Functional müllerian tissue within the conus medullaris generating cyclical neurological morbidity in an otherwise healthy female.

PURPOSE: Endometriosis is a common disease; however, ectopic müllerian tissue within the spine is a rare entity with the potential for producing significant neurological compromise. There are several postulated etiologies for this phenomenon, and only a few case reports are available in the world literature. Knowledge of this rare phenomenon is of paramount importance, since early diagnosis can lead to lessened neurological morbidity. METHODS: In this manuscript, we present a case report, discuss gynecological and neurosurgical perspectives relating to the treatment strategies for managing this entity, and propose an alternative explanation for such an occurrence from a neurogenetic standpoint. RESULTS: We present a case of spinal müllerianosis within the conus medullaris which was managed symptomatically for several years with an intracystic drain and subcutaneous reservoir. Over the years, it became clear that there was a cyclical presentation to her clinical malady, which at times was severe. Ultimately, she required surgical resection which aided in her diagnosis and subsequent treatment. CONCLUSION: Intraspinal müllerianosis is a rare location for an otherwise common disease in women and has the potential to create significant neurological morbidity by creating a mass lesion. Although the exact etiology remains unclear, the histogenic theories of embryologic origin appear most plausible. Treatment strategies for this condition may include hormonal therapy, obstetrical surgery, or open spinal surgery. This unusual and poorly understood disease should be considered in the differential diagnosis for intraspinal lesions presenting with hemorrhage in the clinical context of cyclical neurological symptoms.

A comparative study of short- and long-TE ¹H MRS at 3 T for in vivo detection of 2-hydroxyglutarate in brain tumors.

2-Hydroxyglutarate (2HG) is produced in gliomas with mutations of isocitrate dehydrogenase (IDH) 1 and 2. The (1) H resonances of the J-coupled spins of 2HG are extensively overlapped with signals from other metabolites. Here, we report a comparative study at 3 T of the utility of the point-resolved spectroscopy sequence with a standard short TE (35 ms) and a long TE (97 ms), which had been theoretically designed for the detection of the 2HG 2.25-ppm resonance. The performance of the methods is evaluated using data from phantoms, seven healthy volunteers and 22 subjects with IDH-mutated gliomas. The results indicate that TE = 97 ms provides higher detectability of 2HG than TE = 35 ms, and that this improved capability is gained when data are analyzed with basis spectra that include the effects of the volume localizing radiofrequency and gradient pulses.

Ossifying fibroma of the nose and paranasal sinuses.

BACKGROUND: The purpose of this work was to perform a systematic review regarding ossifying fibroma and its multiple variants of the paranasal sinuses, and to identify any clinical differences between the multiple variants. METHODS: A search of the U.S. National Library of Medicine (PubMed) database was performed for the non­Medical Subject Heading (MeSH) search term "ossifying fibroma." The bibliographies of the retrieved manuscripts were searched to identify additional potentially relevant articles. Finally, textbooks of head and neck pathology were searched to identify peer-reviewed literature that addresses the histopathology of ossifying fibroma and its variants. Abstracts were screened by 2 of the authors to identify reports of ossifying fibroma lesions (and its variants) that involved the paranasal sinuses. Extracted data from case reports or case series included the clinical presentation, age, gender, site of involvement, surgical approach, treatment outcome, follow-up period, and recurrence rate. Information derived from cases is summarized in tables, and simple descriptive statistics were applied to the data. RESULTS: A total of 137 distinct patients were identified in 103 reports. Extracted data did not show any appreciable difference in clinical presentation or outcomes. Data on recurrence of these lesions was often limited by a lack of follow-up. CONCLUSION: Although differentiation between the subtypes of ossifying fibroma can be made histologically, and a diverse nomenclature exists, there does not appear to be any overriding clinical significance to the histopathologic differentiation of OF variants.

CT venography: use in selecting a surgical approach for the treatment of petrous apex cholesterol granulomas.

