Primary lymphoma of the central nervous system

Eleven patients with primary lymphoma of the central nervous system were seen in the King Faisal Specialist Hospital and Research Centre, Saudi Arabia, between 1986 and 1992. None had previously received immunosuppressive therapy. All cases were confirmed by biopsy and histopathological studies. Of the eleven patients, six had debulking of the tumour, seven received radiation therapy and six received chemotherapy. This report confirms the very poor prognosis for patients with primary lymphoma of the central nervous system, with only a few long-term survivors.     

Primary central nervous system lymphoma. A changeable tumor

We have previously shown that lymphocyte beta 2-adrenoceptors (AR) are under cyclical control of sex-steroid hormones with greater receptor density during the luteal phase of the menstrual cycle. It has also been postulated that abnormal cyclical regulation of beta 2-AR might be a possible mechanism for premenstrual asthma. The effects of exogenous female sex-steroid hormones on lymphocyte beta 2-AR function were studied in eight normal healthy females. They were evaluated at two successive menstrual cycles, during the follicular phase (day 1-6). They were randomized to receive single oral doses of either ethinyloestradiol 50 micrograms or medroxyprogesterone 10 mg in a cross-over study. Lymphocyte beta 2-AR parameters were evaluated at baseline (t0), 24 h (t24) and 72 h (t72) after ingestion. Baseline levels of progesterone and oestradiol were comparable on both cycles. Receptor density (Bmax) increased significantly (P < 0.01) from t0 after progesterone but not oestradiol at t 4: a 1.39-fold geometric mean difference (95% CI 0.96-2.00) between t24 vs t0. Receptor affinity (kd) and maximal cAMP response to isoprenaline (Emax) were not altered by either treatment. These results show that exogenous progesterone but not oestradiol, given during the follicular phase, significantly increased beta 2-AR. This, therefore, suggests that endogenous progesterone is probably responsible for previously observed increase in Bmax during the luteal phase of the female menstrual cycle. These findings may suggest possible therapeutic strategies for modulation of beta 2-AR in premenstrual asthma.     

Primary malignant rhabdoid tumor of the central nervous system

Luteinizing hormone (LH) induces progesterone production in hen ovarian granulosa cells, and this induction is inhibited when chloride ions are removed from the culture medium. This suggests that chloride channels may be involved in the signal transduction pathway responsible for the LH-induced progesterone production. In this report, we examined effects of LH on plasma membrane ion currents in single granulosa cells isolated from the largest preovulatory follicle (Fl) of the hen (Gallus domesticus). Using the perforated patch whole cell voltage clamp technique, we found that addition of LH rapidly activated a chloride current in these cells. This chloride current was present at all voltages tested (-90 to +50 mV), showed outward rectification and showed no obvious time or voltage dependence. Its magnitude was 3.5-fold that of the total resting membrane current measured before LH treatment. LH is known to elevate cyclic AMP in these cells. We found that addition of the cAMP analog Sp-cAMPS mimicked LH in inducing chloride currents in these cells. We conclude that LH can activate a chloride conductance in granulosa cells, and that this action may be mediated by cAMP.     

Multifocal primary ocular and central nervous system malignant lymphoma

A patient had bilateral posterior uveitis of two weeks' duration and rapid neurologic deterioration. Obstructive hydrocephalus developed as a result of compression of the fourth ventricle by an enlarged cerebellar vermis. Autopsy revealed multiple foci of infiltration by malignant lymphomas in the eyes, brain, and cerebellum; no evidence of systemic lymphoma was found.     

Primary central nervous system lymphoma: age and performance status are more important than treatment modality

PURPOSE: To assess prognostic factors and treatment modalities of patients with primary central nervous system lymphoma (PCNSL) in terms of response rates, patterns of failure and overall survival. METHODS AND MATERIALS: Sixty-two patients presenting with PCNSL between 1982 and 1994 at Peter MacCallum Cancer Institute with no evidence of human immunodeficiency virus infection were included in the study. Their median age was 60 years; World Health Organisation (WHO) performance status was > or = 2 in 85%. All patients were planned to receive whole brain irradiation; 7 also received spinal irradiation. The median planned dose to the target volume was 50.4 Gy. Twenty patients were planned to receive chemotherapy as well. Patients were followed up to June 20, 1995, giving a median follow-up for 14 surviving patients of 5.4 years, range 0.3 to 10.2 years. RESULTS: The clinical response rate to treatment was 77% [95% confidence interval (CI) 65 to 87%]. The estimated median overall survival was 20.6 months (CI 12.4 to 33.4 months). On univariate analysis male gender, age <60 years, WHO performance status < or = 1, treatment to the target volume > or = 45 Gy, and treatment with additional chemotherapy, were associated with a significantly better overall survival (p < 0.05). On multivariate analysis only age and performance status remained significant prognostic variables. Relapse involved the central nervous system or cerebrospinal fluid (CSF) in all patients with known sites of relapse except three who had ocular relapse only. There was a low incidence of relapse in the initial brain site (23% of known cases) and a high incidence (50%) of CSF/spinal cord relapse. Of 48 deaths, 15 were related to initial or subsequent treatment. CONCLUSIONS: Patient outcome is strongly influenced by age and performance status. Studies suggesting better survival for patients treated with chemoradiation may reflect patient selection rather than treatment variables. Optimal management remains to be defined. The high CSF/spinal relapse rate deserves particular attention.     

