[Acute disseminated encephalomyelitis--ADEM. A rare, but serious disease].
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Biomedical subjects
Publications and source records attributed to U Bergvall.
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395 patients (236 males, mean age 55.6 years: 159 females, mean age 52.2 years) with suspected transient ischaemic attacks or previous strokes underwent intraarterial digital subtraction angiography (IADSA) over a 3-year period ending in March 1991. All procedures were performed via the femoral approach and the majority consisted of arch studies followed by selective catheterization. 253 (64.1%) of the patients had extracranial vascular disease confirmed at angiography. A retrospective analysis of the patients' records was made to extract all possible complications. Complications were defined as any untoward symptoms or signs occurring within 48 h and which could have been related to the angiogram. Neurological complications occurred in 15 (3.89%) patients. 10 (2.5%) patients had transient complications which resolved completely within 24 h. In three (0.8%) patients the neurological deficit was reversible, recovering fully within 6 days. Two (0.52%) patients were left with residual disability from stroke at 10 days. The permanent neurological complication rate is in the lower range of the rates recorded in previous conventional angiographic studies. We conclude that IADSA is a relatively safe and reliable form of investigation in patients with suspected cerebral vascular disease.
We describe a boy who presented at the age of 7 years with short stature due to hypopituitarism. Six months after starting appropriate hormone replacement treatment at the age of 8 he suffered his first generalised convulsion. Further neuroradiological investigation led to the diagnosis of moyamoya syndrome.
Each type of cerebral vascular malformation has its own unique clinical, radiological and pathological features, and a different pattern of natural evolution. With greater awareness and advances in neuroimaging, there is increasing recognition of Cerebral Medullary Venous Malformations (MVM), angiographically characterised by an 'Umbrella' or 'Caput Medusae' appearance. The clinical and radiological features of four patients with angiographically demonstrable features of cerebral medullary venous malformations, seen in our departments are presented to illustrate the features of cerebral MVMs. Three patients have been managed conservatively and one has been treated with stereotactic radiosurgery. The literature on cerebral MVMs has been reviewed and presented in this paper. It appears that most are benign and even in those cases where there has been a haemorrhage the outcome seems to be much better than with arteriovenous malformations. The rationale behind radiosurgical treatment is discussed. Before embarking upon any form of interventional therapy the natural course of these lesions should be considered.
Although primary malignant lymphoma is a rare entity in the gamut of intracranial tumors, it is more frequently seen than the secondary intracranial spread of a primary extracranial lymphoma. In general, the occurrence of lymphomas seems to be provoked by immunosuppression, as with medication (predominantly after transplantation) or with immunodepressive disease such as acquired immunodeficiency syndrome (AIDS). The usual age of onset of this disease is 55 to 65 years; and the male:female patient distribution is roughly 2:1. Characteristically, computerized tomography (CT) scans of lymphomas show a mass which is often large with regular contours, moderate mass effect, and hyper- or isodensity with marked and often homogeneous enhancement. In the series of 30 patients reported, the locations of lesions, in order of decreasing frequency, were the frontocallosal and temporal regions, the basal ganglia, and the cerebellum. Multiple lesions were present in 15% of these cases (20% to 40% in the literature). The following features should raise the suspicion of intracranial lymphoma: mirror lesions of the basal ganglia, bilateral subependymal infiltration, and leptomeningeal involvement contiguous with an intracerebral mass. According to the literature, the angiographic finding typical of lymphoma is an avascular tumor. A blush or vascular encasement of the mass seems to be rare, and the present series was in accordance with other reports in this respect. Differential diagnostic consideration should include meningioma, glioblastoma, metastatic disease, and focal infectious lesions such as toxoplasmosis or multifocal progressive leukoencephalitis, particularly in immunodepressed subjects. Diagnosing lymphoma from CT scans offers the alternative of substituting stereotaxic biopsy and neuropathological diagnosis for the more aggressive open surgical approach, since radiation therapy and possibly chemotherapy usually prove to be the treatment of choice.
