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Biomedical subjects

R Nyberg-Hansen

Publications and source records attributed to R Nyberg-Hansen.

At least 55 records · Page 3Linked to original sources
PubMed1988

Neurological disorders and phytanic acid metabolism.

Fifty patients with neurological symptoms and signs resembling those of Refsum's disease were analyzed for phytanic acid in serum. In addition, the phytanic acid oxidase capacity in skin fibroblasts was determined. The patients suffered from retinitis pigmentosa, cerebellar ataxia and/or chronic polyneuropathy of unknown cause. The serum level of phytanic acid was not increased, and the alpha-oxidation of (1-14C) phytanic acid was found to be normal in all patients. The present investigation lends no support to the theory that so-called atypical or mild cases of Refsum's disease exist. This disorder appears to be a disease entity with a specific genetic phenotype.

Adolescent↗
PubMed1988

Visual evoked responses in hereditary motor and sensory neuropathies.

The P2 latency of the pattern reversal visual evoked response was measured in 11 patients with hereditary motor and sensory neuropathy (HMSN). The P2 latency was inversely related to the peripheral nerve conduction velocity, but no significant relation was found between the P2 latency and the age of the patients or the duration of their symptoms. When the whole group was considered the mean P2 latency was longer but not significantly different from a control group. When the 11 patients were differentiated into HMSN I (5 males) and HMSN II (2 females, 4 males) the P2 latencies of the patients with HMSN I were significantly longer than those with HMSN II. The data support earlier findings that subclinical involvement of the optic nerve may occur in HMSN and suggest that measurement of P2 latency may be of value in the differentiation of HMSN. More than two subtypes, however, may exist.

Adolescent↗
PubMed1988

Myasthenia gravis treated with ciclosporin.

Six patients (4 females, 2 males) with generalized myasthenia gravis (MG) have been treated in an open study with ciclosporin (5-10 mg/kg/day), originally for 28 weeks. The age of the patients ranged from 16-61 years and the duration of symptoms from 5-30 years. Four of the patients had been thymectomized. Ciclosporin was added to prednisolone in 5 of the patients. Three of these had in addition previously used azathioprine without satisfactory improvement. The efficacy was measured by scoring the degree of muscle weakness on clinical examination and by scoring of the patients' personal assessment of their fatigability and muscle strength. In 5 patients there was a moderate to marked improvement in muscle strength and fatigueability on ciclosporin therapy. In one patient the effect was slight to moderate. The degree of improvement was similar in the one patient who received ciclosporin alone and in those also using prednisolone. The levels of anti ACh-r antibodies decreased in 3 patients, increased in 2, and were unchanged in one patient. Side effects known to occur on ciclosporin therapy were noticed, but they were tolerable in all patients. An increase in serum creatinine (mean 10%) and decrease in creatinine clearance (mean 21%) were seen in all patients. The treatment with ciclosporin has been continued in all 6 patients, but the dosage has been reduced to 5 mg/kg/day. Deterioration 3-4 weeks after discontinuation of ciclosporin was seen in one case, but resumption of therapy resulted in improvement as before. The total treatment period is now 24-34 months (March, 1987).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Amaurosis fugax: clinical, Doppler and angiographic findings.

Clinical, Doppler and angiographic findings are described in 53 consecutive patients who presented with amaurosis fugax (AF) in a total of 57 eyes; 4 had non-simultaneous attacks in both eyes. Atherosclerotic lesions were detected on Doppler or angiographic (conventional arteriography and/or intravenous digital subtraction angiography) examination in 36 (63%) of the relevant precerebral internal carotid arteries (ICA) in 34 patients. Nineteen (53%) of these lesions caused a diameter reduction of more than 75%. Patient age was the most important factor in predicting the presence of relevant carotid occlusive disease, all 36 lesions being found in patients over 50 years of age. Two unusual cases of AF are described; in one AF was caused by stenosis of the ipsilateral ophthalmic artery, and in another by occlusion of the brachiocephalic artery with a steal syndrome from the right common carotid artery (CCA) to the right subclavian artery.

Adolescent↗
PubMed1986

Multimodal evoked responses and cerebrospinal fluid oligoclonal immunoglobulins in patients with multiple sclerosis.

One hundred patients with possible, probable and definite multiple sclerosis (MS) were examined with somatosensory (SER), visual (VER) and brain stem auditory (BAER) evoked responses. Paired samples of cerebrospinal fluid (CSF) and serum were examined with agarose gel electrophoresis to detect intrathecally synthesized oligoclonal immunoglobin bands. Comparison of the number of abnormal CSF and evoked response tests showed that the CSF examination was slightly more sensitive in all diagnostic groups when compared to the results of multimodal evoked responses but that the two sets of test were in part supplementary. Oligoclonal immunoglobulin bands in the CSF were present in all patients with a duration of the disease of less than six months. VER seemed to be the most sensitive of the evoked tests in this particular group of patients.