OBJECTIVE: To demonstrate the use of computed tomographic (CT) venography in selecting a surgical approach for cholesterol granulomas. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three patients presented with symptomatic petrous apex cholesterol granulomas with extensive bone erosion involving the jugular fossa. INTERVENTION(S): Computed tomographic venography was performed on each patient before selecting a surgical approach for drainage. MAIN OUTCOME MEASURE(S): Localization of the jugular bulb in relation to the petrous carotid artery and basal turn of the cochlea was ascertained in each subject. RESULTS: Three patients with large symptomatic cholesterol granulomas were identified. Conventional CT demonstrated extensive bone erosion involving the jugular fossa in each patient. The location of the jugular bulb and its proximity to the petrous carotid artery and basal turn of the cochlea could not be determined with conventional temporal bone CT and magnetic resonance imaging. Computed tomographic venography provided the exact location of the jugular bulb in all 3 patients. The favorable position of the jugular bulb in all 3 cases permitted drainage of these lesions using an infracochlear approach. CONCLUSION: Computed tomographic venography provided invaluable information in 3 patients with large symptomatic cholesterol granulomas. All 3 patients were previously thought to be unsuitable candidates for an infracochlear or infralabyrinthine approach because of the unknown location of the jugular bulb.

Concurrent vestibular schwannoma and meningioma mimicking a single cerebellopontine angle tumor.

Vestibular schwannomas account for ~;80% of cerebellopontine angle (CPA) tumors, with meningiomas being the second most common tumor of the CPA. The occurrence of both a schwannoma and a meningioma in the cerebellopontine angle is rare. After obtaining Institutional Review Board approval, we present a case of a concurrent vestibular schwannoma and meningioma in the CPA mimicking a single tumor.

Use of sagittal images for localization of the subthalamic nucleus. Technical note.

Direct observation of the subthalamic nucleus (STN) is not always possible, and physicians at many centers rely on indirect methods that relate the position of the STN to more easily recognized structures such as the red nucleus (RN). In this paper the authors describe an indirect method of viewing the STN based on the anatomy depicted on sagittal magnetic resonance (MR) images. A review of sagittal slices appearing in standard stereotactic atlases showed that the STN lies within the angle formed by the descending internal capsule (IC) and the substantia nigra (SN). The authors' technique consists of marking the location of the STN in this nigrocapsular angle on each sagittal MR image between the RN and the lateral border of the brainstem, and transferring these points to axial MR images to build a locus of points used to describe the STN. A point is chosen in the center of this locus as the stereotactic target. Two hundred eighty-two sagittal images obtained from 71 MR imaging studies performed in 29 patients by using a fast-spin echo inversion-recovery technique were examined for the presence of the STN, the SN, and the IC. The descending IC could be detected in 97% of the slices, the SN in 95% of the slices, and the STN in 73% of the slices. This indirect method involving sagittal anatomy can be used to refine localization of the STN.

Multilevel intramedullary spinal neurocysticercosis with eosinophilic meningitis.

BACKGROUND: Cysticercal involvement of the spinal cord is a very rare form of neurocysticercosis. Intramedullary cysts are even less common. OBJECTIVE: To describe a novel presentation of multilevel intramedullary neurocysticercosis with eosinophilic meningitis. DESIGN: Case report. PATIENT: A 35-year-old man with a history of cerebral neurocysticercosis who presented with both cauda equina and Brown-Sequard syndromes associated with cerebrospinal fluid findings of eosinophilic meningitis. RESULTS: Magnetic resonance imaging confirmed the multilevel intramedullary cord lesions. The patient was treated medically with dexamethasone and albendazole and had a good recovery. CONCLUSION: Intramedullary neurocysticercosis should be considered as a potentially treatable cause of multilevel spinal lesions with subacute meningitis.

Transverse myelitis in systemic sclerosis.

BACKGROUND: Neurological involvement occurs rarely with systemic sclerosis (SSc). Only a few cases of transverse myelopathy have been reported in the setting of SSc. OBJECTIVE: To describe a patient with SSc who developed transverse myelitis that improved during a course of immunosuppression. RESULTS: A 30-year-old woman with SSc presented with subacute onset of bilateral lower extremity weakness and numbness. Results of magnetic resonance imaging and cerebrospinal fluid studies supported a diagnosis of transverse myelitis. The patient responded favorably to a course of corticosteroids and cyclophosphamide. No overlapping autoimmune disorders were evident. Clinical follow-up showed significant recovery, with resolution of radiological abnormalities. CONCLUSION: Transverse myelitis can occur as a rare manifestation of SSc and may respond favorably to immunosuppressive therapy.

