[Paraneoplastic cerebellar degeneration].
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Animals↗
Biomedical subjects
E Ohama
Publications and source records attributed to E Ohama.
[Neuropathology of syringomyelia].
Eighteen autopsy cases of syringomyelia were studied neuropathologically. In 5 cases associated with Chiari type I malformation, the syrinx was irregular in shape and communicated with the subarachnoid space at the entry zone of the posterior nerve roots. The central canal above the level of the syrinx was patent in 2 but closed in 3 out of 5 cases. In cases associated with Chiari type I malformation, communication between the syrinx and the subarachnoid space was considered to play an important role in the pathogenesis of syringomyelia. In 6 cases associated with Chiari type II malformation, the syrinx central canal, and the central canal was patent from the 4th ventricle to the syrinx in all cases. In these cases, direct continuity between the 4th ventricle and the syrinx was essential for the development of the syrinx. In cases associated with posterior fossa or spinal canal tumors, the local circulatory disturbance and/or edema due to the tumors were thought to cause the syrinx.
Adolescent↗
Brain tumor: immunohistochemical studies on the stress-response proteins, p53 protein and proliferating cell nuclear antigen.
This retrospective immunohistochemical study compares the expression of five stress-response (heat-shock) proteins (srp's) [srp 90, srp 72, srp 27, alpha B-crystallin and ubiquitin], p53 protein and proliferating cell nuclear antigen (PCNA) in 118 primary brain tumors and 21 carcinoma metastases to the central nervous system. Serial sections of formalin-fixed, paraffin-embedded tissues were used. Most astrocytomas (9/13), ependymomas (5/5), glioblastoma multiforme (GBM) (11/12), schwannomas (19/21), meningiomas (22/23) and breast carcinoma metastases (Br-Mt) (9/10), and some medulloblastomas (5/15), primitive neuroectodermal tumors (PNETs) (5/11), pituitary adenomas (4/7) and lung carcinoma metastases (6/11), but none of 10 oligodendrogliomas had tumor cells that expressed one or more (up to five) srp's. The percentage of tumors with p53-positive cells was variable; the proportion was highest among srp-expressing GBMs (mean: 16.1%) and Br-Mts (mean: 15.3%). The mean PCNA-labeling index (LI) also varied, ranging from 1.2% in the group of pituitary adenomas to 24.5% in Br-Mts, with GBMs (20.4%) and medulloblastomas (18.4%) approaching the latter value. PCNA-LI was higher in the astrocytomas, GBMs, medulloblastomas and PNETs that expressed srp's than in those did not. A high proportion of p53-positive cells (31.3 to 59.0%) and the highest PCNA-LIs (41.0 to 49.0%) were seen in two GBMs and one Br-Mt that expressed all five srp's. We conclude that primary and metastatic tumors of the brain produce one or more stress-related proteins, and that a variable proportion of the tumor cells have immunohistochemically-detectable p53, the expression of which may depend, at least in part, on the growth potential of a given tumor.
Brain Neoplasms↗
Human inositol 1,4,5-trisphosphate type-1 receptor, InsP3R1: structure, function, regulation of expression and chromosomal localization.
We have isolated cDNA clones encoding an inositol 1,4,5-trisphosphate receptor type 1 (InsP3R1) from human uteri and a leukaemic cell line, HL-60. Northern-blot analysis showed that approx. 10 kb of InsP3R1 mRNA is expressed in human uteri, oviducts and HL-60 cells. The predicted amino acid sequence of human InsP3R1 (2695 amino acids) has 99% identity with that of the mouse SI-/SII- splicing counterpart. Western-blot analysis with anti-(mouse InsP3R1) antibodies showed that InsP3R1 protein of human uteri and oviducts of approx 220 kDa is immunostained. Northern-blot analysis of HL-60 cell differentiation along the neutrophilic lineage induced by retinoic acid or dimethylsulphoxide showed an accompanying enhanced expression of InsP3R1 mRNA. Immunohistochemical analysis of the cerebella of spinocerebellar degeneration patients showed a variable loss of Purkinje cells with an altered pattern of immunostaining. The InsP3R1 gene (Insp3r1) was localized to the 3P25-26 region of human chromosome 3. The data presented here clearly show that InsP3R1 exists widely in human tissues and may play critical roles in various kinds of cellular functions.
