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1/10. The neurological complications of cardiac transplantation.

    review of the neurological complications encountered in 83 patients who received cardiac homografts over a seven-year period leads to the following conclusions: (1) Neurological disorders are common in transplant recipients, occurring in over 50 per cent of patients. (2) infection was the single most frequent cause of the neurological dysfunction, being responsible for one-third of all CNS complications. (3) The infective organisms were typically those considered to be usually of low pathogenicity: fungi, viruses, protozoa and an uncommon bacterial strain. (4) Other clinical neurological syndromes were related to vascular lesions, often apparently from cerebral ischaemia or infarction occurring during the surgical procedure, metabolic encephalopathies, cerebral microglioma, acute psychotic episodes and back pain from vertebral compression fractures. (5) The infectious complications and probably the development of neoplasms de novo, are related to immunosuppressive therapy which impairs virtually all host defence mechanisms and alters the nature of the host's response to infective agents or other foreign antigens. (6) Because neurological symptoms and signs were usually those of behavioural changes or deterioration in intellectual performance, the neurological examination was often of little value in diagnosing the nature or even the anatomical site of the neuropathological process. (7) The possibility of an infectious origin of the neurological manifestations must be aggressively pursued even in the absence of fever and a significantly abnormal spinal fluid examination. The diagnostic error made most frequently was to ascribe neurological symptoms erroneously to metabolic disturbances or to "intensive care unit psychosis" when they were in fact due to unrecognized CNS infection. (8) maintenance of mean cardiopulmonary bypass pressures above 70 mmHg, particularly in patients with known arteriosclerosis, may reduce operative morbidity. (9) Though increased diagnostic accuracy is possible with routine use of a variety of radiological and laboratory techniques, two further requirements probably must be met before a significant reduction in the frequency of neurological complications will occur: the advent of greater immunospecificity in suppressing rejection of the grafted organ while preserving defences against infection; and a more effective armamentarium of antiviral and antifungal drugs.
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2/10. Transient stenoses and occlusions of main cerebral arteries in children--diagnosis and control of therapy by transcranial Doppler sonography.

    Flow disturbances in main cerebral arteries may cause severe neurological symptoms. Using transcranial Doppler sonography (TCD) the blood flow velocities in the basal cerebral arteries (BCA) can be recorded at any age. Transient stenoses or occlusions of main cerebral arteries were detected in 11 children by this method and confirmed by other techniques. Vasospasm produced a marked increase in flow velocities in the affected arteries which was reduced by nimodipine, the calcium channel blocker. Vasospasm also occurred in severe bacterial meningitis. In acute hemiplegia due to cerebral arterial obstruction no flow velocities could be recorded at the corresponding site. If distal branches were obstructed reduced flow velocities were found proximally. Increased flow velocities or reversed flow in anastomoses indicated the collateralization. The transient nature of the occlusions was shown by repeated recordings. TCD is a reliable, noninvasive and rapidly available technique for diagnosing or excluding transient flow disturbances in the main cerebral arteries as the cause of neurological symptoms in children. It indicates the necessity and most advantageous stage for therapy.
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3/10. Clinical presentations of vascular malformations of the brain stem: comparison of angiographically positive and negative types.

    Clinical and radiographic features of 63 patients with a vascular malformation of the brain stem are described. On radiological grounds they were divided into two groups: one with angiographically visible lesions (AVAVMs), the other with lesions not seen angiographically, that is, occult (AOVMs). In the first group the initial clinical manifestation was due to haemorrhage in 20 of the 33 cases and consisted of a progressive neurological deficit in 12. In the second group 29 of the 30 initially presented with a brain stem haemorrhage. The latter was often characterised by development of symptoms over two days or more (16 cases), absence of headache (48 cases) and tendency to recurrence (20 cases). Clinical diagnosis was difficult in many cases especially in the AOVM group. Several of the patients were misdiagnosed as having multiple sclerosis. Clinical data in conjunction with magnetic resonance imaging were helpful in determining the nature of these lesions.
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4/10. Failure of downward gaze: the site and nature of the lesion.

    We report four patients with paralysis of downward gaze but with intact upward gaze, including one with detailed clinico-pathological studies and another with a focal computerized axial tomographic (CT) scan abnormality confirming the presence of bilateral lesions of the dorsomedial red nucleus, including the fasciculus retroflexus. It is suggested that sudden, permanent selective failure of downward gaze accompanied by transient disturbance of consciousness is an embolic syndrome of the posterior thalamosubthalamic or rubral artery.
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5/10. Fat overload syndrome. An autopsy study with evaluation of the coagulopathy.

