Masanori Uono

β-Galactosidase deficiency in juvenile and adult patients

SummarySix juvenile and adult patients with progressive neurological diseases and β-galactosidase deficiency were reported. Any diseases known to date were denied. These cases together with ten case reports in the literature were reviewed and were classified into three groups from clinical and biochemical points. Group 1 patients were characterized by progressive ataxia and myoclonus with gargoyle changes and macular cherry-red spots. In this syndrome β-galactosidase activity seems to be secondarily affected by other biochemical defects. A group 2 patient showed similar neurological manifestations without gargoyle changes or macular cherry-red spots. Patients with these clinical features not associated with β-galactosidase deficiency have also been described in the literature. Group 3 patients had progressive pyramidal and extrapyramidal disease without gargoyle changes or macular cherry-red spots. These cases may represent juvenile and adult type GM1-gangliosidosis. Accumulation of GM1 has not yet been demonstrated.

Effects of bromocriptine on parkinsonism. A nation-wide collaborative double-blind study.

ABSTRACT – The effects of bromocriptine in patients with Parkinsons disease manifesting various problems in levodopa therapy were tested in a double‐blind manner with the collaboration of 59 institutions. The slow and low principle was in part adopted. Either bromocriptine or placebo was added to levodopa.

Concanavalin A binding sites on the erythrocytes of normal and genetically dystrophic chickens

Red blood cells (RBCs) were obtained from genetically dystrophic chickens (Dy) and age-matched controls (C). Dy-RBCs had a lower titer of agglutination to concanavalin A (Con A) compared to C-RBCs. In order to ascertain the difference in agglutination, Con A binding on RBCs was studied, using 125I-labeled Con A ([125I]Con A) and ferritin conjugate to Con A (Fer-Con A). Kinetic analysis of [125I]Con A binding to Dy-RBCs showed a reduction of major binding sites of Con A. There was no difference in the apparent association constant for the major binding sites of Con A between Dy-RBCs and C-RBCs. Quantitative analysis of Con A binding site distribution on RBCs using Fer-Con A showed a remarkable diminution of ferritin particles tagged on the surface of Dy-RBCs. There was no significant difference in the distribution pattern of ferritin particles between Dy-RBCs and C-RBCs.

Multiple sclerosis co-existent with myxedema: An autopsy case report

A 64-year-old woman developed impaired consciousness and vision, sensorimotor paresis, hypothermia, bradycardia, and edema. Symptoms fluctuated with seasonal exacerbations in winter and terminated in coma with respiratory insufficiency at age 69. High CSF protein content and low serum T4 and TSH levels were noted. Treatment with prednisolone and thyroxin considerably improved her consciousness and edema. The patient suddenly expired of pulmonary embolism. Postmortem examination revealed a marked atrophy of pituitary and thyroid glands, while multiple demyelinating plaques were disclosed in the optic tract and cervical cord. A review of the literature indicates that this is the first report of the co-existence of two such disorders.

Studies on the neurohumoral control of the human body and its clinical applications: Histochemical study on cholinesterase of the human hypothalamus

1. The distribution of ChE in human hypothalamus was investigated withKoelles histochemical method. 2. Ganglionic cells of the paraventricular and supraoptic nuclei have strong ChE activity. Cells of the mamillo-infundibular nucleus and of the tuber nucleus have also strong ChE activity, though somewhat weaker than those of the former nuclei. 3. The small autonomic cells of the ventromedian and dorsomedian nuclei have markedly weaker ChE activity than those of the aforementioned nuclei. 4. ChE activity is found in protoplasm and nucleolus of ganglionic cells. However, nucleus of ganglionic cells shows no ChE activity. 5. Glial cells also have usually rather strong ChE activity. 6. The activity of ChE in the ground substance of various portions of hypothalamus is variable. 7. ChE activity of the corpus mamillare is as strong as those of putamen and caudate nucleus. ChE is present not only in ganglionic cells but also abundantly in the ground substance in the corpus mamillare.

Histochemistry of Catecholamines-Continued Report. Methodological Investigation and Application of Wood's Method

A methodological investigation and an application were presented on histochemical procedures for adrenal medullary catecholamines with new potassium dichromate technique. Woods method for light microscopy and Wood-Barrnetts method for electron microscopy were employed. Combination of these procedures was also tried. Changes of adrenal norepinephrine and epinephrine were observed after reserpine, insulin and α-methyldopa administration. Characteristics of these method were discussed, compared with Hillarp-Hockfelts method.