Conductive hearing loss caused by hereditary incus necrosis: a study of familial expansile osteolysis.
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Biomedical subjects
Publications and source records attributed to F J Wippold.
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Six patients are presented who died with active cytomegalovirus (CMV) encephalitis in whom brain MR scans within 25 days of death failed to reveal distinctive findings suggesting the diagnosis of CMV encephalitis. While MR scanning is crucial to evulation of AIDS patients developing neurologic complications, it is not sensitive to the presence of CMV encephalitis even when it is quite severe. The diagnosis of CMV encephalitis during life rests on characteristic clinical findings supported by a typical laboratory measures including the presence of CMV DNA in cerebrospinal fluid as detected by polymerase chain reaction.
BACKGROUND: Intracranial metastases commonly complicate oncologic care affecting 140,000 patients per year in the United States. Treatment of these tumors is difficult and often unsuccessful. Hyperthermia is a treatment alternative that has shown promise in treating cancer in other areas. Therefore it was employed in an attempt to provide increased tumor control in CNS metastases. METHODS: This Phase I and Phase II clinical trial of interstitial hyperthermia with recurrent or progressive intracranial metastatic disease was undertaken to evaluate complications, delivery of heat and patient outcome. RESULTS: Feared complications of clinically significant bleeding, increased mass, or infection from the interstitial implant and treatment did not occur. The seizures which occurred in 4 patients were controlled with additional anticonvulsants. Three venous thromboembolic events were treated medically and with percutaneously placed inferior vena cava filters. The KPS of the majority of patients declined slightly with treatment but rebounded to near baseline within several months. CT scans demonstrated decrease or stabilization of tumor volumes in 7 of the 13 patients. In 4 of these patients, regression or stabilization persisted until death from nonCNS disease. CONCLUSIONS: Interstitial hyperthermia therapy for intracranial metastases is technically feasible and may provide increased tumor control. In this small series, it did not cause unreasonable complications. This therapy has some positive effect, but requires study of more patients before its role is definitively known. Combining hyperthermia with brachytherapy and/or chemotherapy is being evaluated.
OBJECTIVE: Myxopapillary ependymomas are highly vascular tumors that arise almost exclusively in the thoracolumbar region and produce symptoms that may mimic discogenic pathology. The purpose of our study was to define the typical MR features of myxopapillary ependymoma and to determine the value of MR imaging in identifying the tumor, establishing its extent, and defining its relationship to intraspinal structures. MATERIALS AND METHODS: MR studies performed on 20 patients (13 men and seven women; mean age, 35 years) with 24 pathologically proven myxopapillary ependymomas were evaluated retrospectively for size and location of lesions, expansion of the central spinal canal and neural foramina, and MR signal characteristics and enhancement patterns. T1-weighted (16 patients), proton density-weighted (15 patients), T2-weighted (14 patients), and enhanced T1-weighted (nine patients) sequences were examined. RESULTS: In all patients, masses were detected on MR images. MR imaging defined solitary masses in 17 patients and multiple lesions in three. Of the 24 tumors, MR imaging characterized 21 as predominately intradural extramedullary, two as intramedullary and intradural extramedullary, and one as extradural postsacral. Two lesions had associated syrinxes. Lesions occurred most often at the level of the L2 vertebral body, and no tumor extended above T9. Tumors spanned an average of four vertebral segments. MR imaging showed expansion of the spinal canal in five of eight tumors that extended over five or more vertebral segments. Two tumors extended into the neural foramina. The T1-weighted signal of the tumor was isointense in 12 patients, hypointense in three patients, and hyperintense in one patient. The T2-weighted signal was always hyperintense. All tumors imaged after administration of IV contrast material showed enhancement. CONCLUSION: MR imaging was valuable in identifying the extent of tumors and in defining their relationship to the intraspinal structures. Although the MR findings in myxopapillary ependymoma are nonspecific, the diagnosis can be suggested by a large, intensely enhancing, intradural extramedullary thoracolumbar mass that extends for several vertebral levels. Imaging protocols should examine the entire thoracolumbar region and include IV contrast.
Cerebrospinal fluid fistula into the upper airway often results in meningitis. Closure of fistulas is usually effective using conventional surgical techniques to reconstruct the defect. We report a case of cerebrospinal fluid fistula into the sphenoid sinus and nasopharynx secondary to resection of a clivus chordoma that resisted conventional attempts at closure, including a rectus abdominus free flap. Closure of the fistula was accomplished with the use of a novel alloplast, biocompatible osteoconductive polymer. Follow-up for more than 1 year shows no evidence of rejection, infection, or recurrent cerebrospinal fluid rhinorrhea.
High-resolution computed tomography (CT) slices of the temporal bone of the Macaca mulatta monkey were performed and key anatomical landmarks were identified and labelled. The scans provided detailed anatomical definition of the monkey temporal bone and were considered a useful basis for studying the anatomy of a primate model.
