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Biomedical subjects

H Tanabe

Publications and source records attributed to H Tanabe.

At least 271 records · Page 15Linked to original sources

Changes of lysosomal proteinase activities and their expression in rat cultured keratinocytes during differentiation.

The cathepsins B, H and L, lysosomal cysteine proteinases, play a major role in intracellular protein degradation. These proteinase activities and expressions were examined in a Ca2+ regulated epidermal culture system which consists of two morphological cell types: undifferentiated cells grown in low Ca2+ (0.1 mM concentration) and differentiated cells grown in high Ca2+ (1.8 mM concentration), respectively. Cathepsin B and L activities of the differentiated cells showed a several-fold increase compared to that of the undifferentiated cells. In addition, by using CM-cellulose column chromatography, cathepsin B and L were separated and the level of cathepsin L activity increased significantly. Cathepsin B, L and H were also detected by using an immunoblotting procedure in which their bands were expressed after differentiation was induced by the increasing calcium concentration. Cathepsin L activity and immunostaining intensity reached a maximum at 1 or 2 days of differentiation. In contrast, cystatin alpha (an endogenous inhibitor of cysteine-dependent cathepsins) appeared in the final stage of differentiation. These results indicate that the expression of epidermal cathepsins and their endogenous inhibitor are involved in part of the program of cell differentiation and the terminal differentiation process in cultured rat keratinocytes.

Animals↗

Somatosensory evoked potentials to median nerve stimulation after partial section of the corpus callosum.

Cortical somatosensory evoked potentials (SEPs) to electrical stimulation of the median nerve were studied in four patients with intractable epilepsy who had undergone callosotomy and in a patient with infarction in the corpus callosum in order to determine whether the corpus callosum was involved in the generation of ipsilateral frontal components. Both pre- and postoperative SEPs were recorded in three of four epileptic patients. There were no significant differences in the latencies and amplitudes of the bilateral frontal components (P20, N26) between pre- and postoperative recordings. Furthermore, irrespective of the extent of the section or lesion in the corpus callosum, the nature of the impairment and the existence of the disconnection syndrome, the SEP findings showed no significant differences compared with those of normal subjects. It thus appears unlikely that the ipsilateral SEP responses are transmitted from the contralateral hemisphere through at least the anterior portion of the corpus callosum.

Adult↗

Sleep-related periodic leg movements (nocturnal myoclonus) due to spinal cord lesion.

Ten patients with involuntary leg movements due to myelopathy were studied clinically and polysomnographically. The clinical manifestation and polysomnographical findings of involuntary leg movements were identical to sleep-related periodic leg movement (PLM) (nocturnal myoclonus). Since 2 patients had complete transection of spinal cord due to injury or vascular accident, the spinal cord deprived of supraspinal influences was considered to generate the rhythm of PLM. Suppression of the leg movements during REM sleep was not obvious in the patients with complete transection of spinal cord. In addition, PLM alternated from one side to the other 1-4 times a night with intervals of 1-4 h in all patients. This alternation also seemed to be from the spinal cord. This PLM of spinal cord origin was different from spinal myoclonus in their clinical features although both were generated within the spinal cord. PLM of spinal cord origin showed a triple flexion of the ankle, knee and hip, and this was very similar to a flexor withdrawal reflex which all patients exhibited. Therefore, it was suggested that PLM of spinal cord origin has a common mechanisms with spinal automatism. Although all patients had extensor plantar responses, PLM preceded the paresis in three patients and the severity of paresis was variable. There was no laterality of left and right PLMs even in patients with weakness of the leg on one side. This suggested that PLM of spinal cord origin might be induced by the interruption of the tract which was separate from, but runs near the corticospinal tract.

Adult↗

Geotropic ocular deviation with skew and absence of saccade in Creutzfelt-Jakob disease.

