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Biomedical subjects

J Tateishi

Publications and source records attributed to J Tateishi.

At least 19 recordsLinked to original sources
PubMed1992

Successful transmission of Creutzfeldt-Jakob disease from human to mouse verified by prion protein accumulation in mouse brains.

The accumulation of prion protein (PrP) was revealed in the brains of mice inoculated with the brain homogenate from seven patients with Creutzfeldt-Jakob disease (CJD) by immunohistochemistry using hydrolytic autoclaving. It was not found in the brains of mice inoculated with material from either two patients with Gerstmann-Sträussler syndrome or two with other dementing illnesses. PrP accumulation took the forms of diffuse neuropil accumulation in the gray matter and plaque-like accumulation in the white matter and was observed in particular areas in the supratentorial structure. Its distribution was narrower than that in the brains of mice infected with a mouse-adapted CJD strain. PrP accumulation was found not only in all histopathologically positive mice, but also in some histopathologically negative mice. In all groups of mice inoculated with the material from each CJD patient, the percentage of mice with PrP accumulation was equal to or exceeded that of mice with the histopathological findings. PrP immunohistochemistry using formic acid pretreatment stained such plaque-like accumulation less intensely than that using hydrolytic autoclaving and did not stain diffuse neuropil accumulation. Therefore, PrP accumulation which can be revealed in the brains of first-passage CJD mice by this new immunohistochemical method may be the most sensitive hallmark of successful transmission.

Animals↗
PubMed1992

Accumulation of alpha B-crystallin in brains of patients with Alexander's disease is not due to an abnormality of the 5'-flanking and coding sequence of the genomic DNA.

alpha B-Crystallin is a major protein component of Rosenthal fibers, which massively accumulate in the brains of patients suffering from Alexander's disease. To examine whether or not accumulation of alpha B-crystallin is due to any abnormality of the gene structures, we determined the sequence of the alpha B-crystallin gene in two cases of pathologically confirmed Alexander's disease. Direct sequencing of the promoter and coding regions of the alpha B-crystallin gene in patients revealed them to have a normal sequence. Northern blotting showed a single alpha B-crystallin mRNA species expressed in the Alexander's disease brain.

Aged↗
PubMed1992

Alzheimer's amyloid precursor protein accumulates within axonal swellings in human brain lesions.

By using a monoclonal antibody and polyclonal antibodies to human beta amyloid precursor protein (APP), we immunohistochemically examined axonal swellings (spheroids). The immunoreactivity of APP was observed in numerous spheroids, which were found around the areas of old cerebral infarctions, in the globus pallidus with Hallervorden-Spatz disease as well as in or around brain abscesses. The axonal swellings were not immunolabeled with beta protein. These results implied that APP accumulated in spheroids regardless of beta protein depositions.

Aged↗
PubMed1992

Presence of HTLV-I proviral DNA in central nervous system of patients with HTLV-I-associated myelopathy.

The polymerase chain reaction (PCR) method was used to determine the presence and amount of human T lymphotropic virus type I (HTLV-I) proviral DNA in central nervous system (CNS) tissue obtained at autopsy from 6 patients with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP), 1 patient with adult T-cell leukemia (ATL) and CNS infiltration of leukemic cells, and 9 control subjects with other neurological disorders. HTLV-I pX and env but not pol DNA were detected in CNS tissue from 5 of 6 patients with HAM/TSP. The ATL samples were positive for pX, env, and pol DNA by PCR. None of the control samples was consistently positive for HTLV-I by PCR, but all showed positive bands on beta-globin PCR. Quantitative PCR combined with histological studies showed no correlation between HTLV-I proviral DNA amounts and extent of perivascular mononuclear cell infiltration in the HAM/TSP CNS. Also, the amounts of pX and probably env DNA were greater in the HAM/TSP samples than in the ATL sample, although the extent of mononuclear cell infiltration was far less in the HAM/TSP samples than in the ATL sample. Therefore, in addition to infiltrating mononuclear cells, constituent cells of the CNS may harbor the HTLV-I genome, at least in the pX and env regions, in patients with HAM/TSP.

