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Biomedical subjects

M Konagaya

Publications and source records attributed to M Konagaya.

At least 37 records · Page 2Linked to original sources

The role of histamine in ethanol-induced gastric mucosal injury in the rat.

It has been suggested that changes in the micro circulatory system are related to the early production of acute gastric mucosal injury and inflammatory factors such as prostaglandins, histamine, etc., have been considered as contributing to the development of the injury. We assessed the permeability of the gastric mucosa in rats with ethanol-induced acute mucosal injury by measuring the leakage rate of 51chronium-ethylene-diamine-tetraacetic acid (51Cr-EDTA) into the gastric juice. Histamine concentrations in the gastric mucosa was measured by high performance liquid chromatography. The enterochromaffin-like (ECL) cell counts in the gastric mucosa was performed following histamine staining with an enzyme-labeled antibody, and the histamine released due to degranulation was observed. We also investigated the kinetics of endogenous histamine in the gastric mucosa. Five minutes after the administration of ethanol, an increase in permeability, an increase in histamine concentration, and a decrease in ECL cell count were found in the gastric mucosa. These results suggest that endogenous histamine in the gastric mucosa is closely related to the early development of acute gastric mucosal injury.

Animals↗

Transmission of dystrophinopathy by X-chromosome inversion.

A woman had a karyotype of 46Xinv(X)(p21.2;q26.1) and mosaicism of dystrophin-negative muscle fibers. The X-chromosome inversion was transmitted to her daughter, who had an elevated serum CK. The genetic transmission as well as the probable random inactivation of the abnormal X chromosome in this mildly affected patient distinguish this case from those of dystrophinopathy with X;autosome translocations.

Chromosome Inversion↗

[An autopsied case of dominantly affecting upper motor neuron with atrophy of the frontal and temporal lobes--with special reference to primary lateral sclerosis].

In this paper, the autopsy findings of a 78-year-old man mimicking primary lateral sclerosis (PLS) are reported. His clinical symptoms were slowly progressive spasticity, pseudobulbar palsy and character change. He died of sepsis 32 months after protracting the disease. The autopsy revealed severe atrophy of the frontal and temporal lobes. The histological findings were severe neuronal loss with gliosis in the precentral gyrus and left temporal lobe tip, loss of Betz cell, prominent demyelination throughout of the corticospinal tract, axonal swelling in the cerebral peduncule, severe degeneration of the amygdala, mild degeneration of the Ammon horn, normal substantia nigra, a few neuronal cells with central chromatolysis in the facial nerve nucleus and very mild neuronal cell loss in the spinal anterior horn. The anterior horn cell only occasionally demonstrated Bunina body by H & E and cystatin-C stainings, as well as, skein-like inclusion by ubiquitin staining. Thus, this is a case of uncommon amyotrophic lateral sclerosis (ALS) dominantly affecting the upper motor neuron including the motor cortex and temporal limbic system. In analysis of nine cases of putative primary lateral sclerosis in the literature, six cases showed loss of Betz cell in the precentral gyrus, and four cases very mild involvement of the lower motor neuron such as central chromatolysis and eosinophilic inclusion body. Degeneration of the limbic system was observed in two cases. We indicated a possible subgroup with concomitant involvement in the motor cortex and temporal lobe in motor neuron disease dominantly affecting the upper motor neuron.

Aged↗

Clinical and magnetic resonance imaging study of extrapyramidal symptoms in multiple system atrophy.

Slit-hyperintensity in the outer margin of the putamen on T2 weighted MRI was found in 17 out of 28 patients with clinically diagnosed multiple system atrophy. Thirteen of these 17 patients showed extrapyramidal signs. Five patients had only unilateral slit-hyperintensity; four of them had contralateral rigidity; and one had bradykinesia. Despite mild rigidity, one case showed no slit-hyperintensity. One of the 14 cases with parkinsonism showed no hyperintensity, and four of the 14 cases without parkinsonism showed hyperintensity. On the other hand, slit-hyperintensity was not seen in any of 25 patients with clinically diagnosed Parkinson's disease. Putaminal slit-hyperintensity is a useful MRI feature in the differential diagnosis between Parkinson's disease and multiple system atrophy predominantly affecting the extrapyramidal system.

Aged↗

[Significance of serum myosin light chain-1 level in neuroleptic malignant syndrome].

