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Biomedical subjects

S Tsuji

Publications and source records attributed to S Tsuji.

At least 631 records · Page 35Linked to original sources

[MRI findings of posterior spinal artery syndrome--report of a case].

A 58-year-old woman presented with sudden onset of numbness and weakness of the lower limbs. She showed paraparesis associated with hyperreflexia and pathological reflexes in lower limbs. She showed decreased sensation of vibration and proprioception in lower limbs, as well as tingling sensation below Th11 level. Pinprick and thermal sensations were spared. Magnetic resonance imaging (MRI) of the spinal cord revealed a symmetric high signal intensity area at the posterior medial part of the spinal cord spanning Th9 to Th11 on T2-weighted and proton density images. On the basis of clinical findings as well as MRI findings, we made a diagnosis of posterior spinal artery syndrome. The MRI findings are considered to be highly useful for the diagnosis of PSAS.

Diagnosis, Differential↗

[A case of post-rubella Guillain-Barré syndrome associated with ulcerative colitis].

A 56-year-old female with post-rubella Guillain-Barré syndrome (GBS) associated with ulcerative colitis (UC) was described. The patient dramatically recovered with plasmapheresis. It is very rare to encounter the neurological complication following rubella, especially polyradiculoneuropathy as observed in the present case. Significantly high titer of anti rubella antibody at admission and subsequent decrease in the titer suggest that rubella infection is likely to involve the development of GBS. Although GBS and UC are currently regarded as autoimmune disorders, association of GBS and UC is extremely rare and only a few cases have been reported. All of them including the present case developed GBS while UC was in remission. Similarly to previous reports of GBS or UC, our case also had an HLA type of B35 and DR2. Thus the present data suggest that infectious agents as well as individual susceptibilities are important for the development of GBS.

Colitis, Ulcerative↗

[Molecular basis of heterogeneities of clinical presentation of dentatorubral pallidoluysian atrophy (DRPLA)].

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder affecting primarily dentatorubral as well as pallidoluysian atrophy. From a view point of clinical genetics, DRPLA is characterized by prominent anticipation and considerable heterogeneities in its clinical manifestations. Given the fact that unstable CAG repeat expansion is the causative genes for Huntington disease and spinocerebellar ataxia 1, in which anticipation and parental bias of transmission of the severest form are present, we hypothesized that DRPLA might be caused by unstable expansion of a gene containing a CAG repeat. By screening published genes containing CAG repeats, were able to identify the gene for DRPLA located on the chromosome 12. All the DRPLA patients had expanded CAG repeats, and furthermore, there is a good correlation between the degree of CAG repeat expansions and the ages of onset. There are also good correlation in the ages of onset of each of the neurological of manifestations including myoclonus, choreoathetosis, epilepsy, dementia, and psychiatric symptoms. We concluded that the unstable expansion of the CAG repeat in the DRPLA gene is intimately involved in the pathogenesis of DRPLA.

Age of Onset↗

[Transcranial magnetic stimulation].

The technique of transcranial magnetic stimulation of the motor cortex is a new neurophysiological examination of the central motor pathways. In clinical application a reduced MEP/M wave amplitude ratio had a higher correlation with hyperreflexia than did a delayed MEP onset latency. The inhibitory period (IP) which is a transient suppression of muscle action potentials following motor evoked potentials (MEP) by magnetic cortical stimulation is caused primarily by inhibitory process in the motor cortex, because the duration of IP did not depend on the amplitude of MEPs, MEP distribution over the motor cortex or severity of voluntary contraction. Furthermore, the IP was shorter and the amplitude of MEP was increased when ipsilateral motor cortex stimulation to the thenar muscle preceded the contralateral cortex stimulation. C response in a patient with familial essential myoclonus was significantly suppressed by the magnetic stimulation of the sensory cortex locally and during the IP. The uncrossed pyramidal tract was proved by motor cortex stimulation in a patient with hemorrhage in the right thalamus and internal capsule who showed a right hemiparesis. The enhancement of cortical SEP amplitudes (P23 and N30) were noted when the median nerve was stimulated at 50 msec following magnetic stimulation over the sensory cortex locally. SEPs by twin coil stimulation at thoracic and lumbar root levels are useful in detecting the lesions of spinal cord or roots. Sympathetic skin responses and sudomotor potentials are recorded easily by the neck magnetic stimulation, and are useful method for evaluating sympathetic nerve function.

Cerebral Cortex↗

[A case of unilateral brain-stem tumor and impaired ventilatory response].

