Biomedical subjects

N Amano

Publications and source records attributed to N Amano.

At least 55 records · Page 3Linked to original sources

PubMed1995

[Familial early onset cerebellar ataxia with hypoalbuminemia].

We describe two brothers with early onset cerebellar ataxia associated with hypoalbuminemia (EOCAH). Choreo-athetoid movements preceded the cerebellar ataxia, and serum pseudocholinesterase elevation preceded the hypoalbuminemia. The parents are first cousins. Patient 1, the 22-year-old elder brother, developed choreoathetoid movements of the neck and extremities at the age of eighteen months. He later developed slowly progressive cerebellar ataxia with decreased tendon reflexes. The choreoathetoid movements ceased at about 16 years of age. A recent examination revealed cerebellar ataxia, action myoclonus of the neck and upper limbs, diminished tendon reflexes, mild sensory disturbance in the lower extremities, and very slight amyotrophy of the feet. Patient 2, the 18-year-old younger brother, developed choreo-athetoid movements at the age of 6 years, followed by slowly progressive cerebellar ataxia with disminished tendon reflexes. No scoliosis, ECG abnormalities, or edema was detected. Serum biochemistry studies revealed elevated pseudocholinesterase (6,226 to 2,390 IU) in the patient's early teens. Serum albumin levels tended to be low (3.7 to 4.1 g/dl). Serum triglyceride and beta-lipoprotein levels were elevated in the patients' late teens. Genetic studies showed slight linkage of D9S15. The maximum lod score was 0.289 (recombination fraction rate was 0.14).

Adolescent↗

PubMed1995

[Siblings of early onset cerebellar ataxia with hypoalbuminemia].

Recently, a new syndrome of early onset cerebellar ataxia with hypoalbuminemia (EOCA-HA) was reported in Japan. The clinical features of EOCA-HA overlap with those of Friedreich's ataxia (FA), and primary hypoalbuminemia is a characteristic laboratory finding of this syndrome. Genetic linkage analysis of EOCA-HA including this newly reported family revealed that the gene for EOCA-HA is located on the long arm of chromosome 9 as FA. However, several recombination events were observed between D9S15 in EOCA-HA, whereas no recombination events were seen in FA. We report on two siblings with EOCA-HA and discuss the clinical and laboratory features. The patients were a 25-year-old man (patient 1) and a 23-year-old man (patient 2). Their parents marriage was non-consanguineous. The mode of inheritance is compatible with autosomal recessive mode. Clinically, they showed cerebellar ataxia as the initial symptom in the late infantile period and subsequently showed choreoathetosis and ocular motor apraxia at the age of approximately fifteen years. Deep tendon reflexes were reduced in late infancy and finally disappeared. Amyotrophy and sensory impairment of the legs developed at approximately twenty. Abnormal electrocardiogram and diabetes mellitus were not observed. On X-ray CT scan or MRI, the cerebella of both patients were mildly atrophic. Clinical features in these siblings were indistinguishable from those of ataxia telangiectasia, but immunodeficiency syndrome was absent.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1995

[Additional doses of oral morphine on demand].

It is very important to relief the pain of terminally ill patients. Populalization of WHO "Cancer Pain Relief" saved many cancer pain suffered patients. The patients in hospital may get enough pain relief as soon as they suffer the pain by their medical stuffs. But It spend much more time to relieve the pain, I write prescription of regular use morphine and additional morphine. Additional dose is 1/4 of regular dose. When they suffer any pain, they may take additional morphine on their demand. Six terminally ill patients were given additional morphine. Four of 6 used the additional morphine for once to 4 times. They were satisfied the effect of additional, they did not use. Their pain did not increase. I suppose, they knew the means of pain relief then they were free from the anxiety of increasing pain. Decreasing the anxiety reduce their pain. Additional doses of morphine on demand are very useful to relieve the pain of the patients at home.

Administration, Oral↗

PubMed1994

A novel procedure for the preparation of biologically active recombinant peptides using a cyanylation reaction.