In Parkinson disease, the interruption of medication often results in neuroleptic malignant syndrome, which is caused by acute inhibition of the dopaminergic system. It is a serious question in the management of Parkinson disease, whether episode of pyrexia is derived from neuroleptic malignant syndrome or other origins. In this syndrome, muscle damage enhances serum titers of myogenic enzymes and proteins. Myosin light chain-1 (MLC-1), which is a small fragment of myosin, has been reported to show long lasting elevation compared with CK in neuromuscular diseases and cardiac infarction. Then, we evaluated the clinical significance of serum MLC-1 in six patients with neuroleptic malignant syndrome, i.e., four cases of Parkinson disease, one multiple system atrophy and one schizophrenia with acute administration of haloperidol. Muscle breakdown was observed by the elevation of serum MLC-1 titer, which sustained several days after normalization of serum CK titer. In two cases of Parkinson disease, high level of serum MLC-1 was observed in spite of normal body temperature, which suggested pre-clinical stage of neuroleptic malignant syndrome. Thus, we concluded that the estimation of serum MLC-1 is useful in those patients with high risk of neuroleptic malignant syndrome.

Adult↗

Recurrent polyradiculoneuropathy with hyperthyroidism.

A 30-year-old woman, who had had two episodes of distal dominant sensorimotor disorders in the extremities, developed again sensorimotor involvement in the distal portion of all limbs. She was also found to have hyperhidrosis, tachycardia and goiter. Neurological and endocrinological examinations led to a diagnosis of coexistence of recurrent polyradiculoneuropathy and hyperthyroidism. Treatment with thiamazole resulted in improvement of the neurological features as well as of hyperthyroidism. The relationship between polyradiculoneuropathy and hyperthyroidism is discussed.

Adult↗

[Asynchronous Bell's phenomenon in progressive supranuclear palsy].

A 60-year-old man suffering from progressive supranuclear palsy showed asynchronous Bell's phenomenon and apraxia of eye-lid opening. He showed synchronous upward rotation of the eyeball at closing eyes. Although apraxia of eye-lid opening was observed in both sides, eye opening of the right side took longer duration than that of the opposite side. When he tried to open his eyes, unilateral Bell's phenomenon was sustained in the side of involuntary eye closing for a several seconds. These findings indicated disorders of the coordination of eye opening-and-closing, which regulate the orbicural muscle of eye, levator muscle of upper eyelid and external ocular muscles. And the mechanism of synchronous eye movement between the both sides was also disturbed. The administration of L-droxydopa was effective to gait disturbance, but not to ocular movement disorder including impaired Bell's phenomenon. The pharmacological results suggested different neuron system between locomotion control and conjugation affected in this disease.

Apraxias↗

[A clinico-MRI study of extrapyramidal symptoms in multiple system atrophy--linear hyperintensity in the outer margin of the putamen].

We did a clinico-MRI study concerning extrapyramidal symptoms and T2-weighted MRI findings of the putamen in twenty patients with multiple system atrophy (MSA) as well as twenty-five with idiopathic Parkinson's disease. Nine out of twenty MSA patients showed extrapyramidal symptoms. And we could not observe cerebellar ataxia in two of these patients because of severe rigidity and akinesia. Eight out of nine MSA patients with extrapyramidal symptoms showed linear hyperintensity in the outer margin of the putamen. This abnormal intensity was bilateral and symmetric in most patients. However, in MSA patients without extrapyramidal symptoms, only one patient showed the linear hyperintensity. We could not find such abnormal intensity in any patients with Parkinson's disease. On proton density MRI, the signal intensity in the lesion was higher than that in the gray matter, which leads the speculation that the hyperintensity is gliosis of the putamen or increased extracellular fluid space caused by severe shrinkage of the putamen. This characteristic MRI finding is useful to distinguish MSA with extrapyramidal symptoms from Parkinson's disease.

Aged↗

Quadriceps myositis.

A young woman with slowly progressive muscular weakness and atrophy localized in both thighs is reported. Laboratory, electromyographic and histological findings suggested that the patient suffered from chronic myositis with a background of autoimmune disorder. Quadriceps myositis is a rare condition. The previously reported cases of this disease in the literature are reviewed.

Adult↗

[Central nervous system lesion in myotonic dystrophy--demonstrated by X-ray computed tomography and magnetic resonance imaging].

