Spontaneous drainage in syringomyelia.
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Biomedical subjects
Publications and source records attributed to K Tashiro.
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Proteasome, a large protein complex with ATP-dependent protease activities, is composed of non-identical but closely related multi-subunits. Using cDNAs for rat proteasome subunits as probes, we obtained three cDNA clones for two Xenopus proteasome subunits from ovary cDNA library. The primary structures of the three cDNAs showed high homology to the corresponding proteasome subunits of other mammalian species (above 90%) and also considerable homology to those of Drosophila and yeast. These results indicate that the sequences of proteasome subunits are well conserved during evolution. Northern blot hybridization revealed that RNAs for the newly isolated subunits (XC8 and XC9) and the previously isolated subunit (XC3) occur at very high levels in testis and ovary, at moderately high levels in lung, skin kidney and spleen, and at low levels in liver, stomach and muscle. It was also shown that relative amounts of the mRNAs for the three subunits are similar in all the adult tissues examined. From these results, we concluded that the expression of the genes for the three subunits (XC3 XC8 and XC9-1) takes place in a roughly coordinated manner in different adult tissues.
Androgens play an important role in motoneuron growth, development and regeneration. We proved the existence of androgen receptor (AR) in the motoneurons of the rat spinal cord by the immunohistochemical stain and Western blotting. The possibility that AR protein in spinal cord is expressed in tissue-specific form is proposed, being different from other androgen-dependent tissues. AR abnormality in X-linked spinal and bulbar muscular atrophy (SBMA) among a variety of motor neuron diseases were reported recently. Our study may give some clue to the AR abnormality leading to the degeneration of motoneurons.
A method was developed to clone, without the use of specific functional assays, complementary DNAs (cDNAs) that carry specific amino-terminal signal sequences, such as those encoding intercellular signal-transducing molecules and receptors. The vector used in this system directed the cell surface expression of interleukin-2 receptor fusion proteins when inserts with signal sequences were cloned in-frame with the correct orientation. An expression cDNA library was constructed from a bone marrow stromal cell line, which contained 5' portion-enriched cDNAs (the average size was 400 base pairs). Two cDNAs that encoded putative cytokine molecules, stromal cell-derived factor-1 alpha (SDF-1 alpha) and SDF-1 beta, which belong to the intercrine-macrophage inflammatory protein superfamily, were cloned.
A beta-lactosyl residue was linked to the amino groups of L-lysyl-L-lysine through spacer arms of three different lengths (C2, C4, and C9) to give trivalent beta-lactosyl clusters in order to increase the inhibitory activity of the beta-lactosyl group against tumor cell colonization. Thus, O-(2,3,4,6-tetra-O-acetyl-beta-D-galactopyranosyl)-(1-->4)-2,3, 6-tri-O-acetyl-glucopyranosyl trichloroacetimidate was treated with methyl or benzyl hydroxyethanoate, methyl or benzyl 4-hydroxybutanoate, and methyl 9-hydroxynonanoate, respectively, in the presence of trimethylsilyl trifluoromethanesulfonate to give the corresponding beta-lactosides. These were coupled to L-lysyl-L-lysine, after conversion to the N-hydroxysuccinimide esters, to yield the corresponding trivalent beta-lactosyl-L-lysyl-L-lysine conjugates in good yields. The beta-lactosyl group with a C4 spacer arm was also coupled similarly to poly(L-lysine) (M(r) 3800) to form a polyvalent beta-lactosyl cluster. Coinjection of the trivalent (with C2 and C4 spacer arms) and polyvalent beta-lactosyl clusters with the highly metastatic B16 murine melanoma cells inhibited the formation of lung colonies in C57/BL mice, whereas the trivalent cluster with a C9 spacer arm displayed no activity.
In order to determine if there are any residual effects of long-term exposure to mercury vapor, neurobehavioral tests were given to ex-mercury miners about 18 years after the cessation of mercury exposure. Seventy-six male ex-mercury miners who had been exposed to relatively high concentrations of mercury vapor (over 1.0 mg/m3) and with a past history of mercury intoxication were compared to age (+/- 3 years)-, sex-, and years of education-matched controls. Although the extent of the workers' symptoms caused by mercury poisoning, termed erethismus merculialis, markedly decreased after the cessation of exposure, the prevalence of neurological symptoms (such as hand tremors, headaches, and slurred speech) and symptoms of senility (such as low-back pain, loss of sexual desire) in the ex-miners was significantly higher than those in the controls. Matched-pair analysis showed that performances of motor coordination, Simple reaction time, and Short-term memory in the ex-miners were significantly deteriorated compared to those of controls. There are slight but persistent effects on neurobehavioral function, especially on motor coordination function, among mercury miners more than 10 years after the cessation of exposure.
We carried out a survey on the cases of motor neuron disease (MND) occurring in Hokkaido between 1980 and 1989 in order to establish whether there is a relationship between MND and poliomyelitis. Hokkaido, the northern-most island of Japan, was one of the most affected areas during the poliomyelitis epidemic of the late 1940s to 1950s. We ascertained 389 cases of MND for these 10 years and 2,171 cases of paralytic poliomyelitis for the period 1949-1958, and analysed their geographic distributions. No significant correlation was found between MND and poliomyelitis in their geographic distributions.
Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine-starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.
