Sjogren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in Sjogren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. Central nervous system symptoms may be presented as one of extraglandular manifestations, though rare in incidence, and need differential diagnosis from multiple sclerosis. We report a case of a 45-year-old woman diagnosed as multiple sclerosis at first but later as neurologic manifestation of primary Sjogren's syndrome, showing signs of multiple sclerosis and cutaneous erythematous lesions.
Case Report ; Diagnosis, Differential ; Erythema/pathology ; Erythema/diagnosis* ; Female ; Human ; Middle Age ; Multiple Sclerosis/pathology ; Multiple Sclerosis/diagnosis* ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis*

Balo's concentric sclerosis is regarded as a rare variant of multiple sclerosis. Traditionally, Balo's concentric sclerosis was a post-mortem diagnosis, but the recent introduction of brain magnetic resonance imaging (MRI) scans may allow noninvasive access without biopsy. Brain MRI findings of Balo's concentric sclerosis is characteristic concentric configuration of alternating bands of white matter of different pathology, with relatively preserved myelination alternating with regions of demyelination in the cerebral white matter. We report a case of Balo's concentric sclerosis with recurrent optic neuritis.
Biopsy ; Brain ; Demyelinating Diseases ; Diagnosis ; Diffuse Cerebral Sclerosis of Schilder* ; Humans ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Myelin Sheath ; Optic Neuritis* ; Pathology

Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.
Abnormalities, Multiple ; Adolescent ; Elastic Tissue/pathology ; Fingers/abnormalities ; Humans ; Hyperpigmentation/pathology ; Male ; Micrognathism/pathology ; Rothmund-Thomson Syndrome/*diagnosis/pathology ; Sclerosis/pathology ; Skin Diseases/diagnosis/pathology

Sclerosing encapsulating peritonitis (SEP) is a poorly understood and rarely documented cause of small bowel obstruction. Although recurrent peritonitis has been reported as the main contributory factor leading to secondary SEP, the pathogenesis of primary (idiopathic) SEP is still uncertain. A 40-year-old woman with a history of total abdominal hysterectomy due to gestational trophoblastic disease presented with progressive lower abdominal pain and abdominal distension. Ultrasonography and contrast-enhanced abdomen-pelvis computed tomography of the abdomen revealed encapsulation of the entire small bowel with a sclerotic capsule. At laparotomy, a fibrous thick capsule encasing small bowel loops was revealed. Extensive adhesiolysis and removal of the capsule from the bowel loops were performed. The patient recovered uneventfully; she was discharged without complications. SEP is a rare cause of small bowel obstruction. We treated a case of abdominal cocoon with intestinal partial obstruction in a woman with a history of abdominal hysterectomy due to gestational trophoblastic disease. Surgical treatment was effective and the patient recovered without complication.
Adult ; Female ; Humans ; Hysterectomy/*adverse effects ; Intestinal Obstruction/diagnosis/*etiology ; Intestine, Small/*pathology ; Peritonitis/diagnosis/*etiology/surgery ; Sclerosis/*pathology

Encapsulating peritoneal sclerosis (EPS) is a rare but severe complication of peritoneal dialysis. A total of 50% of the patients died within 12 months after being diagnosed. There are no obvious clinical symptoms in the early stage of EPS, which is easy to be missed. And there are few case reports of EPS in early stage. On December 22, 2018, a 70-year-old male patient undergoing peritoneal dialysis for 17 months, who was diagnosed as EPS, was admitted to the Department of Nephrology, the Third Xiangya Hospital, Central South University. The patient's peritoneal dialysis catheter was obstructed after peritonitis. The peritoneal dialysis fluid couldn't be drain in and out of the abdominal cavity. Therefore, the laparoscopy was performed to repair the catheter. The operation in progress showed that the peritoneum was slightly thickened and the ileocecal intestinal tube was closely adhered to the parietal peritoneum where the catheter was wrapped, indicating the early stage of EPS. Peritoneal relaxation was performed. The patient's catheter was normal after adhesiolysis. He underwent hemodialysis, nutritional supporting as well as peritoneal dialysis transition, etc. The peritonitis was controlled after 10 days and the peritoneal dialysis was resumed. After discharge from hospital, the patient took moxifloxacin for 2 more weeks. We followed up the patient for 6 months. The automated peritoneal dialysis is maintained, and everything remains normal. Clinicians need to improve understanding of EPS. Early diagnosis and laparoscopic adhesiolysis is helpful to continue peritoneal dialysis treatment.
Aged ; Early Diagnosis ; Humans ; Male ; Peritoneal Dialysis/adverse effects* ; Peritoneal Fibrosis/pathology* ; Peritoneum ; Peritonitis/pathology* ; Sclerosis/pathology*

