1/30. Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
2/30. Nasopharyngeal extension of a craniopharyngioma in a 4 year old girl.Nasopharyngeal extension of a craniopharyngioma is very rare and usually presents with headache, nasal obstruction and visual disturbances. We present a case of a 4 year old girl, who became symptomatic with visual deterioration. MRI showed a huge supra - and infrasellar cystic craniopharyngioma with extension into the sphenoid sinus. Primary treatment was a transnasal puncture of the cyst followed by a subfrontal approach with removal of the tumour preserving the chiasm and optic nerves. The visual acuity postoperatively improved while she needed hormone replacement due to panhypopituitarism. Follow-up 12 months after the operation showed no recurrence. This is the youngest patient of about 27 patients reported so far in the literature.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
3/30. Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches.OBJECTIVE: Superficial siderosis of the central nervous system (CNS) is a rare chronic progressive disorder caused by chronic subarachnoid hemorrhage. We present four patients with superficial siderosis of the CNS to describe the characteristic symptoms, and to discuss the pathogenetic heterogeneity and possible new therapeutic approaches. RESULTS: The causes of chronic subarachnoid bleeding in superficial siderosis were different. In two patients surgical treatment of ependymoma or cerebral cavernomas were the underlying diseases. No cause was detected in one patient. For the first time, we present one patient with vasculitis of the central nervous system associated with systemic hemochromatosis in superficial siderosis. Therapeutic approaches included exstirpation of cavernomas as the source of chronic bleeding in one patient, immunosuppressive therapy and venupunctures in the patient with vasculitis and hemochromatosis, and symptomatic treatment with chelating agents and antioxidants. The patients remained clinically stable for the follow-up period of up to 2 years. CONCLUSIONS: Our cases underline the pathogenetic heterogeneity of superficial siderosis and favor the early diagnosis for prompt initiation of therapy. Besides treatment of the underlying condition, antioxidants and radical scavengers may be effective in halting the progression of the disease.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
4/30. Primary nodular meningeal glioma mimicking metastatic tumor of the cerebellum with diffuse infra- and supratentorial leptomeningeal spread.A 62-year-old man presented with acute headache, blurred and double vision, nausea, and ataxia. magnetic resonance imaging showed an enhancing mass on the inferior aspect of the right cerebellar hemisphere as well as a thin, widespread leptomeningeal enhancement and T2-weighted hyperintense lesions in the left occipital lobe and both thalami. Lumbar puncture revealed clusters of anaplastic cells. Therefore, metastatic tumor of unknown origin was suggested. Despite whole brain irradiation and intrathecal chemotherapy the patient deteriorated gradually and died four months later. Post-mortem examination of the brain revealed a nodular, high-grade astrocytic tumor within the subarachnoid space on the lower portion of the right cerebellar hemisphere. Diffuse leptomeningeal spread was noted, but cerebellar parenchyma was not infiltrated.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
5/30. Metastatic carcinoma: an unusual cause of focal brain lesions in hiv infection.The most common causes of focal brain lesions (FBLs) in patients with hiv infection are cerebral toxoplasmosis, primary central nervous system lymphoma (PCNSL), and progressive multifocal leukoencephalopathy. neoplasms other than PCNSL are uncommon. We report a rare case of metastatic carcinoma causing an FBL in a patient with hiv infection. The diagnostic workup and further management of FBLs in hiv are outlined in this review. The standard approach includes a lumbar puncture and cerebrospinal fluid (CSF) analysis for cytology and Epstein-Barr virus (EBV) dna testing by polymerase chain reaction. Empiric therapy for PCNSL is justifiable for patients with positive CSF EBV-dna test results and a positive single-photon emission computed tomography (SPECT) scan, especially if there has been no response to antitoxoplasmosis therapy. brain biopsy may be indicated, however, in select cases that do not meet these criteria in order to identify potentially treatable infections and PCNSL.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
6/30. Intratumoral methotrexate kinetics in a patient with intracranial anaplastic ependymoma.Little is known about intratumoral anticancer drug concentration in childhood brain tumors. We were able to measure methotrexate (MTX) tumor concentrations directly in a cystic anaplastic ependymoma. cyst fluid was obtained by puncture of a subgaleal Rickham reservoir connected to a catheter in the tumor cyst. MTX concentrations were determined by fluorescence polarization immunoassay and compared to serum concentrations. Maximum MTX concentrations in tumor and CSF were found at the end of MTX infusion. Twenty-four hour after MTX infusion the mean tumor concentration was significantly higher than in the serum indicating MTX retention and accumulation in the tumor cyst. An AUC(tumor)/AUC(serum) ratio of 1.95 was obtained. In response to the applied multiagent chemotherapy the clinical condition of our patient improved and the tumor showed partial response on MRI. Cystic ependymomas might benefit from high dose MTX especially because of drug retention in the tumor cyst.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