Primary Langerhans' cell histiocytosis of the central nervous system with fatal outcome. Case report

An unusual case of primary parenchymal Langerhans' cell histiocytosis of the central nervous system is reported. The definitive diagnosis was obtained by ultrastructural detection of Birbeck granules and by immunohistochemical evidence of CD1a expression. Despite complete surgical resection, there was an early recurrence with multiple central nervous system metastases leading to a fatal outcome.     

Primary central nervous system lymphoma in a child with acute B-cell lymphoblastic leukaemia: consecutive Epstein-Barr virus-related malignancies

OBJECTIVE: The aim of the study was to evaluate the optimization of injection rates with an automatic power injector versus manual injection for contrast-enhanced breath-hold three-dimensional (3D) MR angiography of the abdominal aorta and its branches. SUBJECTS AND METHODS: In a prospective study, 50 patients underwent breath-hold 3D MR angiography (5/2 [TR/TE]; flip angle, 30 degrees) of the abdominal vessels on a 1.5-T system. Each patient received 0.15 mmol/kg of gadopentetate dimeglumine. All patients were randomly assigned to one of five equally sized groups. The contrast bolus was injected manually in group 1, always by the same investigator, who tried to perform a steady injection rate of 2 ml/sec. An automatic injector was used in groups 2-5 with injection flow rates of 0.5 ml/sec, 2 ml/sec, 4 ml/sec, and 6 ml/sec. The start of the MR sequence was tailored individually to the applied volume of contrast material after determination of circulation times by a test bolus. We measured the signal-to-noise and contrast-to-noise ratios as well as the relative vascular enhancement. The visualization of different abdominal vessel segments was independently ranked on a scale of 1-5 (1 = not visible; 5 = excellent visualization) by three reviewers who were unaware of the applied contrast material injection rate. RESULTS: The signal-to-noise and contrast-to-noise ratios of groups 3 and 4 (2 ml/sec and 4 ml/sec, respectively) were significantly (p < .05) higher than the ratios of groups 1, 2, and 5. The average relative vascular enhancement of groups 3 and 4 was significantly higher (p < .05) than the enhancement of all other groups. The contrast bolus applied with a faster injection rate (group 5) did not cover large parts of the K-space, resulting in increased blurring of the vessel contours. The subjective evaluation of large and small diameter vessels showed significantly better results in groups 3 and 4 than in groups 1, 2, and 5. CONCLUSION: The use of an automatic MR power injector proved superior to manual injection of contrast material. The optimal injection rate was 2 ml/sec for 3D breath-hold MR angiography of the abdominal vessels.     

Intravenous methotrexate for primary central nervous system non-Hodgkin's lymphoma in AIDS

OBJECTIVE: To evaluate high-dose intravenous methotrexate in primary central nervous system (CNS) lymphoma in HIV-infected patients. DESIGN: An uncontrolled pilot trial. SETTING: An infectious diseases department in Paris, France. PATIENTS: All consecutive AIDS patients with primary CNS lymphoma attending the same unit from August 1994 to March 1996. INTERVENTIONS: Methotrexate was intravenously administered at a dose of 3 g/m2 every 14 days with leucovorin rescue. A maximum of six cycles was planned. Steroids were given to all patients and haematological growth factors were administered as required. MAIN OUTCOME MEASURES: Rate of response, time to response and survival. RESULTS: Fifteen patients (10 with histological documentation) were recruited. The median time since clinical onset was 27 days (range, 7-69 days), median Karnofsky score was 51 (range, 30-70), and mean CD4+ cell count was 30 +/- 19 x 10(6)/l (range, 7-69 x 10(6)/l). Complete responses, defined as clinical improvement and disappearance of contrast-enhancing brain abnormalities on computed tomography or magnetic resonance imaging, were obtained in seven out of 15 patients (three out of 10 patients with histological diagnosis and four out of five patients without histological confirmation). The Karnofsky score of these seven patients improved to 80 +/- 10 (range, 70-100). The mean time taken to respond was 62 +/- 20 days (range, 45-90 days). One patient relapsed at 6 months. Six patients failed to respond, and two died of severe sepsis on days 15 and 45. The median survival time was 290 days (range, 11-570 days): 73 days (range, 11-570 days) in the 10 patients with histological diagnosis, and 347 days (range, 286-409 days) in the five patients without histological confirmation. Side-effects occurred in 10 patients, with gastrointestinal disorders in five, mucositis and skin rash in two, and fever in three patients; however, these events were mild and did not require cycle postponement or dose changes. No cognitive dysfunction occurred. CONCLUSION: Methotrexate appears to be an attractive alternative to radiation therapy for primary CNS lymphoma and is associated with a far greater improvement in quality of life relative to historical series of radiation therapy.     

