Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center

BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Sixty-one of these patients were followed-up for more than 1 year and received corticosteroid therapy with or without intravenous cyclophosphamide pulse therapy for 6 to 18 months. Medical records of the 61 patients including demographic data, clinical features, laboratory and pathological findings, treatments, and outcomes were reviewed. RESULTS: Peripheral neuropathy as a manifestation of EGPA was present in 46 (75%) of the 61 patients. The mean follow-up duration of the patients with neuropathy was 6.4 years (range 1.2–18.8 years). The scores on the neurological functional disability scale before and after the combination treatment with corticosteroid and cyclophosphamide were 2.43±0.86 and 0.54±0.95 (mean±SD; p<0.001), respectively. The peripheral neuropathy relapsed in one patient. CONCLUSIONS: The long-term clinical outcome of peripheral neuropathy in patients with EGPA receiving initial corticosteroid and cyclophosphamide combination therapy was favorable with a very low relapse rate.

Glycogen Storage Disease Presenting as Fetal Hydrops: A Case Report / 대한주산의학회잡지

Glycogen storage disease (GSD) is a group of heterogeneous disorders of glycogen metabolism that results in abnormal storage of glycogen in multiple organs. Clinical manifestations of GSD vary according to the basic enzyme defect. Only types II, IV, V or VII of GSD have been known to manifest in the infantile period. Of the 11 types of GSD, the congenital subtype of GSD type IV is characterized by severe neonatal hypotonia, multiple contractures, polyhydramnios, and fetal hydrops. We report a case of a patient born at a gestational age of 34 weeks and 3 days with fetal hydrops, joint contractures, and akinesia. Muscle biopsy results were highly indicative of GSD. This is the first case of suspected GSD in Korea presenting as fetal hydrops. The possibility of other disorders associated with glycogen metabolism should be considered in fatal fetal hydrops patients with severe hypotonia and arthrogryposis, and aggressive investigations such as muscle biopsy should be performed for early diagnosis.

Neuroblastoma Originating from Extra-abdominal Sites: Association with Favorable Clinical and Biological Features

Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (+/-95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4+/-10.6% vs. 69.4+/-9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.

Neuroblastoma Originating from Extra-abdominal Sites: Association with Favorable Clinical and Biological Features

Neuroblastomas originating from different sites might have different clinical and biological characteristics. In the present study, the clinical (age, sex and stage) and biological (N-myc amplification, Shimada pathology and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of patients with newly diagnosed neuroblastoma were compared according to the site of tumor origin (extra-abdominal versus abdominal). The event-free survival rate (EFS) was also compared between the two groups. Among 143 neuroblastomas, 115 tumors originated from the abdomen, 26 from extra-abdominal sites and 2 from unknown primary sites. Frequencies of stage 4 tumor and N-myc amplified tumor were lower in the extra-abdominal group than in the abdominal group (34.6% vs. 60.0%, P=0.019 and 4.2% vs. 45.0%, P<0.001, respectively). Levels of lactate dehydrogenase, ferritin and neuron-specific enolase were significantly lower in the extra-abdominal group than in the abdominal group. The probability of 5-yr EFS (+/-95% confidence interval) was higher in the extra-abdominal group than in the abdominal group (94.4+/-10.6% vs. 69.4+/-9.4%, P=0.026). Taken together, neuroblastomas originating from extra-abdominal sites might be associated with more favorable clinical and biological characteristics and a better outcome than neuroblastomas originating from abdomen.

A case of the primary ovarian malignant melanoma arising from a cystic teratoma / 대한내과학회지

Primary malignant melanoma arising in a cystic teratoma of ovary is extremely rare. Approximately 25 cases of primary malignant melanoma arising from a cystic teratoma in ovary have been reported in the literature. The malignant transformation of benign cystic teratoma of ovary usually develops unilaterally in postmenopausal women. The common presenting symptoms are abdominal distention, lower abdominal pain and a palpable abdominal mass. We report a case of primary malignant melanoma of ovary in a 45-year-old patient who underwent laparotomy for evaluation of a cystic mass in right ovarian cystic teratoma. Histopathological examination of the cyst showed that it was melanoma with pre-existed teratomatous components. Extraovarian primary site was not found. Nine years after the excision, brain metastases developed which were pathologically confirmed as metastatic melanoma. The patient subsequently developed pulmonary metastases and received immunochemotherapy consisting of cisplatin, dacarbazine, vinblastine, interleukin-2 and interferone-alpha. She is on a regular follow-up and continues to have stable disease for 9 months.

