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Biomedical subjects

H Tsukagoshi

Publications and source records attributed to H Tsukagoshi.

At least 181 records · Page 10Linked to original sources
PubMed1987

Adult-onset spinocerebellar syndrome with idiopathic vitamin E deficiency.

A 62-year-old man and his maternal uncle had a selective vitamin E deficiency without generalized fat malabsorption. A progressive neurological disorder comprising ataxia, areflexia, and loss of proprioception developed in their sixth and seventh decades. The vitamin E deficiency is thought to be due to abnormally accelerated utilization, excretion, or degradation of the vitamin. This adult-onset spinocerebellar syndrome is due to vitamin E deficiency not caused by malabsorption.

Age Factors↗
PubMed1987

Cerebral amyloid angiopathy in the aged.

Cerebral amyloid angiopathy (CAA) was found in 57% of 123 autopsy brains removed from patients aged 59-101 years. The incidence of CAA increased with age. CAA was seen most frequently in the occipital cortex. Immunohistochemically, amyloid of CAA was positive for amyloid P component and negative for human AA protein and human prealbumin. The presence and severity of CAA were significantly correlated with the number of senile plaques and neurofibrillary tangles. The incidence of CAA in 17 patients with dementia of Alzheimer type (DAT) was estimated to be 88% and was significantly higher than that in 26 patients with dementia of non-Alzheimer type. CAA had a pathogenetic relationship with both brain ageing and DAT. Lobar cerebral haemorrhage was found in 3 patients with CAA of marked or moderate degree. Lobar cerebral haemorrhage in the aged and in patients with DAT suggest the presence of CAA.

Aged↗
PubMed1987

"Sporadic" prealbumin-related amyloid polyneuropathy: report of two cases.

Two "sporadic" cases of amyloid polyneuropathy are reported. There was no family history or plasma cell dyscrasia. Both showed sensorimotor and autonomic polyneuropathy with onset in the seventh decade. Amyloid deposits in both cases reacted with anti-human prealbumin sera but not with antisera to human AA and anti-human immunoglobulin light-chain amyloids, including A kappa and A lambda. One patient had the abnormal serum prealbumin and abnormal DNA sequence found in type I familial amyloid polyneuropathy (FAP)(Japanese type). Investigations in "sporadic" amyloid polyneuropathy should include immunohistochemistry, using antisera to the different amyloid proteins, and the radioimmunoassay and recombinant DNA techniques for diagnosis of FAP.

Aged↗
PubMed1987

Eye-fixation patterns in homonymous hemianopia and unilateral spatial neglect.

Eye-fixation patterns while viewing simple patterns were quantitatively analysed in homonymous hemianopic subjects with or without unilateral spatial neglect (USN), using an eye camera. Hemianopic subjects without USN used the strategy to look at the hemianopic side of the patterns longer in order to compensate for their visual field defect. However, those with USN lacked this compensatory eye-fixation pattern.

Adult↗
PubMed1987

Hereditary generalized amyloidosis with polyneuropathy. Clinicopathological study of 65 Japanese patients.

A clinicopathological study was made on 65 patients from a small area of Nagano Prefecture, Japan, with hereditary generalized amyloidosis with polyneuropathy to clarify the clinical variety of the disease. Forty-five patients from Ogawa village showed similar clinical features. The age of onset ranged widely from 16 to 62 years. The main neurological manifestations were polyneuropathy starting in the legs and autonomic dysfunction. Lower cranial nerves were also affected in the advanced stages. Severe cardiac and renal involvement was uncommon. All these clinical features are consistent with type I familial amyloid polyneuropathy (FAP). The remaining 20 patients from five unrelated kinships showed unique clinical pictures. Two families from Ogawa village had type I FAP, but 4 out of the 5 affected patients showed marked nephropathy with heavy proteinuria from an early stage. Of the three other families, one, with 10 patients, was notable for the involvement of the central nervous system. Most of the patients showed cerebellar ataxia and pyramidal tract signs in addition to a sensorimotor and autonomic peripheral neuropathy. Another family had 2 siblings who had severe amyloid heart disease from the onset and developed polyneuropathy with autonomic features at an advanced stage. In the third family, onset occurred in the sixth decade in all 3 patients and the course was mild in 2, although the clinical features were those of typical type I FAP. Immunohistochemical study revealed that the amyloid fibril proteins in the patients with all four unusual clinical phenotypes were related to plasma prealbumin. The most common form of hereditary generalized amyloidosis in Japan is type I FAP, but the disease shows considerable variety in the age of onset and involves more systemic organs than previously recognized. The newly recognized clinical forms of hereditary generalized amyloidosis with severe amyloid heart disease or central nervous dysfunction indicate clinical heterogeneity of hereditary amyloidosis with polyneuropathy.

Adolescent↗
PubMed1986

Chronic manganese poisoning: a neuropathological study with determination of manganese distribution in the brain.

An autopsy case of a 52-year-old man suffering from chronic manganese poisoning (CMP) is reported with determination of the manganese distribution in the brain. The patient had been working in a manganese ore crushing plant since 1965. In 1967 he began to complain of difficulties in walking and diminished libido. Later, he developed various neuropsychiatric symptoms including euphoria, emotional incontinence, masked face, monotonous speech, "cock-walk", increased muscle tone, weakness of upper and lower extremities, tremor of the eye lids, and exaggeration of knee jerks. The major neuropathological change was degeneration of the basal ganglia, in which the pallidum was severely affected. The pallidum disclosed a loss and degeneration of nerve cells, which was especially marked in the medial segment, a prominent decrease of myelinated fibers, and moderate astrocytic proliferation. The substantia nigra was intact. Distribution of manganese in the brain of the present case of CMP was determined using flameless atomic absorption spectrometry and compared with control cases and also a case of Parkinson's disease (PD). There was no significant difference between the control cases and the case of PD in average concentration of manganese and its distribution in the brain. The present case of CMP showed no elevation in average concentration of manganese in the brain. However, there were some changes in its distribution. Thus, the continuance of neurological disorders in CMP is not linked to an elevated manganese concentration itself in the brain. CMP appears to be different from PD in neuropathology and manganese behavior in brain.

Aged↗
PubMed1986

Primary cultures of human myasthenia gravis thymus and normal thymus. Studies of cell morphology, cell proliferative pattern and localization of alpha-bungarotoxin binding sites on cultured thymic cells.

We have established primary cultures of human myasthenia gravis (MG) thymuses and normal thymuses. In cultures of 19 thymuses with hyperplasia among 23 MG thymuses and 12 thymuses among 13 normal thymuses, epithelial cells migrated in a mosaic-like arrangement and were maintained for more than 5-10 weeks. There were, among mononuclear epithelial cells, some multinucleated cells, some of which were considered to be derived from epithelial cells because they had a desmosome-like structure and contained tonofilaments in their cytoplasm. There was no significant difference in the morphology of epithelial cells between MG thymuses and normal thymuses. The growth rate of thymic epithelial cells was identified by [3H]thymidine autoradiography(ARG), labeling indices rising to a peak around a week and falling to low levels gradually. There was no significant difference in the growth rate of epithelial cells between MG thymuses and normal thymuses. An autoradiographic method with 125I-labeled alpha-bungarotoxin was used to detect the presence of acetylcholine receptor (AChR) on the cultured cells. ARG of human MG thymuses and normal thymuses, which were cultured for 4 weeks, revealed diffusely distributed silver grains on the epithelial cells. Toxin binding sites (AChRs) were considered to be present on the epithelial cells. There was no significant difference in the distribution of AChR on the epithelial cells between MG thymuses and normal thymuses.

Adolescent↗