Adult↗
Familial juvenile parkinsonism: clinical and pathologic study in a family.
We describe a family with juvenile-onset parkinsonism, which improved following sleep. Four of the five siblings in this family developed a similar onset of parkinsonism at an early age, and the parents were first cousins. In one of the siblings, a 67-year-old woman, pathologic changes at autopsy were confined to the substantia nigra pars compacta (SNPC) and locus ceruleus. The SNPC revealed obvious neuronal loss and gliosis in the medial and ventrolateral regions. In the remainder of the SNPC and the locus ceruleus, the population of neurons was reduced and there was low melanin content in most of the neurons but no detectable gliosis or extraneuronal free melanin pigment suggestive of a neurodegenerative process. There were no Lewy bodies. The entire pathologic picture was different from that of Lewy body Parkinson's disease.
Adult↗
Association of IgG anti-GD1a antibody with severe Guillain-Barré syndrome.
We earlier reported cases of 2 patients with severe acute Guillain-Barré syndrome (GBS) associated with high-IgG anti-GD1a antibody titer. We now have investigated the autoantibody against GD1a or GM1 in 37 GBS patients using the enzyme-linked immunosorbent assay and have found a statistically significant association between IgG anti-GD1a antibody and the severity of the disease (need of a respirator for more than 1 month and a poor functional prognosis 3 months after neurologic onset). An autopsy which showed severe GBS associated with IgG anti-GD1a antibody produced the following findings: (1) severe axonal degeneration and segmental demyelination of peripheral nerves; (2) lymphocytic infiltration; and (3) marked central chromatolysis of the lower motoneurons.
Adolescent↗
Comparative study on the expression of stress-response protein (srp) 72, srp 27, alpha B-crystallin and ubiquitin in brain tumours. An immunohistochemical investigation.
This immunohistochemical study compares the expression of stress-response (heat-shock) protein (srp) 72, srp 27, alpha B-crystallin and ubiquitin in 86 primary human brain tumours and 21 carcinoma metastases to the central nervous system. Normal brain tissues were included for control purposes. Serial sections of formalin-fixed, paraffin-embedded tissues were used. Most meningiomas (17/23), glioblastomas (11/12) and breast carcinoma metastases (9/10) and some astrocytomas (7/13), pituitary tumours (4/9) and lung cancer metastases (5/11) had tumour cells that reacted with one or more of the antibodies used. Around 43% of the meningiomas and 25% of the glioblastomas expressed srp 72 only. Sole expression of srp 27, alpha B-crystallin or ubiquitin was seen in several tumours. Some meningiomas (3/23) and breast cancer metastases (4/10) co-expressed srp 72 and srp 27, and 1/3 of the glioblastomas co-expressed srp 27 and alpha B-crystallin. We conclude that primary and metastatic tumours of the brain produce stress-related proteins and that certain tumours concurrently express two or more srp's.
Brain Neoplasms↗
Immunohistochemical studies on choroidal epithelial cyst and neuroepithelial cyst: in comparison with normal choroid plexus and ependyma.
Immunohistochemical evaluations with antibodies against S-100 protein, glial fibrillary acidic protein, cytokeratin and prealbumin were applied to 2 intracranial cysts of choroidal epithelial and neuroepithelial origin, and to the normal choroid plexus and ependyma. All specimens showed various immunoreactivities with the 4 antigens: especially, prealbumin-positive cells were detected in normal choroid plexus, part of normal ependyma, and part of choroidal epithelial cyst. The epithelium of neuroepithelial cysts presumably has multipotentials in cellular differentiation.
Brain Diseases↗
Malignant nerve sheath tumor of the jugular foramen followed by meningeal dissemination in a patient with neurofibromatosis.
A 20-year-old female with von Recklinghausen's disease became aware of hoarseness and dysphagia with a right neck mass, and received subtotal resection of a jugular foramen neurofibroma. One year later the jugular foramen tumor recurred and pathologic examination at excision showed a malignant transformation. Despite postoperative chemotherapy and radiotherapy, it recurred soon and followed the metastatic cerebral tumor by meningeal dissemination. Malignant nerve sheath tumors of the jugular foramen are very rare and the clinical course and pathological pictures of this tumor are reviewed.