Three patients with Creutzfelt-Jakob disease (CJD) showed characteristic ocular manifestations. The head was turned left or right with the eyes deviated downward and skewed. When the head was turned to one side, the eyes very slowly deviated to that side. In addition, spontaneous ocular movements were very slow with no saccadic component early in the apathetic stage. Caloric stimulation produced tonic deviation to the appropriate side without nystagmus. At autopsy one patient showed lesions compatible with the panencephalopathic type of CJD. Although bilateral pretectal areas had marked gliosis, other nuclei and structures associated with oculomotor system in the brainstem, including the oculomotor, trochlear, abducens, vestibular and perihypoglossal nuclei, medial longitudinal fasciculus and para-median pontine reticular formation were preserved. These patients had a supranuclear disorder, probably caused by combined disruption of the direct and indirect frontal eye field to the brainstem pathways plus impairment of the superior colliculus-mediated saccade pathways.

Creutzfeldt-Jakob Syndrome↗

Pseudopseudohypoparathyroidism with recurrent polyneuropathy: an autopsy report with special reference to the peripheral nervous system.

The clinical and pathological findings of a 21-year-old girl suffering from pseudopseudohypoparathyroidism (PPHP) with relapsing neuropathy are described. Episodic exacerbations were accompanied by intracranial hypertension and were relieved by the administration of corticosteroids. At autopsy, pathologic changes were almost restricted to the peripheral axons and showed distal dominant depletion of myelinated fibers without any active myelin breakdown or inflammatory changes. The neuropathy is thought to be similar to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, the relationship, if any, between PPHP and CIDP is unknown.

Adult↗

Morphological changes in unmyelinated nerve fibres in the sural nerve with age.

Quantitative changes in unmyelinated nerve fibres in sural nerves obtained at autopsy were evaluated in 28 normal adults. The following conclusions were reached. (1) The density of unmyelinated axons showed no significant correlation with age, but the densities of (2) Schwann cell subunits with axons, (3) Schwann cell subunits without axons, (4) single protrusions of Schwann cells and (5) collagen pockets, and (6) the mean number of Schwann cell profiles per axon, all showed positive correlations with age. Additionally, (7) the percentage of subunits containing unmyelinated axons and (8) the mean number of axons in single axon-containing Schwann cell subunits demonstrated negative correlations with age. The density of Schwann cell nuclei related to unmyelinated fibres did not show a significant change with age. The age-dependent changes in unmyelinated nerve fibres thus mainly consist of an increased production of processes by Schwann cells in the absence of cell multiplication. A decrease in unmyelinated nerve fibre density or a compensatory increase of small unmyelinated axons did not occur in these normal adults. In terms of relative sensitivity for the detection of the earliest changes in unmyelinated fibres, the indices (6) and (7) are considered to be useful and superior to the conventional assessment of unmyelinated axon density and diameter distribution. These two indices are not influenced by postmortem swelling of the axons and Schwann cells. Measurements of unmyelinated axon density and size distribution will continue to be useful in the assessment of more advanced pathological conditions.

Adult↗

Sympathetic skin response in patients with multiple sclerosis compared with patients with spinal cord transection and normal controls.

In 28 patients with definite multiple sclerosis (MS) and 21 patients with a functionally complete transection of the spinal cord sympathetic skin responses (SSR) from both the palms and soles were studied. In the patients with a complete transection at T3 or 4, SSRs were impaired in the palms as well as the soles, and most patients with a transection at T11 or T12 had normal SSRs in the soles. These findings indicate that the central pathway mediating the SSR descends to the upper thoracic cord where connections are made with the sympathetic distribution to the palms, and then to the lower thoracic cord to reach the sympathetic outflow to the soles. This is compatible with the anatomical knowledge of the sympathetic system. Of the MS patients, 75% had abnormal SSR results. The SSR for the soles was more sensitive than that for the palms. The incidence of SSR abnormality was as high as those of somatosensory evoked potential (SEP) (79%) and visual evoked potential (VEP) (75%) abnormalities. Of the patients with normal SEPs 14% had impaired SSRs and 21% had abnormal SSR with normal VEPs. When the results of these 3 tests were combined, the abnormality increased to 100%. From our study, the SSR probably reflects not only postganglionic sympathetic activity but also preganglionic function. The SSR is therefore useful in evaluating myelopathy, providing information different from that given by assessment of the somatic nervous system. Since the SSR detected a few MS lesions which were not demonstrated by other evoked potentials, it is potentially a new tool for the detection of MS lesions in addition to conventional evoked potential studies.

