Search PubMed⌕ Search

Biomedical subjects

R Haller

Publications and source records attributed to R Haller.

At least 37 records · Page 2Linked to original sources

[Clinical early symptoms and CT findings in Fahr syndrome].

We present the results of an investigation on initial symptomatology of patients with bilateral, symmetrical intracerebral calcification of the basal ganglia (Fahr's syndrome). 62 patients, who because of various neurological or psychiatric symptoms or other reasons were referred to cranial computer-tomography, revealed clear manifestation of Fahr's syndrome. In these cases estimations of the volume of the opaque bodies were made, based on computer-integrated programmes (SO-MATOM D2). In 9 cases there was a history of thyroidectomy, on average 25 years previously. As a rule patients were referred in the age range between 40 to 60 years, those who had undergone thyroidectomy being on average somewhat older than the others. As presenting symptoms extrapyramidal syndromes, apoplexias, cephalea, affective organic alterations, alcoholism and dementia were noted. Cerebral localisation of bilateral, symmetrical calcification was most frequent in the pallidum, though this localisation may be responsible for numerous different neuropsychiatric symptoms. The dimensions of the opaque structures can be very greatly. Symptomatology seems to be practically independent of either cerebral localisation or volume, except that greater volumes of calcification seem to cause more pronounced neurological (extrapyramidal) symptomatology and dementia. The time-span between manifestation of the initial symptoms and diagnosis was shorter in neurological syndromes than in psychiatric cases. Like earlier workers, we found a high incidence (21%) of organic affective syndromes as an initial manifestation of Fahr's syndrome.

Adult↗

[Polythelia in alcoholics. Preliminary report].

We present a study comparing 650 alcoholics with 1074 patients with miscellaneous other psychiatric (665) and medical (409) diagnoses for the frequency of supernumerary nipples (SN). Also 716 adolescents and school-children in a general medical screening were investigated for this phenomenon. In alcoholics SN were found in over 6%, in the non-alcoholic group in approximately 1%. The latter figure correlates well with other findings in literature, 6% being significantly higher. In the group of adolescents/children (1,8-3,2%) polythelia correlates with familial alcoholism, albeit of the father. As polythelia may be part of fetal alcohol-syndrome, the addiction should be expected on the mother's side, the more so, as the local rate of female/male alcoholism is approximately 1:3 (administrative incidence). We suggest that polythelia may be more frequent among alcoholics. Apart from SN occurring in alcoholic embryopathy, alcoholism of the father may be an additional factor for their development. Polythelia may in certain cases offer a diagnostic clue not only for mammo-renal syndromes but also for individual or familial alcoholism.

Adolescent↗

[Results of a study of a family with aggregated occurrence of bilateral symmetrical basal ganglia calcinosis].

We present a familial study (45 members), in which 31 members have been examined. Seven were afflicted with bilateral symmetrical calcification of the basal ganglia (Fahr's syndrome), as verified by CT scans. The case history and biochemical results for one additional proband, who had died, strongly indicate that this patient also had Fahr's syndrome. The wide range of examinations used in our study were aimed at excluding differential diagnoses of bilateral symmetrical calcification of the basal ganglia, other than when the origin was suspected to be in the parathyroid. The examinations had to be undertaken on an outpatient basis. Some of the variables, such as Fe, Cu, and Mg in the plasma, are connected with results that have been published on the composition of the apatite deposits. Psychiatric, psychological, neurological, and EEG examinations are emphasized. Together with the case reports they are meant to illustrate the neuropsychiatric aspects of this syndrome, the composite view of which has often been previously neglected. Nineteen of 31 probands showed neurological, psychopathological, psychological, and encephalographical deviations. We also noted a high incidence of organic brain syndromes that are phenomenologically similar to affective disorders. We believe these deviations to be directly related to morphological alterations of the basal ganglia, possibly due to errors in phosphorous and calcium metabolism. Although the CT scan has greatly facilitated the diagnosis of intracerebral calcifications, we assume that basal ganglia alterations under the CT-density threshold may also be of clinical importance. Our metabolic results, genetic issues, and neuropsychiatric findings are discussed.

Adolescent↗

[Effects of metacaine and its decomposition products on the excitation mechanism of isolated myelinated nerve fibers].

The blocking effects of metacaine-methanesulfonate (MS-222; abbreviated MMS) and of the corresponding hydrochloride (MHC) on the ionic currents in potential clamped myelinated nerve fibres were investigated. The dose-response relationships of MMS for blocking the Na-currents and K-currents could be satisfactorily fitted to the Langmuir adsorption isotherm with KNa = 0.7 mmol/l and KK = 1.8 mmol/l. The observed loss of efficacy in blocking ionic currents of 3-day-old solutions containing MMS was assumed to be due to a partial decomposition of the drug. We separated the water-soluble decomposition products by extraction of the metacaine base. The remaining aqueous solution showed an anti-blocking effect on metacaine blocked Na-currents. From corresponding experiments on the blocking effect of MHC we conclude that the anti-blocking effect is due to yet unknown decomposition products, formed in MMS but not in MHC solutions.

Aminobenzoates↗

[Headache and EEG changes caused by acetate and bicarbonate dialysis].

