A case of pandysautonomia with associated sensory ganglionopathy.
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Adult↗
Biomedical subjects
Y Mimori
Publications and source records attributed to Y Mimori.
Sarcoidosis: correlation of HRCT findings with results of pulmonary function tests and serum angiotensin-converting enzyme assay.
We examined correlations between findings on chest high resolution computed tomography (HRCT), pulmonary function and values of serum angiotensin converting enzyme (ACE) in 25 patients with sarcoidosis. The most frequent CT features were small nodular opacities. The small nodules, representing the confluence of epithelioid granulomas, are strongly correlated with peribronchovascular, perilobular, and centrilobular lesions, where there is an abundance of lymphatic plexus. This strongly suggests the importance of the lymph vessels in the pathogenesis of sarcoidosis. The pulmonary functions tests showed obstructive defects in 6 and mixed-type defects in 2 of the 25 patients. Furthermore, an elevation of V50/V25 ratio suggesting small-airway disease was detected in many patients who showed normal values of FEV1.0% and %VC. This fact indicates that small-airway disease was manifested earlier in sarcoidosis patients. Statistically significant negative correlations were found between visual score and %VC, %FVC, FEV1.0%, %TLC, and %DLco, but there was no significant correlation between visual score and serum ACE. ACE is derived from granuloma-forming epithelioid cells, and the activity of ACE decreased rapidly in mature granulomas. Epithelioid cells in the mature granulomas which can be recognized on HRCT scan have stopped or are about to stop the release of ACE. In this study, serum ACE activity was found to be elevated and correlated with %V25 and V50/V25 at an early stage of the disease. The results of this study provide meaningful insights into the process of sarcoidosis in lung.
Adult↗
Abnormalities of acetylcholinesterase in Alzheimer's disease with special reference to effect of acetylcholinesterase inhibitor.
In brains from Alzheimer's disease patients, a high activity of acetylcholinesterase (AChE) was detected in the senile plaque-rich fraction and its isozyme pattern was mainly type A, containing a collagen-like tail. AChE inhibitors, including physostigmine, E-2020, amiridin, tetrahydroaminoacridine (THA) and Nicergoline had a poor effect on AChE present in the senile plaque-rich fraction isolated from Alzheimer brain than that either in the soluble fraction of Alzheimer brain or in the control brain. However, AChE purified from rat skeletal muscle (type A) was significantly more susceptible to AChE inhibitors than that purified from rat brain (G4 form) or from human erythrocytes (G2 form). E-2020 inhibited all 3 types of isozymes more effectively than physostigmine, amiridine, Nicergoline or THA. The inhibitory effect of AChE inhibitors on AChE solubilized from senile plaque was also small as compared with AChE in normal human brain, rat brain, human erythrocytes or rat skeletal muscle. These results suggest that the characteristics of AChE present in senile plaques are abnormal or different from that in normal brain or skeletal muscle. As AChE in the Alzheimer brain seems to contain a higher degree of glycosylation, the hydrophobic property of anomalous AChE may serve a seed of amyloid fibril in senile plaques.
Acetylcholinesterase↗
Creutzfeldt-Jakob disease associated with autonomic nervous system dysfunction in the early stage.
A 54-year-old man presented with tremor and unusual behavior. He was admitted two months later because of dementia and myoclonus. Periodic synchronous discharges were observed on the electroencephalogram. Based on these findings, we diagnosed the case as Creutzfeldt-Jakob disease. About two weeks after admission, decubitus, bowel dysfunction and hypohidrosis occurred. We observed various autonomic nervous system dysfunctions such as abnormal pupillary response to autonomic drugs, reduced coefficient of variation of R-R interval, and abnormal diurnal blood pressure variation.
Atrophy↗
[A case of vitamin B12 deficiency with broad neurologic disorders and canities].
The patient was a 48-year-old woman with gait disturbance as her initial symptom. Two years after the onset of the gait disturbance, she developed motor weakness and a sensory disturbance in her limbs, and dementia. On admission to our hospital, the patient's serum vitamin B12 and folic acid levels were low, and she was found to have normochromic anemia. She also had widespread coarse hair and canities was diagnosed. The patient's paresthesia resolved in response to injection of 500 micrograms mecobalamin every other day, and the Hasegawa dementia scale score and the patient's hand grip strength improved. Her gait also improved, and she became able to walk on tiptoe. Her hair returned to normal. Supplementation with high-dose methyl B12 appeared to be effective to some extent in treating a broad range of neurologic disorders besides subacute combined degeneration of the spinal cord. In addition, the hair abnormality may be a marker of vitamin B12 deficiency.
Dementia↗
Thiamine therapy in Alzheimer's disease.