Adult↗

Evolutionary rate of immunoglobulin alpha noncoding region is greater in hominoids than in Old World monkeys.

Recent studies on the molecular evolution of primates show that the evolutionary rate among hominoids is considerably slower than that among nonhominoid primates. However, this observation at the nucleotide-sequence level is restricted to the beta-globin family region. In this study, we sequenced orthologous immunoglobulin alpha (C alpha) genes of chimpanzee, gorilla, orangutan, and crab-eating macaque (an Old World monkey) and compared them with that of the human by using noncoding regions for analysis. Since significant differences in rates among hominoids were not found by using the relative rate test, we evaluated the ratio (R) of the evolutionary distance between Old World monkey and human to the distance between orangutan and human. The R value (1.12) for the C alpha gene was much smaller than the expected value (1.38-2.33), showing that the nucleotide substitution rate (= mutation rate per year under selective neutrality) of the C alpha gene is greater in the human lineage than in the Old World monkey lineage. We also did a similar analysis for the gamma 1-, gamma 2-, psi eta-, and delta-globin genes and found a considerable heterogeneity (1.12-2.37) among the R values, including that for the C alpha gene. This indicates that the hominoid slowdown of the evolutionary rate is not a universal phenomenon in primate evolution.

Animals↗

Memory loss due to transient hypoperfusion in the medial temporal lobes including hippocampus.

A typical case of transient global amnesia (TGA) was investigated with single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRI) with 1.5-tesler scans. During the amnesic episode, a marked decrease of cerebral blood flow was observed in the areas confined to the territory of the bilateral posterior cerebral arteries including the hippocampus. After the episode, cerebral blood flow returned to normal and a circumscribed lesion was detected in the middle portion of CA 1 field of the left hippocampus. The SPECT findings prove direct evidence that the medial temporal structures are involved in the establishment of new memories, as well as in process of recalling only recently acquired memories, but not in retrieval of memories acquired long ago. The MRI findings indicate that a unilateral partial damage to CA 1 sector of the hippocampus does not develop a definite memory impairment and that high-resolution MRI study on the hippocampus is necessary in TGA patients.

Amnesia↗

Somatosensory evoked potentials in patients with selective impairment of position sense versus vibration sense.

Ten patients with selective impairment of either position sense or vibration sense were studied with somatosensory evoked potential (SEP). Five patients with spinal cord lesion (three with MS, one with spinal cord tumor and one with spinal cord injury) lost the vibration sense below the iliac crests without impairment of the position sense. However, five patients with cerebral vascular lesions involving thalamus unilaterally showed severe impairment of position sense, though there was no asymmetry as to the vibration sense. In all these cases with spinal and cerebral lesions, SEPs showed abnormalities in the distributions where the position sense was impaired and were not related to the impairment of vibration sense. Our study indicates that SEP is much better correlated with the position sense than with the vibration sense at any lesion level.

Adult↗

Characterization of intracytoplasmic neurofilament accumulation in hamster brain caused by Alzheimer buffy coat inoculation: comparison with experimental neurofibrillary changes produced by aluminum intoxication.

Neurofilament accumulation was produced in neuronal perikarya of the lower brain stem nuclei in the hamster brain by inoculating buffy coat from a patient with Alzheimer's disease. The abnormally proliferated neurofilament was characterized by immunostaining and electron microscopy. The intracytoplasmic neurofilament accumulation was positively stained with anti-tau and anti-ubiquitin, but not with antibody against paired helical filaments. Experimental neurofibrillary changes were produced in the rabbit brain by aluminum injection, and the proliferated neurofilaments in the rabbit brain were compared with those in the hamster brain inoculated with Alzheimer buffy coat. The results indicate that the long pathological process in the hamster brain is triggered by Alzheimer's buffy coat inoculation which results in intracytoplasmic accumulation of modified neurofilaments.

