Biomedical subjects
Publications and source records attributed to I Steiner.
The frequency of isolated atrial amyloid (IAA), a form of 'senile' cardiac amyloidosis, was determined in younger age groups. For the detection of amyloid the Sirius red staining technique was used. IAA first appears in the fourth decade; its prevalence increases roughly linearly by 15-20 per cent with each subsequent decade, reaching 95 per cent in the 81-90 years age group. The prevalence is higher in females than in males.
Chronic neuropathic intestinal pseudoobstruction is a rare entity, characterized by recurrent episodes of bowel obstruction without a mechanical obstructive cause. We report five members of two Jewish-Iranian families in whom chronic neuropathic intestinal pseudoobstruction was associated with an identical and unique progressive severe neuronal disease. It appeared within the first two decades of life. The disease consisted of external ophthalmoplegia, ptosis, and severe sensory and motor peripheral neuropathy. Three patients also had neuronal hearing loss. There was no evidence of central nervous system involvement and all patients were mentally intact. The combined disease was confirmed by radiologic, electrophysiologic, and histologic studies. Specific nutritional deficiencies, toxic elements, and systemic diseases affecting both the gastrointestinal tract and the nervous system were ruled out. It seems that these patients suffer from an autosomal recessive, presently unrecognized variant, of chronic neuropathic intestinal pseudoobstruction. In a patient with severe peripheral neuropathy of unknown etiology associated with symptoms suggestive of intestinal obstruction, the possibility of chronic neuropathic intestinal pseudoobstruction has to be considered.
Methanobacterium thermoautotrophicum grown on mineral medium contains 120 nmol of Co alpha-(5-hydroxybenzimidazolyl)cobamides (derivatives of factor III) per g of dry cell mass as the sole cobamide. The bacterium assimilated several corrinoids and benzimidazole bases during autotrophic growth. The corrinoids were converted into factor III; however, after three transfers in 5,6-dimethylbenzimidazole (200 microM)-supplemented mineral medium, derivatives of factor III were completely replaced by derivatives of vitamin B12, which is atypical for methanogens. The total cobamide content of these cells and their growth rate were not affected compared with factor III-containing cells. Therefore, the high cobamide content rather than a particular type of cobamide is required for metabolism of methanogens. Derivatives of factor III are not essential cofactors of cobamide-containing enzymes from methanogenic bacteria, but they are the result of a unique biosynthetic ability of these archaebacteria. The cobamide biosynthesis include unspecific enzymes, which made it possible either to convert non-species-derived corrinoids into derivatives of factor III or to synthesize other types of cobamides than factor III. The cobamide biosynthesis is regulated by its end product. In addition, the uptake of extracellular cobamides is controlled, and the assimilated corrinoids regulate cellular cobamide biosynthesis.
Central neurogenic hyperventilation is a rare but important cause of the frequently observed phenomenon of hyperventilation. Its diagnosis demands that primary respiratory or metabolic causes, as well as cerebrospinal fluid abnormalities, be ruled out. We hereby describe a patient whose attacks of hyperventilation were the presenting sign of her disease and might be consistent with the central neurogenic mechanism. This patient suffered from a medulloblastoma which compressed the pons. After removal of the tumor the hyperventilation attacks ceased.
Three patients with severe athetoid-dystonic type of cerebral palsy involving the neck musculature developed in their fourth to fifth decade progressive cervical radiculomyelopathy associated with vertebral spondylarthrotic compressive lesions in addition to their long-standing neurological syndrome. It is likely that the late-onset myelopathy is linked to continuous torsion, compression and 'wear and tear' of the cervical spinal cord, induced by the involuntary movements. In 2 patients decompressive cervical spinal surgery was ineffective, suggesting irreversible damage.
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Chronic idiopathic intestinal pseudo-obstruction (CIIP) is characterized by recurrent episodes of bowel obstruction without mechanical cause. In five members of two Jewish-Iranian families, CIIP was associated with progressive neuronal disease, starting before age 30, with ophthalmoplegia, sensorimotor peripheral neuropathy, and hearing loss. There was no evidence of CNS involvement. The pattern suggested autosomal recessive inheritance.
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Three patients developed acute Guillain-Barré syndrome while on steroid treatment. The first patient suffered from ulcerative colitis and developed Guillain-Barré syndrome when the steroid dosage was being tapered. Another patient with long-standing multiple sclerosis received steroids during relapse and the Guillain-Barré syndrome appeared while treatment was reduced. The third patient, with aqueductal stenosis and ventriculoatrial shunt, who received steroids when malfunction of the shunt was suspected, developed Guillain-Barré syndrome while steroids were being tapered. Based on the putative immune pathogenesis of inflammatory demyelinating polyneuropathy, the occurrence of the syndrome in these patients could have been due to a selective effect of low-dose steroids on a specific, maybe suppressor lymphocyte subpopulation.
Cyanocobamides were extracted from diverse bacterial species, purified by XAD-4 and neutral aluminum oxide column chromatography, and separated by isocratic reversed-phase high-performance liquid chromatography (HPLC). Retention times are given for seven cobamide types: dicyanocobinamide (factor B), Co alpha-(alpha-benzimidazolyl)-Co beta-cyanocobamide, Co alpha-(5-hydroxybenzimidazolyl)-Co beta-cyanocobamide (factor III), Co alpha-(5-methoxybenzimidazolyl)-Co beta-cyanocobamide (factor IIIm), Co alpha-(5-methylbenzimidazolyl)-Co beta-cyanocobamide, cyanocob(III)alamin (vitamin B-12) and Co alpha-(naphthimidazolyl)-Co beta-cyanocobamide. Other Co beta-ligandyl-cobamides such as hydroxycobamide and the light-sensitive methyl-, acetyl-, propyl-, and adenosylcobamides were separated by HPLC in a gradient mode. The recovery of total cell cobamide after extraction, purification, and separation was 75-80%. The method was useful in preparative and analytical work. Less than 10 ng cyanocobamide was detectable.
A case of chronic calcific constrictive pericarditis in a 39-year-old Ugandan is presented. According to recent views this condition is only rarely caused by tuberculosis.
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Multiple parameters for brain volume and mass were studied in 85 parkinsonian patients and in 149 normal controls aged 24 to 84 using CT scanning. In controls there was reduction in brain substance with advancing age. Increased brain atrophy in patients with Parkinson's disease (PD) was mainly observed in the younger age group of 24 to 49. This included parameters evaluating the size of the lateral and third ventricles (P less than 0.05 and P less than 0.01 respectively) and the size of the subarachnoid space in the frontal interhemispheric and Sylvian fissures (P less than 0.005 and P less than 0.01). With computed canonical correlation analysis a formula was obtained which expressed the tendency of the atrophic process in PD to involve the areas surrounding the third ventricle and the mesial aspect of the frontal lobes more than during normal aging.
In two elderly patients with ulcerative colitis, acute Guillain-Barré syndrome developed while the inflammatory bowel disease was in remission. The patients were treated with corticosteroids and recovered. Since both disorders are relatively uncommon, we suggest that the Guillain-Barré syndrome may have been causally related to the preexisting chronic inflammatory bowel disease. Guillain-Barré syndrome may be regarded as a possible extraintestinal complication of chronic ulcerative colitis.