Functional magnetic resonance imaging of activation in subcortical auditory pathway.

OBJECTIVES: Functional magnetic resonance imaging (fMRI) has been used to investigate activation of the auditory cortex; however, assessment of activation in the subcortical auditory pathway has been challenging. The aim of this study was to examine neural correlates of cortical and subcortical auditory activation evoked by pure-tone stimulus using silent fMRI. STUDY DESIGN: Prospective analysis. METHODS: Seventeen normal-hearing volunteers (7 male, 10 female; age range, 14-37 yrs) underwent silent fMRI. An audiometer was used to deliver pure tones of 1000 Hz to the left ear. Pure tones were presented at hearing thresholds determined in the scanner. Brain regions showing increased activation during pure-tone stimulus presentation were mapped and auditory activations exceeding P <.001 were included in the analysis. RESULTS: Pure-tone stimuli evoked bilateral activation in cortical regions of the transverse and superior temporal gyri and the planum temporale. Activation in subcortical structures included the medial geniculate body, inferior colliculus, lateral lemniscus, superior olivary complex, and cochlear nucleus. CONCLUSIONS: Silent functional magnetic resonance imaging findings documented the feasibility of detecting activation elicited by pure tone along the cortical and subcortical auditory pathway. The use of this technique in the assessment of disorders with auditory dysfunction merits further investigation.

Hypoxia imaging in brain tumors.

Assessment of the oxygenation status of brain tumors has been studied increasingly with imaging techniques in light of recent advances in oncology. Tumor oxygen tension is a critical factor influencing the effectiveness of radiation and chemotherapy and malignant progression. Hypoxic tumors are resistant to treatment, and prognostic value of tumor oxygen status is shown in head and neck tumors. Strategies increasing the tumor oxygenation are being investigated to overcome the compromising [figure: see text] effect of hypoxia on tumor treatment. Administration of nicotinamide and inhalation of various high oxygen concentrations have been implemented. Existing methods for assessment of tissue oxygen level are either invasive or insufficient. Accurate and noninvasive means to measure tumor oxygenation are needed for treatment planning, identification of patients who might benefit from oxygenation strategies, and assessing the efficacy of interventions aimed to increase the radiosensitivity of tumors. Of the various imaging techniques used to assess tissue oxygenation, MR spectroscopy and MR imaging are widely available, noninvasive, and clinically applicable techniques. Tumor hypoxia is related closely to insufficient blood flow through chaotic and partially nonfunctional tumor vasculature and the distance between the capillaries and the tumor cells. Information on characteristics of tumor vasculature such as blood volume, perfusion, and increased capillary permeability can be provided with MR imaging. MR imaging techniques can provide a measure of capillary permeability based on contrast enhancement and relative cerebral blood volume estimates using dynamic susceptibility MR imaging. Blood oxygen level dependent contrast MR imaging using gradient echo sequence is intrinsically sensitive to changes in blood oxygen level. Animal models using blood oxygen level-dependent contrast imaging reveal the different responses of normal and tumor vasculature under hyperoxia. Normobaric hyperoxia is used in MR studies as a method to produce MR contrast in tissues. Increased T2* signal intensity of brain tissue has been observed using blood oxygen level-dependent contrast MR imaging. Dynamic blood oxygen level-dependent contrast MR imaging during hyperoxia is suggested to image tumor oxygenation. Quantification of cerebral oxygen saturation using blood oxygen level-dependent MR imaging also has been reported. Quantification of cerebral blood oxygen saturation using MR imaging has promising clinical applications; however, technical difficulties have to be resolved. Blood oxygen level dependent MR imaging is an emerging technique to evaluate the cerebral blood oxygen saturation, and it has the potential and versatility to assess oxygenation status of brain tumors. Upon improvement and validation of current MR techniques, better diagnostic, prognostic, and treatment monitoring capabilities can be provided for patients with brain tumors.