This report describes a case of cerebrotendinous xanthomatosis (CTX) accompanied by clinical manifestations of parkinsonism, including oily and masked face, marked akinesia, muscle rigidity and resting hand tremor. Magne...This report describes a case of cerebrotendinous xanthomatosis (CTX) accompanied by clinical manifestations of parkinsonism, including oily and masked face, marked akinesia, muscle rigidity and resting hand tremor. Magnetic resonance imaging (MRI) of the brain showed high intensity areas on T2 weighted imaging, and slightly low intensity areas on T1 weighted imaging in the right globus pallidus and the left putamen. Cerebral cortical atrophy with slight ventricular dilatation and cerebellar atrophy were present as well. This is a case report of CTX which manifested parkinsonism. Parkinsonism may not be a coincidental manifestation in CTX, but rather represent a symptom of the same underlying diathesis.
A 30-year-old woman with thyroid crisis and impaired renal function presented with severe hypocalcemia. She had already had hypocalcemia one yr previously when her serum creatinine level was normal. A normal level of ser...A 30-year-old woman with thyroid crisis and impaired renal function presented with severe hypocalcemia. She had already had hypocalcemia one yr previously when her serum creatinine level was normal. A normal level of serum amino terminal fragment of parathyroid hormone in spite of severe hypocalcemia suggested impairment of parathyroid function. Since her serum magnesium was normal and she had no history of neck surgery or radioiodine therapy, her hypoparathyroidism was considered to be idiopathic. This is the seventh case with co-existing Graves' disease and idiopathic hypoparathyroidism reported in the literature.
Literature reviews have indicated that thrombotic complications in leukemic patients are rare that prompt diagnosis is very difficult, and that morbidity and mortality are extremely high. We describe the successful treat...Literature reviews have indicated that thrombotic complications in leukemic patients are rare that prompt diagnosis is very difficult, and that morbidity and mortality are extremely high. We describe the successful treatment of a 36-year-old housewife suffering from chronic myelogenous leukemia with secondary myelofibrosis.
A pair of 37-year-old identical twins with diabetes mellitus are described. One of the brothers was admitted for heart failure without pain, and autonomic neuropathy was found. The clinical diagnosis was inferior myocard...A pair of 37-year-old identical twins with diabetes mellitus are described. One of the brothers was admitted for heart failure without pain, and autonomic neuropathy was found. The clinical diagnosis was inferior myocardial infarction with anteroseptal healed myocardial infarction. Cardiac catheterization revealed triple coronary vessel involvement. The diagnosis was confirmed at autopsy after sudden death. The other brother was also examined by cardiac catheterization, which revealed total right coronary occlusion and hypokinesis of the wall. There had been no previous pain nor upper body discomfort until that time in either twin. Thus, genetic factors should possibly be considered in the genesis of asymptomatic or silent myocardial infarction.
A 70-year-old woman with Cogan's syndrome first presented with central diabetes insipidus and then developed secondary hypothyroidism. Magnetic resonance imaging revealed a diffuse pituitary swelling without evidence of...A 70-year-old woman with Cogan's syndrome first presented with central diabetes insipidus and then developed secondary hypothyroidism. Magnetic resonance imaging revealed a diffuse pituitary swelling without evidence of tumor. High-dose glucocorticoid therapy administered to treat Cogan's syndrome was very effective in suppressing the inflammatory process, and resulted in the reversal of the pituitary swelling and partial recovery of thyroid stimulating hormone secretion. This is the first case of hypopituitarism associated with Cogan's syndrome, a form of autoimmune vasculitis. The glucocorticoid-responsive pituitary lesion is best explained by autoimmune hypophysitis which shows pituitary swelling and is known to often associate with other autoimmune phenomena.
Factor V deficiency associated with rheumatoid arthritis was found in a 54-year-old woman. Hereditary factor V deficiency is very rare; only about 150 cases have been reported since its discovery in 1943. This appears to...Factor V deficiency associated with rheumatoid arthritis was found in a 54-year-old woman. Hereditary factor V deficiency is very rare; only about 150 cases have been reported since its discovery in 1943. This appears to be the first case report of factor V deficiency associated with rheumatoid arthritis in the literature.
A case of infective endocarditis of the tricuspid valve is described in a young female patient with no history of intravenous drug abuse. The patient suffered from symptoms of septic emboli of the lung and right heart fa...A case of infective endocarditis of the tricuspid valve is described in a young female patient with no history of intravenous drug abuse. The patient suffered from symptoms of septic emboli of the lung and right heart failure. She was ultimately treated by tricuspid valve replacement. There was no recurrence of symptoms following surgery.
A patient with subarachnoid hemorrhage who exhibited changes suggestive of myocardial infarction by electro- and echocardiography and underwent coronary angiography is reported. Echocardiography demonstrated marked hypok...A patient with subarachnoid hemorrhage who exhibited changes suggestive of myocardial infarction by electro- and echocardiography and underwent coronary angiography is reported. Echocardiography demonstrated marked hypokinesis in the left ventricular anterior wall to the septum. Since the possibility of concomitant myocardial infarction could not be excluded, coronary angiography was performed with cerebral angiography. No abnormalities were observed in the coronary arteries, and the myocardial damage was considered to be due to subarachnoid hemorrhage. Echocardiograms showed improvements in left ventricular wall motion within a short time after operation of the intracranial lesion.
