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Biomedical subjects

T Shirabe

Publications and source records attributed to T Shirabe.

At least 37 records · Page 2Linked to original sources
PubMed1994

[Diagnostic neuropathology supported by immunohistochemistry].

There are various histological components in the central nervous system. When making a neuropathological diagnosis, it is difficult to identify each component. Recently, with the adoption of immunohistochemistry in diagnostic neuropathology, the identification of each component has become relatively easy. Immunohistochemistry is a useful method for staining objective tissues or cells employing the antigen-antibody reaction. Its specificity is very high. In the field of diagnostic neuropathology, immunohistochemical techniques have developed in the diagnosis of brain tumors. With the aid of immunohistochemistry, various findings have been obtained. At the present time, however, the number of available antibodies is rather restricted. As a result, accurate differentiation of microglia and brain macrophage is difficult, and the origin and definition of microglia and brain macrophages are controversial. In the near future, with further progress in immunohistochemical techniques, the number of antibodies will rapidly increase and their sensitivity should improve. When this is achieved, it will be possible to elucidate the pathophysiology of the central nervous system morphologically.

Animals↗
PubMed1994

[Relationship between thermal threshold and population of peripheral nerve fibers].

The relationship skin thermal threshold (TT) and density of both myelinated and unmyelinated fibers were investigated in 12 patients with various peripheral nerve diseases including Crow-Fukase syndrome, carcinomatous neuropathy and Charcot-Marie-Tooth disease. The TT was measured at a site above the medial tibial condyle using a thermal threshold tester. The TT was defined as the threshold at which a slight rise or fall in temperature could be detected. The size of the skin stimulation area was 13.5 cm2, the temperature change rate was 1 degrees C/sec, and the basal temperature was set at 34 degrees C. A sural nerve biopsy was performed and an image processor (IBAS) was used to measure the nerve fiber density. The TT in the patients with peripheral nerve disease was found to be elevated when compared with that of the 74 healthy controls (mean age of 38.1 +/- 13.3 years). Elevation of the TT has an intimate relation to damage of the small myelinated nerve fibers. In conclusion, unmyelinated fibers may play a relatively minor role in conveying thermal sensation.

Adult↗
PubMed1991

A family of familial hypercholesterolemia with cerebral infarction and without coronary heart disease. An unusual case with corneal opacity, polyneuropathy and carpal tunnel syndrome in the family: therapy with probucol and tocopherol nicotinate.

A study is presented of a 48-year-old female patient and her three siblings with familial hypercholesterolemia. The family members had episodes of cerebral infarction and apparently had atherosclerosis of the internal carotid artery, but no coronary heart disease due to their almost normal level of cholesterol. The laboratory studies of the family members revealed the elevations of serum lipid peroxides, serum lipoprotein(a), leukotriene C4 in blood, the thromboxane B2/6-keto-prostaglandin F1 alpha ratio in plasma and serum hydroxyl radical. Therefore, it is suspected that these factors accelerating atherosclerotic process caused the cerebral infarction. The patient demonstrated corneal opacities, palpebral xanthomas, thickened Achilles tendons, polyneuropathy and the carpal tunnel syndrome. Laboratory studies revealed an elevation in the OKT4/8 ratio, monocyte dysfunction with respect to phagocytosis and chemotaxis, and the presence of the 46XX/45XO mosaic chromosome. Lipid deposits were observed in the Achilles tendon, the transverse carpal ligament, the Schwann's cells and axons of the sural nerve, and in the keratocytes and stroma of the cornea. Following the administration of tocopherol nicotinate and probucol, the patient's serum lipid peroxide normalized and there was improvement in her palpebral xanthomas, thickening of the Achilles tendons and polyneuropathy. We conclude that the lipid deposits in this patient were due to the abnormal oxidative metabolism of low-density lipoprotein and a disturbance of the scavenger pathway due to the monocyte dysfunction.

Arteriosclerosis↗
PubMed1991

[Clinico-pathological comparative study between brainstem encephalitis of Iizuka type and neuro-Behçet's disease].