Fursultiamine (TTFD), a derivative of thiamine, at an oral dose of 100 mg/day had a mild beneficial effect in patients with Alzheimer's disease in a 12-week open trial. The improvement could be observed not only in their emotional or other mental symptoms but also in intellectual function. Only mildly impaired subjects showed cognitive improvement. Alzheimer patients' blood levels of thiamine before the trial were within the normal range. No adverse reactions were observed and all patients tolerated the trial well. TTFD could afford an alternate treatment to large doses of thiamine hydrochloride in Alzheimer patients. However, further investigations of the therapeutic implications of thiamine and its possible etiologic clues to Alzheimer's disease are necessary.
Aged↗
Radial mass analysis of the flagellar filament of Salmonella: implications for the subunit folding.
X-ray fiber diffraction patterns of the R-type straight flagellar filament of Salmonella typhimurium SJW1655 strain showed layer-lines with an axial spacing of 1/437 A-1, which could be resolved only due to very small disorientation angles (< 2 degrees) of the filaments in oriented sol specimens. Although the equatorial layer-line was situated between the relatively strong first layer-lines right above and below it, these small disorientation angles and a new method of two-dimensional angular deconvolution allowed us to determine the equatorial layer-line intensities quite accurately. The equatorial data were phased by using the amplitude difference between the native flagellar filament and its heavy atom derivatives. One of the heavy-atom derivatives was prepared by introducing a cysteine residue by site-directed mutagenesis and applying a mercury compound. From the equatorial structure factors, the radial density distribution of the filament was calculated at 11 A resolution. A prominent feature was two pairs of high density peaks at radii of around 25 and 45 A and a deep density trough between them, which corresponds to the concentric double tubular structure in the core region that has been found in the density map recently deduced by helical image reconstruction from electron micrographs of frozen hydrated filaments. The molecular masses were estimated for four radial segments that correspond to the morphological domains identified in the map of helical image reconstruction. Then the domains were assigned to sequence positions by correlating the estimated masses with those of proteolytic fragments of flagellin. The assignment is consistent with the distributions of secondary structures and in particular alpha-helical coiled-coils that were predicted from the sequence. It also helps to understand how the polymerization behaviour is affected by truncation of the disordered terminal regions of flagellin and why mutations in a specific region are responsible for changes in the polymorphic shape of the filament.
Base Sequence↗
The structure of the R-type straight flagellar filament of Salmonella at 9 A resolution by electron cryomicroscopy.
The supercoiled forms of the flagellar filaments are thought to be constructed from a mixture of two distinct subunit conformations arranged in a regular manner. We analyzed the structure of one of the two straight flagellar filaments, each of which is built up with all its subunits in one of the two conformations. The filament we studied was isolated from the strain SJW1655 of Salmonella typhimurium and had a right-handed helical symmetry. With recent advancements in electron cryomicroscopy, such as a liquid helium temperature stage for frozen hydrated specimens and a stable field emission source, and also by averaging high resolution data with a proper correction of the contrast transfer function, the density distribution map of this straight flagellar filament was generated in far more detail than before by including data up to 9 A resolution. The structure shows a densely packed core region from about 15 to 55 A in radius, where a pair of concentric tubular features of high density is present without well-defined subunit boundaries, and an outer part from 55 to 115 A, where the subunits are mostly well separated from each other. The outer tube in the core region, from 35 to 55 A in radius, contains many rod-like features with near-axial orientation and closest lateral distances of around 10 A, which are most likely to represent the alpha-helical bundles that were predicted in our previous report. In the inner tube, from 15 to 30 A in radius, the rod-like features are less clear. Between the inner and outer tubes are the short spoke-like densities, which are radially tilted and are connecting the two tubes. The outer part, from 55 to 115 A, contains an axially elongated column density and a slewed projection with a narrow neck region. When compared with the other straight filament having left-handed helical symmetry, this outer part does not show any significant changes in orientation, suggesting that the switch in the subunit conformation and packing involved in the polymorphic transitions is quite subtle and only occurs within the core region. Reassignment of each structural domain to the amino acid sequence is suggested, based on the volume of each domain, which was determined rather precisely by a proper correction of the contrast transfer function for both amplitudes and phases.
Bacterial Proteins↗
Extrusion of rotating microtubules on the dynein-track from a microtubule-dynein gamma-complex.
