Biomedical subjects
Publications and source records attributed to J G Chi.
Subependymoma is a rare, benign intraventricular tumour and a case of septum pellucidum origin examined with CT and MR is reported. Well demarcated non-enhancing mass with multiple small intratumoral cysts is demonstrated on CT and MR images. The differential diagnosis from ependymoma has some therapeutic implications but may not be possible by CT or MRI.
Congenital obstruction of the pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veins and pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which were accompanied by other cardiac anomalies. Right pulmonary veins were involved in all seven cases; one case was bilateral. Pulmonary veins were occluded totally in five and partially in three lungs. Diagnostic pulmonary catheterization and angiography were performed. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural changes, which were quite specific, whereas pulmonary venous dilatation was the dominant finding in partial obstruction cases. Pulmonary perfusion scan (n = 3) showed total perfusion defects in the cases with total occlusion of pulmonary veins. Magnetic resonance (MR) imaging (n = 2) demonstrated totally occluded pulmonary veins at the venoatrial junction in two lungs and membranous focal obstruction in one lung. Two children underwent pneumonectomy and had the diagnosis histologically confirmed. Although catheterization and angiography are essential for the diagnosis of pulmonary vein obstruction, MR imaging is a useful adjunct.
We report a 5-year-old boy with endodermal sinus tumour associated with benign cystic teratoma of the common bile duct (CBD). To our knowledge, there has been one case of teratoma of the CBD in the English literature with no morphological or radiological description. Our case presented a lobulated polypoid mass obstructing the distal CBD on sonography and computed tomography, which resembled the botryoid masses of rhabdomyosarcoma.
Aspergillosis of the central nervous system is a rare disease, especially if the patient's immune system is not compromised. The authors report three cases of cerebral aspergillosis in the immunocompetent state: a rhinocerebral form in a diabetic patient, a direct extension from chronic Aspergillus otitis media, and a postoperative Aspergillus brain abscess after brain tumor surgery. In spite of the poor prognosis of cerebral aspergillosis, two of the patients survived. The pathogenesis, predisposing factors, radiologic findings including magnetic resonance image, and the outcome are presented. The pertinent literature of cerebral aspergillosis is also reviewed.
We report a case of eosinophilic cystitis caused by vesical sparganosis. The patient had a history of eating raw frogs and presented with a mass in the bladder associated with peripheral eosinophilia and increased serum antibody titer against sparganum. Pathologically, a characteristic sinuous necrosis representing worm tracks was noted with massive diffuse infiltration of eosinophils in the wall of the bladder. The serum titer of sparganum-specific antibody returned to normal after surgical resection of the lesion. This finding emphasizes the importance of suspecting parasitic origin in cases of eosinophilic cystitis with a history of raw meat ingestion in endemic areas.
Two childhood cases of spinal cord ganglioglioma are described. Both cases showed a remarkable similarity in presentation, radiologically and morphologically. The first case was a 6 1/2-year-old boy who had ganglioglioma involving the entire length of the spinal cord. He has been suffering from a long span of scoliosis and gait disturbance since he was 1 year. The tumor contained multiple syrinx. The second case was a 3-year-old girl with ganglioglioma involving a large portion of the spinal cord from lower medulla to T3. This case also contained syringobulbia and syringomyelia in the multiple levels. She suffered from progressive motor weakness of all extremities. Owing to a good demarcation from normal spinal cord, the tumors could easily be removed by the microsurgical dissection through second stage operation in both cases. Histopathologically and ultrastructurally, the tumors were benign gangliogliomas. The symptoms improved a lot after surgery. Based on our two cases and literature review we confirmed that the spinal cord gangliogliomas are slowly growing tumors occurring in childhood or young adulthood. They often involve usually extensive level or entire length of the spinal cord and may be accompanied by scoliosis and gait disturbance when the tumor presents since infancy or early childhood as in our cases. They also often exhibit a cystic change of the tumor associated with large or small syringomyelia. Nearly total removal is possible due to a good demarcation from the normal spinal cord. Therefore, early diagnosis and surgical management are fundamental for the avoidance of unnecessary delay of surgery and a good result.