We experienced one necropsy case of brainstem encephalitis of Iizuka type (BSE) and one necropsy case of the brain-stem syndrome (BSS) of typical neuro-Behçet's disease, and compared them clinically and neuropathologically. Clinically both of these cases showed chronic progressive mental disturbance, pseudobulbar paresis, spastic tetraparesis, cerebrospinal fluid pleocytosis, increased protein, and brainstem atrophy observed by X-CT. Neuropathologically, irregular, boundary-indistinct demyelinating lesions and obsolete softening lesions were sporadically found, associated with perivascular lymphocytic infiltration and gliosis centering on the brainstem. In this way, both cases were similar in many points except for the presence or absence of cutaneo-muco-ocular signs specific for Behçet's disease. Also BSE and BSS reports in the literature showed that both diseases were similar not only in clinical findings consisting of mental disturbance and brainstem signs but also in neuropathological findings with similar topographical distribution of the same histopathological changes, including the variations and diversity of these characteristics. Especially of much interest is their similarity in characteristic mental disturbance. In discriminating BSE from multiple sclerosis and other diseases with exclusive involvement of the brainstem, it is important to understand their clinical characteristics. The characteristic mental disturbance includes damage to memory and sentiment, a change in personality, and lowering in spontaneity, but calculation ability and orientation are comparatively preserved. Of course the similarity in clinical and neuropathological findings does not necessarily mean the identical etiopathogenesis. However, it is possible to consider that neuro-Behçet's disease (syndrome) may form a wide spectrum with BSE and typical neuro-Behçet's disease at the both ends, regarding the time and spatial diversity of the appearance of cutaneo-muco-ocular signs.

Atrophy↗
PubMed1990

[A mitochondrial encephalomyopathy due to partial cytochrome c oxidase deficiency with giant evoked potentials--a case report].

A case of mitochondrial encephalomyopathy with a partial cytochrome c oxidase deficiency was reported with special reference to electrophysiological studies. A 56-year-old man was readmitted to Himeji Central Hospital due to mental deterioration and character change. At the age of 44 when he was attacked by his first epileptic seizure, he was admitted to Himeji Central Hospital, where EEG abnormalities and cerebral atrophy were found. Anticonvulsants helped to relieve his generalized convulsions but the EEG abnormalities persisted. At age 46, he had the second generalized seizure, so he quit his job as a crane operator. His family began to notice deterioration of his intellectual function and hyperaggressive behavior. His daily activities, intellectual performance and mental condition gradually deteriorated (WAIS FIQ less than 60). Other clinical and laboratory findings are as follows: bilateral impaired hearing, no optic nerve atrophy, no disturbance of extra ocular muscle movements, mild wasting and weakness of his extremities, normal coordination and sensation, no myoclonus or other involuntary movements, normal laboratory data of serum creatinine kinase, lactate dehydrogenase and aldolase, and increased amount of lactate and pyruvate in serum and cerebrospinal fluid (CSF), no abnormal amino acids in urine. A biopsy specimen of right biceps brachii muscle revealed numerous ragged-red fibers in frozen sections stained by the Gomori trichrome method. These fibers did not react to a cytochrome c oxidase staining. An ATPase staining demonstrated an atrophy of type-2 fibers. An electron micrograph showed many mitochondria in the sarcoplasm but few paracrystalline inclusions. A biochemical analysis of the muscle biopsy also revealed a significant decrease in the cytochrome c oxidase activity.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Diseases↗
PubMed1989

Mitochondrial encephalomyopathy (MELAS): pathological study and successful therapy with coenzyme Q10 and idebenone.

Two patients with mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes (MELAS) in one family are reported. Pathological examination of case 1 showed ragged-red fibers, with 7% of the fibers being unstained by cytochrome c oxidase stain, peripheral nerve damage, multiple areas of softening in the cerebrum and midbrain, and spongy changes in the cerebrum, optic nerve and pons. Electron microscopic examination revealed abnormal accumulations of mitochondria in the skeletal muscle, smooth muscle and cardiac muscle. The activity of cytochrome c oxidase in the brain and liver showed a tendency to decrease. In case 2 (maternal aunt of case 1), muscular weakness and peripheral nerve damage improved by treatment with coenzyme Q10. By adding idebenone to the coenzyme Q10 therapy, the EEG and Wechsler's Adult Intelligence Scale (WAIS) improved. Furthermore, in the cerebral spinal fluid (CSF), the protein, lactate, and pyruvate decreased, and the monoamines and monoamine metabolites increased.

Acidosis, Lactic↗
PubMed1989

[Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report].

