Compensatory function of central dopaminergic system for suppressing psychic seizures--repeated plasma homovanillic acid measurement in refractory temporal lobe epilepsy

OBJECTIVE: To determine the long term efficacy of laparoscopic ovarian diathermy in the management of refractory anovulatory infertility in women with the polycystic ovary syndrome. DESIGN: Open study of 50 women treated over a period of 3 years and 3 months, with follow up until 18 months after the last woman was treated. SETTING: Teaching Hospital. SUBJECTS: Fifty consecutive women with refractory anovulatory infertility (mean duration 5.3 (SD 3.5) years). All had been treated unsuccessfully with anti-oestrogens and more than half with gonadotrophins. INTERVENTIONS: Laparoscopic ovarian diathermy. MAIN OUTCOME MEASURES: Ovulatory cycles and pregnancies. RESULTS: Forty-three women (86%) ovulated following ovarian diathermy; the mean time to ovulation was 23 (SD 6.2) days. Three nonresponders ovulated following anti-oestrogen treatment to which they were previously resistant. Thirty-three women have conceived 58 pregnancies; 22 had no treatment other than ovarian diathermy prior to their first post-operative conception; in seven an anti-oestrogen was given because of lengthening cycles; two were treated elsewhere with gonadotrophins without prior postdiathermy anti-oestrogen therapy and conceived; four had the operation repeated and two of these conceived. Twenty-six women conceived within the first 8 post-operative months. Forty-two pregnancies ended in the birth of normal live healthy babies, eight are ongoing and eight miscarried. Of the 22 women who had no pelvic abnormality other than polycystic ovaries, 19 (86%) have had one or more successful pregnancies. CONCLUSION: Laparoscopic ovarian diathermy is a very effective treatment for anti-oestrogen resistant anovulatory infertility in women with the polycystic ovary syndrome and should be considered as the next step in those who fail to respond to anti-oestrogen treatment.     

The crystallite-gel-model for microcrystalline cellulose in wet-granulation, extrusion, and spheronization

The effect of idoxifene, a novel anti-oestrogen with less agonist activity than tamoxifen, was compared with that of tamoxifen on the growth of hormone-dependent MCF-7 breast cancer xenografts. Forty tumours were established with oestradiol support in ovariectomized athymic mice, allowed to grow to a median volume of 420 mm3 and then continued with oestradiol, no support, tamoxifen or idoxifene delivered by 1.5-cm silastic capsule. Tumour regression occurred with both anti-oestrogens, although maximum regression was observed following oestradiol withdrawal alone. While prolonged anti-oestrogen therapy was associated with static growth, tumour volumes were significantly lower with idoxifene (P=0.01). After 6 months, 0/10 idoxifene-treated tumours developed acquired resistance compared with 3/10 tumours treated with tamoxifen. In separate experiments, 94 animals were treated initially with oestradiol, tamoxifen, idoxifene or placebo following implantation with 1-mm3 pieces of either wild-type (WT) or tamoxifen-resistant (TR) MCF-7 tumour. After 4 months, only 1/11 WT tumours became established with idoxifene compared with 4/11 with tamoxifen, 8/12 with oestradiol and 0/12 with placebo. Likewise, fewer TR tumours were supported by idoxifene (3/12) than by tamoxifen (8/12) or oestrogen (11/12). These data indicate that, compared with tamoxifen, idoxifene shows reduced growth support of MCF-7 xenografts and may share only partial cross-resistance. Furthermore, the development of acquired anti-oestrogen resistance may be reduced during long-term idoxifene therapy. The drug's reduced agonist activity may, in part, explain these observations and indicate a preferable biochemical profile for breast cancer treatment.     

Two cases of esophageal carcinoma metastatic to the pineal region with a review of the literature

BACKGROUND: Metastasis to the pineal region is rare, and there are no previously reported cases of esophageal carcinoma metastatic to the pineal region. CASE DESCRIPTION: We now present two cases of solitary esophageal carcinoma metastatic to the pineal region. In each case there was no evidence of disease progression at the primary site at the time of presentation, and neurologic symptoms were the first indication of recurrent disease. Both patients underwent infratentorial/supracerebellar resection of the pineal lesions and were subsequently referred for radiation therapy. CONCLUSIONS: These two cases emphasize that metastatic disease must be a likely differential consideration in a patient with a CNS lesion and a history of previous malignancy, even if the lesion is in an unusual location.     