Base Sequence↗
PubMed1992

Creutzfeldt-Jakob disease with codon 129 polymorphism (valine): a comparative study of patients with codon 102 point mutation or without mutations.

We examined 7 patients with Creutzfeldt-Jakob disease (CJD) with a methionine-to-valine change at prion protein (PrP) codon 129 (CJD129 patients). These CJD129 patients did not have either a codon 117 or 198 point mutation. For comparison, we also examined 7 patients with Gerstmann-Sträussler syndrome (GSS) with a proline-to-leucine change at PrP codon 102 (GSS102 patients) and 13 patients without any known mutations at codons 102, 117, 129, 178, or 200 (CJDwild patients). CJD129 patients had a long clinical duration and ataxia at onset, but rarely had any periodic synchronous discharge in their electroencephalogram. Unlike CJDwild patients, all CJD129 patients have typical congophilic PrP plaques in their brain. These clinicopathological findings were similar to those of GSS102. However, the distribution and morphology of PrP deposits revealed by immunohistochemistry were different between CJD129 and GSS102. In GSS102 more numerous and various types of PrP plaques are seen throughout the brain, while in CJD129 patients a unicentric core was the major feature of PrP plaques. The change in codon 129 influences the clinical course and pathological findings in CJD.

Adult↗
PubMed1992

The coexistence of Alzheimer's disease and Creutzfeldt-Jakob disease in a patient with dementia of long duration.

We report here a 75-year-old-male with a slowly progressive dementia of 5-year duration along with a rapid exacerbation of symptoms in the terminal 3 months. Neuropathological examinations revealed findings consistent with conspicuous Alzheimer's disease and mild Creutzfeldt-Jakob disease (CJD). The plaque amyloid was exclusively composed of beta-protein. The immunohistochemistry of prion protein using hydrolytic autoclaving pretreatment showed diffuse gray matter stainings in the sections of both the cerebral and cerebellar cortices. This method was thus considered useful in confirming the diagnosis of CJD for this case.

Aged↗
PubMed1992

Anaplastic large cell Ki-1 lymphoma in the central nervous system: report of an autopsy case.

A 45-year old immunocompetent man presented with multiple lesions in the brain. A histological examination of the tumors showed a diffuse infiltrate of lymphoid cells with cellular polymorphism and of multinucleated giant cells. These cells were immunolabeled with antibodies against B cell lineage and with a monoclonal antibody, Ber-H2 (CD30), which showed the presence of Ki-1 antigen. Recently, among systemic non-Hodgkin's lymphomas, attention has been given to Ki-1-positive lymphomas, which have been incorporated in the up-dated Kiel classification. We report here a case of Ki-1-positive lymphoma arising in the CNS and review previously reported cases.

Humans↗
PubMed1992

Creutzfeldt-Jakob disease with amyloid angiopathy: diagnosis by immunological analyses and transmission experiments.

It was difficult to make a definite pathological diagnosis in a 73-year-old man with Creutzfeldt-Jakob disease (CJD) due to extensive amyloid angiopathy which lacked any severe spongiform changes. Immunostaining using anti-prion protein (PrP) antibody revealed fine granular deposits in the gray matter, after hydrolytic autoclaving pretreatment on tissue sections. Western blotting also revealed an abnormal isoform of PrP, but PrP gene analysis did not show any abnormalities. The primary transmission experiments were repeated three times and induced spongiform encephalopathy in a few mice after a long incubation period.

Aged↗
PubMed1992

Colocalization of prion protein and beta protein in the same amyloid plaques in patients with Gerstmann-Sträussler syndrome.