A rare case of pulmonary hypertension due to glycogen storage disease type II (Pompe's disease) was reported. An 18-year-old girl was admitted to Kawasaki Medical School Hospital because of cyanosis, dyspnea on exertion and amenorrhea. She was 149 cm in height and 29 kg in body weight. Clinical examination revealed that pulmonary artery pulse and right ventricular heave were palpable over the precordium. On auscultation, an accentuated pulmonic second heart sound, pulmonic ejection sound and diastolic decrescendo murmur (Levine III/VI) were heard in the second intercostal space at the right sternal border. Her skeletal muscles, especially her intercostal muscles were generally weak and atrophic. Her electrocardiogram showed a pulmonary P-wave and right ventricular hypertrophy. The chest X-ray revealed right ventricular enlargement and a dilated pulmonary trunk. On echocardiography, the right ventricle and the main pulmonary artery were dilated, and a systolic notch of the pulmonary valve was found. Swan-Ganz catheterization disclosed that pulmonary artery pressure, right ventricular pressure and mean pulmonary capillary wedge pressure were 76/35 (50) mmHg, 76/12 mmHg and 10 mmHg, respectively. Respiratory function tests showed severe restrictive ventilatory impairment with hypercapnea and hypoxemia. On biopsy of the left quadriceps femoris muscle, the most striking finding was numerous intracytoplasmic vacuoles. The small vacuoles were stained with PAS and acid phosphatase. Electron microscopy showed massive glycogen accumulation in the sarcoplasm and membrane bound vacuoles (glycogenosome). Alpha-1, 4-glucosidase activity in the peripheral lymphocytes was definitely decreased. Her pulmonary hypertension resulted from respiratory muscular atrophy and alveolar hypoventilation caused by Pompe's disease.

Adolescent↗
PubMed1988

[A case of triple cancer--gastric, esophageal and hepatocellular carcinoma].

A case of triple cancer involving a tubular adenocarcinoma of the stomach, a squamous cell carcinoma of the esophagus, and hepatocellular carcinoma of the live is reported. These three cancers had been diagnosed while the patient was alive and later were confirmed by autopsy. The esophageal cancer and hepatocellular carcinoma were found almost simultaneously, 6 years after surgery for the gastric cancer. Although many cases of triple cancer have been reported, a triple cancer of this combination is very rare (0.3% of all cases of triple cancer), our case being the fifth such case in Japan.

Adenocarcinoma↗
PubMed1987

Subcortical vascular encephalopathy in a normotensive, young adult with premature baldness and spondylitis deformans. A clinicopathological study and review of the literature.

Progressive subcortical vascular encephalopathy (PSVE) usually occurs in elderly individuals, suffering from hypertension. We here describe a male, born of consanguineous parents, who first showed signs of PSVE at the age of 30. Despite the absence of hypertension or known metabolic causes, the degenerative cerebral vascular disease developed progressively. Several cases, surprisingly identical to the one reported here, were traced using Japanese medical records. They are clinically characterized by: early onset of PSVE (at age 25-30), absence of persistent hypertension, diffuse alopecia since youth, spondylitis deformans with early onset, often so severe as to necessitate surgery, and the possible existence of an autosomal recessive transmission. Cases with these features appear to constitute a distinct clinical entity, possibly a new form of premature aging syndrome.

Adult↗
PubMed1986

Electrophysiological and pathological studies on Creutzfeldt-Jakob disease with retinal involvement.

In a case of Creutzfeldt-Jakob disease in a 59-year-old female, multifocal degeneration from the cerebral cortex through the visual pathway to the retina was detected clinically, electrophysiologically, and pathologically. Visual evoked cortical potentials (VECPs) showed a peculiar huge negative wave in the early stage but the amplitude reduced gradually. The a- and b-waves of the ERG were detectable in the final stage. Dynamic topography of VECPs revealed a delay of excitation in the visual cortex in the early stage, but a complete defect of the cortical potential and diminished reactivity of the brain stem were apparent in the later stage. Pathological findings were the spongy degeneration of the cerebral cortex, demyelination of the white matter and the optic pathway, and the degeneration of the nerve fiber layer and ganglion cell layer of the retina.

Brain↗
PubMed1986

Disseminated Trichosporon beigelii infection in a patient with malignant histiocytosis.

A case of disseminated Trichosporon beigelii infection is reported. Trichosporon inhabits the soil, but may also be present in the human skin and mouth as a normal flora. Occasionally it causes superficial mycotic infection, but it is rarely invasive. A review of literature disclosed 21 reported cases of invasive Trichosporon beigelii infection, to date. Of these, two cases were from Japan. The scarcity of reports on this invasive infection may be due to a lack of awareness of the organism. Histologically, it should be carefully differentiated from Candida.

Adult↗