Biomedical subjects

F Ikuta

Publications and source records attributed to F Ikuta.

At least 73 records · Page 4Linked to original sources

PubMed1992

[Dentatorubropallidoluysian atrophy (DRPLA): comparative pathological study on clinical groups classified into juvenile, early adult and late adult types].

We performed a clinicopathological study on 12 autopsied cases of dentatorubropallidoluysian atrophy. They were divided into 3 groups according to the age at onset: juvenile type (6 cases), early adult type (4 cases) and late adult type (2 cases). In juvenile type showing progressive myoclonus epilepsy (PME) syndrome, degeneration of the globus pallidus was more marked than that of the dentate nucleus. Mild to moderate atrophy was seen in the brain stem and spinal cord. In early adult type showing milder symptom of myoclonus and epilepsy, the globus pallidus and dentate nucleus were equally degenerated to various extents in most cases. Atrophy of the brain stem and spinal cord was mild to moderate in degree. In late adult type without PME syndrome, degeneration of the dentate nucleus was more marked than that of the globus pallidus. The brain stem and spinal cord were severely atrophic. On the other hand, the cases showing severe dentate lesion had a tendency to show severe atrophy of the brain stem and spinal cord. We consider that development of myoclonus, epilepsy and choreoathetoid movement in DRPLA patients has close relation to the extent of not only degeneration of the globus pallidus and dentate nucleus, but also atrophy of the brain stem and spinal cord.

Adolescent↗

PubMed1992

[Hereditary dentatorubropallidoluysian atrophy--clinical variants in a family and degeneration of cerebral white matter in a proband].

We describe a family with hereditary dentatorubropallidoluysian atrophy (DRPLA). 4 patients through 3 successive generations showed a wide clinical variety. The female proband with onset in the elderly developed choreiform involuntary movement, dementia, hyperreflexia and, at the progressive stage, mild ataxia. However she had never displayed epilepsy and myoclonus. The 2 sons showed dementia, choreoathetoid movement and ataxia. The grandson developed typical signs and symptoms of progressive myoclonus epilepsy. The brain CT in the proband showed severe cerebellar and brain stem atrophy, moderate cerebral cortical atrophy and diffuse low density lesions in the deep cerebral white matter. Her neuropathological examination revealed the atrophy and gliosis of cerebral and cerebellar white matter concomitant with both dentatorubral and pallidoluysian system degeneration. The present study indicates that hereditary DRPLA can include multiple clinical variants even in the same family and the degeneration of cerebral and cerebellar white matter besides dentatorubral and pallidoluysian system.

Adolescent↗

PubMed1991

Large neurons in the neostriatum in Alzheimer's disease and progressive supranuclear palsy: a topographic, histologic and ultrastructural investigation.

Large neurons in the neostriatum of patients with Alzheimer's disease (AD) and progressive supranuclear palsy (PSP) were investigated topographically, histologically and ultrastructurally. The number of large neurons whose nuclear area is greater than 101 microns2 was uniformly decreased in the neostriatum in PSP, but the decrease of these neurons in AD appeared to be more marked in the nucleus accumbens. Most of the remaining large neurons in both diseases contained neurofibrillary tangles (NFTs). In addition, some of the small neurons in PSP were positive for tau-immunostaining. Curly fibers were frequently observed in AD, but were absent in PSP. Ultrastructurally, NFTs in AD were composed mainly of paired helical filaments, whereas those in PSP contained straight tubules.

Aged↗

PubMed1991

Galactosialidosis: neuropathological findings in a case of the late-infantile type.

The neuropathological findings in a 13-year-old Japanese male showing decrease of sialidase and beta-galactosidase activities are reported. The patient was the product of normal pregnancy to consanguineous parents. He started to sit at 8 months, stand at 20 months and walk at age of 2; mental retardation, visual disturbance, cerebellar ataxia, myoclonus and epilepsy developed by the age of 10, and he died at 13. Neuropathological investigation revealed neuronal loss and storage. Severe loss of neurons was observed in the thalamus, globus pallidus, lateral geniculate body, gracile nucleus, Purkinje and retinal ganglion cells. Marked ballooning was seen in the Betz cells and neurons in the basal forebrain, the motor neurons in the cranial nerve nuclei and spinal cord, and in the trigeminal and spinal ganglia. The storage material varied in staining from region to region and from neuron to neuron. Electron microscopic investigation revealed a variety of intracytoplasmic and intranuclear inclusions: membranous cytoplasmic bodies, parallel, wavy-lamellar or tortuous tubular structures, lipofuscin-like irregular-shaped pleomorphic bodies, and cytoplasmic vacuoles with fine granules and lamellar materials. The severity of the neuronal loss did not seem to correlate with the amount of the storage materials, but with the presence of tortuous tubular inclusion.

