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Biomedical subjects

I Tominaga

Publications and source records attributed to I Tominaga.

At least 19 recordsLinked to original sources
PubMed2002

Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.

We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.

Adult↗
PubMed2001

Charge microheterogeneity of the beta-trace proteins (lipocalin-type prostaglandin D synthase) in the cerebrospinal fluid of patients with neurological disorders analyzed by capillary isoelectrofocusing.

Charge microheterogeneity of the beta-trace protein (beta-TP = lipocalin-type prostaglandin D synthase) in the cerebrospinal fluid (CSF) of patients with various neurological disorders was analyzed by capillary isoelectric focusing (CIEF). Under the conditions employed, beta-TP in the low-molecular-weight protein fraction of CSF was separated into at least four isoforms with different p/ values. An isoform with the pl value of 4.6-4.8 was usually the most abundant. The total beta-TP level in the CSF was determined by enzyme-linked immunosorbent assay (ELISA) to be elevated in patients recovering from organic damage to the CNS and those with pathological brain atrophy. Changes in the total beta-TP level in the CSF were occasionally accompanied by those in its charge microheterogeneity, as revealed by CIEF. Such quantitative and qualitative changes in beta-TP in human CSF indicated changes in its pathophysiological roles in association with various neurological disorders.

Adult↗
PubMed2000

Sodium dodecylsulfate capillary gel electrophoretic measurement of the concentration ratios of albumin and alpha2-macroglobulin in cerebrospinal fluid and serum of patients with neurological disorders.

Sodium dodecylsulfate capillary gel electrophoresis (SDS-CGE) was applied to measure the concentration ratios of albumin (Alb) and alpha2-macroglobulin (alphaMG) in the cerebrospinal fluid (CSF) and concurrent serum samples from patients with various neurological disorders. The values of the alphaMG index in individual patients were calculated on the basis of the peak area ratios of Alb and an alphaMG subunit on the CSF and serum electropherograms. The alphaMG index value thus obtained was most prominently raised in patients with inflammatory diseases of the brain and/or meninges, suggesting that the function of the blood-brain barrier (BBB) was disturbed under the pathological conditions in the central nervous system. The measurement of the concentration ratios of Alb and alphaMG in CSF and the concurrent serum samples by the present SDS-CGE system seems to be useful as an aid in the biochemical examination of the BBB function in patients with neurological disorders.

Adolescent↗
PubMed1998

Sodium dodecyl sulfate-capillary gel electrophoretic analysis of molecular mass microheterogeneity of beta-trace protein in cerebrospinal fluid from patients with central nervous system diseases.

Molecular mass (M(r)) microheterogeneity of beta-trace protein (beta TP) in cerebrospinal fluid (CSF) from patients with various neurological disorders was analyzed by sodium dodecyl sulfate capillary gel electrophoresis. Under the conditions employed, beta TP with a M(r) distribution of 23,000-30,000 was roughly separated into two subfractions containing the major peaks with M(r) of 26,000 and 28,500, respectively. The peak area ratios of the two subfractions of the electropherograms varied among the samples examined, and elevation in the total beta TP level in the CSF from patients with organic diseases in the central nervous system (CNS) was often accompanied by changes in the ratios of the subfractions. The quantitative changes in the subfraction level in CSF beta TP are considered to reflect the pathological alterations in the CNS.

Adult↗
PubMed1997

Analysis of low-molecular-mass proteins in cerebrospinal fluid by sodium dodecyl sulfate capillary gel electrophoresis.

Proteins of low molecular (M(r)) in human cerebrospinal fluid (CSF) were analyzed by capillary electrophoresis in a sodium dodecyl sulfate-containing polymer solution. Under the conditions employed, peaks of beta 2-microglobulin (beta MG) (M(r): 11,700), gamma-trace protein (12,300), myelin basic protein (18,000), beta-trace protein (beta TP) (23,000 to 30,000) and alpha 1-acid glycoprotein (42,000) were detected on the electropherograms. The concentrations of beta MG and beta TP were determined based on the peak area relative to that of an internal standard, Orange G, which was added at a constant amount as the front marker. It was demonstrated that their levels in CSF change under various pathological conditions in the central nervous system.

Adolescent↗
PubMed1997

Capillary electrophoretic analyses of beta-trace protein and other low molecular weight proteins in cerebrospinal fluid from patients with central nervous system diseases.