Pericarditis in chronic inflammatory bowel disease: underlying disease or side effects of therapy? Clinical problem solving

Pericarditis and myocarditis are rare extraintestinal manifestations of chronic inflammatory bowel disease (ulcerative colitis and Crohn's disease). Pericarditis as a side effect induced by sulfasalazine or 5-aminosalicylic acid, drugs used in the therapy of these diseases, was first described only 7 years ago. In older case reports the relationship between the use of these drugs and pericarditis is unclear. We analyze the reported cases of 68 patients (38 men, 24 women) with ulcerative colitis (n = 45) or Crohn's disease (n = 15) who had one or more episodes of pericarditis or myopericarditis. Pericarditis was not associated with high activity of bowel disease in all cases. In most cases therapy with corticosteroids led to uneventful recovery. In drug induced pericarditis omission of the 5-ASA therapy was sufficient in a few cases. There was one fatal case (with myocarditis). The decision whether pericarditis is a symptom of the underlying disease or a side effect of the drug used for the treatment of the disease is not always easy. We present an analysis (clinical problem solving) of a pertinent observation in a patient with Crohn's disease and pericarditis, showing the dilemma of pericarditis in chronic inflammatory bowel disease and its therapy.     

Effect of cessation of erythromycin therapy on diffuse panbronchiolitis]

The effect of cessation of erythromycin (EM) therapy against diffuse panbronchiolitis was studied. Nine cases were examined. After cessation of EM therapy, the manifestations of disease were stable in five cases, but worsened in the other four. In the former five, the period from the onset of disease until EM therapy began was relatively short; when EM therapy was stopped the manifestations of disease had almost completely disappeared and chest roentgenography revealed resolution of diffuse, small, nodular opacities without remarkable bronchiectasis. In contrast, in the latter four cases, the clinical manifestations of disease did not disappear, and chest-roentgenographic evidence of bronchiectasis was common before the cessation of EM therapy. In conclusion, EM therapy for diffuse panbronchiolitis may be stopped if the clinical manifestations of disease (especially purulent sputum) disappear, if diffuse, nodular opacities resolve almost completely and if there is no evidence of bronchiectasis.     

Ursodeoxycholic acid therapy for primary biliary cirrhosis. A 10-year British single-centre population-based audit of efficacy and survival

The effect of ursodeoxycholic acid treatment on survival in primary biliary cirrhosis was studied in 40 patients with symptomatic disease. Two patients developed early exacerbation of symptoms and stopped therapy in days; they are both alive 4 and 4 1/2 years later. The other 38 patients have continued on treatment for up to 10 years. Results were compared with 12 other similar cases previously seen but not given specific therapy. Kaplan-Meier analysis showed that ursodeoxycholic acid treatment was associated with better survival (p < 0.05) after the first two years of therapy. Predictors of favourable outcome included histological stage I disease. In 26 patients with primary biliary cirrhosis stage II, III or IV, therapy showed a trend to improved survival, but this was still significantly worse than the general population. Prognosis was not different between these different advanced stages. Symptoms improved in 28 out of 40 patients on ursodeoxycholic acid, but 50% had a recurrence by two years.     

Intravenous gentamicin therapy in bilateral Menière disease

BACKGROUND: The treatment of severe forms of bilateral Menière's disease remains an especially challenging task. Similar problems also occur in debilitating Menière's disease in the only hearing ear. The intramuscular titration therapy with streptomycin has been the means of choice since 1984 to minimize the risk of total hearing loss in cases of severe bilateral disease. METHOD: Since 1989 we have treated six out of 21 cases of bilateral Menière's disease by intravenous application of 2 x 120 mg gentamicin in Ringer's solution for several days. Additionally we reported on two cases in 1988. Only minor amounts of gentamicin were applied to sedate the function of both vestibular organs while avoiding damage to the cochlea. RESULTS: In two cases hearing approved approximately about 10 dB, in two cases hearing remained stable, and in two cases hearing worsened about 10 dB. Five of six patients showed minor excitability in caloric tests on both sides, they did not complain of vertigo attacks one to five years after therapy. CONCLUSION: Given that only very small amounts of gentamicin are applied to sedate the function of the vestibular organ while causing almost no damage to the cochlea, this method seems to be an excellent means for treatment of bilateral Menière's disease. Patients do not experience severe problems with equilibrium afterwards, and the treatment can be repeated as often as necessary.     