We made an attempt to detect organic brain lesions in eight patients with myotonic dystrophy (MyD) using X-ray computed tomography (CT) and magnetic resonance imaging (MRI). The patients comprised seven men and one woman with ages ranging from 41 to 58 years (average 47 +/- 5.3 years). Seven patients had intellectual impairment assessed using an intelligence quotient test (WAIS-R < 70). CT scans were obtained in the axial plane with a slice thickness of 10 mm, and MRI scans were performed on a 1.5 tesla system in the axial and coronal sections with a slice thickness of 8 mm. CT scans displayed both marked sulcal and prominent ventricular enlargement in 6 of the 8 patients, indicating the presence of brain atrophy, and 3 cases had a few paraventricular low density area in the cerebral hemispheres. In contrast to CT, MRI study revealed more widespread brain parenchymatous lesions in all the 8 cases, showing the multiple foci of high signal intensity with varying size in the white matter on T2-weighted images and proton density-weighted images. No visible changes were detected on CT or MRI in the brain stem and cerebellum. The present study demonstrated that there exist organic cerebral lesions, predominantly located in the white matter in patients with MyD, regardless of focal neurological symptoms. The parenchymatous lesions in the brain can be detected with MRI, and lesser extent with CT, and are considered to be intimately correlated with intellectual deterioration commonly seen in patients with MyD.

Adult↗

[CSF acetylcholinesterase activity in central neurological diseases involving cholinergic systems].

The enzymatic activity of acetylcholinesterase (AchE) in the cerebrospinal fluid (CSF) is considered to be a marker of central cholinergic neuron integrity. Then, we evaluated CSF AchE activity in 90 cases of neurological diseases involving cholinergic system and their related disease, and 28 control cases without central organic lesions or abnormal findings in routine CSF study. AchE activity was evaluated according to Ellman's method using acetylthiocholine iodide as a substrate and tetraisopropyl-pyrophosphoramide, a specific inhibitor of butyrylocholinesterase. CSF AchE of Alzheimer type dementia (AD/SDAT, N = 12: 21.9 +/- 4.7 nmol/ml/min) showed no significant change from those of both control group (22.1 +/- 3.9) and vascular dementia (9: 21.7 +/- 6.7). In extrapyramidal diseases, reduction of the activity was observed in Huntington's chorea (HC, 4: 16.3 +/- 1.4) and progressive supranuclear palsy (PSP, 4: 17.6 +/- 1.7), whereas normal activity was shown in Parkinson's disease (PD, 19: 22.5 +/- 4.6), dentatorubropallidoluysian atrophy (DRPLA, 4: 22.6 +/- 4.2) and striatonigral degeneration (SND, 4: 20.4 +/- 4.3). In olivopontocerebellar atrophy (OPCA, N = 16), we disclosed reduced CSF AchE activity (15.8 +/- 2.4) which had significant correlations with the atrophy of the pontine base (r = 0.6017, p less than 0.02) and cerebellar vermis (r = 0.5450, p less than 0.05) in MRI. AchE activity in cerebellar cortical atrophy (CCA, 5: 20.6 +/- 2.2) remained within the control values. Normal activity was demonstrated in both amyotrophic lateral sclerosis (6: 24.3 +/- 7.3) and spinal muscular atrophy (4: 22.9 +/- 3.9).(ABSTRACT TRUNCATED AT 250 WORDS)

Acetylcholinesterase↗

[Abnormality of hypothalamic dopaminergic system in neuro-degenerative diseases--evaluation of alpha-melanocyte-stimulating hormone-like immunoreactivity in cerebrospinal fluid].