Immunostaining of monoclonal antibody (MoAb) of smooth muscle actin in paraffin sections and fluorescence of actin-specific phalloidin in cryostat sections were utilized to demonstrate the myoepithelial cells in prenatal and adult salivary glands of humans. In the early developmental stage (10-18 weeks) MoAb actin was weakly positive in the basal cells of the gland epithelium, and the positivity gradually accentuated at the basal portions of the terminal ducts and acini as the gestational period advanced. In the early intermediate developmental stage (19-24 weeks) the polyhedral myoepithelial cells were arranged in the basal portions of the acini and intercalated ducts. At this stage the myoepithelial cells produced phalloidin-positive spindle cytoplasmic processes. In the late intermediate developmental stage (25-32 weeks) the myoepithelial cells became flattened and formed dendritic processes to surround the acini and intercalated ducts. In the late developmental stage (33-40 weeks) numerous myoepithelial cells with well developed dendritic processes were demonstrable in the acini and intercalated ducts. In conclusion, it was found that the myoepithelial cells began to develop at 15-16 weeks of gestation when the acinar cells were still immature. The primitive myoepithelial cells were polyhedral in shape to form compact basal layer beneath the developing acinar cells during 19-24 weeks of gestation. In late gestational period the myoepithelial cells almost matured like the dendritic ones of adult salivary glands. However, the myoepithelial cells were never demonstrated in the striated and excretory ducts of the fetal salivary glands as opposed to its normal presence in the adult salivary glands. A possible aging process of myoepithelial cells was discussed in accordance with the histogenesis of transformed myoepithelial cells of salivary gland tumors.
Developing human fetal salivary glands of gestational age from 10 to 40 weeks (n = 100) and normal adult glands (n = 10) were examined for immunoreactivity to S-100 protein and its subunits S-100 alpha, S-100 beta, glial fibrillary acidic protein (GFAP) and neuron specific enolase (NSE). In the early intermediate developmental stage (19-32 weeks) some acinar basal cells showed immunoreactivity to S-100 protein which rapidly disappeared in the late developmental stage (33-40 weeks). Adult salivary glands were negative for S-100 protein. The S-100 alpha subunit was strongly positive in the glandular ducts and acini of both fetal and adult glands. The S-100 beta, although present in some acini and ductal cells during the late intermediate developmental stage, was rarely seen in the adult glands. GFAP and NSE was positive at the developing salivary epithelium in the early developmental stage (15-18 weeks). The above findings indicated that the developing salivary epithelia showed transient appearance of the neuronal phenotype during active cytodifferentiation stage of glandular acini and ducts. Therefore, after evaluation of normal developmental and neoplastic transformation of the salivary glands a suggestion that neuronal differentiation of ductal reserve cells is responsible for the production of modified myoepithelial cells in both normal developmental salivary gland and neoplastic transformation is made.
Histopathological changes of the brain were observed in 22 mongrel cats each of which was subjected to intracranial inoculation of 3 or 5 plerocercoids of Spirometra erinacei. The brains were examined at 2 weeks, 1, 3, and 6 months post-inoculation (PI). Grossly, the brain lesion consisted of a mass of worm capsule and/or hemorrhagic tract. Microscopically hypertrophied astrocytes, lymphocytes, plasma cells, RBCs, polymorphonuclear leukocytes, and hemosiderin laden macrophages were found around the worms or tracts in all groups. The number of reactive astrocytes increased mainly in the white matter. In the lesions 3 months PI, the sectioned worms were encircled with fibrous tissue along with numerous RBCs, inflammatory cells, and necrotic debris. Collagenous fibers were also demonstrated. Outside the inflammation, a wide edematous zone developed and numerous neuroglial fibers were also found. The location of the worms or the tracts was random in the brain lobes, but the worm seemed to migrate mainly along the white matter.
