Multiple system atrophy with autonomic failure and human T-lymphotrophic virus type I infection.
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Adult↗
Biomedical subjects
T Miyatake
Publications and source records attributed to T Miyatake.
Molecular mimicry between GQ1b ganglioside and lipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher's syndrome.
We isolated Campylobacter jejuni from 2 patients with Fisher's syndrome subsequent to enteritis. Crude lipopolysaccharide fractions were extracted from the bacteria and separated by thin-layer chromatography. Monoclonal antibodies to GQ1b ganglioside (GMR13 and 7F5) reacted with both lipopolysaccharide fractions, indicating that the lipopolysaccharides bear the GQ1b epitope. This is the first report of molecular mimicry between neural tissue components and the antecedent infectious agents of Fisher's syndrome.
Antibodies, Monoclonal↗
Mutational analysis of the amyloid precursor protein gene in Japanese familial Alzheimer's disease kindreds.
We sequenced the entire coding region of the amyloid precursor protein (APP) genes of 11 unrelated patients with Japanese familial Alzheimer's disease (FAD) in order to determine the exact frequency of known APP gene mutations and to search for novel mutations responsible for FAD. Three out of 11 (27.3%) FAD patients showed the known Val to Ile mis-sense mutation at codon 717, but no other mutations were detected in the entire coding region. Analysis of exons 16 and 17 in 30 Japanese with sporadic AD revealed no mutations. Moreover, there were no significant differences in the allele frequencies of the DNA polymorphism in intron 9 among the 11 FAD, 39 sporadic AD, and 110 control subjects.
Adult↗
Motor neuron disease with dementia and ophthalmoplegia. A clinical and pathological study.
Ophthalmoplegia and dementia are not usually observed in patients with amyotrophic lateral sclerosis. We report the case of a 60-year-old female with ophthalmoplegia and frontal-type dementia which appeared at an early stage of her illness that presented with dysarthria and weakness in the upper extremities. Notable autopsy findings in the central nervous system were, in addition to the degeneration of upper and lower motor neurons, moderate neuronal loss and spongy degeneration in layer II of the frontal cortex with prominent astrocytosis, and moderate neuronal loss with astrocytosis in both the substantia nigra and the red nucleus. Central chromatolysis of a few neurons in the oculomotor nucleus was seen. This case is considered to be a new subtype of motor neuron disease.
Atrophy↗
Increased central motor tract excitability in Creutzfeldt-Jakob disease.
Central motor tract excitability was examined in 2 patients with Creutzfeldt-Jakob disease (CJD) and 8 normal subjects by measuring change in the motor evoked potential (MEP) by transcranial magnetic stimulation of the motor cortex after peripheral nerve stimulation. Conditioning stimulation of the median nerve at the wrist greatly increased MEP size (500-1700%) as compared to the size for normal subjects (140-380%) at conditioning-test (C-T) intervals of 30-60 msec for patient 1 and 40-70 msec for patient 2. Moreover, stimulation of the contralateral median nerve at the wrist and of the second and third digits also increased MEP size in the CJD patients; whereas, there was no increase in size in the normals. The time courses of abnormal MEP potentiation were very consistent with the course of the corresponding C reflex. One of the CJD patients had a normal size SEP, and neither patient showed hyperexcitability in SEP-recovery at C-T intervals of 20-60 msec at which there was marked MEP potentiation. These results indicate that there was hyperexcitability of the central motor tract in the CJD patients after the conditioning of muscle and cutaneous peripheral afferents and that it extended to the ipsilateral cortex on the conditioning side as well.
Aged↗
Expression of a blood group B antigen-related glycoepitope in human dorsal root ganglion cells.
Carbohydrate epitopes of glycoconjugates are expressed on sensory neurons of dorsal root ganglion (DRG). A possible role of antibodies directed at carbohydrate determinants of the glycoconjugates has been suggested in some patients with sensory neuropathy. We investigated expression of blood group antigen-related epitopes in human DRG immunohistochemically using monoclonal antibodies to A, B, and H antigens. A blood group B determinant [Gal alpha 1-3(Fuc alpha 1-2)Gal beta-]-related glycoepitope was demonstrated in the neurons and surrounding satellite cells of DRG obtained from subjects with any ABO blood group phenotype. The treatment with trypsin or chloroform/methanol prior to the immunostaining suggested that the glycoconjugate exhibiting the blood group B determinant-related epitope consisted mainly of glycoprotein and included glycolipid. The glycoconjugates with the blood group B determinant-related epitope may play a role in the physiological function and pathophysiology of human DRG neurons.
ABO Blood-Group System↗
Conduction slowing without conduction block of compound muscle and nerve action potentials due to sodium channel block.