Ordinary capillary-zone electrophoresis (CZE), as well as CZE in a sodium dodecylsulfate-containing polymer solution (SDS-CZE) and capillary isoelectrofocusing (CIEF), was applied to the analysis of low molecular weight proteins in cerebrospinal fluid (CSF) from patients with various neuropsychiatric disorders. Under the CZE conditions employed, a peaks of beta-trace protein (beta TP), which is the most abundant low MW protein in CSF, was clearly detected on the electropherograms of all the samples examined, and the CSF beta TP level could be tentatively determined using allylamine added at a constant concentration as the internal standard. The results revealed that beta TP in CSF was non-specifically increased in organic disease in the central nervous system (CNS), especially in ones giving severe physical damage to the brain tissues. On the other hand, SDS-CZE allowed us to determine simultaneously the CSF minor low MW proteins other than beta TP, such as beta 2-microglobulin, gamma-trace protein, myelin basic protein, etc., while the CIEF electropherograms suggested that beta TP were separated into several fractions with the different PI values. These capillary electrophoresis systems seem to be powerful as aids in the biochemical examinations of beta TP and other low molecular weight proteins in CSF from patients with CNS diseases.

Beta-Globulins↗
PubMed1997

Reduction in blood free carnitine levels in association with changes in sodium valproate (VPA) disposition in epileptic patients treated with VPA and other anti-epileptic drugs.

Reduction in the blood free carnitine (FC) level as a side effect of sodium valproate (VPA) given epileptic patients was pharmacokinetically studied in connection with changes in the VPA disposition. The serum FC level in patients taking at least one of phenobarbital (PB), phenytoin (PHT) and/or carbamazepine (CBZ) in addition to VPA was significantly lower than that in the controls given only these other anti-epileptic drugs (AEDs). Patients medicated only with VPA also tended to have a lower serum FC level than the controls, although the difference was not significant. Among all the patients taking VPA with or without other AED(s), a significantly positive correlation was observed between the serum FC level and the value of dose and level ratio (L/D) of VPA, indicating that both the serum FC concentration and the L/D value of VPA were remarkably reduced in those patients receiving both medications. These results suggested that reduction in the blood FC level as a side effect of VPA reflected FC deficiency associated with the accelerated degradation of VPA in liver; such a condition appears to result from medication with VPA and other AED(s) which induce(s) enzyme(s) for the VPA metabolism.

Adult↗
PubMed1996

[A 46-year-old man with right-side dominant parkinsonism, who suffered a sudden death].

We report a 46-year-old man with right side dominant parkinsonism who died suddenly two years after the onset. The patient was well until the age of 42 years in January of 1993, when he noted an onset of difficulty in using his right hand and then the right leg. Soon after he noted nocturnal urinary incontinence. In January of 1994, a local doctor prescribed 200 mg of levodopa with benserazide and 5 mg of bromocriptine. The patient noted some improvement. Cystometry revealed 300 ml of residual urine. He visited our clinic on 24th of December, 1996. He was alert and oriented. BP was 106/60. He showed masked face and small voice. He walked in stopped posture dragging his feet; retropulsion was noted. He showed moderate bradykinesia and rigidity more on the right side. No resting tremor or cerebellar ataxia was noted. Ankle jerks were somewhat exaggerated but no Babinski sign was noted. He continued to show residual urine, but orthostatic hypotension was absent. Routine laboratory examination was unremarkable, however, his cranial MRI showed atrophy of the left putamen and a T2-linear high signal intensity lesion along the lateral border of the left putamen. On January 15, 1997, he ate certain amount of rice cake and drank alcohol. After coming back home and while changing his clothes, he suddenly complained of chest discomfort and lost consciousness. He was pronounced dead in the afternoon. The patient was discussed in a neurological CPC. Opinions were divided between Parkinson's disease and striatonigral degeneration. The chief discussed arrived at a conclusion that the patient had Parkinson's disease, because he responded to levodopa to some extent and except for nocturnal incontinence he did not have wide spread autonomic failure. Postmortem examination revealed marked loss of neurons and extensive gliosis in the left putamen. The right putamen did not show such changes. The substantia nigra showed gliosis in the lateral part on both side, however, neuronal loss was not apparent. The locus coeruleus was well retained. No Lewy bodies were found. The pontine nucleus and the cerebellum were intact. However, glial cytoplasmic inclusions were seen in oligodendrocytes of the cerebral white matter and the pontine base. The heart and lungs were intact and the cause of the sudden death could not be determined. The pathologic diagnosis is striatonigral degeneration. Such a marked asymmetry of the pathologic change is quite unusual. Probably, the death in the early stage of the disease is the reason for this asymmetry.