Brain↗

PubMed1991

Construction of spinal cord cDNA library and application for subtractive cloning of spinal cord-specific cDNAs.

Neurodegenerative diseases are characterized by neuronal degeneration of specific neurons, e.g., degeneration of motoneurons in amyotrophic lateral sclerosis. As an approach to understand molecular mechanisms of neuronal degeneration of human spinal cord motoneurons in various motor neuron diseases, we have constructed a human spinal cord cDNA library and developed a strategy for isolating spinal cord-specific genes by subtractive cloning. We constructed human spinal cord and brain cDNA libraries from postmortem human spinal cord and brain. To isolate human spinal cord-specific cDNAs, a spinal cord-enriched [32P]cDNA probe was generated by the phenol emulsion reassociation technique. Forty-eight cDNA clones out of 10,000 colonies gave strong signals with the subtracted probe, and individual spinal cord cDNA clones were isolated. Northern blotting analysis confirmed that two spinal cord cDNA clones are, in fact, more abundant in spinal cord compared to brain.

Blotting, Northern↗

PubMed1991

Are bunina bodies of endoplasmic reticulum origin? An ultrastructural study of subthalamic eosinophilic inclusions in a case of atypical motor neuron disease.

We carried out an electron microscopic study of eosinophilic intracytoplasmic inclusions in the subthalamic neurons in a case of atypical motor neuron disease. These inclusions were identical in light microscopic morphology and staining characteristics to Bunina bodies. Ultrastructurally, most of the intracytoplasmic inclusions observed were divisible into two different types (I and II). Type I inclusions had features essentially identical to those of Bunina bodies demonstrated previously in the anterior horn cells in cases of motor neuron disease; they consisted of electron-dense, granular material without a particular limiting membrane and often contained a number of translucent areas with entrapped cell organelle-like structures. Type II inclusions were very similar to structures known as multilaminated bodies; at their margin, they sometimes showed continuity with the cisternae of endoplasmic reticulum. The remainder of the inclusions were considered to be transitional forms between these two types, and some of them showed the respective features of both types I and II. These findings suggest that Bunina bodies are of endoplasmic reticulum origin.

Adult↗

PubMed1991

An autopsy case of atypical motor neuron disease with Bunina bodies in the lower motor and subthalamic neurons.

We report a 37-year-old male without any family history of neurological disease who suffered progressive muscular atrophy and sensory impairment of 4 years' duration. Autopsy revealed neuronal loss in the anterior horns of the spinal cord and in the hypoglossal and facial nuclei of the brain stem. The corticospinal tracts of the spinal cord showed only mild degeneration. In addition, there were obvious degenerative lesions manifested by loss of neurons, myelin and axons in the spinal posterior columns, Clarke's column, spinocerebellar tracts and dorsal root ganglia as well as in the subthalamic nucleus, globus pallidus, substantia nigra and cerebellar dentate nucleus. Furthermore, we frequently encountered Bunina bodies not only in the lower motor neurons but also in the subthalamic neurons. We consider this case to be an atypical example of motor neuron disease with features of multisystem degeneration. The fact that Bunina bodies were observed in both lower motor and subthalamic neurons in this case suggests a common etiology of neuronal degeneration in these two different systems.

Adult↗

PubMed1991

Creutzfeldt-Jakob disease in a patient with a cadaveric dural graft.

We report a 26-year-old woman with Creutzfeldt-Jakob disease (CJD) who had received cadaveric dural material 33 months before the onset of neurologic symptoms. This is the fourth case in which a dural graft was the putative source of the CJD agent. All four cases had the grafting before changes in the sterilization procedure adopted in 1987 to inactivate the CJD agent.

Adult↗

PubMed1991

[Ultracytochemical study of Ca(2+)-ATPase activity in developing rat brain].

We carried out the ultracytochemical study of Ca(2+)-ATPase activity for the developing rat brain at embryonic day 11 (E 11), E 14, E 19 and postnatal day 5(P 5), and for the adult rat cerebrum. Matrix cells at E 11 and neuroblasts at E 14 showed no cytochemical reaction (CR) for Ca(2+)-ATPase activity. Glioblasts at E 19 showed very weak CR. Reaction product (RP) was observed on the surfaces of the neuronal and glial cells at P 5 and adult rat cerebrum. Microvessels at E 14 demonstrated no CR, but microvessels at E 19 showed CR, in which RP was, of a fine granular appearance, diffusely observed along the luminal and abluminal surfaces. At P 5, coarsened RP was diffusely observed on the luminal and abluminal surfaces. The CR for capillary Ca(2+)-ATPase activity in the adult rat cerebrum was found to be mild and the RP formed continuous and discontinuous thin layer on the abluminal surface. The results suggest that Ca(2+)-ATPase activity is closely related with development of the cells in the central nervous system.

