Recovery and Adsorption Rate of Murine Norovirus Using NanoCeram(R) Filters

This study investigated the recovery and absorption rates of murine norovirus, a surrogate for human norovirus, by using NanoCeram(R) filters which served as a tool for recovering viruses. In the study, two types of NanoCeram(R) filters were employed: one was a cartridge type and the other was a disc type (phi 47 mm) whose surface area is 75 times smaller than the cartridge type. The analytical method was the real-time reverse transcription-polymerase chain reaction (RT-PCR). The study found that the average recovery rates of the cartridge type and the disc type were 30.9% and 29.5% respectively. Since these two rates were very close to each other, the adsorption rate of the cartridge type could be predicted with the disc type. Analyzing recovery and absorption rates of the disc type based on different filtered volumes showed that when the volume increased from 0.5 L to 20 L, the average recovery rate rose from 14.78% to 30.41 %, while the average absorption rate dropped from 56.33% to 10.48%. The increase in turbidity from less than 1 NTU to less than 3 NTU raised the average recovery rate from 47.23% to 82.84%.

The Clinical and Pathological Characteristics of Polymyositis Using ENMC Diagnostic Criteria / 대한류마티스학회지

OBJECTIVE: Polymyositis (PM) has known to be the most common type of idiopathic inflammatory myopathy (IIM). However, recent immunopathological studies demonstrated that PM was overdiagnosed previously due to suboptimal classification system. Using newly proposed classification system, we investigated the frequency, clinical and pathological characteristics of PM. METHODS: Among the patients diagnosed as IIM during past 6 years, we classified a 'definite' or 'probable PM' using the European Neuromuscular Center (ENMC) diagnostic criteria. The findings of clinical, laboratory and pathological findings were analyzed. Response to treatment was assessed at 6 months after treatment. RESULTS: Of total 97 cases with IIM, twenty-three cases (24%) were satisfactory to the diagnostic criteria for PM (definite=5 and probable=18). Most cases were young adults, and female predominance was found. All cases showed proximal muscle weakness, and about two-thirds of patients showed extramuscular manifestation. One (4%) had breast cancer, and accompanying connective tissue disorders (CTDs) were found in 3 cases (13%), two of which had systemic sclerosis. Interstitial pneumonia was found in one case (4%). All cases showed marked elevation of serum creatine kinase level. On muscle biopsy, there were endomysial mononuclear cell infiltrations in all cases. Three-fourths of patients responded to immunosuppressant therapy (74%). CONCLUSION: Using ENMC criteria, the frequency of PM was lower than that had been reported previously. The results of clinical characteristics, response to therapy and clinical outcome were similar to the previous reports. However, association of malignancy or CTDs was low in PM.

Hemispherotomy in Childhood Medically Refractory Epilepsy / 대한소아신경학회지

PURPOSE: Surgical approach with medically intractable epilepsy has increased with recent advances in neurodiagnostic modalities. This study was designed to review the clinical manifestations and surgical outcome of patients who received hemipherotomy. METHODS: We performed a retrospective study of 19 patients under 15 years old who had medically intractable epilepsy and underwent hemipherotomy at Severance Children's Hospital between 2003 and 2008. RESULTS: Eleven patients(57.9%) showed Engel Class I outcome, 6 patients(31.6%) showed Engel Class II and 1 patient(5.3%) showed Engel Class III outcomes. Preoperative evaluation revealed concordance in all 19 patients(100%) on MRI, 17 patients(89.5%) on long-term EEG monitoring, 17 patients(89.5%) on PET and 13 patients(68.4%) on interictal SPECT. Malformation of cortical development was the most common etiology(4 patients, 21.5%). Other etiologies included hemimegalencephaly, Sturge-Weber syndrome, hemorrhage, infarction and schizencephaly(2 patients each, 10.5%). The most common pathologic finding was cortical dysplasia(9 patients, 49.4%). Other pathological findings included microdysgenesis(2 patients, 10.5%), gliosis(4 patients, 21.1%), leukomalacia, Lafora body and calcification in cortex(1 patient each, 5.3%). CONCLUSION: Surgical outcome of hemipherotomy in infant and children were favorable. Better evaluation through utilization of advanced neurodiagnostic modalities including EEG monitoring and neuroradiologic studies will greatly improve postsurgical outcome of hemipherotomy.