Applying a new in vitro motility assay system for microtubules and 22S dynein, we recently reported on an ATP-induced extrusion of microtubules from microtubule-dynein alpha- and beta-complexes [Mimori and Miki-Noumura, 1994: Cell Motil. Cytoskeleton 27:180-191]. In the present study, we prepared a gamma-complex by copolymerizing porcine brain tubulin and Tetrahymena ciliary 22S dynein, and examined the ATP-induced microtubule movement from the gamma-complex. The extrusion process appeared quite similar to that of the beta-complex. The sliding velocity was 18.39 +/- 2.20 microns/sec, which was a value comparable to that of trypsin-digested flagellar axonemes [Yano and Miki-Noumura, 1980: J. Cell Sci. 44:169-186]. Higher velocity may be due to a densely arranged dynein-track with the same polarity, which was detached from the gamma-complex and absorbed in rows on a glass surface of the slide. Sometimes a free-floating microtubule in the perfusion chamber was observed riding and sliding on the dynein-track remaining on the slide after extrusion. Unexpectedly, we found that when the front part of the microtubule was fixed to a glass surface, a continuous sliding microtubule at the rear part on the dynein-track often transformed into a left-handed helix, and subsequently a twisted helix with several turns. The helix formation may be due to some rigidity in the microtubule and a right-handed torque component in the sliding force of 22S dynein. The addition of ATP may release some distortion accumulated in the complex structure during copolymerization of tubulin and 22S dynein, inducing reverse rotation of the microtubule.
Adenosine Triphosphate↗
[Relationship between impaired blood pressure control and multiple system involvement in chronic alcoholics].
Chronic ethyl alcohol (ethanol) abuse is associated with different types of neurological involvement. Impaired blood pressure control such as in hypertension and/or orthostatic hypotension is a known autonomic dysfunction in chronic alcoholics. Although abstinence appears to improve blood pressure, the precise mechanisms underlying the effects of alcohol remain unknown. We suspected that abnormal blood pressure might influence the outcome of alcohol-related neurological symptoms. We therefore reviewed the alcohol history of 28 male patients admitted to our hospital with multiple system involvement. No family history of spinocerebellar degeneration was found. All of the patients had a history of chronic alcohol abuse. The duration of alcohol abuse in 28 patients was 28.8 + 11.2 yrs. Daily ethanol consumption was less than 83 ml in 6 patients between 83 ml and 138 ml in 11 and more than 138 ml in 11. We assessed the relationship between impaired blood pressure control, clinical characteristics, and outcome after abstinence for a period ranging from 30 days to 6 years in 22 chronic alcoholics who had drunk more than 83 ml/day. All of the patients had various types of autonomic dysfunction, and a high incidence of impaired blood pressure control and decreased CVR-R were noted. The symptoms improved or even completely resolved after cessation of alcohol consumption, however, 8 of the 22 patients in our study reached a plateau and have permanent symptoms. Patients with no improvement as a result of abstinence had a long history of excessive drinking. Brain CT/MRI, SPECT and P300 studies were conducted. SPECT and P300 were performed in both the supine and standing position in 5 and 7 patients, respectively. Five patients who had severe orthostatic hypotension with syncope were found to have cerebrovascular lesions on MRI. Changes in P300 amplitude and/or distribution and latency were noted after a fall in blood pressure. Although the etiology of impaired blood pressure control in alcoholics remains unknown, we believe that abnormal blood pressure affects the outcome of alcohol-related neurological symptoms.
Adult↗
[Vasomotor impairment in patients with diabetic neuropathy--evaluation by laser Doppler flowmetry].
Using laser Doppler flowmetry, we evaluated cutaneous microcirculation in 28 patients with diabetic neuropathy and 18 control subjects. Skin blood flow in the hand was significantly reduced in the patients compared with that in age-matched controls. Skin blood flow in the hand of the patients correlated significantly with the amplitude of sensory nerve action potential of the median nerve. Significant correlation was shown in the patients between skin blood flow in the hand and changes in systolic blood pressure on standing from a supine position. However, there was no correlation between skin blood flow in the hand and variation in the R-R interval. These results suggest that the skin blood flow may decrease with the progression of diabetic neuropathy, particularly sympathetic autonomic neuropathy, and that neural control may be an important factor in the regulation of skin blood flow. Cold water immersion test revealed that there was no significant difference in vasoconstrictive response during the cold challenge, while recovery time of vasodilatory response after withdrawal of the thermal challenge was significantly prolonged in the patients as compared with that in the controls. In addition, it is suggested that the patients with a small increase or normal in skin blood flow of the hand may possess abnormalities predominantly in vasoconstrictive tone, whereas the patients with a decrease in skin blood flow of the hand in vasodilatory tone. Consequently, microcirculation in skin of extremities changes under the balance of vasoconstrictive and vasodilatory tones in patients with diabetic neuropathy.(ABSTRACT TRUNCATED AT 250 WORDS)
Diabetic Neuropathies↗
ATP-induced sliding of microtubules on tracks of 22S dynein molecules aligned with the same polarity.