Dopaminergic neuron controls CNS functions such as meso-limbic, striato-nigral and tubero-infundibular systems. The purpose of the present study is the evaluation of the hypothalamic dopaminergic neuron activity in neuro-degenerative disorders. alpha-melanocyte-stimulating hormone (alpha-MSH) is synthesized in the arcuate nucleus and lateral part of the hypothalamus, and its secretion is under the inhibitory control of the dopaminergic neuron both in the hypothalamus and pituitary. alpha-MSH-like-immunoreactivity (alpha-MSH-LI) in CSF is thought to be representative to the dopaminergic neuron activity in the hypothalamus. We therefore evaluated CSF levels of alpha-MSH-LI in spinocerebellar degenerations and extrapyramidal diseases. The subjects are 11 patients with Parkinson's disease, 16 with Shy-Drager syndrome (SDS), 16 with cerebellar cortical atrophy, 3 with Machado-Joseph disease, 3 with dentato-rubro-pallido-luysian atrophy and 2 with Huntington's disease as well as 24 controls. All patients with Parkinson's disease were administered levodopa and carbidopa. CSF was sampled through lumbar puncture in the morning. After the centrifugation, supernatant of CSF was stored at -40 degrees C until used. alpha-MSH in CSF was extracted by Rainero's method and measured by RIA. alpha-MSH-LI levels in control was 23.9 +/- 2.6 pg/ml (mean +/- SD). The significant elevation was observed in Parkinson's disease (40.3 +/- 7.5, p less than 0.001) and SDS (42.3 +/- 9.4, p less than 0.001). The levels showed not significant correlation with age, duration of illness or severity of autonomic disorder. Most of other diseases demonstrated the levels within normal range.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A case of HTLV-1 associated myelopathy with diffuse white matter lesion of the frontal lobe and continuous lesion of the pyramidal tract on cranial MRI].

In this report, the characteristic findings of cranial MRI of a case with HTLV-1 associated myelopathy (HAM) is described. The patient was a 62-year-old woman with 6 years history of paraplegia. Her main clinical signs were bilateral spastic paraplegia in the lower limb girdle muscles and extremities, paresthesia below the tenth thoracic cord level and urinary disorder. There was bilateral blepharoptosis. Hyperreflexia was observed in the examination of the cranial nerves and upper extremities. She showed no dementia or any other higher cortical dysfunctions. Positive anti-HTLV-1 antibody in the serum and cerebrospinal fluid established a diagnosis of HAM. T2 weighted MRI study revealed the symmetrical diffuse hyperintensity in the subcortical white matter of the frontal lobe and temporal lobe. The hyper-intensity was also observed in the bilateral internal capsule-peduncular base junction and pontine base, which indicated the continuous lesions in the intracranial pyramidal tracts. These MRI findings were different from those of reported cases of scattered lesion in central nervous system. There are some speculations for the nature of the diffuse lesion in the white matter, i.e., fusion of solitary gliosis and/or perivascular cuffing, or diffusely advanced spongy state. The continuous lesion of the pyramidal tract is suspected a systemic demyelination. Although the pathomechanism still remains uncertain, HTLV-1 infection generally affects the spinal pyramidal tract. It seems that this case is selectively affected in the pyramidal tracts in CNS.

Female↗

Abscessed leiomyosarcoma of the ileum.

A-49-year-old man was admitted because of a lower abdominal mass. During the five days prior to admission, he had noted fever and lower abdominal pain. Palpation of the abdomen revealed a tender mass, 10 cm in diameter, in the suprapubic region. An x-ray revealed an irregular collection of gas, 9 cm in diameter, in the pelvic cavity, which appeared as a mirror image when the patient was upright. Based on the physical finding and the results of a barium enema, abdominal CT, MRI, and small intestine imaging, a diagnosis of leiomyosarcoma of the ileum was made. Excision of the tumor and part of the small intestine was performed. A saccular tumor, 11 x 6 x 5 cm, was found 1.0 m from the cecum and growing out of the wall of the ileum. A large amount of pus was found inside the cavity. The pathological diagnosis was leiomyosarcoma.

Abscess↗

Successful transcatheter embolization of pseudoaneurysm associated with pancreatic pseudocyst.

Hemorrhage into a pancreatic pseudocyst is a rare event, but is the most rapidly lethal complication of chronic pancreatitis. Visceral-vessel aneurysms are an unexpectedly common finding in arteriography of patients with chronic pancreatitis. This case report describes bleeding from an anterior superior pancreaticoduodenal artery aneurysm, caused by chronic pancreatitis. The aneurysm was successfully treated by embolization with a steel coil.

Aneurysm↗

Magnetic stimulation study in mirror movements.

A young man with congenital mirror movements was studied by non-invasive magnetic stimulation. Radiological examination showed no craniocervical or pituitary abnormality except for mild atrophy of the right hippocampus. Magnetic stimulation of the motor cortex caused large amplitudes in the ipsilateral hand muscles, indicating the possibility of functional disorder in the motor pathways from the motor cortex to muscles in the upper extremities.

Adult↗