1/132. Aortic dissection in young patients with chronic hypertension.We describe four patients aged 14 to 21 years who developed acute aortic dissection. In three of the four patients, the course was fatal, despite aggressive medical and surgical intervention. All four patients had sustained systemic hypertension related to chronic renal insufficiency. The patients had no other identifiable risk factors for aortic dissection, including congenital cardiovascular disease, advanced atherosclerosis, vasculitis, trauma, pregnancy, or family history of aortic dissection. Although aortic dissection is rare in individuals younger than 40 years of age, young patients with sustained systemic hypertension are at increased risk for this serious and often fatal condition. physicians must be aware of this rare complication of hypertension and consider aortic dissection in the differential diagnosis of unusual chest, abdominal, and back pain in hypertensive children, adolescents, and young adults.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/132. Internuclear ophthalmoplegia following minor head injury: a case report.Internuclear ophthalmoplegia (INO) is a common sign of multiple sclerosis in young patients and of vascular diseases in older people. Traumatic bilateral internuclear ophthalmoplegia following severe head injuries may occur. We present the unusual case of a young patient suffered from bilateral INO as an isolated finding after a minor head injury, without other signs of brain stem or cortical injury. The ophthalmoplegia has persisted for 22 months.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/132. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/132. Immunoadsorption--a new therapeutic possibility for multiple sclerosis?A 46 year old woman suffers from chronic progressive multiple sclerosis. She was diagnosed in 1993. Because of many complications seen in conservative treatment, plasma exchange was started. The expanded disability status scale by Kurtzke could be improved but the interval between the treatments became shorter and shorter. It was therefore decided to commence protein A immunoadsorption treatment. With this treatment the patient shows good and stable improvement in neurological and functional status with an acceptable treatment frequency of once every 3 weeks.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
5/132. Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy: a clinicopathological study.We report an autopsy case of amyotrophic lateral sclerosis (ALS) clinically diagnosed as spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness of the distal part of the left lower extremity at age 42, followed by muscle weakness and atrophy of the right lower extremity and upper extremities. At age 57, she needed transient ventilatory support. Slight weakness in the facial muscles and fasciculation of the tongue appeared at age 60. At age 61, she died of sudden respiratory arrest. During the clinical course, neurological examination revealed neither Babinski signs nor hyperreflexia. The neuropathological examination revealed not only neuronal loss with gliosis in the facial nucleus, hypoglossal nucleus, and anterior horns of the spinal cord, but also loss of Betz cells and degeneration of the pyramidal tracts. Based on these clinicopathological findings and review of literature, we conclude that sporadic ALS mimicking SPMA is present.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/132. A case of renal pseudotumor associated with chronic pachymeningitis.BACKGROUND: A 56-year-old woman was referred to our hospital with a left renal mass. methods/RESULTS: Radiologic studies demonstrated a solitary space-occupying lesion in the left kidney and a malignant tumor was suspected. Left radical nephrectomy was then performed. Pathological examination revealed a sclerotic fibrous lesion with a rather distinct margin and no evidence of malignancy. These pathological findings were consistent with the diagnosis of a renal pseudotumor. CONCLUSIONS: This patient had a history of chronic pachymeningitis that formed a thoracic epidural focus causing spinal cord compression and the histologic appearance of this focus was similar to the renal lesion. It was concluded that this was a rare case of a renal pseudotumor associated with multifocal fibrosclerosis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/132. Watermelon-stomach as a cause of chronic iron deficiency anemia in a patient with systemic sclerosis.Watermelon-stomach is a rare cause of gastrointestinal bleeding. There has been an increasing number of reports on the association of this lesion with diseases of the scleroderma group, causing chronic, sometimes severe gastrointestinal blood loss. The present report presents the case of a 75-year-old female with limited cutaneous systemic sclerosis and watermelon-stomach, which was the cause of her long-standing sideropenic anemia.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
8/132. Optic-spinal form of multiple sclerosis and anti-thyroid autoantibodies.The optic-spinal form of multiple sclerosis (OSMS), characterized by recurrent involvement of optic nerve and spinal cord with rare brain magnetic resonance imaging lesions, is relatively common among Asians. While individual cases of OSMS with anti-thyroid autoantibodies (ATABs) have been reported, the frequency of ATAbs in OSMS and classical multiple sclerosis has not been studied. We studied serum ATAbs and anti-nuclear antibodies (ANA) in 46 Japanese patients with multiple sclerosis: 14 with OSMS, and 32 with non-OSMS. Six patients were positive for ATAbs: five women with OSMS and one man with non-OSMS. The frequency of ATAbs in OSMS (5/14) was significantly higher than that in non-OSMS (1/32; P = 0.007), but the frequency of ANA did not differ between OSMS (3/14) and non-OSMS (6/32; P = 0.99). There may be a pathogenetic link between anti-thyroid autoimmunity and a subgroup of OSMS in Japanese.- - - - - - - - - - ranking = 7keywords = sclerosis (Clic here for more details about this article) |
9/132. Chronic sclerosing sialadenitis of the submandibular gland associated with idiopathic retroperitoneal fibrosis.We report a case of a 57-year-old man who developed a fibrosclerosing lesion in the submandibular gland and idiopathic retroperitoneal fibrosis (IRF) involving the unilateral periureteral region within a year. Both lesions were resected surgically because of the suspicion of neoplasm. Pathologic examination revealed similar histologic and immunohistochemical features for both lesions, namely, fibrosclerosis with prominent hyalinizing collagen bundles and proliferation of myofibroblastic cells, and a non-neoplastic reactive nature. There was infiltration by lymphocytes with prominent lymph follicles, plasma cells and macrophages. The histologic and immunohistochemical findings suggest that the two lesions were of a similar pathogenesis, which was possibly mediated by macrophages. We think that the present case may be an unusual form of multifocal fibrosclerosis. Although sialolithiasis is thought to be a major pathogenic factor for chronic sclerosing sialadenitis of the submandibular gland, the present case suggests that certain cases might have an etiology similar to IRF.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
10/132. Chronic intrauterine meconium aspiration causes fetal lung infarcts, lung rupture, and meconium embolism.Three neonates with chronic intrauterine meconium aspiration are reported. All had distinctive subpleural plate-infarcts of the lungs caused by meconium-induced vasoconstriction of peripheral preacinar arteries. These vessels showed plexogenic arteriopathy with medionecrosis and obliterative hyaline sclerosis. Organized thrombi and systemic-pulmonary arterial anastomoses were numerous. The infarcts contained inspissated meconium with a granulomatous reaction.In one case, lung rupture occurred, causing meconiumthorax and meconium embolism to hilar lymphatics and lymph nodes; this suggests that particulate meconium may enter the circulation. This fetus had rubella and probable acute twin-twin transfusion following the intrauterine death of the co-twin. The cause of the hypoxia that led to intrauterine passage of meconium in the other cases is unknown. meconium-stained amniotic fluid was noted in only one case.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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