Cortical brain R/L asymmetry was assessed by spin echo lateral sagittal sections on magnetic resonance imaging (MRI) classified by visual comparison and by measuring the length of the parietal operculum and the inclination of the sylvian fissure. The results were related to handedness, selected as a lateralized function. The results indicate that assessment of the cortical surface by sagittal MRI is feasible. Comparison between images from two imaging systems (40 hemispheres: 0.15 T/0.75 and 1 cm section thickness versus 4 hemispheres: 0.5 T/0.5 cm section thickness) indicates a minimum standard for this approach on a large scale to be set by the more powerful system, mainly dependent on image noise and volume averaging factors.
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In 69 patients with supratentorial cerebral infarction findings at computed tomography (CT) were related to clinical state on admission and course of recovery. In patients with initial disability of slight to moderate degree CT did not provide prognostically useful information; in patients with severe symptoms at onset location of lesion to the internal capsule or the right parietal lobe and the presence of the mass effect, as single factors or in combination, were ominous CT indicators in terms of functional recovery.
A female, aged 32, with facial birthmarks and suffering migraine headaches and a slight hemiparesis was examined with isotope scanning, CT and angiography. She was found to have a massive, unilateral angiodysplasia, transforming the right cerebral hemisphere into an expanding vascular sponge-like structure. There was a family history of red facial birthmarks. The patient and her father were analysed cytogenetically by estimating the frequency of chromosome aberrations and sister chromatid exchanges (SCE). The frequency of gaps and breaks was normal compared to healthy controls, but the patient showed a significantly higher level of spontaneous SCE's. The patient and her father also had a higher than normal number of SCE's after addition of mitomycin C (MMC) to the blood culture. Numerical and structural chromosome deviations markedly exceeded normal in both, possibly representing a genetic imbalance in this syndrome.
The importance of accurate diagnosis of epileptic manifestations, even if unusual, as complications of diagnostic metrizamide exploration of the CSF space is emphasized on the basis of 2 case histories.
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The time course of enhancement in apparently normal brain tissue, edema and focal lesions during 30 to 60 min after intravenous injection of vascular contrast medium was evaluated in a series of 41 patients with different intracranial lesions. The attenuation of apparently normal unenhanced brain tissue varied with the level of the scan, mainly an effect of beam hardening. Different types of enhancement response are discussed in terms of a 3-compartment model. The differential diagnostic potential of contrast enhancement in the early phase needs further evaluation using instruments with short scanning time, while the late phase of enhancement must be recorded with the use of a reliable head fixation to provide reproducibility of repeat measurements.
Computer tomography (CT) and spectrophotometry of CSF were performed in 30 patients with the clinical diagnosis of cerebral concussion or contusion. The patients with concussion all had normal CT-findings. Spectrophotometry of CSF was sometimes positive for cerebral contusion with normal CT-findings, but the two methods were complementary so that the extent of the lesion was determined by CT and spectrophotometry of CSF indicated the cause.
A case of spontaneous intra-aneurysmal thrombosis, verified angiographically, is reported in a patient with subarachnoid hemorrhage and without surgical intervention. The frequency of such an occurence and the factors involved are reviewed and discussed.
Four-vessel angiography was repeated in 56 patients with confirmed subarachnoid hemorrhage in whom the initial investigation was negative. Only one aneurysm was demonstrated. The results suggested that, with good technique, careful observation, and a complete four-vessel cerebral angiography, a false negative rate of less than 2% can be achieved. It is suggested that to repeat pan-angiography is seldom justified unless further bleeding episodes occur.
A technique is described for post-mortem examination of brain specimens employing computer tomography, for accurate comparison with in vivo scanning, including subsequent cutting of specimens in sections corresponding to tomographic layers measured. Potential application of this technique to diagnosis of mammary tumours is discussed.
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