We studied the effect of lidocaine on nerve conduction in vivo. Recovery of the compound muscle action potential (CMAP), sensory nerve action potential (SNAP), and single motor unit potential (MUP) of median nerve stimulation was recorded in four healthy volunteers after intravenous infusion of 20 ml of 0.5% lidocaine. During loading, CMAP and SNAP amplitudes rapidly decreased and their latencies increased. After recovery of the CMAP and SNAP amplitudes, nerve conduction velocity improved gradually over a period of 3-6 h, the amplitudes and configurations of CMAP and SNAP remaining unchanged. The conduction velocity of the single MUP markedly slowed before it is blocked. This indicates that maximum conduction velocity of CMAP and SNAP could be slowed by the partial inactivation of sodium channels without accompanying conduction block. Prolongation of the rise time of depolarization of the axonal membrane potential may be the active mechanism in this slowing because of sodium channel inactivation. Abnormalities in sodium channels at the nodes of Ranvier should be considered as a mechanism of conduction slowing even when there is no conduction block.
Action Potentials↗
Developmental changes of sialylation of soluble beta/A4 amyloid protein precursor derivatives in human cerebrospinal fluid.
Soluble beta/A4 amyloid protein precursor derivatives (APPs) in cerebrospinal fluid from infants, children, adults and aged individuals were treated with neuraminidase. In the samples from infants, reduction of molecular weight of APPs following neuraminidase treatment was significantly less than those from adults or aged individuals. Hyposialylation of beta/A4 amyloid protein precursor in infants may be relevant to a physiological role of this molecule in the development of the nervous system.
Adult↗
Penner's serotype 4 of Campylobacter jejuni has a lipopolysaccharide that bears a GM1 ganglioside epitope as well as one that bears a GD1 a epitope.
The carbohydrate structures of lipopolysaccharides (LPSs) of Campylobacter jejuni strains belonging to Penner's serotypes (PEN) 1, 2, 4, 19, 23, and 36 were studied by thin-layer chromatography and immunostaining with several monoclonal antiganglioside antibodies. Anti-GM1 and anti-GD1a antibodies reacted with the LPSs of PEN 1, 4, and 19. Aspinall et al. (G. O. Aspinall, A. G. McDonald, T. S. Raju, H. Pang, A. P. Moran, and J. L. Penner. Eur. J. Biochem. 213:1017-1027, 1993) recently reported that the LPS of PEN 4 has a GD1a ganglioside-like structure rather than a GM1-like structure. We found that the LPS fraction of C. jejuni (PEN 4) has an LPS that bears a GM1 epitope as well as an LPS that bears a GD1a epitope.
Antibodies, Monoclonal↗
Complementation of mutant and wild-type human mitochondrial DNAs coexisting since the mutation event and lack of complementation of DNAs introduced separately into a cell within distinct organelles.
The rules that govern complementation of mutant and wild-type mitochondrial genomes in human cells were investigated under different experimental conditions. Among mitochondrial transformants derived from an individual affected by the MERRF (myoclonus epilepsy associated with ragged red fibers) encephalomyopathy and carrying in heteroplasmic form the mitochondrial tRNA(Lys) mutation associated with that syndrome, normal protein synthesis and respiration was observed when the wild-type mitochondrial DNA exceeded 10% of the total complement. In these transformants, the protective effect of wild-type mitochondrial DNA was shown to involve interactions of the mutant and wild-type gene products. Very different results were obtained in experiments in which two mitochondrial DNAs carrying nonallelic disease-causing mutations were sequentially introduced within distinct organelles into the same human mitochondrial DNA-less (rho 0) cell. In transformants exhibiting different ratios of the two genomes, no evidence of cooperation between their products was observed, even 3 months after the introduction of the second mutation. These results pointed to the phenotypic independence of the two genomes. A similar conclusion was reached in experiments in which mitochondria carrying a chloramphenicol resistance-inducing mitochondrial DNA mutation were introduced into chloramphenicol-sensitive cells. A plausible interpretation of the different results obtained in the latter two sets of experiments, compared with the complementation behavior observed in the heteroplasmic MERRF transformants, is that in the latter, the mutant and wild-type genomes coexisted in the same organelles from the time of the mutation. This would imply that the way in which mitochondrial DNA is sorted among different organelles plays a fundamental role in determining the oxidative-phosphorylation phenotype in mammalian cells. These results have significant implications for mitochondrial genetics and for studies on the transmission and therapy of mitochondrial DNA-linked diseases.
Base Sequence↗
[Acute relapsing ataxic polyradiculoneuritis following respiratory tract infections].