A 71-year-old female visited our outpatient unit with chief complaints of anorexia and a sense of abdominal distension. The abnormalities found after admission were conspicious increases in CA125 levels in serum and asci...A 71-year-old female visited our outpatient unit with chief complaints of anorexia and a sense of abdominal distension. The abnormalities found after admission were conspicious increases in CA125 levels in serum and ascites as well as anemia, accelerated erythrocyte sedimentation and increased serum LDH. The patient underwent an emergency operation for ileus. The histological diagnosis was serous papillomatous cancer. Immunohistologic staining of the cancer tissue demonstrated the production of CA125. In this very rare the remnant of the Müllerian duct in the peritoneum is thought to have been cancerous.
Fungal infections are often reported, but Trichosporon infection is very rare. A 78-year-old man with adult T-cell leukemia complicated with pulmonary infections is presented. Bronchial exudate culture revealed many yeas...Fungal infections are often reported, but Trichosporon infection is very rare. A 78-year-old man with adult T-cell leukemia complicated with pulmonary infections is presented. Bronchial exudate culture revealed many yeast-like colonies, which were morphologically and biochemically identified as Trichosporon pullulans.
To investigate the role of the mucus-bicarbonate barrier in the prevention of duodenal ulcer recurrence, duodenal mucosal neutralizing capacity and mucosal prostaglandin (PG) synthesis were examined in 5 normal controls...To investigate the role of the mucus-bicarbonate barrier in the prevention of duodenal ulcer recurrence, duodenal mucosal neutralizing capacity and mucosal prostaglandin (PG) synthesis were examined in 5 normal controls (NC) and 12 duodenal ulcer (DU) patients. DU patients were divided into non-recurrent (NR,7) and recurrent (R,5) groups based on endoscopic follow-up study. The recovery time (RT) was significantly longer and the mucosal PGE2 synthesis was significantly lower in R than in NC and NR groups. There was a proportional correlation between mucosal PG synthesis and RT in DU These results indicate that impaired neutralizing capacity caused by reduced endogenous PG synthesis in duodenal mucosa may contribute to recurrence of DU.
The proband, a 42-year-old woman without any symptoms or hypertension, was admitted for examination of accidentally discovered bilateral adrenal masses. Physical examination disclosed bilateral nodular goiter and mild si...The proband, a 42-year-old woman without any symptoms or hypertension, was admitted for examination of accidentally discovered bilateral adrenal masses. Physical examination disclosed bilateral nodular goiter and mild sinus tachycardia. Pheochromocytomas and medullary thyroid carcinomas were revealed by biochemical and histopathological examinations. Hypercholesterolemia and abnormal glucose metabolism returned to normal after bilateral total adrenalectomy and thyroidectomy. Screening examination showed four affected family members. Three of them were also asymptomatic and normotensive. In pheochromocytoma patients, normotension, hypercholesterolemia, and impaired glucose metabolism might be signs of excess secretion of epinephrine rather than norepinephrine.
Pulmonary sarcoidosis is a granulomatous disorder of unknown cause, characterized by an accumulation of active T lymphocytes in the lung. We measured the levels of soluble interleukin-2 receptor (IL-2R) in bronchoalveola...Pulmonary sarcoidosis is a granulomatous disorder of unknown cause, characterized by an accumulation of active T lymphocytes in the lung. We measured the levels of soluble interleukin-2 receptor (IL-2R) in bronchoalveolar lavage fluid (BALF) of patients with active pulmonary sarcoidosis and normal subjects by a sensitive enzyme-linked immunosorbent assay. Soluble IL-2R was detectable in BALF from 6 of 11 patients with sarcoidosis but in only 1 of 8 normal control subjects, the mean levels of IL-2R in BALF of the two groups being 2.8 +/- 0.9 U/ml and 0.1 +/- 0.1 U/ml, respectively (p less than 0.01). A slight correlation was found between the soluble IL-2R level and the number of CD4-positive cells. Sarcoidosis patients were classified by radiographical staging. Soluble IL-2R was not detectable in the BALF of any stage I patients, but was found in the BALF of 6 of the 7 stage II and stage III patients. These results suggest that in the clinical management of patients with sarcoidosis, measurement of soluble IL-2R in BALF is useful for evaluating the activity of the lung disease is sarcoidosis.