We examined paraffin-embedded brain sections from three patients with Creutzfeldt-Jakob disease (CJD) and four patients with Gerstmann-Sträussler syndrome (GSS) who also had beta protein deposits in the brains. Immunostaining using anti-prion protein (PrP) and anti-beta protein coupled with formic acid pretreatment, revealed PrP deposits and beta protein deposits, respectively. In all four GSS patients examined, sequential double immunostaining and single immunostaining in serial sections or simultaneous double immunofluorescence revealed the colocalization of PrP and beta protein in the same amyloid plaques. The plaques labeled with both antibodies were designated as beta-PrP plaques. Small kuru plaques of less than 15 microns in diameter were rarely found to coexist with beta deposits. The percentages of beta-PrP plaques in larger kuru plaques were not constant among the four GSS patients. The colocalization patterns of both deposits were observed as being roughly of two types as follows: (1) diffuse beta protein deposits located around the PrP core; and (2) a beta protein core and PrP core simultaneously existing in one amyloid plaque. Under an electron microscope, we were able to confirm the presence of both beta protein and PrP in a single plaque in four GSS patients older than 60 years old. In contrast, no colocalization of either deposits was seen in the amyloid plaque core fractions of a young GSS patient who had no beta protein deposits, even at the electron microscopic level. Therefore, the colocalization of both proteins in a single plaque is believed to be age-related and incidental in GSS patients but suggests a similar morphogenesis of both amyloid deposits.

Adult↗
PubMed1992

Gerstmann-Sträussler-Scheinker disease in an Alsatian family: clinical and genetic studies.

The clinical progression of Gerstmann-Sträussler-Scheinker disease in a family of Alsatian origin is reported. The age of onset and the duration of evolution were variable. The clinical picture became more complex over the generations: in the first generations, isolated dementia and in later generations a triad of pyramidal, pseudobulbar syndromes and dementia associated with spinal cord and cerebellar features. Prion gene analysis showed that four surviving patients carry double missense changes at codons 117 and 129, identical to those found in one case at necropsy and 10 other healthy members of the family. The missense changes were not found in 100 controls. No member of the family had modification of condons 102, 178, or 200. The lod score suggests linkage between the missense change at codon 117 and Gerstmann-Sträussler-Scheinker disease in this family.

Adult↗
PubMed1992

The first report of a case with acute myocardial infarction showing familial deficiency of creatine kinase.

A 46-year-old male patient was diagnosed as suffering from acute myocardial infarction, but his serum creatine kinase (CK) level was extremely low and no CK isozymes were detected in the serum. The total CK activities in the skeletal muscle amounted to only 2% of that of the control. Electrophoresis of the CK isozymes in the skeletal muscle showed that CK-MM was absent but the CK-BB and abnormal isozyme bands were present. There was no evidence of myocardial ischemia, although the exercise treadmill test revealed ST segment depression in the chest leads. One of the patient's sisters had an extremely low serum CK level suggesting inheritance of this abnormality. This is the first report of a case showing familial deficiency of CK.

Creatine Kinase↗
PubMed1992

[Impaired cardiopulmonary baroreflex control of forearm vascular resistance in hypertrophic cardiomyopathy].

We tested the hypothesis that the cardiopulmonary baroreflex control of forearm vascular resistance is impaired in patients with hypertrophic nonobstructive cardiomyopathy (HNCM). Forearm vascular responses to lower body negative pressures (LBNP) at -20 mmHg and -40 mmHg and to lower body positive pressure (LBPP) at +20 mmHg and +40 mmHg were compared between 11 patients with HNCM and 6 normal subjects. Forearm blood flow was measured with a strain gauge plethysmograph and forearm vascular resistance was calculated by dividing mean blood pressure by forearm blood flow. The left ventricular end-diastolic dimension and the left atrial dimension changed with the increases in LBNP and LBPP in patients with HNCM and in normal subjects. The ranges of these changes were similar in the 2 groups. Mean blood pressure and heart rate did not change during LBNP and LBPP. LBNP increased but LBPP decreased forearm vascular resistance in normal subjects, but neither LBNP nor LBPP changed the resistance in patients with HNCM. Increases in the resistance to cold pressor tests did not differ between the 2 groups. In patients with HNCM, there was a negative correlation between forearm vascular resistance at LBNP -20 mmHg and the left ventricular wall thickness (r = -0.81, p < 0.01). These results suggest that the cardiopulmonary baroreflex control of forearm vascular resistance is impaired in hypertrophic cardiomyopathy and that this impairment may be associated with left ventricular wall thickness.

Adult↗
PubMed1992

[Left ventricular wall motion mimicking stunned myocardium in hypertrophic obstructive cardiomyopathy with normal coronary arteries: a case report].