A 44-year-old man with a unilateral brain-stem tumor (ganglioglioma) presented with marked hypoventilation and irregular breathing. His respiratory muscle strength was impaired slightly, and his ventilatory responses to chemical stimuli were markedly diminished. Magnetic resonance imaging of the brainstem revealed that the tumor was located in the left superior cerebellar peduncle and the medulla, and involved the left lateral portion of the medullary reticular formation, ambiguous nucleus, and solitary nuclear complex. From these findings, we conclude that the abnormality of ventilatory control may have been caused by damage to both the unilateral respiratory neuron group in the medulla and the afferent pathway to the respiratory motor unit on the opposite side.

Adult↗

[Elevation of cerebral lactate detected by localized 1H magnetic resonance spectroscopy in a patient with migraine].

Localized 1H magnetic resonance spectroscopy was performed in a 45-year-old woman with migraine. She developed throbbing headache attacks without aura since thirteen years ago and the attack was accompanied with right hemiplegia since seven years ago. Brain MRI showed no abnormalities and 123I-IMP SPECT revealed mild frontal dominant decrease of cerebral blood flow. It seemed that her condition was positioned between migraine with prolonged aura and migrainous infarction of complicated migraine in the classification of International Headache Society. Spectra obtained from bilateral frontal lobe interictally showed elevation of lactate at left side. Choline, creatine, and N-acetyl-aspartate were almost equal on both side. The above results suggest that slight ischemia which is not detected by MRI is present or there is a disturbance of oxidative glycolysis, which is induced by mitochondrial dysfunction.

Brain↗

[A patient with aneurysm of extracranial internal carotid artery presenting lower cranial polyneuropathy similar to Tapia's syndrome].

Tapia's syndrome, first described in 1904 by A.G. Tapia, is considered to be a syndrome consisting of ipsilateral hemiplegia of larynx and tongue with spared movement of soft palate. A 61-year-old Japanese woman had been in good health until August 1991, when she developed hoarseness and atrophy of the left side of her tongue. Although she also showed mild disturbance of elevation of bilateral soft palates and loss of taste in the posterior third of her tongue as well, the main symptoms were the paralyses of the ipsilateral larynx and tongue without involvement of the ipsilateral sternocleidomastoid and trapezius muscles. We concluded that she had cranial polyneuropathy similar to that of Tapia's syndrome. A carotid angiography revealed that she has a large aneurysm, which originated from the extracranial internal carotid artery in the region near the skull base. No other abnormal findings were detected by any computed tomography, magnetic resonance imaging or 67Ga-scintigraphy. The patient's lower cranial polyneuropathy was considered to be caused by the aneurysm. A survey of the literature indicates that extracranial carotid aneurysm is an extremely rare cause of Tapia's syndrome. In this case, the location of the aneurysm, which was present considerably distant from the skull base, seems to be the reason for the sparing of the accessory nerve.

Aneurysm↗

[Transcortical sensory aphasia following a left frontal lesion--case report].

We report a patient with transcortical sensory aphasia (TCSA) following a left frontal lesion. The patient was a 63-year-old right handed Buddhist priest admitted to our hospital with a complaint of word-finding difficulty. Neurological examination failed to reveal any abnormal findings except aphasia. The patients spontaneous speech was fluent, not agrammatic, and free of phonemic paraphasia but he sometimes exhibited semantic paraphasia. His ability to repeat sentences was excellent, but he had severe difficulty in word-finding, auditory comprehension and writing. He read aloud correctly, but his reading comprehension of kana (phonograms) was poor in contrast to excellent reading comprehension of kanji (morphonograms). His clinical picture was characterized by poor comprehension but excellent repetition, and a diagnosis of TCSA was made. CT and MRI revealed an infarction involving the pars opercularis and pars triangularis in the inferior frontal gyrus and the anterior portion of the lower prefrontal gyrus and the middle frontal gyrus. Regional cerebral blood flow was measured by single photon emission CT and was found to be decreased in the same region as indicated on CT and MRI. The clinical picture in this patient was comparable to that of TCSA due to a posterior parieto-occipital lesion, but the rapid improvement (within 1 to 2 months) of this patient was characteristic. Auditory comprehension, kana (phonograms) reading comprehension, and word recall, which were severely impaired in this patient, all demand interaction between phonological processes and semantic processes. Thus we conclude that this patient had impairment of the phonological-semantic interaction process.

Aphasia, Wernicke↗

[The detection of anti-Purkinje cell antibody (anti-Yo antibody) by ELISA using recombinant Yo fusion protein].

