Diagnosis of von Hippel-Lindau disease in a patient with blindness resulting from bilateral optic nerve hemangioblastomas.
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Biomedical subjects
Publications and source records attributed to R A Willinsky.
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The causes of tinnitus can be grouped according to whether the noise is continuous or pulsatile, whether it is subjective or objective and whether there is a retrotympanic mass. Imaging algorithms can be based on these symptoms and signs. For patients with nonpulsatile tinnitus and a normal drum, magnetic resonance imaging is preferred if a retrocochlear lesion is suspected, whereas high-resolution computed tomography (HRCT) is recommended if a cochlear abnormality is likely. If a chronic inflammation in the middle ear is suspected, HRCT is the study of choice to differentiate cholesteatoma from chronic otitis media. If the bruit is objective and the tympanic membrane normal, selective cerebral angiography should be the initial investigation, because most such patients have an acquired vascular abnormality, usually a dural arteriovenous fistula. If there is pulsatile tinnitus and a retrotympanic mass, HRCT should be the first examination because this technique allows differentiation of a vascular variation, such as an aberrant carotid artery or jugular dehiscence, from a paraganglioma.
From our series of 203 patients with cerebral vascular lesions, 18 (9%) could be included in the multiple arteriovenous malformation category. There were five patients with Rendu-Osler-Weber, one with Wyburn-Mason syndromes and two with concurrent arteriovenous malformations. The remaining ten patients (4%) had multiple brain arteriovenous malformations. Careful angiography with magnification is necessary to try to diagnose multiple brain AVMs, since these sometimes become apparent only after embolization of a larger dominant AVM. The incidence of multiple brain arteriovenous malformations is likely to have been underestimated due to the failure to recognize micro-arteriovenous malformations associated with larger arteriovenous malformations.
Three patients with hyperflexion sprain of the cervical spine secondary to motor vehicle accidents are discussed. One patient exhibited tetraplegia and the other two only had paresthesia of the upper and/or lower extremities at the scene of the accident. All patients were young with no evidence of degenerative disc disease or osteoarthritis. Diagnosis of hyperflexion sprain is suggested by transient or persistent neurological deficits, local tenderness, or plain film findings, which include interspinous fanning, localized kyphotic angulation, subluxation, or disc space narrowing. Review of our patients' records revealed that some of these findings were evident at their initial presentation. In patients who have no neurological deficits, controlled flexion and extension views after routine plain films may be diagnostic of an unstable cervical spine. If there is a persistent neurological deficit, a magnetic resonance imaging scan is the examination of choice. If there is no compression of the thecal sac or spinal cord, supervised flexion and extension views of cervical spine should be done. Definitive management of the unstable spine is operative fixation.
In the investigation of a pulsatile retrotympanic mass it is important to recognize the high resolution computed tomography findings of an aberrant internal carotid artery. These include an absent exocranial opening of the carotid canal and a tubular density coursing along the medial wall of the middle ear in continuity with the horizontal carotid canal through a dehiscence of the lateral carotid plate. Our report highlights an unusual variation where two anomalous arteries, an aberrant internal carotid and an inferior tympanic to petrous carotid anastomoses, course through the middle ear.
This study examined frontal lobe function in a group of 20 patients with schizophrenia, on and off medication, compared to 20 normals matched for age, sex, handedness, intelligence, and educational level. Schizophrenic patients generally did not perform as well as normals on the Wisconsin Card Sorting Test (WCST). Patients off medication performed less well on this test than those on medication. Those on medication did not perform as well as those off medication on the design and word fluency tests, which suggested that medications may affect various aspects of frontal lobe function differently. During the WCST, normal subjects demonstrated an increase in beta mean frequency of the electroencephalogram in frontal and centrotemporal regions which was not statistically significant in either schizophrenic group. This shift in beta mean frequency was found to correlate positively with performance on the WCST in normals, but not in patients. Patients with more negative symptoms tended to show a smaller increase in beta mean frequency during the WCST. Performance on the WCST was correlated negatively with ventricle-brain ratio in all subjects, suggesting that frontal lobe function might be related to computed tomographic measures in the normal population as well as in schizophrenic patients. There was no correlation with performance on the WCST and length of illness.
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We describe the case of a forty-four year old man who had typical right sided cluster headaches and an arteriovenous malformation supplied mainly by the right anterior cerebral artery. Following endovascular embolisation of this artery the AVM was supplied mainly by the left anterior cerebral artery. Subsequent headaches have always been on the left. Transcranial Doppler studies now suggested an increase of middle cerebral artery flow velocity. We suggest that the change in haemodynamics was responsible for the change in headache side.
The radiological findings in 10 patients with sacral cysts were retrospectively reviewed and classified. The cysts were an incidental finding on computed tomography (CT) in four patients. The expansion of sacral foramina or the sacral canal as seen on plain films suggested the diagnosis in three. In only two of the five patients who had myelograms did the cysts fill with contrast. In eight, CT showed remodelling and expansion of the sacral foramina, or the canal, or both, by a homogeneous mass with a density of 5-20 Hounsfield units. One of the patients underwent magnetic resonance imaging which confirmed that the lesion was fluid-filled. We found that sacral cysts can be either symptomatic or asymptomatic, that they may or may not communicate with the subarachnoid space, and that they have a characteristic CT appearance.
Epidermoid tumors of the spinal canal are rare. We report two patients with intraspinal lesions and radiographically evident calcification. One proved to have an epidermoid tumor. Although the tissue diagnosis in the second was not definitive, it suggested an epidermoid tumor as well. Radiographically visible ossification or calcification has not been reported before in intraspinal epidermoids.