Hematometra after radiotherapy for cervical carcinoma

The development of a hematometra after radiotherapy for cervical carcinoma is often related to recurrent disease. We present two cases in which a hematometra developed during the use of estrogen replacement therapy. This development was related to regained endometrial activity in combination with fibrosis and obliteration of the upper vagina and/or cervix. In one patient a dilatation and curettage could be performed; in the other a hysterectomy was necessary in order to exclude recurrent disease. These two cases show once more that endometrium can regain its proliferative activity after radiotherapy for cervical cancer. Estrogen replacement therapy in these patients should include the use of a progestagen agent in order to avoid continuous unopposed endometrial stimulation. In the absence of progesterone withdrawal bleeding the uterine cavity should be routinely examined for the development of a hematometra.     

Is there a basis for prevention of breast cancer with drugs?

The anti-oestrogen drug tamoxifen has led to one of the most important improvements in therapy for breast cancer patients achieved during the last decades. Tamoxifen reduces risk of relapse and improves survival in women with breast cancer. In addition, tamoxifen has favourable effects on the lipoprotein metabolism, reduces morbidity and death from myocardial infarction, and stabilizes bone density in women after menopause. Owing to the good therapeutic results in breast cancer patients and the additional favourable effects of tamoxifen, studies were started among healthy women with elevated risk of breast cancer. The intention was to examine whether tamoxifen could reduce the risk of breast cancer development in healthy women. Recent studies, however, have demonstrated higher risk of endometrium cancer in breast cancer patients treated with tamoxifen, and higher risk of histological abnormalities in the endometrium in healthy women. Prevention trials, when tamoxifen is given to healthy women, are disputed, owing to the apparent carcinogenic effect of tamoxifen.     

Analysis of renal water excretion in full-term newborn infants

The clinicopathologic characteristics of two patients with Gorham's disease and bilateral recidivant pleural effusions are reported. The first case corresponded to a nine year-old girl with osteolysis in her right clavicle and ribs. The second case was a 55 year-old male with osteolytic lesions in ribs, cranium, pelvis and femurs; a thymic cyst was also documented. In both patients, biopsies were performed from ribs, which showed proliferating, benign, thin walled small vessels that extensively replaced the intertrabecular spaces. Thoracocentesis, instillation of talcum and radiation therapy were used in the former patient and no recidivant pleural effusions have developed after one year. The second patient was not treated with radiation therapy, persisted with pleural effusions and finally died two years after the diagnosis. Because Gorham's disease is rare, it was not immediately suspected in the cases described here. Its association with pleural effusion is even rarer and has been mentioned only occasionally in the literature. The utility of radiation therapy in Gorham's disease is commented.     

Disease caused by Mycobacterium kansasii in patients with HIV infection

BACKGROUND: To describe the clinical features and response to therapy in Mycobacterium kansasii disease among HIV infected patients, an increasing problem in our setting. METHODS: A retrospective survey of all charts from patients with HIV infection with Mycobacterium kansasii infection recorded between April 1985 and December 1991. RESULTS: A total of 13 patients were identified. All of them had clinically significant respiratory tract samples with a definite M. kansasii isolation. Only three had disseminated disease. In all but two cases, CD4 cell count at diagnosis time was lower than 200/mm3. Chest X-ray films showed interstitial pattern (8 cases) or alveolar condensation (3 cases) and lung cavities were seen in 4 patients. All patients with lung disease and one with disseminated disease responded well to anti-tuberculous therapy. CONCLUSION: Mycobacterium kansasii produces disease in advances stages of HIV-induced immunosuppression. The most common primary location is pulmonary, but disseminated forms can also be seen. The infection can be controlled with standard anti-tuberculous therapy.     

The effect of dizocilpine (MK-801) on conditional discrimination learning in the rat

OBJECTIVES: To report the effects of the anti-oestrogen tamoxifen on biochemical and haematological parameters. DESIGN: Randomized, double-blind comparison of tamoxifen 20 mg per day and placebo, over two years. SETTING: A university hospital. SUBJECTS: Forty-six healthy late-postmenopausal women (mean, SD time since menopause; 11, seven years). MAIN OUTCOME MEASURES: Blood specimens were drawn in the fasting state at baseline, six months and two years for measurement of haemoglobin, haematocrit, erythrocyte mean cell volume, mean erythrocyte haemoglobin, leucocyte count, platelet count, urea, electrolytes, creatinine and albumin. RESULTS: There was a significant decline in the haemoglobin concentration in the tamoxifen group (-4.4, 1.2 g/L; mean, SE) and its levels were lower in this group than in those receiving placebo (P = 0.004). Similarly, haematocrit, erythrocyte count and total leucocyte count were lower in those on placebo (P = 0.002, P = 0.01 and P = 0.01, respectively) and platelet count showed a similar trend (P = 0.08). In the tamoxifen group, the level of serum albumin fell significantly (-2.2, 0.4 g/L) and was lower throughout the study than that in the placebo group (P = 0.006). That of serum urea tended to fall (-0.4, 0.2 mmol L) but the between-groups comparison was not significant (P = 0.18). CONCLUSIONS: These data suggest that tamoxifen exerts a haemodilutory effect in normal postmenopausal women. Since a similar effect has been reported in response to postmenopausal oestrogen therapy, it is likely that the observed changes represent another oestrogenic effect of tamoxifen in postmenopausal women. Haemodilution may contribute to the reduced incidence of cardiovascular disease reported in tamoxifen-treated women, and, therefore, its assessment in the new oestrogen agonists/antagonists being developed for cardiovascular disease prevention may be important.     

