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Biomedical subjects

A Tamaoka

Publications and source records attributed to A Tamaoka.

At least 19 recordsLinked to original sources
PubMed2002

Prednisolone (30-60 mg/day) for diseases other than AD decreases amyloid beta-peptides in CSF.

The effect of corticosteroid on the concentration of amyloid beta-peptide (Abeta) in human CSF obtained from 16 patients without dementia treated with prednisolone (> or =30 mg daily) was studied. The concentrations of Abetax-40 and Abetax-42 in CSF decreased after treatment was started (p < 0.002). A moderate- or high-dose regimen of prednisolone decreases Abeta production or increases Abeta degradation in the human brain and deserves further study in AD.

Administration, Oral↗
PubMed2001

[Adult Sandhoff disease presented as a motor neuron disease phenotype with slow progression].

A 35-year old Japanese male with adult Sandhoff disease was described, who was presented as a motor neuron disease phenotype with slow progression. At the age of 15, he first noticed weakness in his thigh. At the age of 28, his upper and lower motor neuron disturbances were disclosed. He was diagnosed as atypical amyotrophic lateral sclerosis. At the age of 34, a slight sensory disturbance appeared in the lower extremities. When he was admitted to our hospital, he displayed marked atrophy and weakness in his quadriceps femoris muscles, but no signs of mental deterioration and cerebellar ataxia. Because of the atypical course of motor neuron disease, hexosaminidase activity in peripheral leukocytes was determined. The assay of total hexosaminidase, hexosaminidase A and hexosaminidase B activities demonstrated low levels of these activities (7-15% of controls), leading the diagnosis of Sandoff disease. He was a member of non-consanguineous family, and the abnormal patterns of hexasaminidase activities were different between his father and mother. These data appear to show that he is a compound heterozygote in the locus of the hexosaminidase B gene. This is the first Japanese case of adult Sanhoff disease presented as a motor neuron disease phenotype.

Adult↗
PubMed2001

[A case of eosinophilic myositis proven by immunohistochemistry using antibodies against eosinophilic granule protein].

A 50-year-old man had been well until three months earlier, when he felt general fatigue, and cutaneous rash with itching. Thereafter a general muscular weakness developed and the patient could not walk for a month. Four weeks before referral to our hospital, he had high fever and could not role over in the bed. On admission, the patient was able to walk. He had no skin rash. Neurologically, he showed mild weakness in proximal muscles. Hematologic examination showed mild eosinophilia and serum creatine kinase was mildly elevated. Needle electromyogram revealed a diffuse myogenic pattern in extremities. Eosinophilic myositis was diagnosed by a biopsy of the left calf muscle showing mild infiltration of eosinophilis which was identified using antibodies against eosinophilic granule protein.

Autoantibodies↗
PubMed2000

Plasma levels of amyloid beta 40 and 42 are independent from ApoE genotype and mental retardation in Down syndrome.

In Down syndrome (DS) brain an early, selective accumulation of amyloid beta (Abeta) peptides ending at residue 42 (Abeta42) occurs. Whether this event depends on an altered processing of amyloid beta precursor protein (APP) or on defective clearance is uncertain. To investigate this issue, we measured Abeta species 40 and 42 in plasma from 61 patients with DS, 77 age-matched normal controls, and 55 mentally retarded subjects without chromosomal abnormalities. The Abeta 40 and 42 plasma levels were then correlated with apolipoprotein E (apoE) genotypes in all groups of cases, and with I. Q. and Mini Mental Status Examination values in DS subjects. Both Abeta species were significantly elevated in DS compared to control groups, and the extent of their increase reflects that expected from APP gene overexpression. Plasma levels of Abeta 40 and 42 did not correlate with apoE genotypes in DS and control cases, and with the extent of mental retardation in DS subjects. The results indicate that accumulation and clearance of plasma and cerebral Abeta are regulated by different and independent factors.

Adolescent↗
PubMed2000

Apolipoprotein E allele-dependent antioxidant activity in brains with Alzheimer's disease.

Thiobarbituric acid-reactive substances (TBARS), an index of lipid peroxidation, were assayed in postmortem brain. Basal TBARS levels were increased and oxidative stimulation produced more TBARS in AD relative to control brains. In addition, apolipoprotein E isoforms showed differing antioxidant activities, with E2 > E3 > E4, suggesting that the lowest antioxidant activity of E4 could contribute to its association with AD.

Aged↗
PubMed2000

Increased amyloid beta protein in the skin of patients with amyotrophic lateral sclerosis.

Distinct vascular and periadnexal immunoreactivity have been observed for amyloid b protein (Abeta) in skin from patients with amyotrophic lateral sclerosis (ALS). We used an enzyme-linked immunosorbent assay to make a more quantitative comparison of Abeta concentrations between ALS patients and controls. The insoluble fractions of skin samples from ALS patients contained significantly higher Abeta concentrations per milligram protein than those from controls. Various alterations in extracellular matrix components have been reported to occur in the skin of patients with ALS, and several matrix constituents have been shown to affect processing and aggregation of Abeta in human brain. Taking these previous findings together with those of the present study, our observations suggest that changes in extracellular matrix in skin of ALS patients may facilitate aggregation and deposition of Abeta.