    Fat overload syndrome is a rare complication of intravenous fat emulsion therapy. It is characterized by sudden elevation of the serum triglyceride level, fever, hepatosplenomegaly, coagulopathy, and variable end-organ dysfunction. The illness is generally discrete, and symptoms regress as the lipemia clears. The transient nature of the syndrome has allowed only speculation as to its pathogenesis. The authors report an autopsy study of a child who died during an acute episode of fat overload and document the causative role of fat sludging in the associated end-organ failure. In addition, they offer evidence that the coagulopathy, previously an enigma, results from primary fibrinolysis, possibly caused by release of tissue plasminogen activators from the damaged endothelial cells.
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6/10. The diagnosis and management of cerebral embolism and haemorrhagic infarction with sequential computerized cranial tomography.

    Haemorrhagic infarction is typically not present immediately after cerebral embolism. Spontaneous haemorrhagic transformation evolves over several days. As a consequence, delayed CT scans are essential to exclude haemorrhagic infarction before initiating anticoagulant therapy. Sequential CT scanning can also help in the diagnosis of cerebral embolism in patients with stroke of unknown cause. In such cases the detection of haemorrhagic infarction on a delayed scan suggest an embolic mechanism. The evolution of haemorrhagic infarction on sequential CT scans graphically demonstrates the dynamic nature of this lesion and thereby indicates why serious brain haemorrhage may result from anticoagulation immediately after cerebral embolism.
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7/10. Capsular infarcts: the underlying vascular lesions.

    In ten patients, 11 infarcts involving mainly the internal capsule have been examined pathologically. Serial sections of the involved basal ganglia were studied in ten infarcts and only a gross dissection was made in the other. The implicated penetrating arteries were traced throughout their length and obstructive vascular lesions were found in nine instances. In two of the nine there was an atheromatous plaque with a superimposed thrombus, in four an atheromatous plaque had caused severe stenosis, in one a destructive arterial process lipohyalinosis had occurred, in one case the nature of the obstruction remained "uncertained," and in one the penetrating arteries were obstructed at their orifices by an atheroma in the superior division of the middle cerebral artery. In two cases the vessels were patent, suggesting embolism. The atheromas consisted almost exclusively of a conglomerate of fat-filled macrophages. The clinical correlate was a pure motor hemiplegia or hemiparesis involving the face, arm, and leg without sensory deficit, homonymous hemianopia, receptive aphasia, or apractognosia. confusion was prominent in one patient.
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8/10. alien hand syndrome: interhemispheric motor disconnection due to a lesion in the midbody of the corpus callosum.

    The neuroanatomic substrate of the alien hand syndrome has remained controversial due to the noncircumscribed nature of cerebral injuries present in most cases. There have been few cases studied in which damage was restricted to portions of the body of the callosum, and most of those involved surgical callosotomy for tumors or epilepsy. We report the case of a woman with a transient alien hand syndrome caused by a stroke limited to the middle and posterior portions of the body of the corpus callosum. This case provides supportive evidence for damage to the midbody of the corpus callosum as the anatomic basis of nondominant alien hand syndrome and conforms to a model of interhemispheric motor disconnection as the essential component of this unusual behavioral syndrome. This disconnection can occur with injuries involving interhemispheric premotor and motor fibers traveling in the midportion of the callosum in individuals with left hemisphere dominance for motor activities.
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9/10. Perioperative stroke in patients undergoing head and neck surgery.

    The risk of perioperative strokes has been demonstrated to be very low in general surgical procedures, and somewhat higher in cardiac and carotid artery procedures. We describe 5 patients who underwent major head and neck procedures not requiring carotid ligation and who postoperatively suffered strokes. These occurred between the first and ninth postoperative days. Four of the patients were thought to have had emboli, 3 to the cerebral hemispheres (2 ipsilateral and 1 contralateral to the neck dissections), and another to the lower brain stem. Hypoperfusion was thought to have caused the stroke in the fifth patient. All patients had risk factors for stroke. The cases in our series were difficult to diagnose because of the delayed onset and subtle nature of symptoms, as well as masking of speech and communication due to the operative involved. Thrombogenesis within the internal carotid and vertebral artery systems due to patient positioning and intraoperative cervical manipulation may be an important etiologic factor in this form of stroke.
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10/10. Metastatic mucinous adenocarcinoma of the heart.

    A case of metastatic mucinous adenocarcinoma to the heart is described. The patient presented with neurological symptoms consistent with an embolic cerebrovascular accident. Evaluation by the referring cardiologist at that time showed what appeared to be a left atrial myxoma. In a review of the English language medical literature, no other case of this nature was found.
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