We evaluated the pathogenicity of mercury (Hg) and selenium (Se) which are supposed to be one of the risk factors in the development of amyotrophic lateral sclerosis (ALS). Hg and Se contents were measured in plasma, blood cells, scalp hair samples of 21 sporadic ALS patients and 36 controls, who included 19 patients with other neurological diseases, in Hokkaido, the northernmost island of Japan. Hg and Se levels in plasma and blood cells of ALS patients were significantly lower in advanced staged ALS patients than controls. Low Hg and Se contents in ALS, being correlated with their disabilities and nutritional conditions, would rather reflect the disease contracted states than the pathogenic roles in ALS.
A 68-year-old woman with parkinsonism showed cortical myoclonus and seizures under antiparkinsonian medication. Myoclonus was induced and enhanced by L-dopa, developing into generalized seizures. EEG was abnormal and somatosensory-evoked potentials (SEPs) showed giant SEPs, transcortical reflex (C reflex) and jerk locked potentials. Myoclonus and seizures disappeared after discontinuation of L-dopa and the introduction of valproate sodium (VPA). We described the occurrence of L-dopa-induced myoclonus and seizures in a case of parkinsonism with its SEPs findings.
A case of reflex epilepsy accompanied by pineal cyst and cavum septi pellucidi induced by hot water bathing is presented. The patient is a 25-year-old male who has had six episodes of convulsions during the last three years. The seizures were diagnosed as a complex partial epilepsy followed by generalized tonic seizures. We have succeeded in recording his electroencephalogram (EEG) during convulsions. Moreover, a magnetic resonance imaging (MRI) study revealed a pineal cyst and cavum septi pellucidi. This is quite a rare form of adult hot water epilepsy accompanied by intracranial malformations.
We analyzed the cerebrospinal fluid (CSF) IgG profiles of 46 Japanese patients with relapsing and remitting types of multiple sclerosis (MS). There were 12, 13 and 21 patients, with inactive, active and both clinical conditions, respectively. The values for IgG, IgG/Alb ratio, IgG index and IgG synthesis rate were significantly higher during active than during inactive stages, but the patients with high values for these IgG profiles during active stages tend to also have high values during inactive stages. None of 33 (0%) and 2 of 34 patients (5.9%) exhibited abnormal IgG levels, 3 (9.1%) and 8 (23.5%) an abnormal IgG/Alb ratio, 9 (27.3%) and 18 (52.9%) an abnormal IgG index, and 5 (15.2%) and 14 (41.2%) an abnormal IgG synthesis rate, during inactive and active stages, respectively. Some of HLA antigen frequencies were significantly different between the patients with and without abnormal CNS IgG profiles. Increased CSF IgG was of lesser diagnostic value for MS in Japan, and seemed to be a function of immunogenetics rather than clinical activity.
We evaluated circulating anticardiolipin antibodies (aCL) in 38 Japanese patients who fulfilled the criteria of clinically definite multiple sclerosis (MS), using a newly developed EIA system with aCL-cofactor. Two of 38 patients (5.3%) had a aCL-cofactor-dependent positive serology, and differences compared with findings in controls were statistically significant. The 2 aCL-positive patients had similar clinical features with acute transverse myelopathy (ATM), optic neuropathy (OPN), normal cranial MRI and negative oligoclonal IgG bands (OCBs) in the cerebrospinal fluid (CSF). Among the 38 patients, 3 had ATM, OPN, normal cranial MRI and negative OCBs, hence, in a significant number of the patients (2/3; 67%) with these distinctive features, serology for aCL was positive. Therefore, they may have another condition associated with aCL, masquerading as MS. Serological testing for aCL with aCL-cofactor is recommended for the patients with clinical diagnosis of MS, especially for those showing OPN and ATM during the clinical course, and in Asian peoples where the incidence of ATM and OPN is relatively high among the patients with diagnosis of MS.
A postmortem case of HTLV-I associated myelopathy (HAM)/tropical spastic paraparesis (TSP) with a history of remission and exacerbation of neurological signs and symptoms, resembling those of multiple sclerosis is reported. MRI analysis revealed lesions in the periventricular white matter in addition to atrophy of the thoracic spinal cord, characteristic of HAM/TSP. The cerebral periventricular areas consisted of ill-defined paucity of myelin sheaths with astrocytic gliosis and hyaline thickening of blood vessels. The poorly demarcated white matter lesions found in both brain and spinal cord were different from plaques found in multiple sclerosis. It is suggested that, in some cases of HAM/TSP, inflammatory lesions that destroy myelin can involve not only the spinal cord but also the cerebral periventricular white matter.
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A 32-year-old man with chronic alcoholism over 10 years developed skin eruptions, dark-red tongue and severe watery diarrhea, followed by weakness of bilateral lower extremities. Physical examination revealed hyperpigmented skin eruptions with scales on the dorsa of his hands and extensor aspects of his forearms. Neurological examination showed proximal muscle weakness of both lower extremities, hyperactive knee and ankle jerks, positive Chaddock reflexes and stocking type sensory disturbances. Laboratory data revealed elevation of myolytic enzyme, hypokalemia and decrease of niacin level in the blood. Diagnosis of hypokalemic myopathy and pellagra was made. With the correction of serum potassium level, muscle weakness improved rapidly and with the supplement of niacin, other physical signs and symptoms improved. In this case hypokalemic myopathy could be attributed to the alcoholic malnutritional state such as pellagrous diarrhea, malnutrition, malabsorption and others.