A cysteine specific cleavage reaction was used for the preparation of biologically active peptides from recombinant fusion proteins. The fusion protein through cysteine was prepared by a recombinant DNA technology and then treated with cyanylating reagents such as 2-nitro-5-thiocyanatobenzoic acid (NTCB) and 1-cyano-4-(dimethylamino) pyridinium tetrafluoroborate (DMAP-CN) to release the desired product. As an example, we have selected a glucagon-like peptide 1 (7-37) (termed insulinotropin). We constructed an expression vector for a fusion protein in which insulinotropin and human basic fibroblast growth factor (hbFGF) mutein (abbreviated as CS 23) are connected by cysteine and then expressed it in Escherichia coli cells. The fusion protein, after refolding, was purified by heparin affinity chromatography, since CS23 has a strong affinity for heparin. The affinity-purified fusion protein was treated with NTCB or DMAP-CN to give crude insulinotropin, which was then purified by reversed phase (rp) high-performance liquid chromatography (HPLC). From various criteria such as amino acid analysis, amino acid sequence and the biological activity, the purified material obtained was found to be methionylated insulinotropin (Met-insulinotropin) with full activity. The specificity and simplicity of the present method make it versatile and convenient for the preparation of biologically active peptides.

Amino Acid Sequence↗

PubMed1994

Renal cell carcinoma producing alpha-fetoprotein (AFP) with a unique lectins-affinity profile.

Renal cell carcinoma (RCC) producing alpha-fetoprotein (AFP) is a rare entity and merely 7 cases have been reported so far. The present case, a 71-year-old woman, showed a high serum AFP level of 204 ng/ml. The RCC of the autopsied right kidney consisted mainly of spindle-shaped or bizarre sarcomatous tumor cells. AFP was immunolocalized only in the concomitant clear cell component. Concanavalin A (Con A)-nonadsorption rate of serum AFP was 42%, which was an intermediate value between those of yolk sac tumors and metastatic liver carcinomas. Lens culinalis agglutinin (LCA)-affinity study of the patient's AFP showed an unknown peak X, which was eluted between the known peaks 2 and 3. These results suggest a certain structural alteration in carbohydrate moieties of the AFP derived from this RCC. A review of the clinicopathologic features of 8 patients with AFP-producing RCC was made to understand the pathophysiology of AFP-producing neoplasms.

Aged↗

PubMed1994

The margin of safety in the use of a straight path centrifugal blood pump.

A new centrifugal blood pump with a rotor that arranges 6 straight paths radially was developed for open heart surgery and temporary circulatory support. We describe comparative studies of the margin of safety in the practical use of the new pump. This pump was evaluated for temperature increase, cavitation, and pressure sensitivity. Two commercially available centrifugal pumps, the Biomedicus cone type and the Sarns 3M impeller type, were used as control pumps. The temperature increase in the new pump was four times slower than in the impeller pump when the outlet and the inlet of the pump was clamped. No sign of cavitation was observed when 0.1 ml air was introduced to the pump inlet under a negative pressure of 200 mm Hg in fresh bovine blood. As for pressure sensitivity of centrifugal pumps in practical applications, circuit resistance was a more essential factor than flow-pressure curves of the pump.

Animals↗

PubMed1994

[Three patients from two families with familial Creutzfeldt-Jakob disease having a point mutation in the prion protein gene at codon 200 (Glu-->Lys)].

We present three patients with Creutzfeldt-Jakob disease (CJD). They lived in Fuji city and its neighboring towns in the eastern part of Shizuoka prefecture. Patient 1 and patient 2 were cousins. Patient 1 developed the illness at the age of 50 in 1987 and died 13 months later. Patient 2 became ill at the age of 73 in 1989 and died seven months later. Patient 3 was related to a familial CJD cases in Yamanashi prefecture, known as Akai's "H" family (Akai et al in 1979, Yamamoto et al in 1986). She became ill at the age of 78 in 1990 and died four months later. Their clinical features were common; rapidly progressive dementia, generalized myoclonus, and periodic synchronous discharges on electroencephalographies. They were autopsied and neuropathologically diagnosed as typical CJD. Molecular genetic analysis of the prion protein (PrP) gene was performed on patient 2 and patient 3 using their frozen brain sections. The results showed a point mutation in the PrP gene at codon 200; GAG to AAG (Glu-->Lys). The eastern part of Shizuoka prefecture is adjacent to Yamanashi prefecture where a large number of patients with CJD including familial cases has been found during the recent 15 years. This study suggests that the patients with CJD in both Yamanashi and Shizuoka prefecture should be re-evaluated by analysis of the PrP gene.

Aged↗

PubMed1994

[Adult-onset dementia with abundant neurofibrillary tangles resembling progressive supranuclear palsy].

