1/23. Metastatic adenocarcinoma masquerading as basal pontine tuberculoma.Tuberculous infection of the central nervous system is common in hong kong. A 39-year-old woman presented with isolated right sixth nerve palsy which was non-progressive for 10 months. Neuro-imaging revealed a right pontine lesion. cerebrospinal fluid (CSF) examination showed lymphocytic meningitis with negative bacteriological and cytological studies. Empirical antituberculous drugs with initial corticosteroid resulted in improved CSF parameters. A diagnosis of cerebral tuberculoma complicated by meningitis was made. She subsequently deteriorated clinically and radiologically. Despite a number of clinical features which were atypical of leptomeningeal metastasis, adenosquamous carcinoma was found on biopsy. Her relatively indolent clinical course might be due to the initial corticosteroid treatment. This report illustrates the importance of early tissue diagnosis in uncertain cases of chronic lymphocytic meningitis.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/23. Bilateral symmetrical enhancing brainstem lesions: an unusual presentation of primary CNS lymphoma.We report a patient with a progressive brainstem syndrome, who on magnetic resonance imaging had large bilateral, symmetrical, contrast-enhancing, infratentorial space-occupying lesions. biopsy of one of the lesions revealed this unusual appearance to be due to a primary central nervous system (CNS) lymphoma of B-cell type. Symmetry of lesions may be a clue to the diagnosis, perhaps reflecting the mechanism by which CNS lymphomas spread.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/23. Extreme lateral supracerebellar infratentorial approach to the posterolateral mesencephalon: technique and clinical experience.OBJECTIVE: Lesions situated posterolaterally along the mesencephalon present neurosurgeons with a special challenge. The midline and paramedian variations of the supracerebellar infratentorial approaches do not adequately expose this region. The subtemporal approach risks injury to the vein of Labbe. An extreme lateral supracerebellar infratentorial approach with more radical resection of bone superiorly and laterally, and skeletonization of the sigmoid and transverse sinuses, was used to approach lesions at this location in eight methods: Five cavernous malformations, two juvenile pilocytic astrocytomas, and one peripheral superior cerebellar artery aneurysm located in this region were approached in eight patients. In this extreme lateral approach, the sigmoid sinus is unroofed more superiorly and the bone flap includes not only a posterior fossa craniotomy but also a portion that extends just above the transverse sinus. The dural opening is based along the transverse and sigmoid sinuses. After the cerebrospinal fluid has been drained, the lateral aspect of the brainstem is approached via the cerebellar surface. A proximal tentorial incision offers additional rostral exposure where needed. RESULTS: Seven patients in this series underwent successful resection of their lesion. The remaining patient's aneurysm was clipped successfully with no major complications. CONCLUSION: The extreme lateral supracerebellar infratentorial approach differs from the midline and paramedian supracerebellar infratentorial variants in the area of exposure, patient positioning, and location of the craniotomy. The technique is effective for approaching the posterolateral mesencephalon.- - - - - - - - - - ranking = 0.17498677273903keywords = malformation (Clic here for more details about this article) |
4/23. Brainstem encephalitis resulting from Epstein-Barr virus mimicking an infiltrating tumor in a child.A case of a child with subacute neurologic features and imaging findings consistent with a brainstem encephalitis that was discovered to be related to a primary central nervous system infection caused by Epstein-Barr virus is presented. A brainstem tumor was initially suspected, but a correct diagnosis was formulated on the basis of the favorable clinical course and the detection of positive Epstein-Barr virus serology. In contrast to a prompt recovery of neurologic signs the neuroimaging alterations persisted for a longer time. The present report emphasizes the possible role of Epstein-Barr virus in the pathogenesis of infectious neurologic disorders in childhood, underlining the unusual presentation of a brainstem encephalitis, and considers the discrepancy between the course of neurologic features and the evolution of imaging alterations.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/23. cerebrospinal fluid oligoclonal IgG bands in patients with spinal arteriovenous malformation and structural central nervous system lesions.OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, israel. patients: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, arnold-chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system.- - - - - - - - - - ranking = 6.3998941819122keywords = nervous system, malformation (Clic here for more details about this article) |
6/23. The juxtaposition of a capillary telangiectasia, cavernous malformation, and developmental venous anomaly in the brainstem of a single patient: case report.OBJECTIVE AND IMPORTANCE: Capillary telangiectasias, cavernous malformations, and developmental venous anomalies are all vascular malformations that occur on the capillary-venous side of the cerebral circulation. The associations of capillary telangiectasias with venous malformations, cavernous malformations with venous malformations, and capillary telangiectasias with cavernous malformations have all been described; however, the association of all three lesions in a single patient is extremely rare. CLINICAL PRESENTATION: A 52 year-old Caucasian woman presented to our clinic with an extended history of confusion, distorted visual perceptions, photophobia, neck pain, swallowing problems, and poor balance. The patient's examination was remarkable for difficulty concentrating, mild rotatory nystagmus, subtle decreased sensation over the left side of the face and body, and brisk reflexes. review of the patient's magnetic resonance imaging examination demonstrated a cavernous malformation, a capillary telangiectasia, and a developmental venous anomaly located adjacent to one another in the brainstem. INTERVENTION: Given the patient's complex constellation of symptoms and relatively mild neurological findings, it was difficult to ascribe any one of them to a specific vascular malformation. Conservative management of this patient's vascular malformations was decided upon. CONCLUSION: Juxtaposition of these three different vascular lesions in the brainstem of an otherwise normal individual suggests a relationship among them. Although there are several theories that link similar associations through physiological mechanisms such as venous hypertension, we propose that a developmental event disrupting local capillary-venous pattern formation is a plausible alternative.- - - - - - - - - - ranking = 2.2748280456073keywords = malformation (Clic here for more details about this article) |