Pleomorphic Variant of Pineocytoma: A Case Report

We herein present a rare case of pineocytoma in a 23-year-old female exhibiting distinct histomorphological features. The tumor contained highly pleomorphic, often multinucleated giant cells in the background of otherwise benign pineocytomatous architecture, which at first led to an erroneous diagnosis of a high grade malignancy. However, the worrisome histological findings turned out to be constituents of a distinct subtype of pineocytoma previously described as pleomorphic variant of pineocytoma. Although it is rare, pathologists should be aware of this entity since the tumor takes on a benign clinical course like any other classic pineocytomas.

Functional Parathyroid Cyst

Parathyroid cysts are uncommon lesions of the neck and superior mediastinum, rarely large enough to be of clinical significance. They frequently present as a clinical diagnostic problem. They may mimic solitary thyroid nodule. They can be classified as either functioning or non-functioning depending on whether or not they are associated with hypercalcemia. Non-functioning or "true" cysts are those associated with normocalcemia (85%), whereas functioning or "pseudocysts" are those associated with hypercalcemia (15%). The presence of a high content of intact parathyroid hormone (iPTH) in the cystic fluid on fine-needle aspiration (FNA), in conjunction with elevated serum levels of calcium and iPTH is diagnostic of a functional parathyroid cyst, which usually requires surgical removal. We report herein two cases of such a functional parathyroid cyst and present a review of the literature.

Posterior Fossa Decompression for Hypertensive Cerebellar Encephalopathy: Case Report / 대한뇌혈관외과학회지

Hypertensive encephalopathy is a medical emergency that is usually presented with manifestating headache, nausea, and visual disturbance. The disease predominantly involves the parieto-occipital white matter. Although cerebellar lesion accompanying hydrocephalus has been occasionally described in the literature, medical management has been sufficient for relieving the posterior fossa hypertension. In the present case, we report a patient who was diagnosed with primary hypertensive cerebellar encephalopathy refractory to medical management and who was successfully treated by surgical decompression of the posterior fossa. A pathologic microscopic inspection of the tissue specimen confirmed hypertensive vascular change.

Primary Intracranial Hemangioendothelioma

Hemangioendothelioma is a recently described uncommon vascular neoplasm, characterized by epithelioid tumor cells and borderline biologic behavior. Its histological features and clinical behavior are intermediate between those of hemangioma and angiosarcoma. Its four principal sites of occurrence are the soft tissue, liver, lung and bone. Its intracranial occurrence is extreamly rare. We report a case of primary cerebral hemangioendothelioma in a 29-year-old male patient

A Case of Congenital Myotonic Dystrophy

Congenital myotonic dystrophy is an almost always maternally-inherited autosomal dominant multisystem disorder of variable clinical expressions characterized by hypotonia and frequent respiratory distress at birth. The muscle weakness may be so severe, it may lead to death in the newborn period. We report a case of congenital myotonic dystrophy in a 34 weeks of gestational age premature infant born to a mother with polyhydramnios, presenting with hypotonia, respiratory insufficiency, feeding difficulties and arthrogryposis. A brief review of literature is given.

A Case of Congenital Myotonic Dystrophy

Congenital myotonic dystrophy is an almost always maternally-inherited autosomal dominant multisystem disorder of variable clinical expressions characterized by hypotonia and frequent respiratory distress at birth. The muscle weakness may be so severe, it may lead to death in the newborn period. We report a case of congenital myotonic dystrophy in a 34 weeks of gestational age premature infant born to a mother with polyhydramnios, presenting with hypotonia, respiratory insufficiency, feeding difficulties and arthrogryposis. A brief review of literature is given.