Brain Stem↗
PubMed1986

Herpes zoster ophthalmicus with cerebral angiitis and reduced cerebral blood flow.

Two patients with herpes zoster ophthalmicus (HZO) who experienced a delayed contralateral hemiparesis, the so-called crossed zoster syndrome, are described. Particular emphasis is paid to the cerebral blood flow (CBF) findings studied with the Xenon-133 inhalation technique using single photon emission computed tomography (SPECT). In a 40-year-old female with right-sided hemiparesis, angiography showed multiple segmental narrowings of the intracerebral arteries. Cerebral computer tomography (CT) scans were normal. The CBF studied 11 months after the HZO showed a generalized reduction of flow which, however, was more pronounced in the left hemisphere. On re-examination 8 months later both the mean hemispheric flow and regional CBF (rCBF) had increased to normal values. In a 66-year-old male with dysphasia and right-sided hemiparesis, cerebral CT scans demonstrated two small deep left-sided infarcts. CBF examination showed a generalized reduction of flow in the left hemisphere. The flow was slightly increased on re-examination 12 months later. These findings suggest that the Xenon-133 inhalation method represents a useful way to demonstrate the CBF pattern in this group of patients.

Adult↗
PubMed1986

Patency and flow velocity profiles in the internal carotid artery assessed by digital subtraction angiography and Doppler studies three months following endarterectomy.

Pulsed Doppler spectral analysis and intravenous digital subtraction angiography have been carried out in 55 patients, three months after a total of 60 carotid endarterectomies. Five patients were operated on both sides and there were no re-operations. There were no re-stenoses and only one of the 60 internal carotid arteries was occluded. Spectral spreading of of the Doppler signal at the site of the endarterectomy was found in 52% of the internal carotid arteries, whereas wall irregularities were demonstrated in 25% by intravenous digital subtraction angiography. A stenosis or occlusion was found in 11 (18%) of the 60 ipsilateral external carotid arteries.

Carotid Artery Diseases↗
PubMed1986

Reduced cerebral blood flow in high altitude climbers.

Cerebral blood flow (CBF) was studied by 133Xe inhalation dynamic single photon emission computer tomography in 8 members of a climbing expedition to the Himalayas. With one exception they had all previously climbed at high altitudes. All stayed above 6,500 m for approximately 3 weeks, and 5 reached the summit of Mt. Everest. CBF was measured in Oslo before, immediately after, and one year after the completion of the expedition. Measurements were made at rest and following the injection of 1 g acetazolamide intravenously. As reference group was used 13 healthy male subjects of similar age from the hospital staff. Ten age-matched male diving instructors formed a second control group. In the climbers a small, but not significant reduction in CBF was seen after the expedition. On the other hand, they had significantly lower CBF than reference subjects already before the expedition. The flow difference was most pronounced corresponding to the perfusion territory of the middle cerebral artery. One year after the completion of the expedition the average CBF in climbers was still more than 15% lower than in the reference group. The climbers had higher relative flow increase after acetazolamide injection than the reference subjects, showing that the functional capacity of the microvascular system of the brain was intact. Whether neuronal activity or number of neurones is reduced in climbers proportional to the decrease in flow, or maintained at normal level by increased oxygen and glucose extraction, cannot be answered by the present data.

Acclimatization↗
PubMed1986

Measurement of cerebral blood flow with 133Xe inhalation and dynamic single photon emission computer tomography. Normal values.

Cerebral blood flow (CBF) was studied by 133Xe inhalation tomography in 25 healthy subjects. Mean age was 41 years and range 23-66 years. Mean hemispheric CBF at rest was 59.8 ml/100 g/min, and cerebellar flow 60.8 ml/100 g/min. This difference was not statistically significant. The distribution of CBF values was skewed and approximated a log normal distribution. Estimated lower and upper normal reference range limits calculated as mean (log) +/- 2 S.D. (log) were 47-74 ml/100 g/min. Women had approximately 5 ml/100 g/min higher CBF values than men. This difference corresponded to the difference in hematocrit. Neither in men nor in women was there any tendency to age dependent reduction or increase in flow. In both sexes hemispheric regional CBF (rCBF) was asymmetric with higher flow values in the right cerebral hemisphere; particularly in the anterior distribution territory of the middle cerebral artery. In this region the maximum individual flow difference was 8 ml/100 g/min and the average difference 3.5 +/- 2.6 ml/100 g/min. Emotional activation as a consequence of the study conditions is assumed to be the cause of this observed asymmetry. Cerebellar flow was not assymetric. No significant difference in cerebellar or hemispheric CBF was found when a second study followed the first by 3-15 months. PCO2 correction of flow improved the reproducibility. The standard deviation of the flow difference between test/retest measurements was 5 ml/100 g/min. After 1 g acetazolamide given intravenously the mean increase in hemispheric CBF was 15.7 +/- 5.1 ml/100 g/min.(ABSTRACT TRUNCATED AT 250 WORDS)

Acetazolamide↗