7/23. Cerebellar mutism associated with a midbrain cavernous malformation. Case report and review of the literature.The authors report a case of cerebellar mutism arising from a hemorrhagic midbrain cavernous malformation in a 14-year-old boy. No cerebellar lesion was identified; however, edema of the dorsal midbrain was noted on postoperative magnetic resonance images. Dysarthric speech spontaneously returned and then completely resolved to normal speech. This case provides further evidence for the theory that involvement of the dentatothalamic tracts, and not a cerebellar lesion per se, is the underlying cause of "cerebellar" mutism.- - - - - - - - - - ranking = 0.87493386369513keywords = malformation (Clic here for more details about this article) |
8/23. Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von hippel-lindau disease. Report of four cases.Hemangioblastomas of the central nervous system (CNS) may occur sporadically or in association with von Hippel-Lindau (VHL) syndrome. The authors present four patients with no family history or clinical evidence of VHL syndrome in whom extensive, progressive, en plaque coating of the brainstem and spinal cord with hemangioblastomas developed 1 to 8 years after complete resection of a solitary cerebellar hemangioblastoma. Analysis included detailed physical, biochemical, radiological, and pathological examinations in all four patients, combined with family pedigree analysis. In addition, a detailed investigation of the VHL gene was undertaken. Allelic loss, comparative genomic hybridization (CGH), single-stranded conformational polymorphism screening, CpG island methylation status, and x chromosome inactivation clonality analyses were performed. Although there was no evidence of germline alterations in the VHL gene on clinical and radiological examination or in the family history (all four patients) or analysis of peripheral blood (three patients), somatic deletion of one copy of the VHL gene occurred in these tumors. These findings indicate that the multiple, separate deposits of tumors were likely derived from a single clone. Results of CGH indicate that one or several additional genes are probably involved in the malignant behavior of the hemangioblastomas in these patients. Furthermore, the malignant biological and clinical behavior of these tumors, in which multiple sites of subarachnoid dissemination developed 1 to 8 years after initial complete resection, followed by progressive tumor growth and death of the patients, occurred despite a histological appearance typical of benign hemangioblastomas. Malignant hemangioblastomatosis developed 1 to 8 years after resection of an isolated cerebellar hemangioblastoma. Alterations of the VHL gene may be permissive in this setting, but other genes are likely to be the source of the novel biological and clinical presentation of the disseminated hemangioblastomas in these patients. This appears to represent a novel condition in which the product of one or more mutations in several genes permits malignant tumor behavior despite retention of a benign histological picture, a circumstance previously not recognized in CNS tumors.- - - - - - - - - - ranking = 5keywords = nervous system (Clic here for more details about this article) |
9/23. glioblastoma multiforme of the brain stem in a patient with acquired immunodeficiency syndrome.glioblastoma of the brain stem is rare and there is no description of such a lesion in patients suffering from acquired immunodeficiency syndrome. The majority of intracerebral mass lesions are due either to toxoplasmosis or primary central nervous system lymphomas so that it is usually not included in the differential diagnosis of enhancing lesions of the central nervous system in these patients. A 31-year-old human immunodeficiency virus (hiv) infected man presented with a four months history of slowly progressive deterioration of brainstem associated symptoms despite antitoxoplasmic therapy. magnetic resonance imaging revealed a large ring enhancing lesion in the brainstem. Clinical and neuroradiological data could not establish a proper diagnosis and a stereotactic serial biopsy was undertaken. Histological examination of the specimen showed a glioblastoma multiforme (GBM) as the first reported case of GBM located in the brainstem in an acquired immunodeficiency syndrome (AIDS) patient. Patient management and effectiveness of stereotactic serial biopsy are discussed.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
10/23. Surgical management of brainstem hemangioblastomas in patients with von hippel-lindau disease.OBJECT: Hemangioblastomas of the brainstem constitute 5 to 10% of central nervous system (CNS) tumors in patients with von Hippel-Lindau (VHL) disease. At present, optimal management of brainstem hemangioblastomas associated with VHL disease is incompletely defined. In an attempt to clarify some of the uncertainty about the operative treatment of these lesions and its outcome, the authors reviewed all cases of VHL disease in which resection of brainstem hemangioblastomas was performed at the National Institutes of health during a 10-year period. methods: Twelve consecutive patients with VHL disease (six male and six female patients [mean age 31.7 /- 9 years; range 15-46 years]) who underwent 13 operations to remove 17 brainstem hemangioblastomas were included in this study (mean follow-up period, 88.4 /- 37.4 months; range 37-144 months). Serial examinations, hospital charts, magnetic resonance images, and operative records were reviewed. To evaluate clinical course, clinical grades were assigned to each patient before and after surgery. Preoperative neurological function was the best predictor of long-term outcome. In addition, patients who underwent CNS surgeries for hemangioblastomas were more likely to improve or to remain neurologically stable. Tumor or cyst size, the presence of a cyst, or the location of the tumor (intramedullary, extramedullary, or mixed; posterior medullary, obex, or lateral) did not affect outcome. No patient was neurologically worse after brainstem surgery. At long-term follow-up review (mean 88.4 months), only one patient had declined neurologically and this was due to the cumulative neurological effects caused by eight additional hemangioblastomas of the spinal cord and their surgical treatment. CONCLUSIONS: Brainstem hemangioblastomas in patients with VHL disease can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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