Brain↗
PubMed1995

[A case of diffuse brain injury involving the medial part of the brain--its difference from diffuse axonal injury].

A 30-year-old male clinico-pathological case survived for 1 year and 9 months after being hit by a truck while riding on his motorbike on Aug. 21, 1988. On admission, his consciousness level was 5 according to the Glasgow Coma Scale, and a traumatic intraventricular hemorrhage and cerebral contusion were revealed by CT scanning. He underwent immediately an operation in order to drain blood from the ventricles at which time a right side dominant quadriplegia was noted. He made a gradual improvement and by January 1989 was able to tell us his name and address correctly. However, he remained incontinent and bedridden owing to the contracture of joints. He was put on rehabilitation exercises in March 1989 which trained him to operate a wheelchair. In April 1990 he regained urinary control, but was remarkably devoid of will power, perseverance and memory. He expired of pneumonia on May 11, 1990. At autopsy, his brain weighed 1180g. The cerebral convexity was discolored, especially the rectal gyri and bilateral olfactory bulbs were brownish-yellow. Old gross contusional scars were observed on the left rectal and orbital gyri, and the 3rd ventricle and inferior horns of the lateral ventricles were enlarged. Holzer's method revealed fibrillary gliosis in the corpus callosum, fornix, cingulate gyrus and a part of the caudate nucleus adjacent to the thalamus. Microscopically, axons were seen to be disrupted in the corpus callosum as well as in the anterior commissure, having the appearance of macrophages. (ABSTRACT TRUNCATED AT 250 WORDS)

Axons↗
PubMed1995

Prion disease with 144 base pair insertion in a Japanese family line.

We describe an insert mutation in the prion protein (PrP) gene in a Japanese family line that encodes six octapeptide repeats. This is the second report to date of an inherited prion disease with a 144-base pair insertion, although the order of the repeat sequences differ from that reported for the disease in an English family line. The clinical features, like those of the English patients, were characterized by a slowly progressive generalized dementia with some neurological signs and cortical focal symptoms. Postmortem examination disclosed diffuse atrophy of cerebral gray matter and the cerebellar cortex; histologically, there were marked patchy and regional neuronal loss with astrocytosis in the frontal cortex, amygdala and hippocampus and PrP-immunoreactive plaques in the molecular layer of the cerebellum. These plaques were different from typical kuru plaques. The prion disease in the present Japanese family line is compared with that in the English family line.

Adult↗
PubMed1994

Capillary zone electrophoretic determination of organic acids in cerebrospinal fluid from patients with central nervous system diseases.

Organic acids in cerebrospinal fluid (CSF) from patients with various central nervous system (CNS) diseases were determined by capillary zone electrophoresis (CZE). Under one of the two sets of conditions employed, several anionic components of CSF were separated into corresponding peaks on the electropherograms and determined. The other conditions employed were also useful in measurement of the lactate contents in CSF. The CSF levels of lactate and pyruvate and the ratios of lactate to pyruvate were elevated in patients with cerebral infarction and bacterial meningitis, whereas CSF ascorbate was reduced mainly in inflammatory disorders of the CNS. The results showed that CZE can become a powerful tool in the biochemical diagnosis of CNS diseases.

Brain Diseases↗
PubMed1994

Proton magnetic resonance spectroscopy of cerebrospinal fluid as an aid in neurological diagnosis.

The proton magnetic resonance (1H-NMR) spectra were obtained from 38 cerebrospinal fluid (CSF) samples from 34 patients with various central nervous system (CNS) diseases and the H2O signals were reduced by the presaturation method. A doublet signal for CH3 protons of lactate, as well as a singlet one for alpha-CH proton of glucose, were clearly detected in all the spectra obtained. The relative lactate concentration values were calculated on the basis of the glucose concentrations which had been measured by a routine laboratory method in the hospital and the ratios of the peak heights (lactate CH3/glucose alpha-CH). In the CSF samples examined, the relative lactate values semi-quantitatively determined in this way were clearly elevated in cerebral infarction and bacterial meningitis, but not in other disorders including viral meningitis. On the other hand, one CSF component whose enhancement was found only in a sample from a hepatic encephalitis patient was identified as glutamine from the double quantum filtered-shift correlation spectrum. These results suggest that 1H-NMR spectroscopy of CSF can become a powerful aid in biochemical diagnosis of CNS diseases.