Clinical Implication of Magnetic Resonance Imaging in the Idiopathic Inflammatory Myopathies / 대한류마티스학회지

OBJECTIVE: The purpose of the study is to describe the magnetic resonance imaging (MRI) findings and to investigate useful sequences of MRI in inflammatory myopathies. A third goal is to correlate MRI findings with the grade of histopathologic severity. METHODS: Sixteen patients (13 women, 3 men), aged 21-74 years (median age of 49) with inflammatory myositis (examined with both MRI and muscle biopsy) were studied. Ten patients were diagnosed with polymyositis and 6 with dermatomyositis. Conventional T1-weighted (SE 366/12) and T2-weighted (SE 4766/90) fast spin-echo and fat-suppressed T1-weighted MR images with contrast enhancement (FSAT T1 CE) were obtained. Ratios between the signal intensities of a muscle and the signal intensities of subcutaneous fat in the same tomographic sections were calculated to objectively represent the degree of signal intensities. Semi-quantitative grading of severities in muscle biopsy specimen was examined based on invasion of inflammatory cells and necrosis, degeneration and regeneration of muscle fiber by a neuromuscular pathologist. RESULTS: FSAT T1 CE could objectively describe the severity of involvement in inflammatory myopathies. The quadriceps muscle group, especially the vastus muscle tended to be most severely and frequently involved in inflammatory myositis with FSAT T1 CE (statistically insignificant). The vastus intermedius muscle was more severely involved in dermatomyositis than polymyositis. The signal intensity of abnormal muscle sampled by biopsy correlated positively with the grade of muscle biopsy. CONCLUSION: MRI shows promise in identifying pathologic muscle in patients suspected of having one of the inflammatory myopathies. The degree of signal intensity on MRI may reflect the severity of grade in muscle biopsy.

Angiosarcoma of the Breast: A report of 2 cases

A primary angiosarcoma is a very rare condition, accounting for 0.04% of all malignant tumors of the breast. It is known to have a poor prognosis and recurrence and distant metastasis are common. We report two cases of primary angiosarcoma. They were first diagnosed in 29 years old & in 34 years old. Preoperative evaluation provided an indication of the disease, and no evidence of merastasis was seen. Both patients underwent a total mastectomy. Radiation therapy was performed as an adjuvant treatment in one patient and in the case of recurrence in the other. Pathologic findings are reviewed and described. The diagnostic approach and treatment options from the literature are discussed.

A Study of the Effects of Clinicobiological Factors upon the Meningioma-Associated Peritumoral Edema Formation

It is thought that the biological aggressiveness of meningioma is closely related to the development of peritumoral edema. However, the effects of mechanical tumor factors on the venous return of underlying brain tissue, as well as those of MIB-1 or p53 immunoreactivity on the brain edema formation are still not clear. To identify factors which may influence meningioma-associated peritumoral edema development, the authors examined 28 histologically proven intracranial meningiomas(22 benign, 4 atypical, and 2 malignant meningioma patients). Correlation between the degree of brain edema and various factors including volume of the tumor, venous sinus involvements, tumor location, histologic subtypes, MIB-1 labeling index(LI), and immunoreactivity of p53 protein was analyzed retrospectively. The degree of brain edema(edema ratio) was measured by maximum edema area to maximum tumor area as seen on T2 and T1 enhanced magnetic resonance images, respectively. Mean maximum tumor area and the volume of the tumor were 15.7cm2 and 50.2cm3, respectively. The mean area of maximum edema extension was 23.3cm2 and the mean ratio between maximum edema area and tumor area was 1.90(range: 0-11.5). Tumor volume and the area of edema showed significant correlation(p=0.015). MIB-1 LI, however, correlated inversely with edema ratio(p=0.039). p53 protein expression, venous sinus involvement, age, sex, and histologic characteristics did not correlated with edema area or ratio. In conclusion, this study showed there was inverse correlation between edema development and MIB-1 LI, and no correlation with p53 expression. It was thus speculated that peritumoral edema in meningioma may not be a sign of biological aggressiveness.