We report a patient with a relapsing form of acute ataxic polyradiculoneuritis. The patient developed marked sensory ataxia with mild limb weakness following a respiratory tract infection. Five similar relapses occurred over 18 years. The symptoms reached their maximum in 2 weeks, followed by subsequent gradual improvement over 1-2 months. Sural nerve biopsy showed mild loss of large myelinated fibers.
Acute Disease↗
[Physiological diagnostic techniques of patients with vasculitis].
This article discusses physiological diagnostic methods in the intractable vasculitis, including Buerger's, Takayasu's and vasculo-Behçet disease. Clinical manifestations of these diseases depend on the lesions of the diseased arteries or veins such as obstruction of the extremities often occurring in Buerger's disease, the obstruction of aortic arch branches in Takayasu's disease and the deep vein thrombosis in vasculo-Behçet disease. Measurements of segmental blood pressure and flow allows a detection of peripheral vascular disease and non-invasive diagnostic techniques, such as the doppler ultrasonic techniques and strain-gauze plethysmography, permit accurate evaluation of arterial occlusive disease and estimation of its physiological significance. Noninvasive diagnostic techniques, together with clinical symptoms and physical examinations, make possible correct diagnosis and subsequently appropriate treatment.
Blood Pressure Determination↗
[Increased unilateral central motor tract excitability in a patient with corticobasal degeneration].
In a 64-year-old woman with a clinical diagnosis of corticobasal degeneration, a hyperexcitability of central motor tract was demonstrated. She had akinesia, regidity, myoclonic movement, hyperreflexia, forced grasping and clumusiness on the right extremities. The asymmetry of these symptoms was striking. Dopa therapy was not effective. On single photon emission computed tomography (SPECT), blood perfusion was reduced especially on the left side in the frontoparietal area. She had large C-reflex after the median nerve stimulation only on the affected side. The intensity threshold of motor evoked potential by stimulation on the left motor cortex was lower than that on the right motor cortex. Conditioning stimulation of the median nerve on the affected side, greatly increased MEP size as compared to those of normal subjects, at conditioning-test (C-T) intervals of 40-80 ms; whereas, stimulation on the contralateral side did not. On the other hand, SEP did not have a marked increase or asymmetry in size and SEP recovery curve showed no hyperexcitability. These results indicate there is a hyperexcitability of the central motor tract on the affected side.
Afferent Pathways↗
[Cervical magnetic motor-evoked potential--the site of the stimulation].
Magnetic stimulation over the cervical spine using the 8-shaped coil was studied to determine the optimal site and intensity of stimulation. Twenty-one healthy volunteers and 9 patients with acute inflammatory demyelinating polyradiculoneuritis (AIDP) or chronic inflammatory demyelinating polyradiculoneuritis (CIDP) were examined. Each wing of the 8-shaped coil had a 5 cm outer diameter and the field (maximum 2.2 tesla) was produced with MAGSTIM200 (MAGSTIM company). The coil was placed along the line from the seventh cervical prominence to the acromion on the recorded side (C7 standard line) to give orthodromic stimulation to the motor nerve. The coil position was marked at the contact point of each wing. Motor evoked potential (MEP) was recorded from abductor digitorum minimi muscle by using surface electrodes. In normals, the latency of MEP did not change while the coil was moved vertically from 2 cm rostral to 2 cm caudal of C7 standard line and horizontally from the midline to 6 cm lateral. Increasing the stimulus intensity, MEP latency never got shorter when the intensity was over 180% of motor threshold. On the other hand, the MEP amplitude was highest when the stimulation was given at 4 cm lateral from the midline on the C7 standard line, but our equipment were unable to give the supramaximal stimulation. Our conclusion was therefore that the optimal stimulus point was 4 cm lateral from the midline on the C7 standard line, and the minimal latency of MEP was obtained by the stimulation with the intensity beyond 180% of motor threshold.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Analysis of cardiac function of discordant heart xenografts using a blood-perfused, isolated, supported heart model].