Adult↗
[Multiple myeloma presenting with a solitary cranial tumor with subdural extension: a case report].
We report a case of multiple myeloma presenting with a solitary cranial tumor in the frontal region, extending from subcutaneous tissue to subdural space. To our knowledge, invasion of a tumor beyond the dura mater has never been described in case of multiple myeloma presenting with a solitary calvarial tumor. In the present case, the subdural extension was clearly visualized by several diagnostic means. A 53-year-old female patient visited the clinic of our University Hospital, complaining of a left frontal mass in May 1991. The mass grew rapidly, and she was hospitalized in June. On admission, neurological examination showed nothing abnormal. Laboratory studies showed normocytic normochromic anemia. Protein electrophoresis disclosed hypergammaglobulinemia with S-spike, and serum paraprotein was specific to IgA with lambda light chains by immunoelectrophoresis. Urinary Bence-Jones protein was not detected. An osteolytic lesion visualized in the frontal bone on plain skull radiographs showed destruction of the frontal bone, and an enhanced mass extending from the epidural to subcutaneous space was shown by computed tomography. The mass had compressed the frontal lobe. T1-weighted magnetic resonance images of the area showed isointensity signals, homogeneously enhanced with gadolinium-diethylenetriamide pentaacetic acid. Subdural extension in the deep area of the tumor was suggested. At operation, we confirmed infiltration of the tumor from the dura mater into the subdural space. Postoperative biopsy of bone marrow of the iliac bone demonstrated myeloma cells. The tumor was histologically diagnosed as plasmacytoma. The patient was highly resistant to postoperative combined therapy, and started on a fatal course leading to respiratory insufficiency caused by interstitial pneumonia in April 1992.(ABSTRACT TRUNCATED AT 250 WORDS)
Female↗
Lewy bodies in the visceral autonomic nervous system in Parkinson's disease.
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Adrenal Medulla↗
Ultrastructural and immunohistochemical studies on ballooned cortical neurons in Creutzfeldt-Jakob disease: expression of alpha B-crystallin, ubiquitin and stress-response protein 27.
This report concerns ultrastructural and immunohistochemical studies on ballooned neurons of ten patients with Creutzfeldt-Jakob disease (CJD). While abundant ballooned neurons and severe white matter degeneration was seen in six Japanese cases, only occasional ballooned neurons and no white matter degeneration was observed in four cases from the files of Montefiore Medical Center. Ultrastructurally, the ballooned neurons contained granule-coated fibrils of 25 to 40 nm in width and 10-nm neurofilaments. The immunohistochemical studies revealed that most ballooned neurons expressed alpha B-crystallin, with deposits of reaction products observed in the cytoplasm. A similar intracellular staining pattern was also seen with the antibody to phosphorylated neurofilament proteins (pNFP). Although the proportion of stained ballooned neurons was less, a positive reaction was also observed with antibodies against ubiquitin, stress-response protein 27 (srp 27) and synaptophysin, but not with an antibody to srp 72. Our findings suggest that expression of pNFP and synaptophysin by ballooned neurons may reflect axonal impairment and that the presence of alpha B-crystallin, srp 27 and ubiquitin may be related to the degenerative processes that neurons undergo in CJD.
Aged↗
Stress-response (heat-shock) protein 72 expression in tumors of the central nervous system: an immunohistochemical investigation.
This report deals with the expression of stress-response (heat-shock) protein 72 (srp 72) in a series of 95 primary human brain tumors and 21 carcinoma metastases to the central nervous system (CNS). Immunohistochemical procedures were employed; cells of the human cervical cancer line HeLa S3 were used as positive controls. The protein was detected in 14/22 meningiomas and in 6/13 glioblastomas. Tumor cells expressing srp 72 were also found in 4/17 astrocytomas, 2/9 pituitary tumors, 2/14 primitive neuroectodermal tumors and 1/10 medulloblastomas. Whereas the majority (8/10) of the breast carcinoma metastases had tumor cells that expressed srp 72, only 2/11 lung tumor metastases were positively stained. These results document srp 72 expression by a variety of primary and metastatic tumors of the CNS.
Antibodies, Monoclonal↗
Increased iron content in the putamen of patients with striatonigral degeneration.