Diffuse mesangial sclerosis (DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.
Denys-Drash Syndrome ; Diagnosis ; Humans ; Infant ; Kidney Failure, Chronic ; Male ; Molecular Biology ; Nephrotic Syndrome* ; Pathology ; Sclerosis*

BACKGROUND: Quantitative measurement of hippocampal T2 relaxation time is an objective means of determining the frequency and severity of signal abnormalities. To evaluate the diagnostic properties of T2 relaxometry in temporal lobe epilepsy(TLE), we measured T2 relaxation time of bilateral hippocampi in pathology-proven TLE patients and normal controls. METHODS: We investigated 10 TLE patients who had temporal lobectomy with MR T2 relaxation mapping. All patients underwent in phase I or II studies, and had pathologic diagnosis. Also we measured T2 relaxation time in 10 normal volunteers. RESULTS: The pathologic findings of 10 TLE patients were followings: 8 hippocampal sclerosis (including dual pathology of necrotic granuloma), 1 calcified fibrous nodule, and 1 normal hippocampus. The mean T2 relaxation time of normal controls is 67.5msec, which is lower value than previous reports. All patients with hippocampal sclerosis in pathology showed increased T2 time greater than 2 SD of mean value of normal controls. But, the T2 values are upper normal range in non-hippocampal sclerosis. The lateralizing value of T2 relaxometry is 50% in TLE patients, and 62.5% in pathology-proven hippocampal sclerosis groups. CONCLUSIONS: There is a clear distinction of T2 relaxation time between the patients of hippocampal sclerosis and normal controls or non-hippocampal sclerosis. These findings suggest that the T2 relaxation time is a reliable objective measurement of hippocampal pathology, especially hippocampal sclerosis in TLE.
Diagnosis ; Epilepsy, Temporal Lobe* ; Healthy Volunteers ; Hippocampus ; Humans ; Pathology ; Reference Values ; Relaxation ; Sclerosis ; Temporal Lobe*

Magnetic resonance (MR) images can be used to detect lesions in the brains of patients with multiple sclerosis (MS). An automatic method is presented for segmentation of MS lesions using multispectral MR images in this paper. Firstly, a Pd-w image is subtracted from its corresponding T1-w images to get an image in which the cerebral spinal fluid (CSF) is enhanced. Secondly, based on kernel fuzzy c-means clustering (KFCM) algorithm, the enhanced image and the corresponding T2-w image are segmented respectively to extract the CSF region and the CSF-MS lesions combinatoin region. A raw MS lesions image is obtained by subtracting the CSF region from CSF-MS region. Thirdly, based on applying median filter and thresholding to the raw image, the MS lesions were detected finally. Results were tested on BrainWeb images and evaluated with Dice similarity coefficient (DSC), sensitivity (Sens), specificity (Spec) and accuracy (Acc). The testing results were satisfactory.
Algorithms ; Brain ; pathology ; Humans ; Image Enhancement ; Magnetic Resonance Imaging ; Multiple Sclerosis ; diagnosis ; Sensitivity and Specificity

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.
Calcinosis/diagnosis ; Colitis, Ischemic/diagnosis/etiology ; Colonoscopy ; Female ; Humans ; Mesenteric Vascular Occlusion/*diagnosis/etiology/pathology ; Mesenteric Veins/*pathology ; Middle Aged ; Sclerosis/pathology ; Tomography, X-Ray Computed

Idiopathic mesenteric phlebosclerosis, rare disease entity causing chronic mesenteric ischemia is a member of non-thrombotic, non-inflammatory stenosis or occlusion of the mesenteric veins. The histologic hallmark is marked fibrous mural thickening and sclerosis of the vessel wall. It is frequently accompanied by calcification in the vessel wall. We report the case of a 61-year-old woman with idiopathic mesenteric phlebosclerosis. To our knowledge, this is the first case reported in Korea.
Calcinosis/diagnosis ; Colitis, Ischemic/diagnosis/etiology ; Colonoscopy ; Female ; Humans ; Mesenteric Vascular Occlusion/*diagnosis/etiology/pathology ; Mesenteric Veins/*pathology ; Middle Aged ; Sclerosis/pathology ; Tomography, X-Ray Computed