Arthroscopic Management and Manipulation of Chronic Idiopathic Adhesive Capsulitis of the Shoulder / 대한정형외과학회잡지

PURPOSE: To describe the clinical efficacy of arthroscopic management followed by manipulation under anesthesia in chronic idiopathic adhesive capsulitis of the shoulder. MATERIALS AND METHODS: We evaluated nineteen patients who had a mean follow-up period of 3 years 1 month using the UCLA shoulder rating score. Arthroscopic management included an arthroscopic examination and debridement of the glenohumeral joint and the subacromial space, as well as the release of the rotator interval, the capsule, the glenohumeral ligaments and the inferior recess. Manipulation was performed after the arthroscopic procedures. RESULTS: Thirteen patients (68%) were completely free from pain. The forward elevation was im-proved from 97 degrees to 175 degrees, the abduction from 69 degrees to 170 degrees, the external rotation from 8 degrees to 51 degrees, and the internal rotation from the range of the buttock and the 3rd-4th lumbar spinous process to the level of the 7th-9th thoracic spinous process. The average UCLA shoulder rating score was 9 points before the operation and 34 points after the operation. CONCLUSION: Our clinical findings suggest that arthroscopic management followed by manipulation is an effective method for the treatment of chronic idiopathic adhesive capsulitis of the shoulder.

Cervical Ganglioglioma

Gangliogliomas are benign tumors, composed of neoplastic astrocytes and nerve cells. They are rare, account for 0.4-6.25% of all primary central nervous system neoplasms. Gangliogliomas affect predominantly infants or young adults. Gangliogliomas are preferentially encountered supratentorially, predominantly affecting the temporal lobe. Spinal gangliogliomas affect predominantly the cervical spinal cord. We report a case of cervical ganglioglioma which was successfully removed surgically, with a review of literature.

Apoptosis of Surrounding Neurons by Brain tumor

BACKGROUND: As brain tumor cells are immunologically active, they release various factors like a cytokine, growth factor and express a death domain on their surfaces. Accordingly they support proliferation, vascularity, invasiveness and maintain immune privileged sites. However, the relationship between tumor cells and surrounding neuron cells have been rarely reported in tumor patients with epilepsy that inhibitory neuron cells have been lost around peritumoral sites. This study was designed to address that tumor cells directly damage neuron cells. METHODS: Using LDH assay and special stain, we investigated whether or not cultured supernatants of astrocytoma cells induce the damage of neuron cells. RESULTS: The neuron cells were killed by tumor cells supernatant and increased by pretreatment of neuron cells supernatant and lysates. Protein extracted tumor cells supernatant also damage neuron cells. It was proved by Annexin-PI stain and DNA fragmentation that neuronal death by tumor cells was apoptosis. The more malignant tumor cells, the more neuronal death was induced and the more their cytokines were expressed. In comparison with various cytokine expressions in tumor cells, it can be assumed that the released protein from tumor cells was associated with TNF (tumor necrosis factor)-alpha. CONCLUSIONS: Brain tumor cells are active processing cells that they recognize surrounding normal neuron cells, release death factors and induce apoptosis of neuron cells. Released death factors are related toTNF-alpha. (J Korean Neurol Assoc 19(3):266~277, 2001)

Serum Neuron Specific Enolase as Early Prognostic Marker of Neurologic Outcome after Cardiac Arrest