Adult↗
PubMed1994

[Encephalomyelitis with cavitary necrosis of the white matter. A clinico-pathologic study].

The authors report the clinico-pathological study of an encephalomyelitis with necrotic cavitation of the white matter occurring in a 61-year-old female. The disease of sudden onset progressed slowly to the death, 20 months after the onset of the first symptoms. They stress the rarity of the entity and the difficulties to establish the diagnosis clinically. The nosological situation of the condition is discussed and compared with the previously reported cases.

Brain↗
PubMed1993

Capillary-zone electrophoretic analyses of the proteins and amino acid components in cerebrospinal fluid of central nervous system diseases.

Analysis systems for protein and amino acid components in cerebrospinal fluid (CSF) from patients with a variety of central nervous system (CNS) diseases were established employing capillary-zone electrophoresis (CZE). Under the conditions employed, the major proteins in CSF and the concurrent sera were separated into several respective fractions, and in the obtained electropherograms of serum proteins, the % values of areas of each major protein fraction (from gamma-globulin to albumin) were in close accord with those in the densitograms of stained protein bands after electrophoresis on cellulose-acetate strips which had been done as a routine laboratory test in the hospital. Some unusual migration patterns of major proteins were observed in various CNS diseases, and an increase of glutamine in hepatic encephalitis was also detected on the electropherograms. A peak responsible for a minor protein component with the molecular weight of 10000--30000, which was tentatively identified as beta-trace protein, was found to be contained in all the CSF samples examined, and its concentration levels were higher in some patients with cerebral infarction and multiple sclerosis. These results suggest that CZE can become a powerful aid in analyses of the protein and amino acid components in CSF for biochemical diagnosis of CNS diseases.

Amino Acids↗
PubMed1993

[Early infantile epileptic encephalopathy (Ohtahara syndrome) with poly-microgyria].

A boy, born after normal pregnancy and delivery, began to have fits at 3 days. The seizures were composed of tonic or tonic-clonic convulsions at the upper extremities but myoclonus was absent. These attacks were not easy to control. There was gross developmental delay. Laboratory investigations were almost normal except for cerebrospinal fluid: pleocytosis and high protein content. EEG showed "suppression-burst" and MRI revealed high signal intensity in the left temporo-occipital region on T2 weighted image. At three and a half months of age, EEG changed into hypsarrhythmia. The child died at 5 months of age. At post mortem neuropathological examination, the cortical ribbon in the bilateral parieto-occipital regions appeared thick, as if there were pachygyria. Microscopically polymicrogyria was noted in these areas as well as in the insular cortex. This lesion showed a symmetrical distribution. The cytoarchitectonic features of the polymicrogyric cortex did not consist of 4 layers. The other structures of the central nervous system were almost devoid of lesion. The number of clinico-pathological reports on Ohtahara's syndrome is very limited and the etiopathogenesis of polymicrogyria is discussed.

Brain↗
PubMed1992

Effects of anti-epileptic drugs on the L-tryptophan binding to human serum albumin.

Effects of four anti-epileptic drugs (AED; phenobarbital, phenytoin, carbamazepine and sodium valproate) on the L-tryptophan binding to human serum albumin were studied. Among these drugs examined, only sodium valproate inhibited the binding even within the concentrations of its therapeutic range, and the Klotz plotting analysis revealed that the inhibition was competitive. The results of examinations with sera from epileptic patients medicated with these AED and drug-free normal controls also suggested that the protein binding ratios of L-tryptophan were decreased in the blood plasma of some patients with the high valproate concentrations and the low albumin contents.

Anticonvulsants↗
PubMed1991

[Prolonged traumatic coma caused by diffuse axonal lesions].

A 22-year old male patient was admitted for deep coma probably of traumatic origin. There was neither fracture of the skull nor expansive intracranial lesion. The patient survived for 6 years and 8 months without any change in consciousness. Post-mortem neuropathological examination showed lesions which predominated in the white matter and had features that were compatible with diffuse axonal injury. The mechanism responsible for these lesions seems to be stretching and shearing of axones at the moment of impact. The exceptionally long duration of survival probably accounts for the severity of the lesions observed.

Adult↗