What is and what is not 'Fahr's disease'

Bilateral almost symmetric calcification involving striatum, pallidum with or without deposits in dentate nucleus, thalamus and white matter is reported from asymptomatic individuals to a variety of neurological conditions including autosomal dominant inheritance to pseudo-pseudohypoparathyroidism. While bilateral striopallidodentate calcinosis is commonly referred to as 'Fahr's disease' (a misnomer), there are 35 additional names used in the literature for the same condition. Secondary bilateral calcification is also reported in a variety of genetic, developmental, metabolic, infectious and other conditions. In autosomal dominant or sporadic bilateral striopallidodentate calcinosis no known calcium metabolism abnormalities are known to date. Clinically, parkinsonism or other movement disorders appear to be the most common presentation, followed by cognitive impairment and ataxia. When presence of movement disorder, cognitive impairment and ataxia are present, a computed tomography scan of the head should be considered to rule-in or rule-out calcium deposits. Calcium and other mineral deposits cannot be linked to a single chromosomal locus. Further genetic studies to identify the chromosomal locus for the disease are in progress.     

Phase II study of temozolomide and cisplatin as primary treatment prior to radiotherapy in newly diagnosed glioblastoma multiforme patients with measurable disease. A study of the Spanish Medical Neuro-Oncology Group (GENOM)

This phase II study evaluates the activity of temozolomide and cisplatin administered before radiation therapy in newly diagnosed glioblastoma multiforme patients, in terms of response, time to progression and survival.Patients and methods: Forty patients with measurable disease after surgery, a Karnofsky status> 60, and Barthel Index> 10 were included. They were treated with three cycles of temozolomide 200 mg/m²/day for 5 days and cisplatin 100 mg/m² on day 1. Conventional focal radiation therapy to 60 Gy was administered after response evaluation.Results: Three patients were not evaluable for central reviewed response but all 40 were evaluable for toxicity, time to progression and survival. Objective responses by Macdonald criteria on an intent to treat basis were 45% including complete response in three patients (7.5%), and partial response in 15 patients (37.5%). Responses were seen in biopsy-only patients (33.4%) as well as in partial surgery patients (52%). Median survival for all patients was 12.5 months. Biopsy-only patients had a median survival of 12.8 months. Grade 3 to 4 neutropenia was the most important toxicity, and occurred in 37.5% of patients. A delay in 18.2% and a dose reduction in 9.6% of cycles were necessary due to myelosuppression on day 28. Two patients had neutropenic fever resulting in one treatment-related death. Eighty-two percent of patients received radiotherapy.Conclusion: This regimen has significant activity, as it induces objective responses even in biopsy-only patients, appearing to improve their median survival. A better combination schedule is needed to improve the toxicity profile.     

The biology of non-small-cell lung cancer: identifying new targets for rational therapy

Lung cancer, and in particular non-small-cell lung cancer (NSCLC), remains the leading cause of cancer death throughout the world. Almost three decades ago, the major concern was to identify whether cisplatin or cisplatin-based chemotherapy enhanced survival in metastatic NSCLC, and whether any survival benefit compensated for cisplatin-related toxicity. Over the last 10 years, significant advances have been achieved in molecular biology, including the identification of critical genes related to the pathogenesis of NSCLC, which have formed the basis for new targeted therapeutic approaches. These new approaches include novel agents against established chemotherapeutic targets such as thymidylate synthetase as well as agents that inhibit novel targets such as growth factor receptors and proteins important in angiogenesis. With the advent of genomic technologies that can identify patterns of gene expression, the hope is that therapy will be tailored to the genetic pattern of the patients's tumor, and individualized treatments that minimize toxicity and maximize efficacy can be developed.     

Methotrexate for cervical pregnancy. A case report

BACKGROUND: The incidence of cervical pregnancy is 1:1,000-95,000 pregnancies and represents < 1% of all ectopic pregnancies. Evacuation of the pregnancy by curettage does not always stop the bleeding because there is little contractile muscle in the cervix. If there is uncontrollable bleeding, hysterectomy is necessary. In selected cases, nonsurgical management offers high success rates. Among medical treatments, the most common is systemic or local administration of methotrexate. CASE: A 36-year-old woman, gravida 2, para 2, presented with vaginal bleeding and subacute pain in the lower abdomen. The patient was 7 weeks' pregnant according to her last menstrual period. Transvaginal ultrasonography showed a gestational sac of approximately 5 weeks' gestational age implanted in the wall of the cervix. Fifty milligrams of methotrexate was injected into the gestational sac and another 50 mg administered intramuscularly. The hCG level decreased continuously and was no longer detectable after 35 days. CONCLUSION: The use of methotrexate for cervical pregnancy is safe and effective and preserves fertility.     

The comparison of clinical and hormonal parameters in PCOS patients treated with metformin and GnRH analogue

In this study, our aim was to compare the effects of metformin and gonadotropin releasing hormone (GnRH) analogues in clinical and hormonal parameters in women with polycystic ovary syndrome (PCOS). There were 50 women with PCOS who were included in our study and they were divided into two groups. In this randomized trial, metformin (850 mg, two times per day) was administered to the first group and GnRH analogue (goserelin 3.6 mg, every 28 days) was given to the second group for 3 months. Because of the previous treatments, PCOS patients served as their own controls. The results of 42 women who completed the study were evaluated. Insulin resistance was not ascertained in patients. Metformin treatment resulted in a significant decline in mean body mass index, body weight, circumferences of waist and hip and total hirsutismus score. There was a significant decrease in luteinizing hormone (LH) levels and a significant increase in follicle stimulating hormone (FSH), progesterone, and sex hormone binding globulin (SHBG) concentrations. No changes in fasting glucose and insulin levels were observed. The GnRH analogue resulted in a significant increase in FSH and SHBG levels and a significant decrease in LH, total testosterone, dehydroepiandrosterone sulfate (DHEAS) levels and LH to FSH ratio and an improvement in hirsutism scores. Metformin and GnRH analogues had effects upon the abnormal steroid-gonadotropin metabolism and the clinical findings of hyperandrogenism with different mechanisms.