Combined therapy for primary central nervous system lymphoma in immunocompetent patients

A retrospective series of 13 immunocompetent patients with histological diagnosis of primary central nervous system lymphoma (PCNSL) is presented. The series was divided into Group A, 6 patients treated with radiotherapy alone, and Group B, 7 patients treated with chemotherapy and radiotherapy. Clinicopathological patterns were similar for the two groups. In Group A, 4 patients achieved complete remission after radiotherapy (45-59.4 Gy) but relapsed within 9 months and died within 21 months of diagnosis. 4 Group B patients received chemotherapy followed by radiotherapy, and three who received a methotrexate-containing regimen are alive and disease-free at 34, 42 and 45 months, while the fourth died after 11 months. The other 3 subjects in this group were treated with radiotherapy followed by chemotherapy, and died within 15 months of diagnosis. Although radiotherapy is the standard treatment, chemotherapy has potentially an important role in the management of PCNSL. The sequence of combined treatment could be crucial to improvement of outcome.     

MR findings of primary central nervous system lymphoma in a child. A case report

Huntington's disease is an autosomal dominant, inherited disorder that results in progressive degeneration of the basal ganglia (especially the neostriatal caudate nucleus and putamen) and other forebrain structures and is associated with a clinical profile of movement, cognitive and psychiatric impairments for which there is at present no effective therapy. Neuropathological, neurochemical and behavioral features of the disease can all be reproduced in experimental animals by local injection of excitotoxic or metabolic toxins into the neostriatum. All these features of the disease can be alleviated, at least in rats, by transplantation of embryonic striatal tissue into the degenerated striatum, which was the basis for commencing the first clinical trials of striatal transplantation in Huntington's patients. However, although rat striatal xenografts may temporarily reduce apomorphine-induced dyskinesias in monkeys, there has been no demonstration that allograft techniques that work well in rats translate effectively to the much larger differentiated striatum of primates. Here we demonstrate good survival, differentiation and integration of striatal allografts in the primate neostriatum, and recovery in a test of skilled motor performance. Long-term graft survival in primates indicates probable success for clinical transplants in Huntington's disease; in addition, our data suggest that graft placement has a direct influence on the pattern and extent of functional recovery.     

Malignant non-Hodgkin lymphoma of the central nervous system with ocular site. Value of vitrectomy for diagnosis

Diagnosis of intraocular lymphoma is difficult and should be considered in middle-aged or older patients with a chronic uveitis. Many explorations (lumbar punctures, brain imaging and vitrectomies) may be needed before correct diagnosis is made. We report the case of a 50-year-old woman with an intraocular lymphoma for whom the diagnosis was confirmed by a vitrectomy.     

Atypical central nervous system lymphoma at the cranial base: report of four cases

OBJECTIVE AND IMPORTANCE: Primary central nervous system lymphoma is a disease with increasing incidence. Atypical presentations are becoming more frequent. CLINICAL PRESENTATION: Three patients bearing cavernous sinus lesions presented initially with periorbital pain and diplopia. Tolosa-Hunt syndrome was the initial presumptive diagnosis for two patients, and meningioma was the diagnosis for the third patient. A fourth patient presented with left ear pain, and a mass in the left internal auditory canal was thought to represent an acoustic neuroma. INTERVENTION: For all four patients, an operative pathological diagnosis was obtained and was compatible with central nervous system lymphoma. All patients were treated with osmotic blood-brain barrier disruption with intra-arterial chemotherapy using a methotrexate-based regimen. CONCLUSION: All four cases included atypical presentations of lymphoma. These cases again illustrate that a correct diagnosis cannot be obtained based only on imaging and clinical findings.     

