0.46, while in two patients it was raised from 250 mgm to 462.5 mgm daily. The present and previous clinical studies show that L-deprenyl is a valuable adjunctive agent for the long-term levodopa treated parkinsonian patient.

Intoxication by the chickling pea (Lathyrus sativus): nervous system and skeletal findings.

Two hundred patients with chronic neurolathyrism were examined 25-35 years after the appearance of signs and symptoms of intoxication of the chickling pea. Their daily food intake, in a German forced labor camp during World War II, consisted of 400 g Lathyrus sativus peas cooked in water plus 200 g bread baked of barley and straw. Apart from the classic signs of neurolathyrism, i.e., a spastic paraparesis, in five cases, the skeletal findings observed were similar to experimental osteolathyrism. There was an absence of ossification centers of the iliac creasts, ischial tuberosities and vertebrae; and bowing with thickening of the femoral shaft also occurred. These bony changes in human lathyrism have not been described before.

Dopamine-beta-hydroxylase (DBH) in idiopathic torsion dystonia (ITD).

Dopamine-beta-hydroxylase (DBH) has been reported to be elevated in the serum of patients with idiopathic torsion dystonia. We have examined the levels in jewish patients suffering from that disease and found levels comparable to those of age-and sex-matched controls.

Human neurolathyrism, a follow-up study of 200 patients. Part I: Clinical investigation.

Two hundred patients with chronic neurolathyrism were clinically examined, 25 to 35 years after exposure to the lathyrus sativus pea. All the patients were prisoners in a labour camp under similar nutritional and physical conditions at the outbreak of the syndome. The main symptoms were spastic paraparesis and a neurogenic bladder, both of varying degree. In addition lower motor neuron involvement with muscular atrophies was noticed in 14 cases and simulated amyotrophic lateral sclerosis. A lathyric sensory neuropathy was found in 12 patients. No cranial lesions or psychiatric disorders were found. Laboratory tests including 5 CSF specimens were normal. HLA typing was unremarkable. Our overall impression was that chronic neurolathyrism in the long run shows a gradually slow progressive course.

Human neurolathyrism, a follow-up study. Part II: Special investigations.

Special investigations were undertaken in the group of patients suffering from chronic neurolathyrism as described in part one of our work. X-ray films of the skeleton were taken of 45 patients. Pathologic findings similar to experimental osteolathyrism were revealed in 5 patients. These observations have not been described before. Sural nerve biopsies were taken from three patients with lathyric sensory neuropathy. Electron microscopy revealed mainly thick myelin sheaths which extended into the axonal regions. The picture is similar to the "tomaculous" neuropathy of Bradley. The lower motor neuron was involved in a few cases, manifested by delayed nerve conduction time and muscular atrophies.