We described an autopsy case of 79-year-old woman with clinically unclassified senile dementia. The patient developed forgetfulness at the age of 73, and later, persecution mania, apathy and episodic stupor, but no extrapyramidal symptoms. Neuropathological examination revealed severe neuronal loss and gliosis of substantia nigra, moderate neuronal loss and marked grumose degeneration of dentate nucleus, and mild astrocytosis of subthalamic nucleus. Abundant neurofibrillary tangles (NFT) were observed in subthalamic nucleus, globus pallidus, substantia nigra, locus ceruleus, tegmentum of brain stem, pontine nucleus, inferior olive, and dentate nucleus. Gallyas silver impregnation method showed a wide distribution of argyrophilic grains and threads in cerebrum, brain stem and cerebellum. Although absence of clinical and neuropathological hallmarks excluded the diagnosis of progressive supranuclear palsy (PSP), the distribution of NFT and argyrophilic grains in this patient resembled PSP.

Age of Onset↗

PubMed1994

[Widespread argentophilic structures in progressive supranuclear palsy--an autopsy case report].

We report an autopsy case of progressive supranuclear palsy (PSP) with a five-year clinical course. A 67-year-old man was suffering from a gait disturbance and mental deterioration. Neurological examination at the age of 71 revealed pseudobulbar palsy, horizontal ophthalmoplegia, and truncal dystonia, and a diagnosis of PSP was made. Mental deterioration including forgetfulness and character change was also noted, and the patient sometimes exhibited intermittent stuporous states. Cranial computed tomography and magnetic resonance images revealed moderate brain atrophy, predominantly in the frontal lobes. The patient died of bronchopneumonia at the age of 71. Neuropathological examination confirmed typical pathological changes of PSP, such as neuronal loss, neurofibrillary tangles, and fibrillary gliosis in the subcortical nuclei. Gallyas-Braak silver impregnation revealed neurofibrillary tangles, silver-positive glia and thread-like structures in degenerating subcortical nuclei. In addition to these classical lesions, the argentophilic structures were detected in the cerebral cortex, cortical white matter and cerebellar white matter. In the cerebral cortex, they were abundant mostly in the precentral gyrus and subcortical white matter. Immunohistochemical studies revealed that most silver-positive structures were also tau 2 antibody-positive. Thus, these argentophilic structures seemed to be closely related to abnormal tau protein. Their distribution in this case implies that lesions related to abnormal tau protein may occur more extensively in the brains of PSP than expected.

Aged↗

PubMed1994

[Autosomal recessive hereditary cortical cerebellar atrophy with striatal degeneration--two siblings showing choreoathetoid movement, ataxia, dementia, and amenorrhea].

We present two siblings with hereditary cortical cerebellar atrophy (CCA), who showed peculiar clinical features. Their unaffected parents are cousins. The mode of inheritance in this family was autosomal recessive. Both patients developed involuntary movement and ataxia during the fourth decade. The proband (patient 1) was the elder sister. She developed choreoathetoid involuntary movement and cerebellar ataxia at the age of 32. At the age of 39, she showed mental deterioration and marked gait disturbance due to severe ataxia and amyotrophy. At the age of 40, she took medication for hypertension. At the age of 42, she was bedridden and had generalized convulsions and dysautonomia. Involuntary movement continued until her death at age 44. She had amenorrhea since the age of 25 years. Neuropathological findings. The brain weighed 1,010 g. We found marked degeneration in the cerebellar cortex including the molecular, Purkinje cells, and granular cell layers, and in the inferior olivary nuclei. In the basal ganglia, the putamen and caudate nuclei were moderately affected, but the substantia nigra and globus pallidus were spared. The cerebral cortex was spared, but the cerebral white matter showed diffuse myelin pallor without fibrillary gliosis. In the pons, the volume of the tegmentum was moderately decreased, but the base was spared. The spinal cord was normal. The findings of the patient differed from those of the case originally reported by Gordon Holmes in 1907. Holmes autopsied a case showing severe degeneration in both the cerebellar cortex including all three layers and the inferior olivary nucleus as in our patient. However, the striatum of his case spared and the patient did not develop involuntary movement as did other patients. The patients presented here should be distinguished from Holmes' original case clinicopathologically.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1993

A new inherited prion disease (PrP-P105L mutation) showing spastic paraparesis.

We report the clinicopathological findings of 5 patients with an inherited prion disease with a codon 105 (Pro to Leu) mutation. All of the patients had a spastic gait disturbance and progressive dementia without either cerebellar signs, myoclonus, or periodic synchronous discharges. Autopsy of 3 patients revealed numerous amyloid plaques in the cerebral cortex, especially in the motor cortex and the frontal lobe where neuronal loss and severe gliosis were observed in the absence of spongiform changes. The cerebellum was preserved histologically except for only a few amyloid plaques. The pyramidal tracts in the brainstem and spinal cord showed vacuolated changes and a loss of myelin, but no prion protein accumulations. Thus, the prion protein codon 105 mutation is considered to correspond to a new variant of the Gerstmann-Sträussler syndrome with spastic paraparesis.