Difference of Bonding Behavior between Four Different Kinds of Hydroxyapatite Plate and Rabbits's Bone / 대한정형외과학회잡지

The change of conditions of hydroxyapatite synthesis can affect not oniy the material properties, but also the body reaction to the hydroxyapatite implants. To find out conditions for preparing more biocompatible hydroxyapatite implants as bone graft substitute. we evaluated the biologic response to the dense synthetic hydroxyapatite implants, made with various synthetic conditions, placed in corticocancellous defects of rabbits' long bone. The hydroxyapatites were synthesized with coprecipitation technique using Ca(NO3) 4H2O and (NH4)2HPO4, made with various Ca/P ratio and aging temperatures. Four kinds of hydroxyapatites were selected to use as implants(HA I: Ca/P ratio 1.5, aging temperature 90degrees C; HA V :1.5 , 30degrees C; HA VI: 1.83, 30degrees C; and HA lX: 1.67, 30degrees C). These hydroxyapatites were pressed and sintered at l300degrees C to fabricate dense plates. Biomechanical test and rnorphological examination were performed using Instron, light microscope and electron microscope. The characteristics of hydroxyapatite powder and sintered body were more significantly affected by siarting Ca/P ratios. The bonding strength of HA IX(1.67, 30degrees C) with bone was grcatest at 4 or 8 weeks after implantation with statistically significant difference(p<0.05). Bonding behavior betweeb HA IX and bone was most excellent in terms of new bone formation and new bone ingrowth into resorbed surface of hydroxyapatite plate.

Cerebral Amyloid Angiopathy Presenting with Progressive Dementia

Cerebral amyloid angiopathy (CAA) can present with lobar hemorrhage, progressive dementia, or transient neurologic symptoms. To date, Pathology-confirmed CAA which presented with progressive dementia has rarely been repored in Korea. A 79-year-old normotensive man presented with progressive dementia for one year. Neurologic examination was remarkable for hyperreflexia and bilateral grasp response. Mild parkinsonian feature was also present. Cognitive domains impaired on neuropsychological test included verbal and nonverbal memory, language, visuospatial function and frontal/executive function. Brain MRI, especially gradiant-echo imaging, showed multiple microhemorrhages in the cortical areas along with leukoaraosis. Six months after initial evaluation, he developed massive ICH involving right frontal lobe with midline shift. Hematoma removal as well as cortical biopsy was performed. Cortical and meningeal vessels were thickened with amorphous materials showing apple-green birefringence, a feature consistent with CAA. CAA should be suspected in patient with subacute dementia when their MRI shows cortical microhemorrhage and leukoaraiosis.

Relapsing sensory ataxia initially manifested by miller-fisher syndrome

We experienced a patient who manifested Miller-Fisher syndrome initially, and progressed relapsing ataxia later, There was electrophysiologic evidence of distal sensory polyneuropathy with completely sparig of somatic motor nerves. Sural nerve biopsy showed severe segmental demyelination. All the symptoms improved by intravenous immunoglobulin dramatically but after a period of remission, progressive ataxia reappeared. Plama exchange had some effect and prednisolone alone showed no definite improvement. High dose of prednisolone combined with IVIgG made remission for a long time. We believe that this patient had an unusual form of inflammatory polyneuropathy, a relapsing variant of the Miller-Fisher syndrome of acute idiopathic polyneuritis. In this case, IVIgG was the most effective therapy and prednisolone was added as a long term therapy successfully.

Secretory Carcinoma of the Breast: A case report

We report a case of secretory carcinoma with axillary lymph node metastasis in a 21-year old woman. She was aware of a mass in her breast for 10 years and noticed a rapid growth of the preexisting mass during the last years. Histologically, the tumor was composed of micropapillary and microcystic or cribriform glandular structures which contained eosinophilic, mucinous, intraluminal secretions. The center had a dense hyalinized strama with a solid infiltrative growth of tumor cells with intracytoplasmic secretory vacuoles at the periphery. In addition, marked intraductal papillary epithelial proliferations were present at the superficial portions of the tumor near the nipple. Prognostic factors and their relationship to juvenile papillomatosis are discussed with a review of the literature.

Malakoplakia / 대한소화기내시경학회지

Malakoplakia is an uncommon granulomatous disease most frequently found in the bladder or ureter. Howevcr, it has been reyorted in almost every ather organ and structure, the colon is the most common site of malakoplakia outside the urogenital tract and colonic carcinoma is the most commonly associated disease. A case of malakoplakia of the colon in a 54-year-old female is reported. Sigmoidoscopy revealed multiple whiteyellowish nodules simulating polypoid lesion at 10cm from the anal verge. The biopsy showed characteristically comprised submucosal proliferations of histiocyte and chronic inflammatory cells with typical cytoplasmic inclusions known as Michaelis-Gutmann bodies. Intravenous pyelogram showed no evidence of involvement of the urinary system. These nodules were removed by endoscopic polypectomy, So far, this is the first case of isolated colonic malakoplakia in Korea.

The clinical manifestations of the five cases of lymphangitic carci-nomatosis of the lung presented as diffuse and interstitial disease / 결핵

No abstract available.