7/30. Pseudouveitis: a clue to the diagnosis of primary central nervous system lymphoma in immunocompetent patients.Primary oculocerebral non-Hodgkin lymphoma (NHL) of the immunocompetent patient is associated with significant morbidity and mortality, but early diagnosis and follow-up may improve prognosis. The eye, anatomically and embryologically part of the central nervous system (CNS), can be the primary site of the lymphomatous process. In patients with symptoms of atypical uveitis, vitrectomy can be of great help for early diagnosis of primary central nervous system lymphoma. We retrospectively reviewed the diagnostic features, treatment, and evolution of 10 patients with primary central nervous system lymphoma who presented with symptoms of pseudouveitis. The patients complained of chronic vitreal opacities, increasing with time. These symptoms contrasted with the absence of the usual signs of inflammation of the anterior segment or of the retina, which characterize true uveitis. vitrectomy was proposed after lumbar puncture and cerebral magnetic resonance imaging. Six vitrectomies were carried out, 3 patients had a stereotaxic biopsy, and 1 patient had a cardiac biopsy. A pathologic diagnosis of large B-cell lymphoma was made on vitrectomy specimens in 100% of the patients who had this procedure. The mean time from onset of ocular symptoms to diagnosis was 24 months. This series was characterized by a rare systemic dissemination of the NHL (negative in 80%), a strong preponderance of B-cell NHL, and the absence of association with Epstein-Barr virus (EBV) among these immunocompetent patients. To our knowledge, this series includes the only reported case of oculocardiac lymphoma. Meningeal dissemination appeared to be associated with a poor prognosis. Neurologic complications of treatment combining radiotherapy and methotrexate were significant among patients older than 60 years of age. The current study suggests that primary central nervous system lymphoma should be suspected in patients with pseudouveitis, and that the diagnosis can be established quickly and without side effects by vitrectomy. These patients should be followed carefully in order to detect meningeal dissemination.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
8/30. Cerebral relapse of metastatic gastrointestinal stromal tumor during treatment with imatinib mesylate: case report.BACKGROUND: The management of unresectable or metastatic gastrointestinal stromal tumors (GISTs) has previously been difficult as they are resistant to conventional chemotherapy and radiation. The development of imatinib mesylate has made a major impact on the management of advanced GISTs. It is apparent that there are sanctuary sites such as the central nervous system where imatinib does not achieve adequate concentrations. We describe the case of a man with metastatic GIST who experienced multiple cerebral relapses of disease while systemic disease progression appeared to be controlled by imatinib. CASE PRESENTATION: A 47-year-old man presented in July 1999 with a jejunal GIST with multiple hepatic metastases. The jejunal primary was resected and after unsuccessful cytoreductive chemotherapy, the liver metastases were also resected in December 1999. The patient subsequently relapsed in August 2001 with symptomatic hepatic, subcutaneous gluteal, left choroidal and right ocular metastases all confirmed on CT and PET scanning. biopsy confirmed recurrent GIST. MRI and lumbar puncture excluded central nervous system involvement. The patient was commenced on imatinib 400 mg bd in September 2001 through a clinical trial.The symptoms improved with objective PET and CT scan response until December 2002 when the patient developed a right-sided foot drop. MRI scan showed a left parasagittal tumor which was resected and confirmed histologically to be metastatic GIST. Imatinib was ceased pre-operatively due to the trial protocol but recommenced in February 2003 on a compassionate use program. The left parasagittal metastasis recurred and required subsequent re-excision in September 2003 and January 2004. Control of the systemic GIST was temporarily lost on reduction of the dose of imatinib (due to limited drug supply) but on increasing the dose back to 800 mg per day, systemic disease was stabilized for a period of time before generalised progression occurred. CONCLUSION: This case illustrates that the brain can be a sanctuary site to treatment of GISTs with imatinib. Maintaining dosing of imatinib in the face of isolated sites of disease progression is also important, as other metastatic sites may still be sensitive.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
9/30. Optic neuropathy due to anaplastic large cell lymphoma.PURPOSE: To report a case of anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) producing an optic neuropathy. methods: Observational case report. RESULTS: A 29-year-old male presented with new onset headaches. Magnetic resonance imaging (MRI) of the brain revealed a large enhancing parietal lobe mass. Ocular exam at that time was normal. Initial diagnoses included possible bacterial cerebritis and fungal abscess. Serial lumbar punctures showed increased white blood cells but cytology was negative. A brain biopsy was non-diagnostic. The patient then presented with a left optic neuropathy. Repeat MRI of the brain and orbits revealed infiltration of the clivus and left orbital apex including the optic nerve. The patient had elevated liver function studies and an abdominal ultrasound disclosed two hypoechoic lesions. liver biopsy confirmed the diagnosis of ALK-1 positive ALCL. The patient was treated with chemotherapy but expired seven months after the initial presentation. CONCLUSION: ALCL should be considered to be a very rare but potential cause of optic neuropathy. To our knowledge, this is the first reported case of ALCL causing an optic neuropathy.- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
10/30. A negative cranial computed tomographic scan is not adequate to support a diagnosis of pseudotumor cerebri.A 10-year-old boy with daily headache for 1 month and intermittent diplopia for 1 week was found to have a unilateral partial abducens palsy and bilateral papilledema; otherwise, his neurologic examination showed no abnormalities. A cranial computed tomographic (CT) scan was normal. Lumbar puncture disclosed a markedly elevated opening pressure of > 550 mm of cerebrospinal fluid with normal cerebrospinal fluid. Medical therapy with acetazolamide for presumed pseudotumor cerebri was begun. magnetic resonance imaging (MRI) of the brain, done several days later because of continuing symptoms, unexpectedly showed multiple hyperintensities of cerebral white matter on T2-weighted and fluid-attenuated inversion recovery images. Despite high-dose intravenous methylprednisolone for possible demyelinating disease, he failed to improve. A left temporal brain biopsy followed and disclosed an anaplastic oligodendroglioma. In a patient with features indicating pseudotumor cerebri, a negative cranial CT scan is not adequate to rule out underlying pathology; thus, MRI of the brain should probably always be performed. A revised definition of pseudotumor cerebri could better include "normal MRI of the brain" rather than "normal neuroimaging."- - - - - - - - - - ranking = 1keywords = puncture (Clic here for more details about this article) |
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