Two cases of necrotizing myelopathy, one with lung carcinoma and the other with adult T cell leukemia (ATL), displayed flaccid paraplegia and sphincter dysfunction. Both cases did not show any direct neoplastic cell inva...Two cases of necrotizing myelopathy, one with lung carcinoma and the other with adult T cell leukemia (ATL), displayed flaccid paraplegia and sphincter dysfunction. Both cases did not show any direct neoplastic cell invasion of the spinal cord. Pathologically, diffuse and random necrosis was found in the spinal cord in both cases. In the case of ATL, small numbers of inclusion bodies in the nerve cell nuclei were demonstrated. Using polyclonal and also monoclonal antibodies, herpes simplex virus type 2 (HSV-2) was clearly demonstrated in the spinal cord in both cases. By electron microscopy, numerous herpes virus particles were observed in the spinal cord in both cases. The usefulness of electrophysiological studies should be emphasized in the differentiation of these cases from others which might also display flaccid paraplegia. The possibility of necrotizing myelopathy caused by HSV-2 should always be considered in the differential diagnosis of spinal cord diseases displaying flaccid paraplegia.
A 45-year-old woman presented with persistent headache which was of acute onset and occurred daily. Laboratory findings included thrombocytosis, elevated levels of antinuclear antibody, rheumatoid factor, and antibodies...A 45-year-old woman presented with persistent headache which was of acute onset and occurred daily. Laboratory findings included thrombocytosis, elevated levels of antinuclear antibody, rheumatoid factor, and antibodies to Epstein-Barr virus (EBV). Magnetic resonance image of the head, left selective external carotid angiography, temporal arterial biopsy, and cerebrospinal fluid revealed no abnormal findings that could explain her headache. Her headache was compatible with NDPH, which has been reported by Vanast, and was thought to be related to EBV reactivation.
A 62-year-old Japanese female was admitted due to dyspnea. She showed a marked increase in CA19-9 (maximum, 192,000 U/ml) and monoclonal IgM-kappa type rheumatoid factor (RF) activity. The patient died of respiratory fai...A 62-year-old Japanese female was admitted due to dyspnea. She showed a marked increase in CA19-9 (maximum, 192,000 U/ml) and monoclonal IgM-kappa type rheumatoid factor (RF) activity. The patient died of respiratory failure 3 months later. Autopsy findings revealed an infiltration of IgM-kappa-positive plasma cells in the pulmonary interstitium, and therefore a diagnosis of lymphoid interstitial pneumonia (LIP) was made. The bronchoepithelial cells were stained with monoclonal antibody-reactive with CA 19-9 antigen. This is the first documented LIP, associated with a marked increase in monoclonal IgM-kappa type RF and CA19-9 in the serum.
A 44-year-old woman with scleroderma-like skin lesions and malignant hypertension following mammoplasty is reported. Sclerotic change is an unusual finding for ordinary finding progressive systemic sclerosis. On admissio...A 44-year-old woman with scleroderma-like skin lesions and malignant hypertension following mammoplasty is reported. Sclerotic change is an unusual finding for ordinary finding progressive systemic sclerosis. On admission, she had severe high-renin hypertension and progressive renal failure, suggesting scleroderma renal crisis. With intensive treatment for hypertension including angiotensin-converting enzyme inhibitor, the blood pressure was well controlled. It was then suggested that she had malignant hypertension due to scleroderma after silicone injection, or the so-called human adjuvant disease after cosmetic surgery.
Diffuse proliferative glomerulonephritis without IgA deposits (DPGN) in patients with pustulosis palmaris et plantaris (PPP) is described. This association has not been previously reported. Although PPP was resolved tran...Diffuse proliferative glomerulonephritis without IgA deposits (DPGN) in patients with pustulosis palmaris et plantaris (PPP) is described. This association has not been previously reported. Although PPP was resolved transiently after tonsillectomy, it was worsened coincidently at the time of exacerbation of DPGN. Improvement of proteinuria and PPP was observed after antiplatelet therapy. It was suggested that the renal manifestation and the development of PPP were associated.
A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse ther...A 26-year-old male patient with pachydermoperiostosis is reported. He had severe anemia with myelofibrosis. Treatment with iron, prednisolone, oxymethorone and 1 alpha (OH)D3 were not satisfactory. But steroid pulse therapy with parenteral iron improved his anemia and pancytopenia, but was not sufficient to relieve the bone marrow fibrosis or splenomegaly. The mechanism of anemia which was considered to be multifactorial including gastro-intestinal bleeding associated with peptic ulcer or erosion and bone marrow failure due to myelofibrosis, is discussed.
The effect of glucagon infusion (15-20 ng/kg/min, for one hour) on renal hemodynamics was examined in normal subjects and in patients with chronic glomerulonephritis (CGN). In normal subjects, the glomerular filtration r...The effect of glucagon infusion (15-20 ng/kg/min, for one hour) on renal hemodynamics was examined in normal subjects and in patients with chronic glomerulonephritis (CGN). In normal subjects, the glomerular filtration rate (GFR) was significantly increased by glucagon with a concomitant rise in effective renal plasma flow (ERPF) and filtration fraction (FF) (GFR/ERPF). In renal patients with a baseline GFR of above 90 ml/min/l/73 m2, GFR was significantly increased in response to glucagon, without a significant rise in ERPF. The patients whose baseline GFR ranged from 40-90 ml/min had no significant increase in GFR, while ERPF rose significantly. These results suggest that the renal hemodynamic response to glucagon infusion were modified by the level of baseline GFR in patients with chronic glomerulonephritis.