替莫唑胺联合利妥昔单抗对复发的老年原发中枢神经淋巴瘤的应用

Objective: The aim of our study was to analyze the long-term results of rituximab combined with temozolomide in treatment of elderly patients (> 60 years) with relapsed primary central nervous system lymphoma (PCNSL). Methods:Twelve postoperative elderly patients (> 60 years) were treated between August 2004 and October 2009. Temozolomide 100 mg/m2 to 200 mg/m2 days 1 to 7 and 15 to 21 and rituximab 375 mg/m2 days 1, 5, 8, 22. The maximum number of rituximab cycles was two. After one or two cycles of this combination, patients with an objective response and an acceptable level of toxicity continued treatment with single agent temozolomide (days 1 to 5, every 28 days). The overall survival was analyzed by using Kaplan-Meier. Results: The overall survival was 9 months. Toxicity was very mild with no grade 3-4 neurotoxicity toxic events. Conclusion: Rituximab combined with temozolomide seems to yields substantial long-term survival with moderate toxicity for the treatment of elderly relapsed PCNSL.     

Primary central nervous system tumors: advances in knowledge and treatment

The ability to diagnose, monitor, and treat CNS tumors has been improved by new imaging techniques such as positron emission tomography (PET) scanning and functional MR imaging, stereotactic surgery, delivery of radiotherapy with brachytherapy and radiosurgery, and novel methods for delivering chemotherapy. These innovations combined with the new information about tumor pathogenesis and behavior revealed by molecular research give hope that more specific treatments for malignant CNS tumors will be developed in the future.     

Schizophrenia and central nervous system pathology.

The Rorschach protocols of 3 groups of patients, typical process schizophrenics, atypical or reactive schizophrenics, and schizophrenics with known central nervous system pathology were examined for incidence of Piotrowski's 10 signs of organic involvement. It was found that organic and process groups could not be distinguished from each other, but both of these groups showed significantly more subjects with at least five signs than did the reactive group. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Risk factors, treatment, and outcome of central nervous system recurrence in adults with intermediate-grade and immunoblastic lymphoma

Effects of gastrocnemius glycogen (Gly) concentration on changes in transverse relaxation time (T2; ms) were studied after 5-min plantar flexion at 25% of maximum voluntary contraction (MVC). Gastrocnemius Gly, phosphorus metabolites, and T2 were measured in seven subjects by using interleaved 13C/31P magnetic resonance spectroscopy (MRS) at 4.7 T and magnetic resonance imaging (MRI; 1.5 T). After baseline MRS/MRI, subjects exercised for 5 min at 25% of MVC and were reexamined (MRS/MRI). Subjects then performed approximately 15 min of single-leg toe raises (50 +/- 2% of MVC), depleting gastrocnemius Gly by 43%. After a 1-h rest (for T2 return to baseline), subjects repeated the 5-min protocol, followed by a final MRI/MRS. After the initial 5-min protocol, T2 values increased by 5.9 +/- 0.8 ms (29.9 +/- 0.4 to 35.8 +/- 0.6 ms), whereas Gly did not change significantly (70.5 +/- 6.8 to 67.6 +/- 7.4 mM). After 15 min of toe raises, gastrocnemius Gly was reduced to 40.4 +/- 5.3 mM (P 相似文献   

Tumors of the central nervous system

Neoplasia of the central nervous system (CNS) can be divided into two main categories: nonpituitary CNS neoplasia and pituitary adenomas. Nonpituitary CNS neoplasias are generally compressive in nature, although some are also invasive. The majority of reported CNS tumors are secondary with only a few originating from nervous tissue. Pituitary adenomas predominantly occur in the pars intermedia of the older horse. Clinical signs, diagnostic testing, and possible treatments are discussed.     

Remyelination of the central nervous system

The three typical stages in the clinical course of multiple sclerosis (relapse, persistent disability and progression) can be explained on the basis of inflammation, demyelination and failure of repair leading to axon degeneration and astrocytosis. Strategies are being evaluated for limiting the inflammatory process using immunological treatments and these may have unexpected dividends in promoting endogenous remyelination. Increasing knowledge on glial lineages and axon-glial interactions needed for stable myelination also offer the prospect for enhancing remyelination through growth factor therapy and cell implantation.     

非霍奇金淋巴瘤中枢神经系统累及的诊断和预防

非霍奇金淋巴瘤(NHL)患者中枢神经系统(CNS)累及预后不良,其中位生存期2～6个月.与NHLCNS累及相关参数是年轻、进展期、累及结外部位数、乳酸脱氢酶(LDH)增高和国际预后指标(IPI)积分.最有希望的治疗为自体造血于细胞移植,可延长中数生存期10～26个月.处于CNS侵袭高危状态的某些NHL亚型患者需要早期进行CNS预防,如伯基特淋巴瘤(BL)和淋巴母细胞淋巴瘤(LBL).弥漫性大B细胞淋巴瘤(DLBCL)初期治疗时是否需应用CNS预防久有争议,因为它属于CNS累及(≈5%)的低危群体.危险模式的确定有助于预示NHL的CNS复发.