Aluminum↗

[Monoparesis due to the brachial plexus neuritis by herpes zoster virus--report of a case].

A 73-year-old woman suffering from the acute onset monoparesis of her right arm which followed the skin eruption with mild sensory disturbance of right C4-6 level, was reported. Electrophysiological examinations revealed the brachial plexus neuritis and axonal degeneration of the proximal portion, with the evidence of herpes zoster infection. Her paresis of the right arm gradually improved without any medication during her hospital course. It was concluded that herpes zoster should be considered to be one of the causes of acute onset brachial plexopathy.

Aged↗

[Clinical studies of 23 patients with multiple system atrophy presenting with vocal cord paralysis].

In order to elucidate the clinical features and the prognosis for life in the patients with multiple system atrophy (MSA) presenting with vocal cord paralysis (VCP), we studied the correlation between VCP and other neurological findings including cerebellar, pyramidal, extrapyramidal and autonomic nervous signs. Subjects were 48 MSA patients: 23 with VCP and age- and illness duration-matched 25 without VCP. MSA in this paper comprised clinically Shy-Drager syndrome, olivopontocerebellar atrophy, and striatonigral degeneration. MSA patients with VCP had in general more severe neurological findings, compared with those without VCP. Urinary incontinence developed in the relatively early stage of illness and preceded VCP in all patients. VCP developed not only in far-advanced stage but at any time in the course of illness. As to swallowing function when a diagnosis of VCP was established, about half of the patients with VCP needed nasogastric tube feeding and the remaining half tolerated oral feeding. VCP correlated strongly with urinary incontinence but not always with the severity of orthostatic hypotension or extrapyramidal tract sign such as parkinsonism. Five of the eight patients without tracheostomy came to sudden death. The mean duration from making a diagnosis of VCP to death was 1.1 years. In contrast, nine of the 11 patients with tracheostomy were alive and the survival periods after tracheostomy reached a maximum, five years. These facts suggest that the prognosis for life in the patients with VCP depends in part upon whether tracheostomy was carried out or not.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Energy metabolism of ALS patients under mechanical ventilation and tube feeding].

We studied the energy metabolism of ALS patients under mechanical ventilation and tube feeding. Gas exchanges (O2 and CO2 content in expiratory and inspiratory gas) were measured all day long by DELTATRAC (Datex, Finland) in 11 ALS patients, and energy metabolism during 24 hours was calculated according to the next formula; 5.67 VO2 + 1.60 VCO2-2.17 UN (VO2; O2 consumption l/min, VCO2; CO2 production l/min, UN; urea nitrogen excretion in urine g/day). All patients were clinically stable under continuous mechanical ventilation and tube feeding, and did not have any infection such as pneumonia. The patients were 23-70 years old (mean 49.3), and had total clinical courses of 3-12 years (mean 7.1), and 2-8 year-long courses under mechanical ventilation (mean 4.6). They were classified into the next 3 groups: group I; totally locked-in state (2 patients), group II; complete tetraplegia (6 patients), group III; incomplete tetraparesis (3 patients). Basal metabolic rate (BMR) of each patient was also calculated from Harris-Benedict's formula; male = 66.47 + 13.75W + 5.0H - 6.76A, female = 665.10 + 0.567W + 1.85H - 4.68A (W; weight kg, H; height cm, A; body surface area m2). And the changes of the body weight by month were examined retrospectively in 26 ALS patients with at least 2 year-duration under mechanical ventilation, which include the previous 11 patients. The calorie consumption of 24 hours were 783.3 kcal (group I), 875.3 (group II), 974.9 (group III), which were all lower than BMR (I; -26.8%, II; -17.6%, III; -11.3%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Autosomal recessive oculopharyngeal "muscular dystrophy"--clinical features and association with reduced activity of myophosphorylase].