OBJECTIVE: The purpose of this study was to evaluate the utility of cranial CT performed emergently in patients with neurologic deficits of acute onset that had resolved by the time of presentation to an emergency department. MATERIALS AND METHODS: Data were evaluated from 1518 patients presenting to level I (967 patients) and level II (551 patients) emergency departments. All patients underwent cranial CT during their visit to the emergency department to exclude potential intracranial hemorrhage or life-threatening mass effect from other causes. Of the 1518 patients who had CT done in one of these two departments and were entered into this study, 71 had histories compatible with resolved neurologic deficits, including 62 with apparent cerebrovascular disease, six with trauma, and three with seizure. The data collected included demographic information, medical history, physical and neurologic examinations, relevant laboratory data, results of CT, and information regarding patients' disposition from the emergency department. RESULTS: All CT scans in patients with resolved deficits were normal except for one in a patient who had had trauma. The scan of this patient showed a left frontal bone fracture and a subtle underlying subdural hematoma. These required no therapy. The rate of abnormal findings on CT scans was significantly lower in the 71 patients in whom acute neurologic deficits had resolved than in the other 1447 patients who underwent CT for other reasons (chi 2: p < .001). CONCLUSION: Immediate cranial CT is not indicated in the evaluation of patients with resolved neurologic deficits, except possibly when the patient has a history of trauma.
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PURPOSE: To compare conventional and spiral CT studies of the larynx. METHODS: Ten patients had both conventional and spiral CT studies of the neck using 5-mm section collimation and 5-mm increments. The spiral scans also were reconstructed at 2-mm increments. Five patients had two spiral CT larynx studies with 5-mm and 2-mm collimation. Two observers independently rated the visibility of laryngeal structures and absence of motion artifact in the studies and assigned values from 1 (poor) to 4 (excellent). RESULTS: The spiral scan images showed less motion artifact (1.9 versus 3.6) and better anatomic detail (2.3 versus approximately 2.6). Thinner reconstruction intervals and sections gave better anatomic detail (2.3 versus approximately 3.3). Interobserver kappa was 0.65. CONCLUSION: Spiral is better than conventional CT scanning when studying the larynx.
BACKGROUND: Vogt-Koyanagi-Harada Syndrome (VKHS) is a systemic disorder consisting of bilateral panuveitis with associated cutaneous and neurologic findings. In the absence of extraocular manifestations, diagnosis of VKHS may be difficult. METHODS: The results of magnetic resonance imaging (MRI) in two patients with VKHS and of computed tomography (CT) in a third patient with VKHS are described. RESULTS: Bilateral, diffuse choroidal thickening with scleral sparing was the norm. Choroidal thickening was demonstrable, despite the normal fundus appearance and fluorescein angiogram, in one eye. Intense enhancement after administration of gadopentetate was seen in the acute stages, but not after corticosteroid treatment. High-intensity periventricular lesions were seen in one patient. CONCLUSION: MRI superbly discriminates the fibrous sclera from the choroid and allows for the detection of subclinical ocular and central nervous system disease. It is therefore a useful adjunct in the diagnosis and follow-up of VKHS.
In preparation for future implantation of the implantable middle ear transducer in patients, a method was sought for preoperatively test fitting a model of the device, using computer generated three-dimensional (3-D) temporal bone images derived from spiral computed tomography (CT) data. A 3-D model of the implantable middle ear transducer was designed using NIH Image software on a Macintosh computer. High resolution human temporal bone CT scans were obtained using a spiral CT scanner (Siemens Somatom Plus S). The 3-D transducer model was superimposed onto 3-D reconstructions of the temporal bone using ANALYZE software on a computer graphics workstation (Sun SPARCstation 10), showing the transducer "implanted" in the temporal bone. Measurements were validated using a cadaver temporal bone. This process produced images demonstrating the "fit" of the current transducer design in the mastoid region of the adult temporal bone. It permitted assessment of the proximity of surrounding structures such as the external auditory meatus, dura, or sigmoid sinus. Preliminary cadaver validation measurements confirmed the accuracy of this method. Three-dimensional CT is a feasible method for preoperative planning of the surgical implantation of devices in the temporal bone. This method of 3-D test fitting will be used in the future to determine optimum orientation and size limitations for human implantable devices.
STUDY PURPOSE: We evaluated the need for emergency noncontrast cranial computed tomography (CT) among patients presenting to an emergency department with a complaint of seizure. METHODS: We retrospectively evaluated the medical records of 115 consecutive patients who presented to a trauma Level I ED with a complaint of seizure and underwent a noncontrast cranial CT. RESULTS: Sixty patients had a known seizure disorder, 38 had new-onset seizure, and 17 had possible seizure. The results of the neurologic examination and CT could be compared in 105 of the patients. An abnormal neurologic examination predicted 19 of 20 positive CT scans (95%) and demonstrated a strong association with CT results (P < .00004). Only a history of malignancy correlated to CT findings (P < .008). No other catalogued variable showed a statistical relationship with CT findings. CONCLUSION: Our data suggest that patients with either a history of malignancy or an abnormal neurologic examination at the time of examination in the ED will derive the greatest benefit from emergency CT.