Immunoreactivity of lysozyme (LY), lactoferrin (LF), alpha 1-antichymotrypsin (alpha 1-ACT), alpha 1-antitrypsin (alpha 1-AT), keratin proteins KL1, PKK1, K8.12, S-100 protein, MAM-3, MAM-6, and epithelial membrane antigen (EMA) were evaluated in lymphoid and glandular tissues of developing salivary gland of human fetus (gestational age ranging from 17 to 40 wk to investigate the role of lymphoid tissue in developing salivary glands. In a total of 79 cases, lymphoid cell aggregations were noted in parotid (57 cases), submandibular (21 cases) and sublingual (5 cases) glands. Mononuclear cells showing intense activity of LY, alpha 1-ACT and alpha 1-AT were present in the lymphoid aggregation. The glandular ducts embedded in lymphoid tissue were negative to MAM-3, MAM-6, EMA and S-100 protein, but showed positive PKK1 and KL1 reaction during early stages of development, and showed degeneration and effacement upon increase in number and LY activity of the mononuclear cells. The lymphoid aggregations progressively emerged as lymph nodes.
Fourteen magnetic resonance (MR) images of 11 patients with cerebral sparganosis were reviewed retrospectively and compared with 13 computed tomographic (CT) scans of 10 of the patients. All patients underwent both nonenhanced and contrast material-enhanced MR imaging. All white matter degenerations, which were slightly hypointense on T1-weighted images and were hyperintense on T2-weighted images, were seen as areas of low attenuation on CT scans. Better contrast between normal and degenerated brain tissues was seen at MR, and two lesions were seen that were missed at CT. The parasitic granuloma was isointense to brain parenchyma on images obtained with all pulse sequences. On two follow-up MR images, location or shape of the enhancing lesions changed, suggesting migration of the worm. A new finding of hyperintensity on T1-weighted images and hypointensity on T2-weighted images in subcortical areas probably due to petechial hemorrhages was demonstrated in eight patients only at MR. Small calcific foci, which were important diagnostic clues of cerebral sparganosis, were detected in four patients only at MR, while they were seen in eight patients at CT.
OBJECTIVE: Gliomatosis cerebri is a diffuse infiltrative glial neoplasm frequently involving both cerebral hemispheres. Diagnosis and evaluation of its extent with CT are known to be difficult. The purpose of this study was to compare the MR and CT findings in gliomatosis cerebri. MATERIALS AND METHODS: MR images of nine patients were reviewed retrospectively and compared with CT scans. Pathology was determined by open or stereotaxic biopsy. The MR images included sagittal T1-weighted, axial proton density-weighted, and T2-weighted images. Contrast material was administered in seven patients. Unenhanced and enhanced CT scans were obtained in eight patients. RESULTS: On proton density-weighted and T2-weighted MR images, the most common findings were poorly defined bilateral areas of diffuse high signal intensity in the cerebral hemisphere. On T1-weighted images, the lesions were isointense to hypointense compared with normal brain. Enhanced T1-weighted images showed focal parenchymal enhancement in three patients and meningeal enhancement in one. On CT scans, the lesions showed poorly defined areas of subtle low density or isodensity, and appeared much smaller than those on T2-weighted MR images. Enhancement was seen in only one case. The extent of disease was evaluated much better on T2-weighted MR images than on T1-weighted MR images and CT scans. CONCLUSION: In gliomatosis cerebri, MR imaging is more sensitive than CT for detecting lesions and shows the extent of disease better than CT does. Accordingly, MR imaging should be used as a primary imaging study in the evaluation of gliomatosis cerebri.
We observed the changes of the gastrocnemius muscle in relation to the percentage of lengthening of the rabbit's tibia by callotasis. 75 rabbits were separated into 3 lengthening groups, 10, 20, and 30 percent lengthening, respectively. Histopathologic observations, based on the fiber size variation, internalization of the nuclei, degeneration, regeneration, and endomysial fibrosis of muscle fibers, revealed that substantial changes occurred in the latter groups. Histomorphometrically, the decrease in the mean size of Types I and II muscle fibers was observed in all lengthening groups, but there was no significant change in the proportion of the muscle fiber types in any of the lengthening groups.