We reported two cases of brothers demonstrating oculopharyngeal muscular dystrophy (OPMD). The cases had consanguineous parents and five healthy siblings, which suggested the autosomal recessive inheritance. The initial symptom was slowly progressive blepharoptosis with onset in the third decade. On examination, total external ophthalmoplegia was observed in both patients. Additionally, the elder, a 57-year-old man, exhibited dysarthria, dysphagia and muscular weakness with atrophy of the face, bilateral proximal upper limbs and diffuse lower limbs. The younger brother, a 55-year-old man, displayed muscular weakness and atrophy distributed in the face and four limbs. Muscle biopsy of both cases revealed rimmed vacuoles and spheroid bodies in the atrophic and normal-sized fibers. Biochemical study of the biopsy specimens of the elder brother disclosed the myophosphorylase activity reduced to about 40% of the normal value, although in the younger brother, that activity was normal. OPMD is usually inherited in the autosomal dominant mode, and autosomal recessive OPMD is rare. The onset age of our cases was younger than that of the autosomal dominant OPMD. There were some differences in the clinical manifestation between the presented cases, which could be interpreted as phenotypic variation. The elder brother was thought to be associated with McArdle's disease.

Blepharoptosis↗

[Congenital facioscapulohumeral muscular dystrophy associated with tongue atrophy and sensorineural hearing disturbance].

An 18-year-old high-school boy presented facial muscle weakness since birth, and then developed wasting around the neck, shoulder girdle, upper arms, and thighs. He was born to un-consanguineous parents. His father had suffered from similar but milder muscle atrophy with predominance on the right side of the face and shoulder girdle since adolescence. His mother and his only sibling were clinically unaffected. Hearing disturbance was detected at the age of 6, and he also noted atrophy of the tongue and the bilateral thighs at the age of 10. The symptom progressed gradually. Neurological examination on admission revealed a well-developed boy (166 cm/60 kg) with a prominent facial diplegia with distinct proximal muscular atrophy of the extremities. Muscles of the tongue, neck, upper arms, shoulder and pelvic girdles, and hamstrings were markedly involved. The anterior tibial muscles were also affected, while the calf muscles were hypertrophic. High arched palate, X legs, mild lordoscoliosis were also noted. Serum CK was slightly increased (424 IU/l), and needle EMG in the extremities including the tongue revealed myopathic changes. Muscle CT demonstrated marked atrophy of the proximal muscles in the lower limbs and hypertrophy of the calf muscles. Audiogram showed bilateral sensorineural hearing disturbance. Muscle biopsy of the gastrocnemius showed myogenic as well as neurogenic changes consisting of atrophic and hypertrophic fibers with interstitial cellular infiltration, and type I fiber predominance. With these family history as well as clinical and laboratory examinations, this case could be diagnosed as "congenital facioscapulohumeral muscular dystrophy".(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Aging process of the acetabular labrum--an electron-microscopic study].

We have studied the aging process of the labrum of the hip using scanning and transmission electron-microscopes. The materials consisted of 22 human hip joint acetabula. Disruption and irregular ridges were evident on the uppermost superficial layer and the superficial layer of the labrum in their fifties of age. Collagen fibers composing the labrum increased in density and sectional area in the specimens of subjects up to their twenties, but decreased in those of subjects in their thirties. At the joint of the labrum and articular cartilage, a bundle of fibers approximately 20 microns in diameter was observed to extend from the labrum to the cartilage, reinforcing the bond of these components. Disruption of this area starting on the acetabular side and leaving fibers of the labrum of about 400 to 500 microns on the cartilage side was more frequent in the specimens of subjects in their fifties of age. The changes with aging in the collagen fibers along with concentration of the stress of weight bearing at the point where the labrum meets the cartilage, may have some effect on the well-fitting of the acetabulum and femoral head.

Acetabulum↗