O.J.S. Buruma

The MPTP model: versatile contributions to the treatment of idiopathic Parkinson's disease

In human and subhuman primates, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) produces irreversible clinical, biochemical and neuropathological alterations highly reminiscent of those observed in Parkinsons disease. The MPTP model has provided the best available tool to date for the assessment of efficacy and side-effects of symptomatic treatments of Parkinsons disease. In addition, the mechanism of action of MPTP has offered a basis for the development of novel therapeutic strategies aimed at the prevention of Parkinsons disease.

Cognitive Function after Spinal or General Anesthesia for Transurethral Prostatectomy in Elderly Men

Cognitive functions in 53 elderly men who underwent a transurethral prostatectomy were assessed pre‐operatively and 4 days and 3 months post‐operatively. Thirteen patients had a preference for one particular type of anesthesia, and the remaining 40 were randomly allocated to receive either spinal or general anesthesia. Cognitive function was not different between the groups receiving different types of anesthesia at either time point and did not decrease post‐operatively. No pre‐ or perioperative variable could distinguish the subgroup of patients who had a post‐operative decrease of 2 points or more on the Mini‐Mental State Examination. No difference in post‐operative performance was found in the patient groups with pre‐operative Mini‐Mental State Examination scores above or under their age‐specific norm. It is concluded that neither hospitalization nor the two forms of anesthesia investigated cause a decrease in cognitive function in elderly men.

SPECT in the diagnosis of Alzheimer's disease and multi-infarct-dementia

SPECT with Tc-99m HM-PAO as a radiopharmaceutical was performed in 17 patients meeting research criteria for Alzheimers disease (AD), in 10 patients with a clinical diagnosis of multi-infarct-dementia (MID) and in 12 healthy volunteers. Regional tracer uptake was measured in frontal, parietal, and temporoparietal regions. A statistically significant decrease of tracer uptake in the temporoparietal region was found in AD-patients compared with controls. AD-patients showed less activity in this region than MID-patients, but this difference did not reach statistical significance. In both AD- and MID-patients decrease of tracer uptake was not correlated with dementia severity. We conclude that SPECT brain imaging is not yet ready for routine use in the distinction between AD and MID.

Respiration during sleep in Huntington's chorea

In view of recent reports on lower brainstem dysfunction in Huntingtons chorea, we studied respiration during sleep in 12 patients with Huntingtons chorea (HC) and in controls. There were no statistically significant differences between patients and controls with respect to apnea periods, respiratory frequency and time elapsed between minimal and maximal value of the respiratory curve. No statistically significant differences in respiratory variability were observed between patients and controls. In the present study, no indication was found for dysfunction of lower brainstem structures involved in respiration in HC.

Diurnal variation of essential and physiological tremor.

Evaluations were made of the diurnal variations of tremor power at rest, after fatigue and after mass loading, and plasma norepinephrine in patients with familial essential tremor and normal subjects. Diurnal tremor power rhythms for both essential and physiological tremor pursued identical temporal profiles. Plasma norepinephrine levels followed a congruent diurnal pattern with later peak values than the peak values of tremor power. Sympathetic nervous system activity is unlikely to be the cause of diurnal tremor power variation. The consistent diurnal rhythm of tremor power may affect dosage schemes of tremorolytic drugs.

Myopathy in familial hypokalaemic periodic paralysis independent of paralytic attacks

Case histories and muscle biopsy findings are reported of two members of a family suffering from hypokalaemic periodic paralysis associated with permanent muscular weakness. the first patient aged 9 years, as well as her affected sibs, gives a history of a slight and slowly progressing impairment from early childhood on. At the age of 8 she exhibited slightly reduced muscle strength occasionally in the morning, with a frank paralytic attack only on two occasions. Physical examination revealed slight proximal muscle weakness, predominantly in the legs. Light microscopic examination as well as ultrastructural analysis of the biopsy revealed large vacuoles in many muscle fibres, mostly containing glycogen. the second patient, a man aged 18 years, never experienced a paralytic attack nor any fluctuation in muscle strength. Though his muscular strength was sligthly below expectation, no frank paresis could be detected on examination. the EMG revealed no abnormalities except for slight myopathic changes bilaterally in the adductor magnus. Light microscopic examination of the muscle biopsy showed no pathological changes. Accumulations of glycogen and secondary atrophy and disappearance of muscle fibrils were seen with the electronmicroscope.

Neurological complications in patients with cutaneous T-cell lymphoma

Five patients with a cutaneous T‐cell lymphoma (four with mycosis fungoides and one with a primary cutaneous T‐immunoblastic lymphoma) who developed neurological sequelae are reported. In four of them, central nervous system involvement could be demonstrated at autopsy. The importance of early cerebrospinal fluid examination in these patients is stressed.

Heart muscle disease in familial hypokalaemic periodic paralysis

The case history, skeletal muscle and heart muscle biopsy findings from an affected member of a family suffering from hypokalaemic periodic paralysis associated with permanent muscular weakness are reported. The patient, a female aged 35 years, while treated with 750 mg and later 1000 mg of acetazolamide daily, developed typical exercise angina pectoris. The ECG during exercise showed ST‐segment depression. A coronary angiography was performed because coronary artery disease was suspected. To exclude cardiac muscle disease a biopsy of the left ventricular wall was taken. The angiography was normal. Ultrastructural analysis of the biopsy specimen showed an unusual amount of intermyofibrillary glycogen resembling, although far less abundant, the increase of glycogen found in the skeletal muscle biopsy specimen obtained from the same patient.

Quantification of choreatic movements by Doppler radar

A simple and effective method is described to quantify involuntary movements. The method is based on the Doppler radar principle. Reproducibility of the method is demonstrated in a group of patients with Huntingtons chorea and Tardive Dyskinesia. It is shown that the method correlates with clinical assessments, but is preferable because of its objectivity and simplicity.

Oculomotor disturbances in extra-pyramidal disorders. A review of the literature

Oculomotor disturbances have been described in Parkinson’s disease, in progressive supranuclear palsy (Steele-Richardson-Olszewski’s disease), in Wilson’s disease and in Huntington’s chorea.