BACKGROUND: The purpose of this study was to determine that the assessment of serum neuron specific enolase(NSE) could provide a reliable early predictor of neurologic outcome after cardiac arrest. METHODS: Prospective, observational study was performed from April 1996 to March 1998 at a university teaching hospital ED. Serum NSE concentrations were analysed twice at 24 and 48 hours after return of spontaneous circulation(ROSC). Neurologic outcome was categorized using cerebral performance category(CPC). RESULTS: Twenty-nine patients(16 were men) were enrolled during the study period. The mean age was 50.8 years. Nine(31%) of them showed good outcome defied as CPC 1-3, and 20(69%) patients showed bad outcome defied as CPC 4-5. In the good outcome group, the serum NSE was revealed 33.8+/-9.3 ng/ml at 24 hours, 34.0+/-4.73 ng/ml at 48 hours. While in the bad outcome group, it was 99.5+/-11.7 ng/ml and 114.6+/-15.8 ng/ml. The NSE at 48hr after ROSC was more prescise than that of 24hr. When the cutoff value of 50 ng/ml at 48 hr, the sensitivity was 82%, and specificity was 93%. CONCLUSION: This study suggest that the serum NSE may represent a valuable, noninvasive, and useful clinical tool for prediction of neurologic outcome after cardiac arrest.

MRI follow-up study and 1H-MR spectroscopic finding in a patient with Kearns-Sayre syndrome

Serial Brain MRI was performed on a seventeen-year-old girl with Kearns-Sayre syndrome. At the age of 11, she complained bilateral ptosis. Two years later, bilateral blepharoplasty was done and brain MRI was taken. T2-weighted MRI sequence showed high signal intensity areas in the brainstem, thalamus and white matter of the cerebrum and cerebellum bilaterally. Four years later, chronic progressive external ophthalmoplegia developed and serial MRI and proton MRS were taken. Follow-up MRI showed similar but slightly progressed findings compared with previous films. The proton MR spectroscopic imaging demonstrated focal localization of abnormally increased lactate content in the involved area of the brain.

None uniformity of muscle pathology in Becker muscular dystrophy

Incontinentia pigmenti (IP) is a rare hereditary neurocutaneous syndrome characterized by typical linear hyperpigmentationed skin lesions, often associated with central nervous system (CNS) involvement, dysplasia in dental and skeletal system, and ocular abnormalities. Thirty to fifty percent of the patients suffer CNS complications such as mental retardation, seizures, spastic paralysis, microcephaly, and cerebellar ataxia. We experienced a case of incontinentia pigmenti in three-month-old female patient who had characteristic linear hyperpigmented skin lesion on both her thighs and partial seizure with secondary generalization. She had family history of typical skin lesions on her maternal relatives. She showed abnormal findings on EEG as well as multiple necrotic lesions on brain MRI. Confirm diagnosis of incontinentia pigmenti was made by skin biopsy.

Histopathologic Re-evaluation of Thymoma with Immunonhistochemical Study for bcl-2 and MIC-2 Protein

We reviewed 86 thymic epithelial tumors and reclassified them according to the Kirchner and Muller- Hermelink classification. They were subtyped as medullary, mixed, predominantly cortical (organoid), cortical, well differentiated thymic carcinoma, and poorly differentiated thymic carcinoma. The frequency of each subtype was determined and histologic findings were related to stage and myasthenia gravis. Immunohistochemical stains for bcl-2 protein as a marker for medullary thymocytes and MIC-2 protein as a marker for cortical thymocytes were performed in each case. The stages and association of myasthenia gravis was significantly different in each subtypes. The results of this study demonstrate that this histogenetic classification is clinically applicable. The bcl-2 protein was specifically demonstrated in lymphocytes within areas of medullary differentiation and MIC-2 protein in cortical differentiation. The expression of bcl-2 and MIC-2 proteins lend histogenetic support for this new classification of thymoma. Bcl-2 protein is strongly expressed in tumor epithelial cells of every case of poorly differentiated thymic carcinoma whereas the other types of thymic epithelial tumors do not show epithelial expression of this protein. The strong expression of bcl-2 protein in tumor epithelium may be considered as a predictor of aggressive behavior in thymic epithelial tumors.