Chlamydomonas and Tetrahymena axonemal dyneins have previously been found to bind to porcine brain microtubules to produce a microtubule-dynein complex. At appropriate microtubule:dynein concentration, microtubules in the complex became covered to saturation by dynein arms of the same polarity and at a spacing of 24 nm [Haimo et al., 1979; Haimo and Fenton, 1988; Haimo, 1989; Porter and Johnson, 1983a]. In the present study, two different types of microtubule-dynein complexes (alpha- and beta-complexes) were prepared from Tetrahymena ciliary 22S dynein and porcine brain tubulin. The characteristics of the adenosine triphosphate (ATP)-induced extrusion of microtubules from these complexes were analyzed, as a simple and direct in vitro assay for the ATP-induced extrusion of singlet microtubules. The alpha-complex prepared by adding dynein to microtubules showed an interrupted sliding movement, which would stop and start several times following the addition of ATP. In the beta-complex, prepared by adding dynein bound to DEAE-tubulin to pre-assembled microtubules, microtubules became covered with dynein molecules whose orientation and binding were uniform with respect to microtubule polarity. The microtubules in the beta-complex extruded at 12 microns/second following the addition of ATP. Dark-field and electron microscopy indicated that the extruded microtubules had undergone sliding on a dynein-track that had become detached from the complexes and had been absorbed onto the surface of the glass slide. At higher light intensity under a dark-field microscope, the dynein-track was seen to be composed of rows of dynein molecules arranged densely. The orientation of dynein molecules in rows appeared to be uniform, considering the images of bound dynein in the beta-complex under electron microscope. The higher sliding velocity of the microtubules on these dynein-tracks compared to that seen on slides coated at random with dynein [Vale and Toyoshima, 1988, 1989], may be due to more efficient force generation by this dense arrangement of dynein molecules with the same polarity on the tracks.
Adenosine Triphosphate↗
[Dystonia].
Dystonia is a persistent attitude or posture in one or other of the extremes of athetoid movement. It may take the form of an over-extension or over-flexion of the hand, torsion of the spine, with arching and twisting of the back or forceful closure of the eyes and a fixed grimace. Dystonia is classified into idiopathic and symptomatic dystonia. Idiopathic dystonia is further divided into generalized, focal and segmental dystonia. Generalized dystonia covers classical torsion dystonia, paradoxical dystonia, myoclonic dystonia, dystonia with diurnal variation and Dopa-responsive dystonia. Dystonic tic, paroxysmal dystonia and hypnotic dystonia show a dystonic posture, although they are also accompanied by various other involuntary movements such as athetosis or chorea. Torticollis, writer's cramp or blepharospasm is assigned to the focal dystonia and Meige syndrome to the segmental dystonia. Symptomatic dystonia is observed in various neurological disorders, including cerebrovascular diseases, Parkinson's disease and Wilson's disease.
Adolescent↗
[Sympathetic skin response (SSR)].
The sympathetic skin response (SSR) is a slow wave resulting from activation of the sudomotor sympathetic efferent fibers. Records are usually made with surface electrodes on hand or foot after the electrical stimulation. Either the amplitude or the latency of the response varies greatly on consecutive stimulations and there is also a remarkable tendency to habituation. Therefore SSR is considered abnormal if no significant responses are detected. SSR is well correlated with other autonomic function tests and its abnormality is documented in a variety of neurologic disorders such as diabetic neuropathy, cerebrovascular disease and Parkinson's disease. In good methodological conditions, SSR is a simple, reliable indicator of sympathetic sudomotor outflow in central and peripheral nervous system disorders.
Autonomic Nervous System Diseases↗
Familial hypobetalipoproteinaemia complicated by cerebellar ataxia and steatocystoma multiplex.
A 55-year-old man with cerebellar ataxia and steatocystoma multiplex was found to have reduced serum concentrations of total cholesterol, betalipoprotein and apolipoprotein B. Computed tomography revealed atrophy of the cerebellum and brain stem. Of the six family members examined, four had hypobetalipoproteinaemia, and one had mild ataxia. Similar skin lesions were noted in five male relatives. This case represents a rare combination of familial hypobetalipoproteinaemia, cerebellar ataxia and steatocystoma multiplex.
Cerebellar Ataxia↗
A micromethod for the isolation of large and small microvessels from frozen autopsied human brain.
Microvessels were isolated from autopsied human brain using a simple procedure involving disruption, sieving, and centrifugation on a sucrose density gradient. The present procedure is characterized by isolation, from frozen autopsied brain, of materials either from the cerebral cortex or white matter, and subsequent separation of the capillary fraction from the large vessel fraction. The preparation appears highly purified under phase-contrast microscopic examination. The purity was also established by the enrichment of gamma-glutamyl transpeptidase activity and by the nearly negligible cerebroside content in the vessel fractions as compared to the brain homogenate.
Brain↗
[Meningoencephalitis during the pre-icteric phase of hepatitis A--a case report].
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Acute Disease↗
[An outbreak of Guillain-Barré syndrome with ophthalmoplegia in Kochi].
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Adolescent↗