Fifteen pediatric (age under 16) cases of oligodendroglioma (ODG) were surgically proven from January 1985 to April 1992 at the Division of Pediatric Neurosurgery, Seoul National University Children's Hospital. To observe the proportion of ODG's in primary intracranial tumors, the location of ODG's and the prognostic significance of the histological grading of ODG's in childhood, the 15 cases of pediatric ODG's were retrospectively analyzed. ODG's accounted for 5.6% of pediatric primary intracranial tumors operated on during the same period. Nine tumors were located in the cerebral hemisphere (3 cases each in the frontal, temporal and parietooccipital lobes), 1 in the thalamus, 2 in the pons-medulla, 2 in the cerebellum and 1 in the thoracolumbar spinal cord. Four tumors were anaplastic and an additional case showed positive cerebrospinal fluid (CSF) cytology for tumor cells. All the cases of anaplasia or positive CSF cytology had a poor outcome. All the seven cases of benign ODG's in cerebral hemispheres presented with seizures which were controlled with or without medication after tumor removal.
We present an autopsy case of a two-day-old female infant with a very unusual combination of neuroblastoma in situ in both adrenals and microcephaly. This baby was born to a 28-year-old mother after 38 weeks of gestation, and died of respiratory difficulty 2 days later. At autopsy, the baby weighted 1,840gm, and the brain was extraordinarily small with a weight of 125gm. The gyral pattern was simplified and irregular. Microscopically massive migration defects, pachygyria, micropolygyria, leptomeningeal glioneuronal islands, small corticospinal tract and heterotopic Purkinje cells in the cerebellum were found. In addition, there were medullary nodules in both adrenals. They measured 0.7 x 0.4cm and 0.7 x 0.3cm, respectively. These nodules showed the typical histological features of undifferentiated neuroblastoma. The tumor nodules were confined to the medullary portion and did not extend to the cortex or contiguous structures meeting the criteria of neuroblastoma in situ. Based on these unusual and seemingly unrelated sets of findings, it is suggested that the histogenesis of neuroblastoma in situ could be a part of the generalized dysontogenic process.
Leigh's disease is a rare progressive neurological disorder that is characterized light microscopically by focal spongy necrosis in the brain and electron microscopically by mitochondriopathy. We report an autopsy case of Leigh's disease that showed abnormalities in the liver, kidney and skeletal muscle as well as the central nervous system. The patient was an 18-month-old girl who has carried a diagnosis of cerebral palsy ever since her birth to a 20-year-old mother. The baby was generally hypertonic and mentally retarded. She died of severe metabolic acidosis. Postmortem examination showed growth retardation, fatty liver, fatty kidney and soft brain. Brain section showed multifocal softenings in the brainstem, basal ganglia and periventricular areas. Microscopically increased capillaries with endothelial proliferation, vacuolar degeneration and mild gliosis were seen in the brain. The axons were relatively preserved. Liver and kidneys showed microvesicular fatty change. Myofiber degeneration of the skeletal muscle was also noted. Electron microscopic examination showed markedly increased mitochondria in the parenchymal cells of the brain, liver and kidney. The mitochondria showed round to ovoid ballooned appearance including electron-dense core-like structures and pseudoinclusions of glycogen granules.
We describe a case of the juvenile form of Pompe's disease that presented as primary alveolar hypoventilation due to respiratory muscle involvement. This 17-year-old girl had been asymptomatic until this admission, although she had a delayed puberty. Arterial blood gas analysis, pulmonary function test as well as physical findings were compatible with chronic alveolar hypoventilation syndrome. Since she had lower extremity muscle weakness and pseudomyotonic discharge on electromyography a muscle biopsy was done, which revealed glycogen storage disease. The patient was managed successfully with nasal intermittent positive pressure ventilation.
The acute peripheral neuropathy as one of hypereosinophilic syndrome is known to be a rare disorder. The authors experienced a dramatic case with acute peripheral neuropathy, hypereosinophilia in peripheral blood, and the positive anti-GM1 antibodies. The serum protein electrophoresis showed a diffusely increased gamma-globulin region and the polyclonal gammopathy was found by the immunoelectropheresis. There was no evidence of inflammatory myopathy in vastus lateralis muscle. The sural nerve biopsy was compatible with vascular neuropathy, as there were a few myelin digestion chambers, mild perineuronal fibrosis, and perivascular lymphoplasmocytic infiltration with focal organizing thrombosis. The clinical response to prednisone therapy was excellent.