Cost-effectiveness analysis of short-term course and regular course of antithyroid drug therapy for Graves' disease]

One hundred and- eighteen newly diagnosed patients with Graves' disease were treated by tapazole for 6 months (short-term course) and followed up for another 12 months. Forty three percent remission rate was found in this group. A regular course of antithyroid drug therapy for 1.5-2 years with 50% remission rate was taken as control. Cost-effectiveness analysis was made to compare the cost in the two different kinds of therapy. The results showed that the total costs of the treatment per 100 cases were 17,746 yuan for the short-term therapy and 30,708 yuan for the regular therapy, with an average of 412.7 yuan and 614.2 yuan per case respectively. In comparison with the short-term therapy, the regular course could remit 7 more cases at a cost of 12,962.40 yuan, with an average of 1851.80 yuan per case. Therefore, the cost was high by prolonged course of therapy to increase slightly remission rate, although it could remit a few more cases. This study suggests that six-month treatment is preferable for those who live in the rural or remote area where the economy was less developed. A longer course of therapy may be unnecessary for those who have been treated by antithyroid drugs for 6 months or longer and are predicted to obtain a possible prolonged remission.     

Immobilization of beta-fructofuranosidases from Aspergillus on methacrylamide-based polymeric beads for production of fructooligosaccharides

Three cases of chondromyxoid fibroma arising in the skull base are reported. The tumors arose in females 34, 65, and 66 (median 55) years of age. Two women presented with headaches, and one with nasal obstruction. Radiographic studies revealed that all three lesions were expansile soft tissue masses centered in the clivus, at least 4 cm in greatest diameter. One lesion involved primarily the clivus, the others extended from the clivus into the sphenoid and ethmoid sinuses. Two of the three cases were initially misdiagnosed as chordoma or chondrosarcoma. The initial treatment was curettage of gross disease in all three cases. One patient also received radiation therapy. One patient had local progression of disease, which was treated with surgery and radiation therapy. All patients are clinically free of disease 11 to 26 months following the most recent treatment. Chondromyxoid fibroma can and should be distinguished from chondrosarcoma and chordoma, two tumors which more commonly arise in the skull base and which have the potential to metastasize.     

Persistent cognitive and motor deficits following acute hydrogen sulphide poisoning

The incidence of thromboembolic complications among 288 patients with atrial fibrillation (AF) who were followed up during an average period of 7.2 years was examined retrospectively. The annual incidence of thromboembolic complications was 1.6% in total, 1.7% in valvular heart disease (n=128), and 2.1% in non-valvular heart disease (n=117). No thromboembolism occurred in lone AF (n=43), defined as the complete absence of any underlying disease. The type of AF before embolic attack was chronic in 26 cases and paroxysmal in 6 cases. The cardiac rhythm at the time of the embolic attack was AF, except in 2 cases in which ECG was not recorded. In all patients with thromboembolic complications who were receiving antithrombotic therapy during the follow-up, the anticoagulant effect just before the embolic attack was found to be insufficient. Major bleeding was not observed in the patients receiving antithrombotic therapy. Thromboembolism in AF in long-term follow-up tends to occur more frequently in patients with underlying heart disease and in those with chronic AF compared rather than paroxysmal AF; it rarely occurs in lone AF. We should not hesitate to administer sufficient anticoagulant therapy in AF patients who are at high risk of developing thromboembolic complications.     

Acute sacroiliitis as a manifestation of calcium pyrophosphate dihydrate crystal deposition disease. A report of two cases

Whereas radiographic lesions of the sacroiliac joints are common in patients with calcium pyrophosphate deposition crystal disease, they are rarely accompanied with clinical symptoms. We report two cases of acute sacroiliitis probably due to calcium pyrophosphate dihydrate deposition disease. The patients were a 53-year-old man and an 82-year-old woman with chondrocalcinosis in other joints and presence on computed tomography studies of the sacroiliac joints of sclerosis and irregularities of the joint margins with a thin linear calcific deposit within the joint. Both patients recovered fully under therapy with colchicine, analgesics and rest. These two cases suggest that acute sacroiliitis can be caused by calcium pyrophosphate dihydrate crystal deposition disease.     