STUDY OBJECTIVE: To study patients with acute trauma retrospectively for clinical predictors of positive cranial computed tomography. METHODS: We reviewed the medical records and noncontrast computed tomography scans of 373 consecutive head trauma patients presenting to a trauma Level I emergency department. Potential criteria for patient selection were analyzed statistically, using both univariate and multivariate models. RESULTS: Our data suggest that relying on four variables--positive neurologic examination, intoxication, and a history of amnesia or focal neurologic deficit--as screening criteria for computed tomography would have saved 58.6% of the scans performed on these patients. Together, these criteria had a sensitivity of 90.1% and a negative predictive value of 98.1% for abnormal computed tomography. Four patients with positive scans would not have been detected using this strategy. None of these patients deteriorated clinically or required operative intervention. CONCLUSION: Our data suggest that it may be possible to effectively screen patients with head trauma for cranial computed tomography using clinical criteria and so reduce the current number of scans performed by more than half. However, a prospective study is required to confirm our results.
RATIONALE AND OBJECTIVES: The accuracy and safety of nonionic myelography, unenhanced computed tomography (CT), and magnetic resonance (MR) in the diagnosis of lumbar disc herniation are reviewed. The comparative costs of these tests are also considered. METHODS: The accuracy of imaging tests that diagnose disc herniations was established by conducting a Medline search between 1985 and 1992. The morbidity associated with these tests are less formally established from selected articles. Finally, the mediocre reimbursement rates of these tests are reviewed. RESULTS AND CONCLUSIONS: The evolution of nonionic contrast media from the first to the second generation has been accompanied by a notable decrease in adverse reactions. Although nonionic myelograms are now better tolerated by patients, noninvasive imaging with CT and MR imaging has become as accurate as or more accurate than lumbar myelography, and should replace it as a screening test. Computed tomography may have advantages over MR imaging when issues of availability and cost are considered. In contrast to lumbar myelography, CT myelography may continue to be a useful method for clarifying ambiguous results of noninvasive tests. The morbidity associated with CT myelography can be minimized by using low doses of a nonionic agent, keeping the patient well hydrated, and using a small lumbar puncture needle. The incidence of adverse reactions can be expected to decrease further as new nonionic dimers become clinically available.
Charts from 1,074 consecutive emergency department patients who underwent cranial computed tomography (CT) were reviewed for predictors of a CT abnormality. Twenty-six clinical variables and the results of neurologic examination were compared with cranial CT findings. Patients with focal neurologic deficit, unresponsiveness, and hypertension had an increased risk of a CT abnormality. Blurred vision, trauma, loss of consciousness, headache, and dizziness were each associated with a lower risk of a CT abnormality. Multivariate analysis showed that only focal neurologic deficit and unresponsiveness effectively helped predict a CT abnormality. In patients with negative neurologic findings, only intoxication and amnesia were associated with greater than 10% positive scans and an increased risk for a CT abnormality. The data indicate that positive neurologic findings coupled with intoxication and amnesia would have helped detect 90.7% of the positive scans and provide an effective initial approximation strategy for selecting patients to undergo CT. Although 15 patients with positive scans (1.4%) would have been missed, this strategy would have yielded a negative predictive value of 97.3% and eliminated 53.9% of the CT scans obtained.
MR imaging can be used to identify abnormalities of the head and spine in patients with neurofibromatosis. In this pictorial essay, we illustrate the craniospinal MR imaging findings in a large series of patients with neurofibromatosis.
OBJECTIVE: The objective of this study was threefold: to determine if the frequency of deep white matter changes and cerebral atrophy seen on MR images is significantly different between patients with and without AIDS dementia complex, to determine if certain patterns of white matter changes are more closely associated with AIDS dementia complex, and to determine if focal lesions within the white matter of the splenium are more common in AIDS dementia complex. MATERIALS AND METHODS: Forty-five patients with AIDS were clinically examined for AIDS dementia complex. MR images from these patients were retrospectively reviewed without knowledge of the clinical results. The presence or absence of white matter abnormalities and cerebral atrophy was evaluated by using graded scales and correlated with the presence or absence of AIDS dementia complex. RESULTS: Ten patients met the criteria for AIDS dementia complex. Eight of 25 patients in whom MR images showed abnormal signal intensity in deep white matter had dementia compared with two of 20 in whom MR showed no changes in deep white matter. The presence of these deep white matter abnormalities was not significantly different between groups with and without dementia (p = .08), although higher grades of deep white matter abnormality were more likely to be associated with AIDS dementia complex. Nine of 19 patients in whom MR images showed atrophy had dementia compared with one of 26 in whom MR showed no atrophy. Atrophy was significantly associated with AIDS dementia complex (p = .001). Eight of 15 patients in whom MR images showed abnormal signal intensity within the white matter of the splenium had dementia compared with two of 30 in whom MR showed normal signal intensity in this area. The degree of abnormality in the splenium was weakly associated with AIDS dementia complex (Kendall's tau = .471, p = .001). CONCLUSION: MR findings of cerebral atrophy and abnormal signal intensity in the splenium are associated with AIDS dementia complex. The presence of generalized deep white matter abnormalities does not differ significantly between patients with and without dementia, although more severe grades of white matter abnormality are more likely to be seen in patients with AIDS dementia complex.