10 patients suffering from severe headache during Acetatedialysis were subsequently treated with Acetatedialysis (AD) and Bicarbonatedialysis (BD). During AD the headaches occurred more frequently and more intensely. After AD a deterioration of EEG-results was also seen more frequently than after BD. Urea, osmolarity and sodium in the blood as well as heart frequency and blood pressure showed no different variation. An essential difference was found in correction of the metabolic acidosis. After AD there could be seen a negative base excess and a fall in PaCO2, after BD the PaCO2 rose and the base excess was positive. Headaches and EEG-changes as signs of a cerebral dysfunction (disequilibrium syndrome) may perhaps be caused by a decrease of the cerebral blood flow. From our experience we recommend a change to BD for patients suffering from headaches during AD.

Acetates↗

SMS 201-995: a very potent and selective octapeptide analogue of somatostatin with prolonged action.

Stepwise modification of a conformationally stabilised analogue of the fragment of somatostatin which had been thought to be essential biologically active moiety has enabled us to synthesise the analogue H-(D) Phe-Cys-Phe-(D) Trp-Lys-Thr-Cys-Thr(ol) code-named SMS 201-995, which in vitro is three times more potent than the native hormone in inhibiting the secretion of growth hormone, which is highly resistant to degradation by pure enzymes and by tissue homogenates, which in vivo in rat and rhesus monkey is (depending on test system) at least 20 times more active than somatostatin, which is much longer acting, and which moreover in both species is much more selective in inhibiting the secretion of growth hormone than that of insulin. The compound is active by several routes of administration including the oral, is well tolerated both in laboratory animals and in man, and is currently undergoing preliminary clinical trial.

Animals↗

[Multidisciplinary diagnostic approach to Fahr's syndrome, a familial disease (author's transl)].

Fahr's syndrome is characterized by idiopathic non-arteriosclerotic, symmetric, intracerebral vascular sclerosis. On the basis of relevant literature and our own research, the necessity of multidisciplinary diagnosis is reviewed: psychiatric, neurological, medical, paediatric and radiological approaches are discussed. The early onset of diverse psychological alterations is regarded as significant. Extensive diagnosis seems justified by the at least theoretically possible means of prophylaxis and treatment in cases of Fahr's syndrome associated with hypoparathyroidism, pseudo-hypoparathyroidism and pseudo-pseudo-hypoparathyroidism. This condition otherwise irreversibly leads to dementia.

Adolescent↗

Hypophosphatemia and rhabdomyolysis.

Clinical observations suggest that overt rhabdomyolysis may occur if severe hypophosphatemia is superimposed upon a pre-existing subclinical myopathy. To examine this possibility, a subclinical muscle cell injury was induced in 23 dogs by feeding them a phosphorus- and calorie-deficient diet until they lost 30% of their original weight. To induce acute, severe hypophosphatemia in the animals after partial starvation, 17 of the dogs were given large quantities of the same phosphorus-deficient diet in conjunction with an oral carbohydrate supplement, which together provided 140 kcal/kg per day. After phosphorus and caloric deprivation, serum phosphorus and creatine phosphokinase (CPK) activity were normal. Total muscle phosphorus content fell from 28.0+/-1.3 to 26.1+/-2.5 mmol/dg fat-free dry solids. Sodium, chloride, and water contents rose. These changes resembled those observed in patients with subclinical alcoholic myopathy. When studied after 3 days of hyperalimentation, the animals not receiving phosphorus showed weakness, tremulousness, and in some cases, seizures. Serum phosphorus fell, the average lowest value was 0.8 mg/dl (P <0.001). CPK activity rose from 66+/-357 to 695+/-1,288 IU/liter (P <0.001). Muscle phosphorus content fell further to 21.1+/-7.7 mmol/dg fat-free dry solids (P <0.001). Muscle Na and Cl contents became higher (P <0.01). Sections of gracilis muscle showed frank rhabdomyolysis.6 of the 23 phosphorus- and calorie-deprived dogs were also given 140 kal/kg per day but in addition, each received 147 mmol of elemental phosphorus. These dogs consumed their diet avidly and displayed no symptoms. They did not become hypophosphatemic, their CPK remained normal, and derangements of cellular Na, Cl, and H(2)O were rapidly corrected. The gracilis muscle appeared normal histologically in these animals. These data suggest that a subclinical myopathy may set the stage for rhabdomyolysis if acute, severe hypophosphatemia is superimposed. Neither acute hypophosphatemia nor rhabdomyolysis occur if abundant phosphorus is provided during hyperalimentation.

Animals↗

Endogeneity and reactivity as orthogonal dimensions in depression.

Endogeneity and reactivity have long been considered polar opposites of a major dimension of depression. The present factor analytic study examines 35 distinct depressive symptoms in three depressed samples and supports the conceptualization of D. F. Klein (Endomorphic depression. Arch. Gen. Psychiatry, 31:447-454, 1974) that endogeneity and reactivity can be construed as orthogonal, independent dimensions. Symptoms consistently loading on an endogeneity factor are: 1) lacking in reactivity to environmental changes; 2)showing no interest in life; 3) retarded (slow, feeling tired, etc.); 4) feeling unable to act; 5) considering self lazy; 6) feeling helpless and powerless; and 7) perceiving depression as qualitatively different from ordinary sadness. Symptoms consistently loading on a reactivity factor are: 1) feeling that he or she is bearing troubles; 2) presence of precipitating stress; 3) crediting problems to excessive family and/or job responsiblitiies; 4) expressing concern for welfare of family and friends; 5) visceral symptoms; 6) feeling at "end of rope"; 7) having middle-of-the-night insomnia; and 8) showing self-pity. The results suggest a methodology for identifying depressives who are high-high, low-high, high-low, and low-low on the endogeneity and reactivity factors.

Adjustment Disorders↗