Critical evaluation of vascular embolization in patients with Rendu-Osler disease

BACKGROUND: As causal therapy of Rendu-Osler-Weber syndrome is not yet possible, there are many different therapeutical approaches. It seemed expedient to investigate the efficacy of super-selective angiography in treating Rendu-Osler-Weber syndrome after this technique proved effective in other therapeutic settings. This method is known to be efficient in controlling tumor bleeding. PATIENTS: Between March 1993 and September 1995, eight patients with an average age of 57 years were treated by super-selective intraarterial embolization. RESULTS: In all cases epistaxis occurred on both sides. Unilateral embolization was performed in ten cases and bilateral embolization in five cases. In two of three patients with nasal packing the embolization permitted removal of the packing. Nevertheless nosebleeds reoccurred on an average of 3.5 days after therapy. Two of eight patients showed long-term improvement after therapy. They suffered fewer nosebleeds than before. Complications included one patient who suffered from partial hemiparesis after embolization, which lasted 1.5 hours and was completely reversible. Another patient died because of pulmonic bleeding caused by manifestation of the disease in this organ. Often patients complained of facial pain or headache which occasionally lasted for a few weeks. CONCLUSIONS: Endovasal embolization obviously is an alternative to artery ligation in emergency cases whereas the long-term success is currently impaired by formation of new anastomoses. Further development of this technique is necessary.     

Burkitt's lymphoma-leukemia in patients treated for Hodgkin's disease

We reviewed all reported cases of Burkitt's lymphoma and/or Burkitt's leukemia (BLL) occurring following therapy for Hodgkin's disease. In addition to the case described in this report, a total of 19 patients have been previously reported. The male/female ratio was 3.75. Treatment for Hodgkin's disease included chemotherapy combined with radiation therapy in 15 patients, chemotherapy in 3 patients, and radiation therapy alone in 1 patient. Median interval between Hodgkin's disease and the diagnosis of BLL was 97 months. Patient characteristics are similar to those with de novo BLL. Bone marrow, abdomen, central nervous system, as well as extranodal organs were commonly involved. Typical cytogenetic translocations seen in patients with primary BLL were found in 6 patients, but 5 of these patients had additional cytogenetic abnormalities. Only 2 patients achieved complete remission after chemotherapy. The mechanism for the development of BLL after treatment for Hodgkin's disease is unknown. Although the majority of cases have been seen in patients treated with combined-modality therapy, the role of previous therapy in causing this complication cannot be assessed in this study.     

Factors associated with the adaptation of parents with a chronically ill child

Improvement and standardization of the conservative therapy of inflammatory bowel disease has lead to a better prognosis for the patients. During the acute flare of Crohn's disease steroids are still the standard therapy, whereas 5-aminosalicylic acid (5-ASA) preparations are used for maintenance therapy during remission. In contrast ulcerative colitis may be treated with 5-ASA also for acute exacerbations. The development of new drugs as for example the topical steroids helps to improve life quality of the patients by reducing adverse side effects. Potent immunosuppressants as azathioprine and methotrexate are useful in chronic active and refractory disease. Cyclosporin A plays a role in severe steroid refractory colitis. In the future immunomodulation by application of antiinflammatory cytokines or antibodies to inflammatory cytokines may have its place in the treatment of IBD patients. In some cases, however, the conservative therapy reaches its limits. Mistakes in the therapy are made, when these limits are not recognized and complications are not discovered in time.     

Immunohistochemical and electrophysiological evidence for omega-conotoxin-sensitive calcium channels in unmyelinated C-fibres of biopsied human sural nerve

Two cases of glomerulonephritis associated with colonization of cerebrospinal fluid shunts inserted for the treatment of hydrocephalus (shunt nephritis) are described and additional 113 cases reported in world literature, are reviewed. Both of our patients were affected by two episodes of shunt nephritis. Complete clinical remission of renal disease followed antibiotic treatment in one patient, and shunt replacement combined with antibiotic therapy in the other. In the 115 cases of shunt nephritis reported to date, the causative organisms, clinical features, pathogenesis, renal histology, treatment, and outcome are discussed in detail. The importance of regular observation for early recognition of this reversible form of glomerulonephritis, is emphasized.