Amyloid beta-Peptides↗
PubMed2000

Novel presenilin-1 mutation with widespread cortical amyloid deposition but limited cerebral amyloid angiopathy.

OBJECTIVE: To clarify the phenotypic heterogeneity in deposition of amyloid beta (Abeta) in the parenchyma and in cerebral vessels of the brains of the patients having presenilin-1 (PS1) mutations. Mutations in PS1 induce increased production of Abeta42(43), resulting in an enhanced overall deposition of Abeta protein within the cerebral cortex. METHODS: Sequence analysis of the PS1 gene of DNA from patients with early onset Alzheimer's disease, and immunostaining of brain tissues by end specific monoclonal antibodies against Abeta. RESULTS: Sequence analysis disclosed a novel mutation (N405S) in the PS1 gene in a Japanese patient with early-onset Alzheimer's disease. Postmortem examination of one patient with N405S showed limited cerebral amyloid angiopathy, whereas postmortem examination of another Japanese patient with Alzheimer's disease with the E184D mutation disclosed severe cerebral amyloid angiopathy. The brains of both patients showed widespread neuritic plaques, neurofibrillary tangles, and neuronal loss. Immunostaining showed that Abeta42 was predominant over Abeta40 in neuritic plaques in both patients, whereas Abeta40 was found to be predominant over Abeta42 in cerebral amyloid angiopathy in the patient with E184D. However, most cortical vessels of the patient with N405S were not reactive with either of the antibodies. CONCLUSION: The N405S mutation of PS1 is a major determinant of cortical Abeta deposition but not cerebral amyloid angiopathy in Alzheimer's disease.

Alzheimer Disease↗
PubMed2000

Cessation of cerebral hemorrhage recurrence associated with corticosteroid treatment in a patient with cerebral amyloid angiopathy.

We report the case of a 65 year old female patient with biopsy-proven cerebral amyloid angiopathy (CAA). She experienced intracerebral hemorrhages 4 times during 23 days but these serious strokes did not recur after corticosteroid therapy was started and her condition greatly improved. Since high titers of antinuclear antibodies and elevated erythrocyte sedimentation rate were found in her serum, she may have an inflammatory disorder involving amyloid-laden cerebral vessels. This is the first report showing the usefulness ofcorticosteroid for the treatment of CAA-related cerebral hemorrhages. Additionally, the concentrations of Abeta40 and Abeta42 in the CSF of this patient decreased rapidly after the use of corticosteroid, and ultimately fell far below normal values.

Adrenal Cortex Hormones↗
PubMed2000

A case with myasthenia gravis (MG) emerging after splenectomy for idiopathic thrombocytopenic purpura (ITP): possible effects of thymectomy on autoantibodies.

The combination of idiopathic thrombocytopenic purpura (ITP) and myasthenia gravis (MG) has been reported infrequently. We report here the development of MG in a patient who underwent splenectomy for ITP ten years earlier, and describe the serum levels of anti-acetylcholine receptor antibodies (anti-AchR) and platelet-associated IgG (PAIgG) followed over 11 years. The concentrations of both autoantibodies have been decreased after thymectomy, suggesting that this procedure is potentially beneficial for treating both MG and ITP.

Adult↗
PubMed1999

Angiotensin-converting enzyme genotype is associated with Alzheimer disease in the Japanese population.

We compared the distribution of an insertion (I)/deletion (D) polymorphism of the gene coding for the angiotensin-converting enzyme (ACE) in 133 Japanese sporadic Alzheimer disease (AD) patients with 257 controls. The association between AD and ACE genotypes or alleles was found to be significant. The frequency of II genotypes was 1.4 times higher in AD than controls, while that of DD genotypes was only 0.4 times as high. The altered distribution of ACE alleles in patients with AD appeared to be independent of apolipoprotein E.

Aged↗
PubMed1999

Exacerbation of myasthenia gravis in a patient after interferon-beta treatment for chronic active hepatitis C.

We report the case of a 53-year-old female patient, who developed bilateral blepharoptosis, limb weakness, dysphagia, and dyspnea several days after human natural interferon-beta (IFN-beta) treatment for chronic active hepatitis C. A positive edrophonium test, an elevated anti-acetylcholine receptor antibody titer, and decrements in the amplitude of muscle action potentials evoked by repetitive stimulation confirmed the diagnosis of myasthenia gravis (MG). Since she had been suffering from drooping of her right eyelid, fluctuating diplopia and easy fatiguability of limbs before receiving IFN-beta, her symptoms of MG were considered to be exacerbated by IFN-beta. It is recommended that IFN-beta should be used with particular care in patients with known MG or its compatible symptoms.

Antiviral Agents↗