Animals↗

PubMed1991

[A case of brain metastasis of rhabdomyosarcoma in a child].

We report here a specific case of metastatic brain tumor orienting from rhabdomyosarcoma. An 11-year-old boy came to our hospital with complaints including headache, exophthalmos and tumors in the right frontal and left occipital region. 6 months previously subtotal removal of rhabdomyosarcoma in his right foot had been performed. CT scan demonstrated tumors in the right orbit, right frontal region and left occipital region. The tumors existed bilaterally in the skull and markedly enhanced by contrast medium. But the changes of the cranium were mild, only the dipole was slightly dilated. MRI showed that the tumors were extraaxial masses. Right external carotid angiogram revealed numerous tumor vessels fed by the meningeal artery. On April 3rd, biopsy was performed at the right frontal region. The tumor had spread in the epidural space but the cortical surface seemed to be intact. Histologically, the tumor consisted of small round cells and revealed multinuclear giant cells. It involved the skull bone, dura mater and subdural tissues. Accordingly the tumor was diagnosed as rhabdomyosarcoma. Although tumor increased rapidly, the intracranial region did not change remarkably. It was an interesting phenomenon. Eventually, rhabdomyosarcoma metastasized to the lung, and the patient died due to respiratory distress.

Brain Neoplasms↗

PubMed1991

[A case of an intracranially located esthesioneuroepithelioma].

A 1-year-8-month-old boy was admitted to our service on September 30, 1986, complaining of nausea, vomiting and consciousness disturbance lasted for about 2 weeks. In CTs, right frontal cystic mass which was homogeneously enhanced by contrast media was revealed. Neither hypervascularity nor tumor staining were seen angiographically. On October 9, 1986, total removal of the tumor was performed. The tumor was located extracerebrally in the right anterior cranial fossa, but was covered with arachnoid membrane. The tumor showed tight adhesion with falx cerebri, particularly at crista galli where an invasive infiltration was seen. Light microscopic examination demonstrated oval or spherical small cells arranged multilobularly with rosette like formation and numerous mitoses. Ultrastructurally, cilia, microvilli and junctional complexes were observed. No dense-cored secretory granules were found in the tumor cells. Immunohistochemical study on this tumor showed negative NF and GFAP; positive NSE, S-100, vimentin and keratin. From these findings, the tumor was diagnosed as esthesioneuroepithelioma. Postoperatively, irradiation and chemotherapies were also performed, and the patient showed uneventful course. On January 31, 1987, he was discharged on his foot, and no recurrent or metastatic signs could be found until the end of March of 1990.

Brain Neoplasms↗

PubMed1990

Parkinson's disease: an immunohistochemical study of Lewy body-containing neurons in the enteric nervous system.

We performed immunohistochemical analysis of specimens from three autopsied patients with Parkinson's disease, using antibodies to tyrosine hydroxylase (TH), vasoactive intestinal polypeptide (VIP), somatostatin, met-enkephalin, leu-enkephalin and substance P in an attempt to reveal the types of neurons that contain Lewy bodies (LBs) in the paravertebral and celiac sympathetic ganglia and in the enteric nervous system of the alimentary tract. In the sympathetic ganglia, almost all LB-containing neuronal cell bodies and processes were immunoreactive for TH. In the alimentary tract, however, most LBs were found in the VIP-immunoreactive (VIP-IR) neuronal cell bodies and processes. In spite of the significant presence of TH-IR neuronal cell bodies and processes in the alimentary tract, LB-containing TH-IR neuronal elements were rarely encountered. These findings indicate that in the alimentary tract, the VIP neuron system is mainly involved in the disease process of Parkinson's disease.

Aged↗

PubMed1990

Lewy bodies in the lower sacral parasympathetic neurons of a patient with Parkinson's disease.

Lewy bodies were observed incidentally in the neurons of the dorsal group of nucleus intermediolateralis of the 3rd sacral segment of the spinal cord in a 74-year-old male with Parkinson's disease. The findings indicate the degeneration of the preganglionic parasympathetic neurons innervating the internal anal sphincter. The correlation between the findings and the mechanism of constipation in this disease are discussed.

Aged↗

PubMed1990

Dural arteriovenous malformation with abnormal parenchymal vessels: an autopsy study.