A 63-year-old woman was admitted to the coronary care unit of Hyogo College of Medicine because of cardiogenic shock. She previously had been hospitalized in the Gynecology Department for the treatment of recurrent uterine cancer. She had poor appetite due to chemotherapy which was given for 10 days prior to her admission. On admission, echocardiography and cardiac catheterization revealed hypertrophic obstructive cardiomyopathy and extensive left ventricular wall motion abnormalities. Coronary arteriography showed no coronary artery disease. Left ventriculography as well as echocardiography performed on the 21st post-admission day revealed that the wall motion abnormalities had completely resolved and the systolic anterior motion of the mitral valve (SAM) was no longer evident. The systolic pressure at the apex of the left ventricle was 200 mmHg on admission. The increased ventricular pressure and the simultaneous resolution of the wall motion abnormality and SAM suggest that marked obstruction of the left ventricular outflow tract is more likely to be involved in transient ventricular wall motion abnormality rather than acute myocardial ischemia. The mechanism of the SAM in the present case seemed to be related to a Venturi effect which was augmented by the decreased preload due to hypovolemia. In addition, papillary muscle contraction seemed to pull the mitral valve toward the interventricular septum during systole.

Cardiac Catheterization↗
PubMed1992

Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease.

A new method, which enabled the first immunohistochemical documentation of abnormal prion protein (PrP) in all patients with Creutzfeldt-Jakob disease (CJD), was established. This method designated as "hydrolytic autoclaving" revealed punctate PrPCJD stainings around the neuronal cell bodies and dendrites in CJD brains. These punctate stainings were almost identical with that of synaptophysin, suggesting PrPCJD accumulations in the synaptic structures. Subcellular fractionation revealed that prion protein in Creutzfeldt-Jakob disease (PrPCJD) was most concentrated in the synaptosomal fraction. In CJD patients with a long clinical course, synaptophysin immunoreactivity decreased, and synaptic PrPCJD accumulated with a wider distribution. These results suggest that synaptic PrPCJD accumulations might be responsible for the neuronal dysfunction and degeneration in CJD.

Creutzfeldt-Jakob Syndrome↗
PubMed1992

The primary structure of the prion protein influences the distribution of abnormal prion protein in the central nervous system.

We immunohistochemically examined tissue sections from patients with prion protein (PrP) polymorphism using hydrolytic autoclaving enhancement. Abnormal PrP accumulations could be classified into plaque formations (plaque-type) and the diffuse gray matter stainings including synaptic structures (synaptic-type). Insertional polymorphism, a point mutation in codon 102 or 117/129, and a polymorphism in codon 129 (Val129) result in plaque-type PrP accumulations. The patients with codon 102 mutation also have synaptic-type PrP accumulations. However, a point mutation in codon 200 did not show plaque-type accumulations, and only showed synaptic-type PrP accumulations. Likewise, sporadic Creutzfeldt-Jakob disease patients without any known mutations only have synaptic type accumulations. These results imply that the primary structures of PrP influence the phenotype of prion diseases, especially in abnormal PrP distributions of the central nervous system.

Base Sequence↗
PubMed1992

Massive accumulation of modified tau and severe depletion of normal tau characterize the cerebral cortex and white matter of Alzheimer's disease. Demonstration using the hydrated autoclaving method.

Using the hydrated autoclaving method, a new immunohistochemical procedure to enhance tau immunoreactivity in formalin-fixed brain tissue, the authors recently reported that tau protein is detected in neuronal cell bodies and proximal dendrites, gray matter neuropil, axons, and glial cells in normal human hippocampus and neocortex. In the this study, the authors performed a comparative study of the distribution of normal and modified forms of tau in Alzheimer's disease (AD) and control brains. In the cerebral cortex and white matter of AD brains, a massive accumulation of modified tau and/or severe depletion of normal tau were documented in all the tau compartments. In mild AD cases, gray matter neuropil, axons, and glial cells were less severely involved than neuronal perikarya. In the controls, neuronal perikarya were often involved by modified tau accumulation, but the other compartments showed normal distribution. These observations suggest that modifications of tau which lead to neurofibrillary lesions in AD may begin in neuronal perikarya and extend to the other tau compartments in advanced stages of the disease.

Adult↗