Paraneoplastic cerebellar degeneration (PCD) is a remote effect of cancer mediated by possibly immunological mechanisms. The sera and cerebrospinal fluid of PCD patients containing high titer autoantibody against cerebellar Purkinje cells had been reported. This antibody binds to 62-kD and 34-kD bands of cerebellar Purkinje cell homogenates (anti-Yo antibody). However, it is not always true that the autoantibody of this character on immunoblot and immunohistochemistry recognizes the same molecule. Recently, the DNA sequence encoding the Yo antigen, whose common epitope is a sequence containing leucine-zipper motif, was reported. We made the recombinant protein deduced from the cDNA clone encoding the leucine-zipper motif of the Yo antigen. Using this recombinant protein as the antigen for ELISA, the anti Yo antibodies in the sera and CSF were examined and 3 new patients with PCD possessing anti-Yo antibody were found. The sera of one patient, serially taken during several kinds of treatment were examined with this ELISA system, which revealed that the anti-Yo antibody titer was increased after plasmapheresis and reduced after tumor resection and anti-cancer chemotherapy. The early resection of malignant tumors may prevent the continuous production of high titer anti-Yo antibody and stop the progression of cerebellar tissue damage.

Adult↗

[Correlation between degrees of the CTG repeat expansion and clinical features of myotonic dystrophy].

The mutation in myotonic dystrophy gene has recently been identified as an unstable expansion of trinucleotide CTG repeat located at the 3'-untranslated region of myotonin protein kinase gene. In this paper we report the correlation between the degree of CTG amplification and clinical features in 35 individuals with myotonic dystrophy. The analysis of CTG repeat expansion was performed with Southern blot hybridization. Genomic DNA from peripheral blood leukocytes was digested with a restriction endonuclease, Pst I, instead of commonly used EcoRI. Since small expansion (about 100 bp) could be detected with PstI digestion and furthermore, the DNA fragment did not contain insertion/deletion polymorphism, we were able to accurately determine the exact sizes of CTG repeat expansion. We have observed a tendency of earlier ages of onset with larger allele sizes. The good correlation between the size of the expansion and the severity in muscle weakness was clearly demonstrated especially if the analysis was focused on the patients at same age group at 40-45 years. The severity of motor disability was classified into three stages. The mean size of expansion was 0.33 +/- 0.17 (M +/- SD) kbp in stage I, 2.58 +/- 1.42 kbp in stage II, and 4.75 +/- 0.93 kbp in stage III. The tendency was also observed when patients were categorized according to the intellectual grade. The anticipation was observed in all the parent-child pairs. When the increases of the repeat expansions were compared between father-child and mother-child transmissions, broader variation of the increases was observed in father-child transmissions.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[A case of acute cholinergic dysautonomia].

A 52-year-old man with hypohidrosis and selective parasympathetic peripheral autonomic nerve disturbances of acute onset is described. In April 1991, he noted a feeling of dryness in the eyes and the oral cavity, disturbance of taste, difficulty in micturition and a feeling of dryness at the distal extremities with acute onset, which were followed by alternating diarrhea and constipation. Clinical and laboratory autonomic examinations revealed decreased sweating in the distal extremities, decreased lacrimation and salivation, a decrease in the average urinary flow with residual urine and hypersensitive pupillary constriction to the instillation of methacholine. On the other hand, the results of upright tilt testing, the Valsalva test and cold pressor test were all normal, and the plasma noradrenaline level was also normal. Therefore, the diagnosis of acute cholinergic dysautonomia was made. He resumed his clerical work without medication 3 months after the onset. One year and 4 months after the onset, clinical and laboratory examinations showed that hypohidrosis in the distal lower extremities remained unchanged. We found ten patients of acute cholinergic dysautonomia in the literature, and concluded that our patient should be classified as a typical case of this disease. Clinical reports of acute cholinergic dysautonomia are relatively rare, and our patient is the oldest among the reported patients.

Acute Disease↗

[Official inspection estimated production of coal-tar dyes (including dye aluminum lakes) in 1993-1994].

The number of official inspections of coal-tar dyes and their aluminum lakes from April 1993 till March 1994 were 734 in total, with the quantity which passed inspection amounting to 213 tons in Japan. Data for production by color for each month are summarised in Table 1, and by each producing company in Table 2. The food coal-tar dye produced in the largest quantity was Food Yellow No. 4, occupying a 42.9% proportion of the total during this period.

Chemical Industry↗

A novel transcript from a pseudogene for human glucocerebrosidase in non-Gaucher disease cells.

Human glucocerebrosidase (GC)-encoding cDNA clones were isolated from a promyelocytic HL-60 cDNA library and analyzed. A novel cDNA clone was found to originate from a gene referred to as a GC pseudogene. Using the polymerase chain reaction (PCR) with primers specific for the GC pseudogene, we found that all the human cell lines examined, HL-60, K-562, WI-38, HepG2 and HeLa, expressed a pseudogene transcript. In vitro translation of RNA synthesized by transcription of the pseudogene cDNA produced a polypeptide of approximately 30 kDa.

Amino Acid Sequence↗