A 48-year-old man with dural arteriovenous malformation (AVM) is reported. Radiologically, the dural AVM was demonstrated mainly in the region of the left transverse sinus. Postmortem examination revealed dural AVM involving the bilateral transverse, superior sagittal and straight sinuses. In addition, numerous malformed venous vessels and extensive necrosis were observed mainly in the parenchyma of the brain stem and cerebellum. The present case suggests that dural AVMs may be associated with malformed venous vessels in the brain parenchyma.

Adult↗

PubMed1990

Involvement of extraocular muscle in mitochondrial encephalomyopathy.

We carried out a histological examination of the extraocular muscles (EOMs) in a case of myoclonus epilepsy associated with ragged-red fibers (MERRF) and two cases of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), which did not manifest external ophthalmoplegia clinically. By light microscopy, many granular and vesicular fibers were seen associated with endomysial fibrosis. Electron microscopy revealed that the fibers showed prominent accumulation of abnormal mitochondria, extensive loss of myofibrils, proliferation of free sarcoplasmic reticulum and an increased amount of lipid vacuoles. These changes were more pronounced in MELAS than in MERRF. Hirano bodies were often seen in the subsarcolemmal area of muscle fibers and also in the intramuscular myelinated nerve fibers and axon terminals. These findings suggest the presence of mitochondrial myopathy of the EOMs in cases of MELAS and MERRF.

Actin Cytoskeleton↗

PubMed1990

Symptomatic cerebrospinal fluid dissemination of cerebral glioblastoma. Computed tomographic findings in 11 cases.

Computed tomography (CT) findings in eleven patients with symptomatic cerebrospinal fluid (CSF) dissemination from cerebral glioblastoma were analyzed and, in seven cases subsequently autopsied, they were compared with histological observations. Each patient had multiple CT abnormalities including periventricular enhancement (5/11), subarachnoid enhancement (10/11) and progressive hydrocephalus (7/9) by cranial CT, and small filling defects with or without block (5/5) by CT myelography. The areas that showed periventricular or subarachnoid enhancement on CT were confirmed to have macroscopically detectable seeding at autopsy. On the other hand, microscopic deposits were more widely distributed than the enhancement suggested, and were hardly visualized on CT. In association with subarachnoid seeding, we found low-density lesions on CT which had resulted from ischemia or reinvasion of adjacent structures by disseminated glioblastoma and resulting parenchymal edema. By cranial CT, subarachnoid enhancement seems to be a very reliable sign of CSF seeding, whereas periventricular enhancement due to CSF metastases should be carefully distinguished from that due to periventricular tumor infiltration. CT myelography is capable of revealing minute metastatic spinal deposits and may be helpful for ruling out spinal seeding as well as its precise evaluation.

Adult↗

PubMed1990

Focal pachygyria with unusual vascular anomaly.

A case of focal pachygyria with an unusual vascular anomaly is reported. Preoperative magnetic resonance imaging demonstrated few and broad gyri, and an abnormally thickened cortex of the right frontal lobe. In addition, T2-weighted imaging showed a high intensity lesion beneath the thickened cortex. In the pachygyric cortex, the peripheral portions of the arteries were tortuous and irregularly dilated, and prominent deep medullary veins were found draining into the subependymal veins. Histological examination revealed a decreased number of neurons with no tendency towards lamination, and degenerative changes with gliosis in the white matter. These findings suggest that the etiology of this anomaly may be gradual perfusion failure restricted to the territory of the anomalous vessels through the period of neuronal migration to the post-migratory, perinatal stage.

Cerebral Angiography↗

PubMed1990

Demyelination and remyelination in spinal cord lesions of human lymphotropic virus type I-associated myelopathy.

We describe postmortem findings in a patient with human T lymphotropic virus type I (HTLV-I)-associated myelopathy (HAM). The patient developed the disease 8 years after blood transfusion and showed good response to corticosteroid treatment but died of cardiac failure. Histologically, chronic, mild meningoencephalomyelitis was noted predominantly involving the bilateral lateral and anterior columns of the middle to lower thoracic segments. The spinal cord lesions showed obvious loss of myelinated nerve fibers and fibrillary gliosis with minimal inflammatory cell infiltration. Electron microscopy of the lesion revealed disintegration of the myelin sheaths, regular separation of the minor dense line of the myelin sheaths, and completely demyelinated axons. In addition, remyelinated fibers with thin central myelin sheaths and disproportionately large axons were seen frequently. These findings indicate that primary demyelination and remyelination by oligodendrocytes occur in the spinal cord lesions of HAM.

Aged↗