Congenital complete atrioventricular block in an infant with Miller-Dieker syndrome.
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Intracranial tumors are rare in the neonatal period, and generally the most common histological types are astrocytoma, medulloblastoma, choroid plexus papilloma and neuroectodermal tumors. The early diagnosis of these tumors is often very difficult. The authors report a case of a full-term newborn who presented with opisthotonus. A subependymal mass was detected by cerebral ultrasonography, and when the child was 1 month of age depigmentations appeared on the trunk and on the right leg, confirming the suspicion of tuberous sclerosis. At 3 months of age the child suffered infantile spasm with hypsarrhythmia. The developmental delay, the marked progressive neurological deterioration and the daily seizures suggested surgical resection. Histologic studies showed a subependymal giant cell astrocytoma such as typically occurs in tuberous sclerosis.
A 64-year-old right-handed man acutely developed elementary visual hallucinations (monochromatic, moving geometrical figures), visual illusions (distortion of the right side of faces) with achromatopsia and blurred vision restricted to the left visual hemi-field. CT of the brain before and after administration of contrast medium and a repeat examination 2 months later showed no abnormalities, while brain mapping (power analysis of EEG) demonstrated theta wave slowing of the curve over the posterior part of the right hemisphere. 99mTC HMPAO SPECT of the brain, however, demonstrated an area of definite focal hypoperfusion in the right occipito-temporal region. Echo-Doppler-duplex and continuous wave examination of the cervical arterial blood vessels disclosed bilateral discrete atheromatous plaques that did not affect the blood flow. Transoesophageal echocardiography demonstrated slight mitral valve insufficiency. Cerebral angiography showed an occlusion of the right posterior cerebral artery. After the visual hallucinations had subsided, SPECT showed partial normalization of the right occipito-temporal perfusion. In the absence of CT evidence for a structural lesion in the clinically suspected areas, only functional imaging revealed an obviously significant lesion. This case furthermore demonstrates that SPECT can contribute to the identification of the pathophysiology underlying visual hallucinosis.
Right-to-left shunt through a patent foramen ovale was searched for in 80 patients with acute ischemic stroke by simultaneously performing transthoracic two-dimensional echocardiography and transcranial Doppler during agitated saline injection. A patent foramen ovale was detected by echocardiography in 14 patients (17.5%). Transcranial Doppler correctly identified all 14 patients, and 7 more patients in whom echocardiographic findings were indeterminate. Prevalence of patent foramen ovale by transcranial Doppler was therefore 26.3% (21 of 80 patients). Concordance between the two tests was 91.3% (73 of 80 patients). The delivery of contrast material to cerebral vessels is therefore demonstrable by transcranial Doppler in all patients diagnosed by contrast echocardiography, suggesting that paradoxical embolization through a patent foramen ovale may be more frequent than previously thought. Transcranial Doppler with contrast injection is a valid alternative in case of poor echocardiographic image quality.
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Explore the source record for details and available documents.
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Ultrasound examinations (US) of the brain are possible in the presence of an acoustic window in the skull, either after craniotomy or through an open fontanelle. In this study 19 children were examined, 11 via a suboccipital craniectomy; 9 cases were examined more than once. Via this suboccipital route most of the contents of the posterior fossa, the third and lateral ventricles could be made visible. In two patients with a temporal or parietal craniotomy the involved hemisphere could be visualized. Six patients were examined via the open fontanelle after insertion of a ventriculoperitoneal shunt (VPS). In three of these cases the examination was undertaken to evaluate also a posterior fossa cyst. The findings were compared with CT if the findings were equivocal.
In 10 patients with lesions of the sensorimotor cortex cortical SEP were registered to identify the postcentral gyrus, and intra-operative ultrasound sonography served to locate the lesion. The combination of both techniques helped to find the optimal approach to the lesion. Postoperative results were considered favourable, as only one patient suffered transient postoperative deterioration, six were unchanged and in three patients the pre-operative motor deficits were improved. The combination of intra-operative ultrasound and neurophysiological identification of the sensorimotor cortex is concluded as being useful in surgery within this region.
The risks which result from hemodynamic changes after surgery of 35 patients with supratentorial arterio-venous angiomas were investigated. With the help of a miniaturized 20 MHz Doppler probe flow and resistance were measured before, during, and after operations. The Doppler findings were compared with pre- and postoperative angiograms and intraoperative surgical findings. Postoperative angiograms were performed immediately after operation. The risk of haemorrhage and oedema was found to be high in cases of large vessels with slow flow and high resistance, in high flow angiomas, in stagnating arteries visible longer than 10 s and a length more than 6 cm, and in cases of pre-operative steal. These hemodynamic risky angiomas must be treated either by multiple step-operation or hypotension.
Twenty cavernous angiomas were surgically removed after being localised by intra-operative echography. 10 patients were males and 10 females, with an average age of 34 years. Four patients had had intracerebral haemorrhage; all the remaining patients presented with headaches or seizures. The size of the lesion ranged from a minimum of 2.5 to a maximum of 4.0 cm. Its localisation was subcortical in 14 cases, paraventricular in 4 and in another 2 originated from the floor of the IV ventricle. Echography showed a blackberry-like cavernous angioma, hyperechogenous with respect to the surrounding parenchyma; the lesion was well-defined in all cases due to the absence of perilesional oedema with a clear demarcation from healthy tissue. After operation, pre-operative cranial nerve deficits (brought on by haemorrhage) only persisted in the two cases where the cavernoma involved the floor of the IV ventricle. In all cases post-operative radiological investigation recorded the complete removal of the cavernous angioma, the diagnosis of which was invariably confirmed by histological analysis. The authors draw the conclusion that intra-operative echography is capable of localising cavernous angiomas, providing the surgeon with real-time guidance during microsurgical removal. It is also useful for establishing as to whether complete removal has been accomplished.
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The effects of acute right internal carotid artery occlusion in a previously healthy young male, was studied over a period of 10 months, with angiography, TransCranial Doppler ultrasonography (TCD) and SPECT-rCBF. A clinically observed inability to meet increased metabolic demand in the right hemisphere was concommitant to a decreased Pulsatility Index (PI) in the right middle cerebral artery (MCA). Autoregulation studies showed almost dilatation of the resistance vessels in the right middle cerebral artery territory, at rest. A decreased blood flow velocity, in the right middle cerebral artery 7 months after the accident, suggesting a decreased rCBF, could not be confirmed by SPECT-rCBF studies. This finding strongly cautions against interpretation of chronical blood flow velocity changes in terms of changes in regional blood flow. The present study shows the benefits in the combined use of angiography, SPECT-rCBF, and TCD.
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