A Case of Hereditary Sensory Neuropathy Type II with Acroosteolysis / 대한류마티스학회지

The hereditary sensory neuropathy is a very rare disease characterized by prominent sensory loss without corresponding motor involvement, but may be associated with autonomic features. Currently, the disease is divided into five main types and most frequent are Type I and Type II. The type II hereditary sensory neuropathy is characterized by autosomal recessive inheritance, onset in utero or in infancy, loss of touch-pressure sense more than paintemperature sense, and almost total absence of myelinated nerve fibers. In this case, we describe a 23 years old female patient with acroosteolysis and heel ulcer who was diagnosed as hereditary sensory neuropathy type II.

Effects of cis - Platin on the Type I Collagen of the Osteoid in the Metaphysis of Rat Tibia / 대한정형외과학회잡지

The present study has been undertaken to pursue the cytotoxic effects of cis-Platin on the osteoid formation in metaphysis of rat tibia. By using the immunohistological staining method for type I collagen in rat tibial osteoid, the author detected the deposition of type I collagen, which is the collagenous constituent of endochondral osteoid, after administration of cis-Platin in experimental animals. For the immunological reactions of type I collagen, we used the rabbit anti-rat collagen type I polyclonal antibody as primary antibody and biotinylated goat anti-rabbit IgG as secondary antibody. The distributions of immunohistological reactions in the each of metaphyseal osteoids were analyzed with an image analyzer, and we studied the variances of type I collagens by statistical probabilities. In 12 hours after cis-Platin injection, immunoreactive area in the osteoid of metaphysis was distinctly decreased. Immunoreactive area of type I collagen in osteoids of 1 day and 3 days group metaphysis was increased more than that of 12 hours group and the type I collagen in the metaphysis showed weak immunoreactions of type I collagens with an image analyzer. In the osteoids of 7 days group after cis-Platin injection, the immunoreactive area was similar to that of control group. It is consequently suggested that cis-Platin would induce the decrease of type I collagen in the osteoid. But the type I collagen in tibial osteoid shows the increase from a few days after cis-Platin injection.

Papillary Neoplasm of the Endolymphatic Sac: A report of two cases

Papillary tumor of the temporal bone or middle ear has been recognized as an aggressive neoplasm because of its invasive growth pattern. The site of origin is controversial so that most cases have been reported under various diagnostic terms. Recently, Heffner(1989) suggested that the endolymphatic sac is a possible site of origin, because the tumor resembles the endolymphatic sac in several aspects. We report two such cases. One patient was a 34-year-old female presenting with tinnitus and hearing difficulty for 1 year. Temporal bone CT revealed extensive bone destruction by the tumor which was located in the posterolateral aspect of temporal bone. The other patient was a 56-year-old female who complained of tinnitus, dizziness and otalgia for 2 years. Cranial MR imaging showed an irregularly marginated mass in the left jugular fossa with extension to the petrous bone. Histologically, both cases showed a papillary pattern and locally destructive growth that are typical of papillary tumor of the endolymphatic sac. The papillae were lined by a single layer of bland-looking cuboidal to low columnar cells. Immunohistochemically the lining cells expressed cytokeratin, epithelial membrane antigen, neuron specific antigen and in one case, S-100 protein, supporting the thesis that these neoplasms might be of endolymphatic sac origin.

A Case of Huge Mature Teratoma Developed in the Middle Cranial Fossa: A Case Report

A case of intracranial mature teratoma found in a 35-year-old man is reported. This tumor was originated from the left middle cranial fossa. The tumor was exceptionally huge and MRI showed whirling appearance of intratumoral content. It seems that the temporal location permitted the tumor to grow into a huge size for a squamous epithelium, sebaceous glands, bony spicules, and neuroepithelial tissue, which were compatible with histologic findings of teratoma. It is very unusual for a teratoma to occur in the middle cranial fossa, whereas most of the teratomas are located in the midline structures, such as the pineal or the suprasellar region.