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Biomedical subjects

I Goto

Publications and source records attributed to I Goto.

At least 217 records · Page 12Linked to original sources
PubMed1986

Thiamine-deficient encephalopathy in rats: effects of deficiencies of thiamine and magnesium.

Thiamine- and vitamin B-deficient encephalopathy was produced in adult Wistar rats and the effect of magnesium deprivation and/or the administration of guanidine, a magnesium antagonist, was studied. These encephalopathic rats had symmetrical lesions, edema, exudation of fibrin, spongy changes, petechial hemorrhages, neuronal degeneration and gliosis in the pontine tegmentum, mainly the lateral and medial vestibular nuclear areas. Mild pathological changes were seen in the purely thiamine- or vitamin B-deficient rats, whereas severe pathological changes occurred in thiamine- or vitamin B-deficient rats combined with magnesium deprivation and/or guanidine administration. However, magnesium-deficient diets or guanidine administration did not produce pathologic changes in rats. These findings suggest that thiamine deficiency is an essential factor in the development of these pathological changes in the brainstem, and that magnesium and guanidine, an antagonist of magnesium, play important roles in the development of these disorders.

Animals↗
PubMed1986

Nonfamilial prealbumin-type amyloid polyneuropathy.

A 53-year-old man with nonfamilial prealbumin-type amyloid polyneuropathy had severe motor, sensory, and autonomic polyneuropathy, beginning at age 48 years. These clinical features closely resembled familial amyloid polyneuropathy (FAP), but abnormal serum prealbumin levels, specific to FAP (Japanese type), were not detected by radioimmunoassay; DNA sequence for prealbumin was normal. Thus, the diagnosis of FAP was excluded. A possible diagnosis of systemic senile amyloidosis was also considered.

Amyloidosis↗
PubMed1986

Proteolytic enzyme activities of macrophages and lymphocytes in neurological diseases.

Studies showed a significant decrease in the macrophage neutral protease and lymphocyte acid protease activities in patients with multiple sclerosis in remission, a significantly decreased neutral protease activity in macrophages in patients with myasthenia gravis and a significantly decreased acid protease activity in macrophages and lymphocytes in patients with polymyositis. No remarkable abnormalities were found in patients with myotonic dystrophy. These results suggest that multiple sclerosis, myasthenia gravis and polymyositis have an abnormality in immunological function.

Adult↗
PubMed1986

Adrenoleukodystrophy and beta-galactosidase deficiency: patient and carrier.

A patient with adrenoleukodystrophy and his mother, a carrier, showed an elevated ratio of very long-chain fatty acids to long-chain fatty acids and decreased beta-galactosidase activity. Other lysosomal enzyme activities were normal except for the borderline level of arylsulfatase-A activity. However, the father and other patients with variant forms of adrenoleukodystrophy showed normal beta-galactosidase and other lysosomal enzyme activities.

Adrenoleukodystrophy↗
PubMed1986

Plasmalogen deficiency in cultured skin fibroblasts from neonatal adrenoleukodystrophy.

The plasmalogen ratio (defined as area ratio of lysophosphatidylethanolamine to the diacyl form of phosphatidylethanolamine) was investigated in cultured skin fibroblasts from neonatal adrenoleukodystrophy (N = 4) and X-linked recessive (N = 3) in addition to Zellweger syndrome (N = 3) because plasmalogen was reported to be reduced in Zellweger syndrome. The ratio was markedly decreased in all cases of Zellweger syndrome studied and in three of the four cases of neonatal adrenoleukodystrophy, whereas it was normal in the X-linked cases. This is the first documentation of a plasmalogen deficiency in neonatal adrenoleukodystrophy.

Adrenoleukodystrophy↗
PubMed1986

Adrenoleukodystrophy and variants. Clinical, neurophysiological and biochemical studies in patients and family members.

Clinical, neurophysiological and biochemical studies were performed in patients with various forms of adrenoleukodystrophy (ALD) and their family members. The patients showed an abnormality in saturated very long chain fatty acids and in the somatosensory and brain stem auditory or visual evoked potentials. Female presumptive carriers without abnormal neurological manifestations also showed abnormality in the somatosensory or brain stem auditory evoked potentials and in saturated very long chain fatty acids. One ALD patient and his mother, a female carrier, had the decreased beta-galactosidase activity. The increase in saturated very long chain fatty acids was found, not only in sphingomyelin, but also in phosphatidylcholine and phosphatidylserine. Our results suggest that a generalized abnormal metabolism of VLFA and an abnormality in the central nervous system exist in our patients and female carriers.

Adrenoleukodystrophy↗
PubMed1986

Plasma and erythrocyte copper, zinc, manganese and magnesium concentrations in Wilson's disease.

Plasma copper was significantly reduced in Wilson's disease with decreased ceruloplasmin levels, while there was no significant difference in erythrocyte copper levels between Wilson's disease and controls. Mean zinc and manganese levels in plasma were increased in Wilson's disease, but these differences were not significant. Mean magnesium level in plasma was normal. The levels of these metals in erythrocytes showed no difference between Wilson's disease and controls. Our results suggest that the abnormal copper level in plasma is not due to the primary metabolic defects, but is probanly due to the level of the binding proteins in plasma to this metal, ceruloplasmin, in Wilson's disease.

Adolescent↗
PubMed1986

The rigid spine syndrome and Emery-Dreifuss muscular dystrophy.

We present five patients, three of whom suffered from a rigid spine syndrome and two from Emery-Dreifuss muscular dystrophy. One patient with rigid spine syndrome showed a nonprogressive course, normal cardiac rhythm and mild myopathic changes in muscle histology, while in the other two patients there was a rapidly progressive course, sinus tachycardia without cardiac conduction defects and marked fiber necrosis in muscle histology. The two patients suffering from Emery-Dreifuss muscular dystrophy showed a very mildly progressing course, myopathic changes in muscle histology, cardiac conduction defects and deviations in electrocardiograms. These findings show that rigid spine syndrome can be distinguished from Emery-Dreifuss muscular dystrophy.

Adolescent↗
PubMed1986

Familial spinocerebellar degeneration as an expression of adrenoleukodystrophy.

A family with adrenoleukodystrophy and clinical manifestations of spinocerebellar degeneration was studied. Two adult male first cousins showed progressive limb and truncal ataxia, slurred speech and spasticity of the extremities. Brain CT scans demonstrated atrophy of the pons and cerebellum, in both cases. Very long chain fatty acids in plasma and erythrocyte membranes were elevated in the affected patients and intermediately increased in an aunt and the mother of one patient, thereby indicating homozygotes and carriers of adrenoleukodystrophy, respectively. This unusual type of adrenoleukodystrophy seems to be transmitted as an X-linked recessive trait.

Adrenoleukodystrophy↗
PubMed1986

Dietary treatment of adrenoleukodystrophy.

We treated two patients with adrenoleukodystrophy (ALD) with a diet that contained 0.636% lipids as soybean oil, and less than 0.01% of the total fatty acids were very-long-chain fatty acids (VLCFA). Although clinical improvement was not evident in either patient, the VLCFA levels of erythrocyte membranes and plasma decreased in patient 1; in particular, the C25:0 in erythrocyte membranes became normal. Therefore, the abnormal accumulation of VLCFA in ALD arises partially from the diet. The VLCFA-free diet should be beneficial in ALD.

Adrenoleukodystrophy↗