We measured the total content of iron, copper, zinc and manganese in the putamen of four patients with striatonigral degeneration (SND) and age- and gender-matched normal controls. The iron content in the SND patients was five times greater than in the controls. Electron microscopic histochemistry revealed the iron reaction products in the pigments showing a triphasic pattern of coarse, electron-dense globules, fine granular and fibrillary materials, and lamellated structures. These findings suggest that increased iron deposition may be related to pigment formation in the putamen of SND.
Aged↗
Does methylmercury intoxication induce arteriosclerosis in humans? A pathological investigation of 22 autopsy cases in Niigata, Japan.
In order to clarify whether or not arterio- and/or arteriolosclerosis is induced or exacerbated in patients with methylmercury (Me-Hg) intoxication, the pathological features of arteries and arterioles in specific areas in 22 patients and 36 control subjects were examined qualitatively and quantitatively. Vessels investigated were: (1) small arteries and arterioles in the subarachnoid space and cortex of the postcentral gyrus, transverse temporal gyrus, first visual area and cerebellar vermis, as well as the myocardium and renal cortex; (2) the lateral striate artery; (3) the internal carotid, anterior, middle and posterior cerebral, basilar and vertebral arteries, as well as the coronary and renal arteries; and (4) the aorta. The arteriosclerotic changes observed in the patients with Me-Hg intoxication were indistinguishable both qualitatively and quantitatively from those of controls. The results indicate that Me-Hg intoxication does not induce or exacerbate sclerotic changes in arteries and arterioles. Thus, the peculiar neurological symptoms and neuropathological features of Me-Hg intoxication are thought to be induced not by ischemia but by selective primary degeneration of the neurons in specific regions.
Adult↗
Clarke's column in sporadic amyotrophic lateral sclerosis.
Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7 of the 12 non-respirator-supported ALS patients and in 3 of the 6 respirator-supported patients. The number of spheroids was significantly higher in the non-respirator-supported patients (P < 0.01) than in the control subjects; however, the number in the respirator-supported patients was about equal to that in the controls. The number of neurons in Clarke's column in the non-respirator-supported ALS patients was not reduced, but in the respirator-supported patients they tended to disappear with time after respiratory support. These findings suggest that Clarke's column neurons are also involved primarily in the disease process in sporadic ALS. However, they may begin to disappear only after the patients require respiratory support.
Adult↗
Primary leptomeningeal glioma: ultrastructural and laminin immunohistochemical studies.
We studied a case of primary leptomeningeal glioma (PLG) on the left parietal lobe of a 74-year-old woman and compared the tissue with heterotopic glial tissue from another case. The PLG tumor consisted of spindle-shaped cells with marked nuclear atypism, which tended to be arranged in a fascicular pattern, and the majority of its cells were positive for glial fibrillary acidic protein. Ultrastructural examination demonstrated that most of the tumor cells contained intermediate filaments and often junctional complexes were present on their plasma membranes. Frequently, basal lamina-like structures surrounding the tumor cell surfaces were observed. Laminin immunohistochemistry clearly demonstrated a fine network of linear positive staining around the cytoplasm and processes of the tumor cells. The ultrastructure of the heterotopic glial tissue consisted of many astrocytes partially surrounded by basal lamina. These findings strongly suggest that PLG is a distinct tumor, which arises from the heterotopic astrocytes within the subarachnoid space.
Aged↗
Problems related to dorsal root entry zone lesions.
Several clinical problems related to the dorsal root entry zone lesions (DREZLs) in 15 patients with chronic pain are presented and discussed in terms of ratings of pain relief following surgery, development of sensory or motor weakness and postmortem histologies. Subjective pain relief exceeding 70% was achieved at around 2 weeks after the operations in most patients (13/15), and then decreased in some to 30 from 70% in the follow-up observations. Our new "objective" pain relief score was tested in these patients. A significant positive correlation between subjective pain relief and our objective pain relief scale was found, but some discrepancies between them were also found during the follow-up. Sensory loss, motor weakness, paraesthesia and a new pain were found as complications in 12, 7, 4 and 6 patients, respectively. Postmortem histological findings of the spinal cord in two patients with systemic lupus erythematosus and uterine cancer, who received bilateral DREZLs twice and bilateral DREZLs plus commissural myelotomy, respectively, indicate that care should be taken to avoid extension of the coagulation beyond the dorsal horn.
Adult↗