Adult↗

PubMed1993

A case of antirabies inoculation encephalitis with a long clinical course.

In 1950, a 24-year-old man developed gait disturbance, incontinence of urine and increasing lethargy a week after a course of antirabies vaccination. He was diagnosed as having post-rabies vaccination encephalitis and had been in hospital for 37 years before he died in 1987. This is one of the rare cases in which the course of this disease was traced for an exceptionally long period. His personality began to deteriorate at an early stage of the illness, but his intellectual faculties seemed to be maintained rather well until a few years prior to his death. A neuropathological study revealed disseminated, patchy and somewhere perivascularly located demyelinated lesions in the cerebral white matter. Inflammatory lymphocytic infiltration was also remarkable in the CNS regions. But while the superimposed lesions due to convulsive attacks, traumatic contusion and terminal anoxia were remarkable, the whole aspect of neuropathological changes in rabies inoculation encephalitis cannot be observed.

Adult↗

PubMed1993

A straight path centrifugal blood pump concept in the Capiox centrifugal pump.

This article describes comparative studies of a newly developed "straight path" centrifugal pump (Capiox centrifugal pump) targeted for open-heart surgery and circulatory support. A unique straight path design of the rotor was very effective in reducing the pump's rotational speed and prime volume. This pump was evaluated for hydraulics, hemolysis, depriming characteristics, cavitation, and heat generation. Two commercially available centrifugal pumps, the Biomedicus cone-type pump and the Sarns 3M impeller-type pump, were used as controls. The new pump required the lowest pump speed to produce the same flow rates under the same pressure loads and demonstrated the lowest hemolysis and the lowest temperature rise with the outlet clamped. The air volume required to deprime the new pump was one-third to one-half that for the other pumps, and no sign of cavitation was observed even if a small amount of air was introduced to the pump inlet under a negative pressure of 200 mm Hg.

Animals↗

PubMed1993

[Intensive care of a postpartum patient complicated with HELLP syndrome].

We reported our intensive care experience of a postpartum patient with HELLP syndrome. We administered haptoglobin immediately after the delivery in order to prevent renal insufficiency following hemoglobinuria. Continuous veno-venous hemofiltration was undertaken and plasma exchange was also employed to overcome hepatorenal insufficiency. After these procedures an improvement was observed in her hepatic and renal functions. Administration of haptoglobin and blood purification would be beneficial for patients complicated with HELLP syndrome.

Adult↗

PubMed1993

[Some problems on the clinical phenotype of Machado-Joseph disease in relation between their ages at onset].

UNLABELLED: This study proposed that three phenotypes of Machado-Joseph disease (MJD) are closely related to the patients' ages at onset. MATERIALS: Six patients from two families. Autopsy performed in three of them (case 2, 4, 5). Two patients are a father (case 4) and his son (case 5). RESULTS: 1. Clinical features. All cases showed cerebellar ataxia and nystagmus. Progressive nuclear oculomotor palsy was common except for one case who killed himself in the early clinical stage. Pyramidal symptom which is increased deep tendon reflexes, spasticity, and extesor plantar response was common for three patients (case 3, 5, 6) whose ages of onset are under 40 years. One case (case 5) developed dystonia of foot at the age of ten and he developed the symptom of type 1 of MJD. However, the other three patients (case 1, 2, 4) who developed ataxia after the middle of the fifth decade showed hypotonia and decreased or absence of deep tendon reflexes from the beginning. The latter did not revealed spasticity or dystonia. Their clinical symptoms were identical with the type 3 of MJD. In spite of ages at onset, they showed general muscular atrophy and sensory disturbance in the advanced clinical course. 2. Neuropathological findings. 1) cerebellar system: Severe degeneration in the spinocerebellar system and mild to moderate one in the pontocerebellar system and dentate nuclei. The inferior olivary nucleus and the cerebellar cortex were almost preserved. 2) extrapyramidal system: Moderate to severe degeneration in the substantia nigra, globus pallidus (prominent in the internal segment) and subthalamic nucleus. 3) Degeneration in the oculomotor nuclei, motor neurons in the anterior horn and dorsal column of the spinal cord. CONCLUSION: These clinico-pathological findings indicate the difference of clinical phenotype is not always reflected those of neuropathological findings. The review of our experience and the literature suggests that the clinical features of MJD symptoms are related to the patients' ages at onset and clinical progression of the disease. When the disease begins before the age of ten, dystonia is an initial symptom, followed by pyramidal symptoms and cerebellar ataxia (type 1). In the early adult cases, the onset in which is earlier than forty, cerebellar ataxia and pyramidal symptoms are the initial symptoms, followed by extrapyramidal symptoms such as dystonia or choreoathetoid movements or both (type 2). In both instances however, decreased DTRs, muscular atrophy and sensory disturbance are common clinical manifestations at the advanced clinical stage. In late adult MJD cases with the age at onset after forty, the initial symptom is progressive cerebellar ataxia with hypotonia, followed by muscular atrophy and sensory disturbance (type 3). In spite of a marked degeneration in the extrapyramidal system, few or no extrapyramidal symptoms are detected in the last cases.