UNLABELLED: Changes in cardiac function during hyperacute rejection are not fully understood because of lack of appropriate models. In this study, a blood-perfused, isolated, supported heart model was employed for the analysis of cardiac function in discordant heart xenografts. METHODS: Experiment 1: Changes in left ventricular end-systolic pressure (LVESP) and coronary perfusion pressure (CPP) were measured for 120 min. Dry heart weight after perfusion was measured in the following 4 groups: group A; isolated rat heart perfused with blood from support rat, group B; guinea pig heart, support guinea pig, group C; guinea pig heart, support rat, and group D; guinea pig heart, support rat with cobra venom factor (CVF) pretreatment. Complement C3 of support rat serum in group C and group D was measured by single radial immunodiffusion (SRID). Experiment 2: Fifteen guinea pig hearts perfused with blood from untreated support rats were analyzed for correlation between survival time and LVESP, and CPP as well. RESULTS: In groups A and B, LVESP and CPP was stable up to 120 min. In group C, 4 out of 6 hearts were stopped beating within 120 min. The difference between LVESP at 10 min in group C and that in group B was not obtained, possibly due to high variation of values in group C, whereas CPP in group C was higher than that in group B (p < 0.05). In group D, CVF was shown to deplete complement C3. Group D showed constant LVESP and CPP, similar to non-xenograft groups. Dry heart weight of group C was larger than those of group B and D. There were positive correlations between survival time and LVESP at 10 min, and increasing rate of LVESP after 10 min as well. A negative correlation between survival time and CPP at 10 min was observed, while no correlation was obtained between survival time and increasing rate of CPP after 10 min. CONCLUSIONS: 1) Decreases in LVESP and increases in CPP in xenograft group are considered to be due to hyperacute rejection. 2) These changes can be abolished by depletion of C3. 3) Guinea pig hearts can work well in xenograft condition as in allograft condition in certain circumstances, i.e. depletion of C3. 4) The blood-perfused, isolated, supported heart model is useful for the analysis of cardiac function in discordant xenografts.
Animals↗
Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis.
We report a 57-year-old woman with familial amyotrophic lateral sclerosis (ALS) with posterior column involvement of 11 years duration. The patient had been on a respirator for more than 5 years before death. In addition to the well established pathology of this form of familial ALS, there were obvious degenerative changes in the brainstem tegmentum, including the reticular formation, cerebellar cortex, dentate and red nuclei, thalamus and mammillary body. Of great interest in this case was intracytoplasmic accumulation of neurofilaments in the remaining neurons in these areas, some of which, for instance, those in the oculomotor and abducens nuclei, still showed only minimal loss. This patient had survived considerably beyond the point of respiratory failure, which is the final stage in the natural history of the disease. We considered that in this patient the underlying degenerative process of the disease had become manifested in wider areas than recognized previously during the prolonged clinical course due to respiratory support. However, whether or not such widespread degeneration would occur in all patients with this disease who survive longer with the help of respiratory support awaits further studies.
Amyotrophic Lateral Sclerosis↗
Absence of linkage disequilibrium at amyloid precursor protein gene locus in Japanese familial Alzheimer's disease with 717Val-->Ile mutation.
To date, eleven independent FAD pedigrees with the 717Val-->Ile mutation have been identified. Interestingly, five pedigrees were of Japanese origin and four were of British origin. The apparent ethnic prediction of this mutation raises the possibility that there is a founder effect in these two island nations. We did not observe any significant linkage disequilibrium in any locus of APP and GT12 loci in the five Japanese FAD pedigrees with the 717Val-->Ile mutation. A founder effect would probably not be present in Japanese FAD pedgrees with the 717Val-->Ile mutation.
Alleles↗
A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure.
There is a strong association between Guillain-Barré syndrome (GBS) and Penner's serotype 19 (PEN 19) of Campylobacter jejuni. Sera from patients with GBS after C. jejuni infection have autoantibodies to GM1 ganglioside in the acute phase of the illness. Our previous work has suggested that GBS results from an immune response to cross-reactive antigen between lipopolysaccharide (LPS) of the Gram-negative bacterium and membrane components of peripheral nerves. To clarify the pathogenesis of GBS, we have investigated whether GM1-oligosaccharide structure is present in the LPS of C. jejuni (PEN 19) that was isolated from a GBS patient. After extraction of the LPS, the LPS showing the binding activity of cholera toxin, that specifically recognizes the GM1-oligosaccharide was purified by a silica bead column chromatography. Gas-liquid chromatography-mass spectrometric analysis has shown that the purified LPS contained Gal, GalNAc, and NeuAc, which are sugar components of GM1 ganglioside. 1H NMR methods [Carr-Purcell-Meiboom-Gill (CPMG), total correlation spectroscopy (TOCSY), and nuclear Overhauser effect spectroscopy (NOESY)] have revealed that the oligosaccharide structure [Gal beta 1-3 GalNAc beta 1-4(NeuAc alpha 2-3)Gal beta] protrude from the LPS core. This terminal structure [Gal beta 1-3GalNAc beta 1-4(NeuAc alpha 2-3)Gal beta] is identical to the terminal tetrasaccharide of the GM1 ganglioside. This is the first study to demonstrate the existence of molecular mimicry between nerve tissue and the infectious agent that elicits GBS.
Adult↗