Adult↗

PubMed1993

[Study on the neuropathological diagnosis of senile dementia of the Alzheimer type].

Trying to define more suitable criteria for neuropathological diagnosis of senile dementia of the Alzheimer type (SDAT), the three histopathological criteria were tentatively made: 1) Laminar neuronal loss in the parahippocampus and medial gyri of the oral temporal lobe, 2) Appearance of senile plaques (SPs) and neurofibrillary tangles (NFTs) in the cerebral cortex more than the 95th percentile value of the age-matched control which were obtained from 12 different gyri of the 135 non-demented brains (60 to 106 years old), 3) Exclusion of the other diseases. There were 106 cases which satisfied the 3rd criterion in 581 consecutive autopsy cases of our hospital. They were divided into two groups. The first group was fulfilled the 1st criterion (46 cases), although approximately a half of the cases did not fulfilled the 2nd criterion. The second group was satisfied the 3rd criterion (60 cases). The first group included 43 cases clinically diagnosed as SDAT (93%), which occupied 72% of the all clinical SDAT cases. On the contrary, only 25% of the second cases were clinically diagnosed as SDAT. This study showed that laminar neuronal loss in the oral parahippocampus and medial gyri of the oral temporal lobe of both sides was the most important criterion for neuropathological diagnosis as well as widespread appearance of many SPs and NFTs in the cerebral cortex.

Aged↗

PubMed1993

[An autopsied Japanese case of hereditary olivo-ponto-cerebellar atrophy compatible with the original one of Menzel's report (1891)].

We report a patient whose clinicopathological findings are compatible with those of the case reported by Menzel in 1891. This case was briefly reported by Kinoshita et al in 1967 as hereditary ataxia of Menzel type. But the concept of this disease has been confused, especially in the early investigations for the hereditary olivo-ponto-cerebellar atrophy (OPCA) in Japan. The Kinoshita's patient should be considered as the first Japanese case whose findings are identical with Menzel's report. This report represents a precise study of the case reported by Kinoshita et al. The patient was a 42-year-old Japanese woman. Her mother and one of her brothers suffered from the same disease. She began to experience progressive ataxia at the age of 30. At age 42, she was admitted to another hospital because of inability to walk and mental deterioration. Neurological examination revealed cerebellar ataxia in the extremities and trunk, childish personality change, dementia, diminished deep tendon reflexes with extensor plantar response bilaterally, slowness and hypokinesia in the movement, generalized muscular atrophy, and sensory disturbance prominent in deep sensory. She had no involuntary movement and dysautonomia. She had no retinal degeneration, nystagmus, nor progressive nuclear oculomotor palsy. She died of pneumonia. Neuropathological findings revealed brain weight of 850g.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1993

[A case report of total intravenous anesthesia for renal transplantation].

A 20-year-old female patient with chronic renal failure received renal transplantation under total intravenous anesthesia with droperidol, fentanyl and ketamine. Anesthesia was induced with droperidol 12.5 mg, fentanyl 100 micrograms, ketamine 100 mg and vecuronium bromide 5 mg, and maintained with fentanyl and ketamine. Ketamine was given continuously at a rate of 2.0 mg.kg-1.h-1. After the renal artery and vein of the donor kidney were anastomosed with the patient's internal iliac artery and vein, the urine output of 7.7 ml.min-1 was obtained. The patient recovered from anesthesia smoothly and her postoperative course was uneventful. Although plasma epinephrine, cortisol and ADH levels showed significant changes during anesthesia mainly due to surgical stress, they returned to normal values after the end of operation. We conclude that this method of anesthesia would be a choice for renal transplantation.

Adult↗